Cutis

Click here to view the articles published in October 2017.

Click here to view the articles published in October 2017.

Click here to view the articles published in October 2017.

To the Editor:

Purpura annularis telangiectodes of Majocchi (PATM) is a type of pigmented purpuric dermatosis (PPD). Patients present with nonblanchable, annular, symmetric, purpuric, and telangiectatic patches, often on the legs, with histology revealing a perivascular lymphocytic infiltrate and extravasated erythrocytes.^1,2 A variety of medications have been linked to the development of PPD. We describe a case of levofloxacin-induced PATM.

RELATED ARTICLE: Granulomatous Changes Associated With Pigmented Purpuric Dermatosis

A 42-year-old man presented with a rash on the arms, trunk, abdomen, and legs of 1 month’s duration. He reported no associated itching, bleeding, or pain, and no history of a similar rash. He had a history of hypothyroidism and had been taking levothyroxine for years. He had no known allergies and no history of childhood eczema, asthma, or allergic rhinitis. Notably, the rash started shortly after the patient finished a 2-week course of levofloxacin, an antibiotic he had not taken in the past. The patient resided with his wife, 3 children, and a pet dog, and no family members had the rash. Prior to presentation, the patient had tried econazole cream and then triamcinolone acetonide cream 0.5% without any clinical improvement.

A complete review of systems was unremarkable. Physical examination revealed scattered, reddish brown, annular, nonscaly patches on the back, abdomen (Figure 1), arms, and legs with nonblanching petechiae within the patches.

A punch biopsy of the left inner thigh demonstrated patchy interface dermatitis, superficial perivascular inflammation, and numerous extravasated red blood cells in the papillary dermis (Figure 2). The histologic features were compatible with the clinical impression of PATM. The patient presented for a follow-up visit 2 weeks later with no new lesions and the old lesions were rapidly fading (Figure 3).

Pigmented purpuric dermatoses are a group of conditions that have different clinical morphologies but similar histopathologic examinations.² All PPDs are characterized by nonblanching, nonpalpable, purpuric lesions that often are bilaterally symmetrical and present on the legs.^2,3 Although the precise etiology of these conditions is not known, most cases include a perivascular lymphocytic infiltrate along with the presence of extravasated erythrocytes and hemosiderin deposition in the dermis.² Of note, PATM often is idiopathic and patients usually present with no associated comorbidities.³ The currently established PPDs include progressive pigmentary dermatosis (Schamberg disease), PATM, pigmented purpuric lichenoid dermatosis of Gougerot and Blum, lichen aureus, and eczematidlike purpura of Doucas and Kapetanakis.^2,4

RELATED ARTICLE: Granulomatous Pigmented Purpuric Dermatosis

The lesions of PATM are symmetrically distributed on the bilateral legs and may be symptomatic in most cases, with severe pruritus being reported in several drug-induced PATM cases.^3,5 Although the exact etiology of PPDs currently is unknown, some contributing factors that are thought to play a role include exercise, venous stasis, gravitational dependence, capillary fragility, hypertension, drugs, chemical exposure or ingestions, and contact allergy to dyes.³ Some of the drugs known to cause drug-induced PPDs fall into the class of sedatives, stimulants, antibiotics, cardiovascular drugs, vitamins, and nutritional supplements.^3,6 Some medications that have been reported to cause PPDs include acetaminophen, aspirin, carbamazepine, diltiazem, furosemide, glipizide, hydralazine, infliximab, isotretinoin, lorazepam, minocycline, nitroglycerine, and sildenafil.^3,7-15

Although the mechanism of drug-induced PPD is not completely understood, it is thought that the ingested substance leads to an immunologic response in the capillary endothelium, which results in a cell-mediated immune response causing vascular damage.³ The ingested substance may act as a hapten, stimulating antibody formation and immune-mediated injury, leading to the clinical presentation of nonblanching, symmetric, purpuric, telangiectatic, and atrophic patches at the site of injury.^1,3

Levofloxacin is a broad-spectrum antibiotic that has activity against both gram-positive and gram-negative bacteria. It inhibits the enzymes DNA gyrase and topoisomerase IV, preventing bacteria from undergoing proper DNA synthesis.¹⁶ Our patient’s rash began shortly after a 2-week course of levofloxacin and faded within a few weeks of discontinuing the drug; the clinical presentation, time course, and histologic appearance of the lesions were consistent with the diagnosis of drug-induced PPD. Of note, solar capillaritis has been reported following a phototoxic reaction induced by levofloxacin.¹⁷ Our case differs in that our patient had annular lesions on both photoprotected and photoexposed skin.

The first-line interventions for the treatment of PPDs are nonpharmacologic, such as discontinuation of an offending drug or allergen or wearing supportive stockings if there are signs of venous stasis. Other interventions include the use of a medium- or high-potency topical corticosteroid once to twice daily to affected areas for 4 to 6 weeks.¹⁸ Some case series also have shown improvement with narrowband UVB treatment after 24 to 28 treatment sessions or with psoralen plus UVA phototherapy within 7 to 20 treatments.^19,20 If the above measures are unsuccessful in resolving symptoms, other treatment alternatives may include pentoxifylline, griseofulvin, colchicine, cyclosporine, and methotrexate. The potential benefit of treatment must be weighed against the side-effect profile of these medications.^2,21-24 Of note, oral rutoside (50 mg twice daily) and ascorbic acid (500 mg twice daily) were administered to 3 patients with chronic progressive pigmented purpura. At the end of the 4-week treatment period, complete clearance of skin lesions was seen in all patients with no adverse reactions noted.²⁵

Despite these treatment options, PATM does not necessitate treatment given its benign course and often self-resolving nature.²⁶ In cases of drug-induced PPD such as in our patient, discontinuation of the offending drug often may lead to resolution.

In summary, PATM is a PPD that has been associated with different etiologic factors. If PATM is suspected to be caused by a drug, discontinuation of the offending agent usually results in resolution of symptoms, as it did in our case with fading of lesions within a few weeks after the patient was no longer taking levofloxacin.

To the Editor:

Purpura annularis telangiectodes of Majocchi (PATM) is a type of pigmented purpuric dermatosis (PPD). Patients present with nonblanchable, annular, symmetric, purpuric, and telangiectatic patches, often on the legs, with histology revealing a perivascular lymphocytic infiltrate and extravasated erythrocytes.^1,2 A variety of medications have been linked to the development of PPD. We describe a case of levofloxacin-induced PATM.

RELATED ARTICLE: Granulomatous Changes Associated With Pigmented Purpuric Dermatosis

A 42-year-old man presented with a rash on the arms, trunk, abdomen, and legs of 1 month’s duration. He reported no associated itching, bleeding, or pain, and no history of a similar rash. He had a history of hypothyroidism and had been taking levothyroxine for years. He had no known allergies and no history of childhood eczema, asthma, or allergic rhinitis. Notably, the rash started shortly after the patient finished a 2-week course of levofloxacin, an antibiotic he had not taken in the past. The patient resided with his wife, 3 children, and a pet dog, and no family members had the rash. Prior to presentation, the patient had tried econazole cream and then triamcinolone acetonide cream 0.5% without any clinical improvement.

A complete review of systems was unremarkable. Physical examination revealed scattered, reddish brown, annular, nonscaly patches on the back, abdomen (Figure 1), arms, and legs with nonblanching petechiae within the patches.

A punch biopsy of the left inner thigh demonstrated patchy interface dermatitis, superficial perivascular inflammation, and numerous extravasated red blood cells in the papillary dermis (Figure 2). The histologic features were compatible with the clinical impression of PATM. The patient presented for a follow-up visit 2 weeks later with no new lesions and the old lesions were rapidly fading (Figure 3).

Pigmented purpuric dermatoses are a group of conditions that have different clinical morphologies but similar histopathologic examinations.² All PPDs are characterized by nonblanching, nonpalpable, purpuric lesions that often are bilaterally symmetrical and present on the legs.^2,3 Although the precise etiology of these conditions is not known, most cases include a perivascular lymphocytic infiltrate along with the presence of extravasated erythrocytes and hemosiderin deposition in the dermis.² Of note, PATM often is idiopathic and patients usually present with no associated comorbidities.³ The currently established PPDs include progressive pigmentary dermatosis (Schamberg disease), PATM, pigmented purpuric lichenoid dermatosis of Gougerot and Blum, lichen aureus, and eczematidlike purpura of Doucas and Kapetanakis.^2,4

RELATED ARTICLE: Granulomatous Pigmented Purpuric Dermatosis

The lesions of PATM are symmetrically distributed on the bilateral legs and may be symptomatic in most cases, with severe pruritus being reported in several drug-induced PATM cases.^3,5 Although the exact etiology of PPDs currently is unknown, some contributing factors that are thought to play a role include exercise, venous stasis, gravitational dependence, capillary fragility, hypertension, drugs, chemical exposure or ingestions, and contact allergy to dyes.³ Some of the drugs known to cause drug-induced PPDs fall into the class of sedatives, stimulants, antibiotics, cardiovascular drugs, vitamins, and nutritional supplements.^3,6 Some medications that have been reported to cause PPDs include acetaminophen, aspirin, carbamazepine, diltiazem, furosemide, glipizide, hydralazine, infliximab, isotretinoin, lorazepam, minocycline, nitroglycerine, and sildenafil.^3,7-15

Although the mechanism of drug-induced PPD is not completely understood, it is thought that the ingested substance leads to an immunologic response in the capillary endothelium, which results in a cell-mediated immune response causing vascular damage.³ The ingested substance may act as a hapten, stimulating antibody formation and immune-mediated injury, leading to the clinical presentation of nonblanching, symmetric, purpuric, telangiectatic, and atrophic patches at the site of injury.^1,3

Levofloxacin is a broad-spectrum antibiotic that has activity against both gram-positive and gram-negative bacteria. It inhibits the enzymes DNA gyrase and topoisomerase IV, preventing bacteria from undergoing proper DNA synthesis.¹⁶ Our patient’s rash began shortly after a 2-week course of levofloxacin and faded within a few weeks of discontinuing the drug; the clinical presentation, time course, and histologic appearance of the lesions were consistent with the diagnosis of drug-induced PPD. Of note, solar capillaritis has been reported following a phototoxic reaction induced by levofloxacin.¹⁷ Our case differs in that our patient had annular lesions on both photoprotected and photoexposed skin.

The first-line interventions for the treatment of PPDs are nonpharmacologic, such as discontinuation of an offending drug or allergen or wearing supportive stockings if there are signs of venous stasis. Other interventions include the use of a medium- or high-potency topical corticosteroid once to twice daily to affected areas for 4 to 6 weeks.¹⁸ Some case series also have shown improvement with narrowband UVB treatment after 24 to 28 treatment sessions or with psoralen plus UVA phototherapy within 7 to 20 treatments.^19,20 If the above measures are unsuccessful in resolving symptoms, other treatment alternatives may include pentoxifylline, griseofulvin, colchicine, cyclosporine, and methotrexate. The potential benefit of treatment must be weighed against the side-effect profile of these medications.^2,21-24 Of note, oral rutoside (50 mg twice daily) and ascorbic acid (500 mg twice daily) were administered to 3 patients with chronic progressive pigmented purpura. At the end of the 4-week treatment period, complete clearance of skin lesions was seen in all patients with no adverse reactions noted.²⁵

Despite these treatment options, PATM does not necessitate treatment given its benign course and often self-resolving nature.²⁶ In cases of drug-induced PPD such as in our patient, discontinuation of the offending drug often may lead to resolution.

In summary, PATM is a PPD that has been associated with different etiologic factors. If PATM is suspected to be caused by a drug, discontinuation of the offending agent usually results in resolution of symptoms, as it did in our case with fading of lesions within a few weeks after the patient was no longer taking levofloxacin.

To the Editor:

Purpura annularis telangiectodes of Majocchi (PATM) is a type of pigmented purpuric dermatosis (PPD). Patients present with nonblanchable, annular, symmetric, purpuric, and telangiectatic patches, often on the legs, with histology revealing a perivascular lymphocytic infiltrate and extravasated erythrocytes.^1,2 A variety of medications have been linked to the development of PPD. We describe a case of levofloxacin-induced PATM.

RELATED ARTICLE: Granulomatous Changes Associated With Pigmented Purpuric Dermatosis

A 42-year-old man presented with a rash on the arms, trunk, abdomen, and legs of 1 month’s duration. He reported no associated itching, bleeding, or pain, and no history of a similar rash. He had a history of hypothyroidism and had been taking levothyroxine for years. He had no known allergies and no history of childhood eczema, asthma, or allergic rhinitis. Notably, the rash started shortly after the patient finished a 2-week course of levofloxacin, an antibiotic he had not taken in the past. The patient resided with his wife, 3 children, and a pet dog, and no family members had the rash. Prior to presentation, the patient had tried econazole cream and then triamcinolone acetonide cream 0.5% without any clinical improvement.

A complete review of systems was unremarkable. Physical examination revealed scattered, reddish brown, annular, nonscaly patches on the back, abdomen (Figure 1), arms, and legs with nonblanching petechiae within the patches.

A punch biopsy of the left inner thigh demonstrated patchy interface dermatitis, superficial perivascular inflammation, and numerous extravasated red blood cells in the papillary dermis (Figure 2). The histologic features were compatible with the clinical impression of PATM. The patient presented for a follow-up visit 2 weeks later with no new lesions and the old lesions were rapidly fading (Figure 3).

Pigmented purpuric dermatoses are a group of conditions that have different clinical morphologies but similar histopathologic examinations.² All PPDs are characterized by nonblanching, nonpalpable, purpuric lesions that often are bilaterally symmetrical and present on the legs.^2,3 Although the precise etiology of these conditions is not known, most cases include a perivascular lymphocytic infiltrate along with the presence of extravasated erythrocytes and hemosiderin deposition in the dermis.² Of note, PATM often is idiopathic and patients usually present with no associated comorbidities.³ The currently established PPDs include progressive pigmentary dermatosis (Schamberg disease), PATM, pigmented purpuric lichenoid dermatosis of Gougerot and Blum, lichen aureus, and eczematidlike purpura of Doucas and Kapetanakis.^2,4

RELATED ARTICLE: Granulomatous Pigmented Purpuric Dermatosis

The lesions of PATM are symmetrically distributed on the bilateral legs and may be symptomatic in most cases, with severe pruritus being reported in several drug-induced PATM cases.^3,5 Although the exact etiology of PPDs currently is unknown, some contributing factors that are thought to play a role include exercise, venous stasis, gravitational dependence, capillary fragility, hypertension, drugs, chemical exposure or ingestions, and contact allergy to dyes.³ Some of the drugs known to cause drug-induced PPDs fall into the class of sedatives, stimulants, antibiotics, cardiovascular drugs, vitamins, and nutritional supplements.^3,6 Some medications that have been reported to cause PPDs include acetaminophen, aspirin, carbamazepine, diltiazem, furosemide, glipizide, hydralazine, infliximab, isotretinoin, lorazepam, minocycline, nitroglycerine, and sildenafil.^3,7-15

Although the mechanism of drug-induced PPD is not completely understood, it is thought that the ingested substance leads to an immunologic response in the capillary endothelium, which results in a cell-mediated immune response causing vascular damage.³ The ingested substance may act as a hapten, stimulating antibody formation and immune-mediated injury, leading to the clinical presentation of nonblanching, symmetric, purpuric, telangiectatic, and atrophic patches at the site of injury.^1,3

Levofloxacin is a broad-spectrum antibiotic that has activity against both gram-positive and gram-negative bacteria. It inhibits the enzymes DNA gyrase and topoisomerase IV, preventing bacteria from undergoing proper DNA synthesis.¹⁶ Our patient’s rash began shortly after a 2-week course of levofloxacin and faded within a few weeks of discontinuing the drug; the clinical presentation, time course, and histologic appearance of the lesions were consistent with the diagnosis of drug-induced PPD. Of note, solar capillaritis has been reported following a phototoxic reaction induced by levofloxacin.¹⁷ Our case differs in that our patient had annular lesions on both photoprotected and photoexposed skin.

The first-line interventions for the treatment of PPDs are nonpharmacologic, such as discontinuation of an offending drug or allergen or wearing supportive stockings if there are signs of venous stasis. Other interventions include the use of a medium- or high-potency topical corticosteroid once to twice daily to affected areas for 4 to 6 weeks.¹⁸ Some case series also have shown improvement with narrowband UVB treatment after 24 to 28 treatment sessions or with psoralen plus UVA phototherapy within 7 to 20 treatments.^19,20 If the above measures are unsuccessful in resolving symptoms, other treatment alternatives may include pentoxifylline, griseofulvin, colchicine, cyclosporine, and methotrexate. The potential benefit of treatment must be weighed against the side-effect profile of these medications.^2,21-24 Of note, oral rutoside (50 mg twice daily) and ascorbic acid (500 mg twice daily) were administered to 3 patients with chronic progressive pigmented purpura. At the end of the 4-week treatment period, complete clearance of skin lesions was seen in all patients with no adverse reactions noted.²⁵

Despite these treatment options, PATM does not necessitate treatment given its benign course and often self-resolving nature.²⁶ In cases of drug-induced PPD such as in our patient, discontinuation of the offending drug often may lead to resolution.

In summary, PATM is a PPD that has been associated with different etiologic factors. If PATM is suspected to be caused by a drug, discontinuation of the offending agent usually results in resolution of symptoms, as it did in our case with fading of lesions within a few weeks after the patient was no longer taking levofloxacin.

To the Editor:

A 69-year-old woman with no history of immunodeficiency presented 1 month after a thorn from her locally grown Madagascar palm plant (Pachypodium lamerei) pierced the skin. The patient developed a painful nodule at the site on the left elbow (Figure 1). An excisional biopsy by an outside dermatologist was performed, which showed granulomatous inflammation within the dermis with epidermal hyperplasia and the presence of golden brown spherules (medlar bodies). The diagnosis was a dermal fungal infection consistent with chromoblastomycosis. A curative surgical excision was performed, and medlar bodies were seen adjacent to a polarizable foreign body consistent with plant material on histology (Figure 2). Because the lesion was localized, adjuvant medical treatment was not deemed necessary. The patient has not had any recurrence in the last 1.5 years since the resection.

The categorization of chromoblastomycosis includes a chronic fungal infection of the cutaneous and subcutaneous tissues by dematiaceous (pigmented) fungi. This definition is such that there are a multitude of organisms that can be the primary cause of this diagnosis. Generally, infection follows a traumatic permeation of the skin by a foreign body contaminated by the causative organism in agricultural workers. The most common dematiaceous pathogens are Fonsecaea pedrosoi, Phialophora verrucosa, and Cladosporium carrionii; however, the specific causative organism varies heavily on geographic location. With inoculation by a foreign body, a small papule develops at the site of the lesion. Several years after the primary infection, nodules and verrucous erythematous plaques develop in the same area, and patients present with concerns of pain and pruritus.¹ Lesions usually are localized to the initial area of inoculation, generally a break in the skin by the offending foreign body, on the legs, arms, or hands, but hematogenous or lymphatic dissemination with distant transmission due to scratching also can occur. Ulceration due to secondary bacterial infection is another possible manifestation, resulting in a foul odor and less commonly lymphedema. Rarely, squamous cell carcinoma is a complication.²

RELATED ARTICLE: Fungal Foes: Presentations of Chromoblastomycosis Post–Hurricane Ike

On histopathology, thick-walled sclerotic bodies termed medlar bodies or copper pennies are pathognomonic for chromoblastomycosis and represent the fungal elements. Grossly, black dots can be seen on the skin in the affected areas from the transepidermal elimination of the fungi.^1,2 However, there is no specificity for determining the causative organism in this manner, or even with culture, as it is difficult to differentiate the species morphologically. More advanced tests can help, such as polymerase chain reaction or enzyme-linked immunosorbent assay, where available.² Hematoxylin and eosin stain also shows epidermal hyperplasia and dermal mononuclear infiltrate.

Treatment modalities include surgical excision, cryotherapy, pharmacologic treatment, and combination therapy. Localized lesions often can be resected, but more severe infections can require pharmacologic treatment. Unfortunately, there tends to be a high risk for relapse with most antifungal modalities. The combination of itraconazole and terbinafine has been shown to offer the best medical therapy with lower risk for refractoriness to treatment by producing a synergistic effect between the 2 antifungals.^2,3 Many surgical treatments often are combined with oral antifungals to try to attain complete eradication in deep or extensive lesions, as seen in a case in which oral terbinafine was used prior to surgery to reduce the size of the lesion, followed by complete resection.⁴ With localized lesions that are resectable, a wide and deep incision often can be curative. Cryotherapy also may be coupled with surgical excision or pharmacologic therapy. Most literature suggests that cryotherapy or the use of antifungals prior to excision offers improved outcomes.^2,5 Prognosis tends to be good for chromoblastomycoses, particularly with smaller lesions. Complete eradication varies greatly on the size and depth of the lesion, independent of the causative pathogen.

Our patient’s presentation with chromoblastomycosis is unique because of the source of infection, which was a plant grown from seeds in a local nursery in South Florida and then sold to the patient. The majority of chromoblastomycosis infections occur in agricultural workers, typically in tropical climates such as South and Central America, the Caribbean, and Mexico.^1,2 Historically, infections in the United States have been uncommon, with the majority presenting in patients on prolonged corticosteroid therapy or with other immunosuppressive conditions.^6,7 This presentation of a chromoblastomycosis infection in a 69-year-old woman with no history of immunosuppression or chronic disease can serve as a teaching point about atypical presentations of the disease. The Madagascar palm plant that was responsible for the initial lesion in our case is a member of the Pachypodium species of plant that is endemic to Madagascar, one of the few regions outside of Latin America that has displayed numerous cases of chromoblastomycosis. In this fashion, a domestic gardener can now become exposed to dematiaceous pathogens that normally are not found in the continental United States, and knowledge of this possible exposure source can be crucial in the diagnosis and management of similar patients.

To the Editor:

A 69-year-old woman with no history of immunodeficiency presented 1 month after a thorn from her locally grown Madagascar palm plant (Pachypodium lamerei) pierced the skin. The patient developed a painful nodule at the site on the left elbow (Figure 1). An excisional biopsy by an outside dermatologist was performed, which showed granulomatous inflammation within the dermis with epidermal hyperplasia and the presence of golden brown spherules (medlar bodies). The diagnosis was a dermal fungal infection consistent with chromoblastomycosis. A curative surgical excision was performed, and medlar bodies were seen adjacent to a polarizable foreign body consistent with plant material on histology (Figure 2). Because the lesion was localized, adjuvant medical treatment was not deemed necessary. The patient has not had any recurrence in the last 1.5 years since the resection.

The categorization of chromoblastomycosis includes a chronic fungal infection of the cutaneous and subcutaneous tissues by dematiaceous (pigmented) fungi. This definition is such that there are a multitude of organisms that can be the primary cause of this diagnosis. Generally, infection follows a traumatic permeation of the skin by a foreign body contaminated by the causative organism in agricultural workers. The most common dematiaceous pathogens are Fonsecaea pedrosoi, Phialophora verrucosa, and Cladosporium carrionii; however, the specific causative organism varies heavily on geographic location. With inoculation by a foreign body, a small papule develops at the site of the lesion. Several years after the primary infection, nodules and verrucous erythematous plaques develop in the same area, and patients present with concerns of pain and pruritus.¹ Lesions usually are localized to the initial area of inoculation, generally a break in the skin by the offending foreign body, on the legs, arms, or hands, but hematogenous or lymphatic dissemination with distant transmission due to scratching also can occur. Ulceration due to secondary bacterial infection is another possible manifestation, resulting in a foul odor and less commonly lymphedema. Rarely, squamous cell carcinoma is a complication.²

RELATED ARTICLE: Fungal Foes: Presentations of Chromoblastomycosis Post–Hurricane Ike

On histopathology, thick-walled sclerotic bodies termed medlar bodies or copper pennies are pathognomonic for chromoblastomycosis and represent the fungal elements. Grossly, black dots can be seen on the skin in the affected areas from the transepidermal elimination of the fungi.^1,2 However, there is no specificity for determining the causative organism in this manner, or even with culture, as it is difficult to differentiate the species morphologically. More advanced tests can help, such as polymerase chain reaction or enzyme-linked immunosorbent assay, where available.² Hematoxylin and eosin stain also shows epidermal hyperplasia and dermal mononuclear infiltrate.

Treatment modalities include surgical excision, cryotherapy, pharmacologic treatment, and combination therapy. Localized lesions often can be resected, but more severe infections can require pharmacologic treatment. Unfortunately, there tends to be a high risk for relapse with most antifungal modalities. The combination of itraconazole and terbinafine has been shown to offer the best medical therapy with lower risk for refractoriness to treatment by producing a synergistic effect between the 2 antifungals.^2,3 Many surgical treatments often are combined with oral antifungals to try to attain complete eradication in deep or extensive lesions, as seen in a case in which oral terbinafine was used prior to surgery to reduce the size of the lesion, followed by complete resection.⁴ With localized lesions that are resectable, a wide and deep incision often can be curative. Cryotherapy also may be coupled with surgical excision or pharmacologic therapy. Most literature suggests that cryotherapy or the use of antifungals prior to excision offers improved outcomes.^2,5 Prognosis tends to be good for chromoblastomycoses, particularly with smaller lesions. Complete eradication varies greatly on the size and depth of the lesion, independent of the causative pathogen.

Our patient’s presentation with chromoblastomycosis is unique because of the source of infection, which was a plant grown from seeds in a local nursery in South Florida and then sold to the patient. The majority of chromoblastomycosis infections occur in agricultural workers, typically in tropical climates such as South and Central America, the Caribbean, and Mexico.^1,2 Historically, infections in the United States have been uncommon, with the majority presenting in patients on prolonged corticosteroid therapy or with other immunosuppressive conditions.^6,7 This presentation of a chromoblastomycosis infection in a 69-year-old woman with no history of immunosuppression or chronic disease can serve as a teaching point about atypical presentations of the disease. The Madagascar palm plant that was responsible for the initial lesion in our case is a member of the Pachypodium species of plant that is endemic to Madagascar, one of the few regions outside of Latin America that has displayed numerous cases of chromoblastomycosis. In this fashion, a domestic gardener can now become exposed to dematiaceous pathogens that normally are not found in the continental United States, and knowledge of this possible exposure source can be crucial in the diagnosis and management of similar patients.

To the Editor:

A 69-year-old woman with no history of immunodeficiency presented 1 month after a thorn from her locally grown Madagascar palm plant (Pachypodium lamerei) pierced the skin. The patient developed a painful nodule at the site on the left elbow (Figure 1). An excisional biopsy by an outside dermatologist was performed, which showed granulomatous inflammation within the dermis with epidermal hyperplasia and the presence of golden brown spherules (medlar bodies). The diagnosis was a dermal fungal infection consistent with chromoblastomycosis. A curative surgical excision was performed, and medlar bodies were seen adjacent to a polarizable foreign body consistent with plant material on histology (Figure 2). Because the lesion was localized, adjuvant medical treatment was not deemed necessary. The patient has not had any recurrence in the last 1.5 years since the resection.

The categorization of chromoblastomycosis includes a chronic fungal infection of the cutaneous and subcutaneous tissues by dematiaceous (pigmented) fungi. This definition is such that there are a multitude of organisms that can be the primary cause of this diagnosis. Generally, infection follows a traumatic permeation of the skin by a foreign body contaminated by the causative organism in agricultural workers. The most common dematiaceous pathogens are Fonsecaea pedrosoi, Phialophora verrucosa, and Cladosporium carrionii; however, the specific causative organism varies heavily on geographic location. With inoculation by a foreign body, a small papule develops at the site of the lesion. Several years after the primary infection, nodules and verrucous erythematous plaques develop in the same area, and patients present with concerns of pain and pruritus.¹ Lesions usually are localized to the initial area of inoculation, generally a break in the skin by the offending foreign body, on the legs, arms, or hands, but hematogenous or lymphatic dissemination with distant transmission due to scratching also can occur. Ulceration due to secondary bacterial infection is another possible manifestation, resulting in a foul odor and less commonly lymphedema. Rarely, squamous cell carcinoma is a complication.²

RELATED ARTICLE: Fungal Foes: Presentations of Chromoblastomycosis Post–Hurricane Ike

On histopathology, thick-walled sclerotic bodies termed medlar bodies or copper pennies are pathognomonic for chromoblastomycosis and represent the fungal elements. Grossly, black dots can be seen on the skin in the affected areas from the transepidermal elimination of the fungi.^1,2 However, there is no specificity for determining the causative organism in this manner, or even with culture, as it is difficult to differentiate the species morphologically. More advanced tests can help, such as polymerase chain reaction or enzyme-linked immunosorbent assay, where available.² Hematoxylin and eosin stain also shows epidermal hyperplasia and dermal mononuclear infiltrate.

Treatment modalities include surgical excision, cryotherapy, pharmacologic treatment, and combination therapy. Localized lesions often can be resected, but more severe infections can require pharmacologic treatment. Unfortunately, there tends to be a high risk for relapse with most antifungal modalities. The combination of itraconazole and terbinafine has been shown to offer the best medical therapy with lower risk for refractoriness to treatment by producing a synergistic effect between the 2 antifungals.^2,3 Many surgical treatments often are combined with oral antifungals to try to attain complete eradication in deep or extensive lesions, as seen in a case in which oral terbinafine was used prior to surgery to reduce the size of the lesion, followed by complete resection.⁴ With localized lesions that are resectable, a wide and deep incision often can be curative. Cryotherapy also may be coupled with surgical excision or pharmacologic therapy. Most literature suggests that cryotherapy or the use of antifungals prior to excision offers improved outcomes.^2,5 Prognosis tends to be good for chromoblastomycoses, particularly with smaller lesions. Complete eradication varies greatly on the size and depth of the lesion, independent of the causative pathogen.

Our patient’s presentation with chromoblastomycosis is unique because of the source of infection, which was a plant grown from seeds in a local nursery in South Florida and then sold to the patient. The majority of chromoblastomycosis infections occur in agricultural workers, typically in tropical climates such as South and Central America, the Caribbean, and Mexico.^1,2 Historically, infections in the United States have been uncommon, with the majority presenting in patients on prolonged corticosteroid therapy or with other immunosuppressive conditions.^6,7 This presentation of a chromoblastomycosis infection in a 69-year-old woman with no history of immunosuppression or chronic disease can serve as a teaching point about atypical presentations of the disease. The Madagascar palm plant that was responsible for the initial lesion in our case is a member of the Pachypodium species of plant that is endemic to Madagascar, one of the few regions outside of Latin America that has displayed numerous cases of chromoblastomycosis. In this fashion, a domestic gardener can now become exposed to dematiaceous pathogens that normally are not found in the continental United States, and knowledge of this possible exposure source can be crucial in the diagnosis and management of similar patients.

Myth: Actinic keratoses are only seen in patients with lighter skin

Actinic keratoses (AKs) are precancerous lesions that may turn into squamous cell carcinoma if left untreated. UV rays cause AKs, either from outdoor sun exposure or tanning beds. According to the American Academy of Dermatology, AKs are more likely to develop in patients 40 years or older with fair skin; hair color that is naturally blonde or red; eye color that is naturally blue, green, or hazel; skin that freckles or burns when in the sun; a weakened immune system; and occupations involving substances that contain polycyclic aromatic hydrocarbons such as coal or tar.

A 2007 study compared the most common diagnoses among patients of different racial and ethnic groups in New York City. Alexis et al found that AK was in the top 10 diagnoses in white patients but not for black patients. They postulated that photoprotective factors in darkly pigmented skin such as larger and more numerous melanosomes that contain more melanin and are more dispersed throughout the epidermis result in a lower incidence of skin cancers in the skin of color (SOC) population.

RELATED ARTICLE: Common Dermatologic Disorders in Skin of Color: A Comparative Practice Survey

However, a recent skin cancer awareness study in Cutis reported that even though SOC populations have lower incidences of skin cancer such as melanoma, basal cell carcinoma, and squamous cell carcinoma, they exhibit higher death rates. Furthermore, black individuals are more likely to present with advanced-stage melanoma and acral lentiginous melanomas compared to white individuals. Kailas et al stated, “Overall, SOC patients have the poorest skin cancer prognosis, and the data suggest that the reason for this paradox is delayed diagnosis.” They evaluated several knowledge-based interventions for increasing skin cancer awareness, knowledge, and protective behaviors in SOC populations, including the use of visuals such as photographs to allow SOC patients to visualize different skin tones, educational interventions in another language, and pamphlets.

RELATED ARTICLE: Assessing the Effectiveness of Knowledge-Based Interventions in Increasing Skin Cancer Awareness, Knowledge, and Protective Behaviors in Skin of Color Populations

Dermatologists should be aware that education of SOC patients is important to eradicate the common misconception that these patients do not have to worry about AKs and other skin cancers. Remind these patients that they need to protect their skin from the sun, just as patients with fair skin do. Further research in the dermatology community should focus on educational interventions that will help increase knowledge regarding skin cancer in SOC populations.

Expert Commentary

Although more common in patients with lighter skin, actinic keratosis and skin cancer can be seen in patients of all skin types. Many patients are unaware of this risk and do not use sunscreen and other sun-protective measures. We, as a specialty, have to educate our patients and the public of the risk for actinic keratosis and skin cancer in all skin types.

—Gary Goldenberg, MD (New York, New York)

Myth: Actinic keratoses are only seen in patients with lighter skin

Actinic keratoses (AKs) are precancerous lesions that may turn into squamous cell carcinoma if left untreated. UV rays cause AKs, either from outdoor sun exposure or tanning beds. According to the American Academy of Dermatology, AKs are more likely to develop in patients 40 years or older with fair skin; hair color that is naturally blonde or red; eye color that is naturally blue, green, or hazel; skin that freckles or burns when in the sun; a weakened immune system; and occupations involving substances that contain polycyclic aromatic hydrocarbons such as coal or tar.

A 2007 study compared the most common diagnoses among patients of different racial and ethnic groups in New York City. Alexis et al found that AK was in the top 10 diagnoses in white patients but not for black patients. They postulated that photoprotective factors in darkly pigmented skin such as larger and more numerous melanosomes that contain more melanin and are more dispersed throughout the epidermis result in a lower incidence of skin cancers in the skin of color (SOC) population.

RELATED ARTICLE: Common Dermatologic Disorders in Skin of Color: A Comparative Practice Survey

However, a recent skin cancer awareness study in Cutis reported that even though SOC populations have lower incidences of skin cancer such as melanoma, basal cell carcinoma, and squamous cell carcinoma, they exhibit higher death rates. Furthermore, black individuals are more likely to present with advanced-stage melanoma and acral lentiginous melanomas compared to white individuals. Kailas et al stated, “Overall, SOC patients have the poorest skin cancer prognosis, and the data suggest that the reason for this paradox is delayed diagnosis.” They evaluated several knowledge-based interventions for increasing skin cancer awareness, knowledge, and protective behaviors in SOC populations, including the use of visuals such as photographs to allow SOC patients to visualize different skin tones, educational interventions in another language, and pamphlets.

RELATED ARTICLE: Assessing the Effectiveness of Knowledge-Based Interventions in Increasing Skin Cancer Awareness, Knowledge, and Protective Behaviors in Skin of Color Populations

Dermatologists should be aware that education of SOC patients is important to eradicate the common misconception that these patients do not have to worry about AKs and other skin cancers. Remind these patients that they need to protect their skin from the sun, just as patients with fair skin do. Further research in the dermatology community should focus on educational interventions that will help increase knowledge regarding skin cancer in SOC populations.

Expert Commentary

Although more common in patients with lighter skin, actinic keratosis and skin cancer can be seen in patients of all skin types. Many patients are unaware of this risk and do not use sunscreen and other sun-protective measures. We, as a specialty, have to educate our patients and the public of the risk for actinic keratosis and skin cancer in all skin types.

—Gary Goldenberg, MD (New York, New York)

Myth: Actinic keratoses are only seen in patients with lighter skin

Actinic keratoses (AKs) are precancerous lesions that may turn into squamous cell carcinoma if left untreated. UV rays cause AKs, either from outdoor sun exposure or tanning beds. According to the American Academy of Dermatology, AKs are more likely to develop in patients 40 years or older with fair skin; hair color that is naturally blonde or red; eye color that is naturally blue, green, or hazel; skin that freckles or burns when in the sun; a weakened immune system; and occupations involving substances that contain polycyclic aromatic hydrocarbons such as coal or tar.

A 2007 study compared the most common diagnoses among patients of different racial and ethnic groups in New York City. Alexis et al found that AK was in the top 10 diagnoses in white patients but not for black patients. They postulated that photoprotective factors in darkly pigmented skin such as larger and more numerous melanosomes that contain more melanin and are more dispersed throughout the epidermis result in a lower incidence of skin cancers in the skin of color (SOC) population.

RELATED ARTICLE: Common Dermatologic Disorders in Skin of Color: A Comparative Practice Survey

However, a recent skin cancer awareness study in Cutis reported that even though SOC populations have lower incidences of skin cancer such as melanoma, basal cell carcinoma, and squamous cell carcinoma, they exhibit higher death rates. Furthermore, black individuals are more likely to present with advanced-stage melanoma and acral lentiginous melanomas compared to white individuals. Kailas et al stated, “Overall, SOC patients have the poorest skin cancer prognosis, and the data suggest that the reason for this paradox is delayed diagnosis.” They evaluated several knowledge-based interventions for increasing skin cancer awareness, knowledge, and protective behaviors in SOC populations, including the use of visuals such as photographs to allow SOC patients to visualize different skin tones, educational interventions in another language, and pamphlets.

RELATED ARTICLE: Assessing the Effectiveness of Knowledge-Based Interventions in Increasing Skin Cancer Awareness, Knowledge, and Protective Behaviors in Skin of Color Populations

Dermatologists should be aware that education of SOC patients is important to eradicate the common misconception that these patients do not have to worry about AKs and other skin cancers. Remind these patients that they need to protect their skin from the sun, just as patients with fair skin do. Further research in the dermatology community should focus on educational interventions that will help increase knowledge regarding skin cancer in SOC populations.

Expert Commentary

Although more common in patients with lighter skin, actinic keratosis and skin cancer can be seen in patients of all skin types. Many patients are unaware of this risk and do not use sunscreen and other sun-protective measures. We, as a specialty, have to educate our patients and the public of the risk for actinic keratosis and skin cancer in all skin types.

—Gary Goldenberg, MD (New York, New York)

Myth: Popping pimples resolves acne faster

Acne patients may be compelled to squeeze or pop their pimples at home thinking it will clear their acne faster, but they should be advised that doing so without using the proper technique can actually make the condition worse.

When over-the-counter or prescription acne medications take too long to work, some patients may use their fingernails or even a physical instrument (eg, tweezers) to clear the contents of the pimple; however, this process often produces lesions that are inflamed and far more visible, slower to heal, and more likely to scar than lesions progressing through the natural disease course. According to the American Academy of Dermatology (AAD), unwanted side effects of popping pimples can include permanent acne scars, more noticeable and/or painful acne lesions, and infection from bacteria on the hands.

The AAD promotes that dermatologists know how to remove bothersome acne lesions safely. Also, the AAD guidelines of care for the management of acne vulgaris reported that comedo removal may be helpful for lesions resistant to other therapies. Acne extraction may be offered when standard treatments fail and involves the use of sterile instruments to clear comedones and microcomedones. For single lesions that are particularly painful, dermatologists may opt to inject the lesion with a corticosteroid to reduce inflammation, speed healing, and decrease the risk of scarring; the strength of this recommendation is level C, according to the AAD acne guidelines work group. Finally, incision and drainage using a sterile needle or surgical blade can be used to open and clear the contents of large or painful pimples, nodules, and cysts.

These procedures are not first-line acne therapies. To minimize the appearance of acne lesions and promote clearance while waiting to see results from prescribed treatment regimens, patients should be advised to keep their hands away from their face and avoid picking at lesions, to apply ice to painful lesions to reduce inflammation and relieve pain, and to be patient with the acne treatment prescribed by a dermatologist. If patients are prone to picking their acne lesions, a more aggressive approach to treatment may be necessary, as a reduced number of inflammatory lesions leaves the patient with fewer spots to manipulate.

Myth: Popping pimples resolves acne faster

Acne patients may be compelled to squeeze or pop their pimples at home thinking it will clear their acne faster, but they should be advised that doing so without using the proper technique can actually make the condition worse.

When over-the-counter or prescription acne medications take too long to work, some patients may use their fingernails or even a physical instrument (eg, tweezers) to clear the contents of the pimple; however, this process often produces lesions that are inflamed and far more visible, slower to heal, and more likely to scar than lesions progressing through the natural disease course. According to the American Academy of Dermatology (AAD), unwanted side effects of popping pimples can include permanent acne scars, more noticeable and/or painful acne lesions, and infection from bacteria on the hands.

The AAD promotes that dermatologists know how to remove bothersome acne lesions safely. Also, the AAD guidelines of care for the management of acne vulgaris reported that comedo removal may be helpful for lesions resistant to other therapies. Acne extraction may be offered when standard treatments fail and involves the use of sterile instruments to clear comedones and microcomedones. For single lesions that are particularly painful, dermatologists may opt to inject the lesion with a corticosteroid to reduce inflammation, speed healing, and decrease the risk of scarring; the strength of this recommendation is level C, according to the AAD acne guidelines work group. Finally, incision and drainage using a sterile needle or surgical blade can be used to open and clear the contents of large or painful pimples, nodules, and cysts.

These procedures are not first-line acne therapies. To minimize the appearance of acne lesions and promote clearance while waiting to see results from prescribed treatment regimens, patients should be advised to keep their hands away from their face and avoid picking at lesions, to apply ice to painful lesions to reduce inflammation and relieve pain, and to be patient with the acne treatment prescribed by a dermatologist. If patients are prone to picking their acne lesions, a more aggressive approach to treatment may be necessary, as a reduced number of inflammatory lesions leaves the patient with fewer spots to manipulate.

Myth: Popping pimples resolves acne faster

Acne patients may be compelled to squeeze or pop their pimples at home thinking it will clear their acne faster, but they should be advised that doing so without using the proper technique can actually make the condition worse.

When over-the-counter or prescription acne medications take too long to work, some patients may use their fingernails or even a physical instrument (eg, tweezers) to clear the contents of the pimple; however, this process often produces lesions that are inflamed and far more visible, slower to heal, and more likely to scar than lesions progressing through the natural disease course. According to the American Academy of Dermatology (AAD), unwanted side effects of popping pimples can include permanent acne scars, more noticeable and/or painful acne lesions, and infection from bacteria on the hands.

The AAD promotes that dermatologists know how to remove bothersome acne lesions safely. Also, the AAD guidelines of care for the management of acne vulgaris reported that comedo removal may be helpful for lesions resistant to other therapies. Acne extraction may be offered when standard treatments fail and involves the use of sterile instruments to clear comedones and microcomedones. For single lesions that are particularly painful, dermatologists may opt to inject the lesion with a corticosteroid to reduce inflammation, speed healing, and decrease the risk of scarring; the strength of this recommendation is level C, according to the AAD acne guidelines work group. Finally, incision and drainage using a sterile needle or surgical blade can be used to open and clear the contents of large or painful pimples, nodules, and cysts.

These procedures are not first-line acne therapies. To minimize the appearance of acne lesions and promote clearance while waiting to see results from prescribed treatment regimens, patients should be advised to keep their hands away from their face and avoid picking at lesions, to apply ice to painful lesions to reduce inflammation and relieve pain, and to be patient with the acne treatment prescribed by a dermatologist. If patients are prone to picking their acne lesions, a more aggressive approach to treatment may be necessary, as a reduced number of inflammatory lesions leaves the patient with fewer spots to manipulate.

The Diagnosis: Merkel Cell Carcinoma

A partial biopsy was performed during the dermatology examination. Histopathology demonstrated a dense dermal infiltrate of small, dark blue, pleomorphic cells (Figure 1). On high power, the individual cells were noted to have vesicular nuclei with finely granular and dusty chromatin (Figure 2). Numerous mitotic figures were present. Immunohistochemical stains were performed and revealed positive staining for cytokeratin 20 (with a perinuclear dot pattern), synaptophysin, and chromogranin.

Merkel cell carcinoma (MCC) is an uncommon carcinoma of the epidermal neuroendocrine cells with approximately 1500 cases a year in the United States.¹ Merkel cell carcinoma has a poor prognosis with approximately one-third of cases resulting in death within 5 years and with a survival rate strongly dependent on the stage of disease at presentation.² A complete surgical excision with histologically verified clear margins is the main form of treatment of the primary cancer.³ Although the effectiveness of adjuvant therapy for MCC has been debated,⁴ retrospective analysis has shown that the high local recurrence rate of the primary tumor can be reduced by combining surgical excision with a form of radiation therapy.⁵

A systematic cohort study of 195 patients diagnosed with MCC summarized its most clinical factors with the acronym AEIOU: asymptomatic, expanding rapidly, immunosuppression, older than 50 years of age, and UV-exposed site on a fair-skinned individual.⁶ The role of immune function in MCC was highlighted by a 16-fold overrepresentation of immunosuppressed patients in the studied cohort as compared to the general US population. The immunosuppressed patients included individuals with human immunodeficiency virus, chronic lymphocytic leukemia, and iatrogenic suppression secondary to solid organ transplantation.⁶

In 2008, Merkel cell polyomavirus (MCPyV) was found in 80% (8/10) of MCC tumors tested.⁷ Since then, many different studies have suggested that MCPyV is an etiologic agent of MCC.^8-10 A natural component of skin flora, MCPyV only becomes tumorigenic after integration into the host DNA and with mutations to the viral genome.¹¹ Although there currently is no difference in treatment of MCPyV-positive and MCPyV-negative MCC,¹² research is being done to determine how the discovery of the MCPyV could impact the treatment of MCC.

The Diagnosis: Merkel Cell Carcinoma

A partial biopsy was performed during the dermatology examination. Histopathology demonstrated a dense dermal infiltrate of small, dark blue, pleomorphic cells (Figure 1). On high power, the individual cells were noted to have vesicular nuclei with finely granular and dusty chromatin (Figure 2). Numerous mitotic figures were present. Immunohistochemical stains were performed and revealed positive staining for cytokeratin 20 (with a perinuclear dot pattern), synaptophysin, and chromogranin.

Merkel cell carcinoma (MCC) is an uncommon carcinoma of the epidermal neuroendocrine cells with approximately 1500 cases a year in the United States.¹ Merkel cell carcinoma has a poor prognosis with approximately one-third of cases resulting in death within 5 years and with a survival rate strongly dependent on the stage of disease at presentation.² A complete surgical excision with histologically verified clear margins is the main form of treatment of the primary cancer.³ Although the effectiveness of adjuvant therapy for MCC has been debated,⁴ retrospective analysis has shown that the high local recurrence rate of the primary tumor can be reduced by combining surgical excision with a form of radiation therapy.⁵

A systematic cohort study of 195 patients diagnosed with MCC summarized its most clinical factors with the acronym AEIOU: asymptomatic, expanding rapidly, immunosuppression, older than 50 years of age, and UV-exposed site on a fair-skinned individual.⁶ The role of immune function in MCC was highlighted by a 16-fold overrepresentation of immunosuppressed patients in the studied cohort as compared to the general US population. The immunosuppressed patients included individuals with human immunodeficiency virus, chronic lymphocytic leukemia, and iatrogenic suppression secondary to solid organ transplantation.⁶

In 2008, Merkel cell polyomavirus (MCPyV) was found in 80% (8/10) of MCC tumors tested.⁷ Since then, many different studies have suggested that MCPyV is an etiologic agent of MCC.^8-10 A natural component of skin flora, MCPyV only becomes tumorigenic after integration into the host DNA and with mutations to the viral genome.¹¹ Although there currently is no difference in treatment of MCPyV-positive and MCPyV-negative MCC,¹² research is being done to determine how the discovery of the MCPyV could impact the treatment of MCC.

The Diagnosis: Merkel Cell Carcinoma

A partial biopsy was performed during the dermatology examination. Histopathology demonstrated a dense dermal infiltrate of small, dark blue, pleomorphic cells (Figure 1). On high power, the individual cells were noted to have vesicular nuclei with finely granular and dusty chromatin (Figure 2). Numerous mitotic figures were present. Immunohistochemical stains were performed and revealed positive staining for cytokeratin 20 (with a perinuclear dot pattern), synaptophysin, and chromogranin.

Merkel cell carcinoma (MCC) is an uncommon carcinoma of the epidermal neuroendocrine cells with approximately 1500 cases a year in the United States.¹ Merkel cell carcinoma has a poor prognosis with approximately one-third of cases resulting in death within 5 years and with a survival rate strongly dependent on the stage of disease at presentation.² A complete surgical excision with histologically verified clear margins is the main form of treatment of the primary cancer.³ Although the effectiveness of adjuvant therapy for MCC has been debated,⁴ retrospective analysis has shown that the high local recurrence rate of the primary tumor can be reduced by combining surgical excision with a form of radiation therapy.⁵

A systematic cohort study of 195 patients diagnosed with MCC summarized its most clinical factors with the acronym AEIOU: asymptomatic, expanding rapidly, immunosuppression, older than 50 years of age, and UV-exposed site on a fair-skinned individual.⁶ The role of immune function in MCC was highlighted by a 16-fold overrepresentation of immunosuppressed patients in the studied cohort as compared to the general US population. The immunosuppressed patients included individuals with human immunodeficiency virus, chronic lymphocytic leukemia, and iatrogenic suppression secondary to solid organ transplantation.⁶

In 2008, Merkel cell polyomavirus (MCPyV) was found in 80% (8/10) of MCC tumors tested.⁷ Since then, many different studies have suggested that MCPyV is an etiologic agent of MCC.^8-10 A natural component of skin flora, MCPyV only becomes tumorigenic after integration into the host DNA and with mutations to the viral genome.¹¹ Although there currently is no difference in treatment of MCPyV-positive and MCPyV-negative MCC,¹² research is being done to determine how the discovery of the MCPyV could impact the treatment of MCC.

The Diagnosis: Phakomatosis Pigmentovascularis With Sturge-Weber Syndrome

The erythematous patches were identified as capillary malformations (port-wine stains) and the slate gray pigmentary changes as dermal melanocytosis (Mongolian spots)(Figure). In fact, the diagnosis of phakomatosis pigmentovascularis (PPV) type II requires dermal melanocytosis and capillary malformation with and without nevus anemicus.1 In one case series, 46% (7/15) of patients with PPV had nevus anemicus² but our patient did not.

Phakomatosis pigmentovascularis was divided into 4 types in 1985,³ then later 5 types.⁴ Subcategories of the 5 types include type A, which denotes a lack of extracutaneous involvement, and type B, which is used when internal manifestations have been exhibited. Since 1947, approximately 222 cases of PPV have been described in the literature.²

A case of PPV associated with Sturge-Weber syndrome (SWS) was reported in 1997.⁵ Since then, PPV occasionally has been linked with SWS,^5-9 though there have been other syndromic associations including Klippel-Trenaunay-Weber syndrome and melanosis oculi.² The incidence and prevalence of overlap of PPV and SWS is unknown but is likely to be rare. In our case, magnetic resonance imaging of the patient's brain did not reveal the characteristic tram-track appearance of SWS; however, the diagnosis of SWS type II only requires facial angioma with or without glaucoma.^9,10 Most cases of PPV originate from Japan, Argentina, and Mexico.² Interestingly, our patient's parents were both of Mexican ancestry. Phakomatosis pigmentovascularis type IIb is the most common, followed by type IIa.² Most cases have been described as sporadic, though our patient's mother also exhibited a port-wine stain on the right neck, suggesting a possible genetic association.

The etiology of PPV has been postulated as twin spotting or didymosis (Greek for twin), most commonly seen in plants and animals. A previous review defined twin spotting as 2 mutant tissues situated adjacent to one another and unique from the normal tissue surrounding both of them.² When the cell loses its heterozygosity, this phenomenon appears. An alternative etiology supplants that a drug or virus toxic to the nervous system causes aberrant angioblasts and melanoblasts.^11,12 The etiology of SWS also is unknown, though vasomotor instability has been postulated as a cause.^6,13

It is important to exclude associated internal organ involvement with both of these syndromes because approximately 50% of PPV cases have extracutaneous organ involvement.^2,14 In fact, PPV is known to involve the brain, skeletal system, and eye, potentially manifesting as deafness, hydrocephalus, extremity overgrowth, scoliosis, cataracts, and more.² Patients with SWS often exhibit brain and eye symptoms including seizures.¹ To screen for extracutaneous involvement, multiple imaging studies should be performed. In our patient, an echocardiogram revealed a patent foramen ovale and normal cardiac anatomy for his age. Brain imaging revealed a hypoplastic left sigmoid and transverse sinus without venous thrombosis and unremarkable appearance of the brain. An ultrasound of the liver, spleen, kidneys, and pancreas revealed no evidence of solid, cystic, or vascular lesions, though the gallbladder exhibited hyperechoic areas.

To manage the skin lesions, some authors recommend Q-switched lasers for pigmented lesions and pulsed dye lasers for capillary malformations.¹⁵ Paller and Mancini¹ cited evidence that pulsed dye laser treatment before the age of 1 year may offer a psychological advantage, while other views have been offered.¹⁶ Some physicians believe that no urgent treatment of capillary malformations is needed unless internal organs are involved.^2,15

The Diagnosis: Phakomatosis Pigmentovascularis With Sturge-Weber Syndrome

The erythematous patches were identified as capillary malformations (port-wine stains) and the slate gray pigmentary changes as dermal melanocytosis (Mongolian spots)(Figure). In fact, the diagnosis of phakomatosis pigmentovascularis (PPV) type II requires dermal melanocytosis and capillary malformation with and without nevus anemicus.1 In one case series, 46% (7/15) of patients with PPV had nevus anemicus² but our patient did not.

Phakomatosis pigmentovascularis was divided into 4 types in 1985,³ then later 5 types.⁴ Subcategories of the 5 types include type A, which denotes a lack of extracutaneous involvement, and type B, which is used when internal manifestations have been exhibited. Since 1947, approximately 222 cases of PPV have been described in the literature.²

A case of PPV associated with Sturge-Weber syndrome (SWS) was reported in 1997.⁵ Since then, PPV occasionally has been linked with SWS,^5-9 though there have been other syndromic associations including Klippel-Trenaunay-Weber syndrome and melanosis oculi.² The incidence and prevalence of overlap of PPV and SWS is unknown but is likely to be rare. In our case, magnetic resonance imaging of the patient's brain did not reveal the characteristic tram-track appearance of SWS; however, the diagnosis of SWS type II only requires facial angioma with or without glaucoma.^9,10 Most cases of PPV originate from Japan, Argentina, and Mexico.² Interestingly, our patient's parents were both of Mexican ancestry. Phakomatosis pigmentovascularis type IIb is the most common, followed by type IIa.² Most cases have been described as sporadic, though our patient's mother also exhibited a port-wine stain on the right neck, suggesting a possible genetic association.

The etiology of PPV has been postulated as twin spotting or didymosis (Greek for twin), most commonly seen in plants and animals. A previous review defined twin spotting as 2 mutant tissues situated adjacent to one another and unique from the normal tissue surrounding both of them.² When the cell loses its heterozygosity, this phenomenon appears. An alternative etiology supplants that a drug or virus toxic to the nervous system causes aberrant angioblasts and melanoblasts.^11,12 The etiology of SWS also is unknown, though vasomotor instability has been postulated as a cause.^6,13

It is important to exclude associated internal organ involvement with both of these syndromes because approximately 50% of PPV cases have extracutaneous organ involvement.^2,14 In fact, PPV is known to involve the brain, skeletal system, and eye, potentially manifesting as deafness, hydrocephalus, extremity overgrowth, scoliosis, cataracts, and more.² Patients with SWS often exhibit brain and eye symptoms including seizures.¹ To screen for extracutaneous involvement, multiple imaging studies should be performed. In our patient, an echocardiogram revealed a patent foramen ovale and normal cardiac anatomy for his age. Brain imaging revealed a hypoplastic left sigmoid and transverse sinus without venous thrombosis and unremarkable appearance of the brain. An ultrasound of the liver, spleen, kidneys, and pancreas revealed no evidence of solid, cystic, or vascular lesions, though the gallbladder exhibited hyperechoic areas.

To manage the skin lesions, some authors recommend Q-switched lasers for pigmented lesions and pulsed dye lasers for capillary malformations.¹⁵ Paller and Mancini¹ cited evidence that pulsed dye laser treatment before the age of 1 year may offer a psychological advantage, while other views have been offered.¹⁶ Some physicians believe that no urgent treatment of capillary malformations is needed unless internal organs are involved.^2,15

The Diagnosis: Phakomatosis Pigmentovascularis With Sturge-Weber Syndrome

The erythematous patches were identified as capillary malformations (port-wine stains) and the slate gray pigmentary changes as dermal melanocytosis (Mongolian spots)(Figure). In fact, the diagnosis of phakomatosis pigmentovascularis (PPV) type II requires dermal melanocytosis and capillary malformation with and without nevus anemicus.1 In one case series, 46% (7/15) of patients with PPV had nevus anemicus² but our patient did not.

Phakomatosis pigmentovascularis was divided into 4 types in 1985,³ then later 5 types.⁴ Subcategories of the 5 types include type A, which denotes a lack of extracutaneous involvement, and type B, which is used when internal manifestations have been exhibited. Since 1947, approximately 222 cases of PPV have been described in the literature.²

A case of PPV associated with Sturge-Weber syndrome (SWS) was reported in 1997.⁵ Since then, PPV occasionally has been linked with SWS,^5-9 though there have been other syndromic associations including Klippel-Trenaunay-Weber syndrome and melanosis oculi.² The incidence and prevalence of overlap of PPV and SWS is unknown but is likely to be rare. In our case, magnetic resonance imaging of the patient's brain did not reveal the characteristic tram-track appearance of SWS; however, the diagnosis of SWS type II only requires facial angioma with or without glaucoma.^9,10 Most cases of PPV originate from Japan, Argentina, and Mexico.² Interestingly, our patient's parents were both of Mexican ancestry. Phakomatosis pigmentovascularis type IIb is the most common, followed by type IIa.² Most cases have been described as sporadic, though our patient's mother also exhibited a port-wine stain on the right neck, suggesting a possible genetic association.

The etiology of PPV has been postulated as twin spotting or didymosis (Greek for twin), most commonly seen in plants and animals. A previous review defined twin spotting as 2 mutant tissues situated adjacent to one another and unique from the normal tissue surrounding both of them.² When the cell loses its heterozygosity, this phenomenon appears. An alternative etiology supplants that a drug or virus toxic to the nervous system causes aberrant angioblasts and melanoblasts.^11,12 The etiology of SWS also is unknown, though vasomotor instability has been postulated as a cause.^6,13

It is important to exclude associated internal organ involvement with both of these syndromes because approximately 50% of PPV cases have extracutaneous organ involvement.^2,14 In fact, PPV is known to involve the brain, skeletal system, and eye, potentially manifesting as deafness, hydrocephalus, extremity overgrowth, scoliosis, cataracts, and more.² Patients with SWS often exhibit brain and eye symptoms including seizures.¹ To screen for extracutaneous involvement, multiple imaging studies should be performed. In our patient, an echocardiogram revealed a patent foramen ovale and normal cardiac anatomy for his age. Brain imaging revealed a hypoplastic left sigmoid and transverse sinus without venous thrombosis and unremarkable appearance of the brain. An ultrasound of the liver, spleen, kidneys, and pancreas revealed no evidence of solid, cystic, or vascular lesions, though the gallbladder exhibited hyperechoic areas.

To manage the skin lesions, some authors recommend Q-switched lasers for pigmented lesions and pulsed dye lasers for capillary malformations.¹⁵ Paller and Mancini¹ cited evidence that pulsed dye laser treatment before the age of 1 year may offer a psychological advantage, while other views have been offered.¹⁶ Some physicians believe that no urgent treatment of capillary malformations is needed unless internal organs are involved.^2,15

The Diagnosis: Inverted Follicular Keratosis

The differential diagnosis for endophytic squamous neoplasms encompasses benign and malignant entities. The histologic findings of our patient's lesion were compatible with the diagnosis of inverted follicular keratosis (IFK), a benign neoplasm that usually presents as a keratotic papule on the head or neck. Histologically, IFK is characterized by an endophytic growth pattern with squamous eddies (quiz images). Inverted follicular keratosis may represent an irritated seborrheic keratosis or a distinct neoplasm derived from the infundibular portion of the hair follicle; the exact etiology is uncertain.^1,2 No relationship between IFK and human papillomavirus (HPV) has been established.³ Inverted follicular keratosis can mimic squamous cell carcinoma (SCC). Important clues to the diagnosis of IFK are the presence of squamous eddies and the lack of squamous pearls or cytologic atypia.⁴ Squamous eddies consist of whorled keratinocytes without keratinization or atypia. Superficial shave biopsies may fail to demonstrate the characteristic well-circumscribed architecture and may lead to an erroneous diagnosis.

Acantholytic SCC is characterized by atypical keratinocytes that have lost cohesive properties, resulting in acantholysis (Figure 1).⁵ This histologic variant was once categorized as an aggressive variant of SCC, but studies have failed to support this assertion.^5,6 Acantholytic SCC has a discohesive nature producing a pseudoglandular appearance sometimes mistaken for adenosquamous carcinoma or metastatic carcinoma. Recent literature has suggested that acantholytic SCCs, similar to IFKs, are derived from the follicular infundibulum.^5,6 Also similar to IFKs, acantholytic SCCs often are located on the face. The invasive architecture and atypical cytology of acantholytic SCCs can differentiate them from IFKs. Acantholytic SCCs can contain keratin pearls with concentric keratinocytes showing incomplete keratinization centrally, often with retained nuclei, but rare to no squamous eddies unless irritated.

Trichilemmoma is an endophytic benign neoplasm derived from the outer sheath of the pilosebaceous follicle characterized by lobules of clear cells hanging from the epidermis.⁷ A study investigating the relationship between HPV and trichilemmomas failed to definitively detect HPV in trichilemmomas and this relationship remains unclear.⁸ Desmoplastic trichilemmoma is a subtype histologically characterized by jagged islands of epithelial cells separated by dense pink stroma and encased in a glassy basement membrane (Figure 2). The presence of desmoplasia and a jagged growth pattern can mimic invasive SCC, but the absence of cytologic atypia and the surrounding basement membrane differs from SCC.^4,7 Trichilemmomas typically are solitary, but multiple lesions are associated with Cowden syndrome. Cowden syndrome is a rare autosomal-dominant condition characterized by the presence of benign hamartomas and a predisposition to the development of malignancies including breast, endometrial, and thyroid cancers.^9,10 There is no such association with desmoplastic trichilemmomas.¹¹

Pilar sheath acanthoma is a benign neoplasm that clinically presents as a solitary flesh-colored nodule with a central pore containing keratin.¹² Histologically, pilar sheath acanthoma is similar to a dilated pore of Winer with the addition of acanthotic epidermal projections (Figure 3).

Warty dyskeratoma (WD) is a benign endophytic neoplasm traditionally seen as a solitary lesion histologically similar to Darier disease. Warty dyskeratomas are known to occur both on the skin and oral mucosa.¹³ Histologically, WD is characterized as a cup-shaped lesion with numerous villi at the base of the lesion along with acantholysis and dyskeratosis (Figure 4). The dyskeratotic cells in WD consist of corps ronds, which are cells with abundant pink cytoplasm, and small nuclei along with grains, which are flattened basophilic cells. These dyskeratotic cells help differentiate WD from IFK. Although they are endophytic neoplasms, WDs are well circumscribed and should not be confused with SCC. Despite this entity's name and histologic similarity to verrucae, no relationship with HPV has been established.¹⁴

The Diagnosis: Inverted Follicular Keratosis

The differential diagnosis for endophytic squamous neoplasms encompasses benign and malignant entities. The histologic findings of our patient's lesion were compatible with the diagnosis of inverted follicular keratosis (IFK), a benign neoplasm that usually presents as a keratotic papule on the head or neck. Histologically, IFK is characterized by an endophytic growth pattern with squamous eddies (quiz images). Inverted follicular keratosis may represent an irritated seborrheic keratosis or a distinct neoplasm derived from the infundibular portion of the hair follicle; the exact etiology is uncertain.^1,2 No relationship between IFK and human papillomavirus (HPV) has been established.³ Inverted follicular keratosis can mimic squamous cell carcinoma (SCC). Important clues to the diagnosis of IFK are the presence of squamous eddies and the lack of squamous pearls or cytologic atypia.⁴ Squamous eddies consist of whorled keratinocytes without keratinization or atypia. Superficial shave biopsies may fail to demonstrate the characteristic well-circumscribed architecture and may lead to an erroneous diagnosis.

Acantholytic SCC is characterized by atypical keratinocytes that have lost cohesive properties, resulting in acantholysis (Figure 1).⁵ This histologic variant was once categorized as an aggressive variant of SCC, but studies have failed to support this assertion.^5,6 Acantholytic SCC has a discohesive nature producing a pseudoglandular appearance sometimes mistaken for adenosquamous carcinoma or metastatic carcinoma. Recent literature has suggested that acantholytic SCCs, similar to IFKs, are derived from the follicular infundibulum.^5,6 Also similar to IFKs, acantholytic SCCs often are located on the face. The invasive architecture and atypical cytology of acantholytic SCCs can differentiate them from IFKs. Acantholytic SCCs can contain keratin pearls with concentric keratinocytes showing incomplete keratinization centrally, often with retained nuclei, but rare to no squamous eddies unless irritated.

Trichilemmoma is an endophytic benign neoplasm derived from the outer sheath of the pilosebaceous follicle characterized by lobules of clear cells hanging from the epidermis.⁷ A study investigating the relationship between HPV and trichilemmomas failed to definitively detect HPV in trichilemmomas and this relationship remains unclear.⁸ Desmoplastic trichilemmoma is a subtype histologically characterized by jagged islands of epithelial cells separated by dense pink stroma and encased in a glassy basement membrane (Figure 2). The presence of desmoplasia and a jagged growth pattern can mimic invasive SCC, but the absence of cytologic atypia and the surrounding basement membrane differs from SCC.^4,7 Trichilemmomas typically are solitary, but multiple lesions are associated with Cowden syndrome. Cowden syndrome is a rare autosomal-dominant condition characterized by the presence of benign hamartomas and a predisposition to the development of malignancies including breast, endometrial, and thyroid cancers.^9,10 There is no such association with desmoplastic trichilemmomas.¹¹

Pilar sheath acanthoma is a benign neoplasm that clinically presents as a solitary flesh-colored nodule with a central pore containing keratin.¹² Histologically, pilar sheath acanthoma is similar to a dilated pore of Winer with the addition of acanthotic epidermal projections (Figure 3).

Warty dyskeratoma (WD) is a benign endophytic neoplasm traditionally seen as a solitary lesion histologically similar to Darier disease. Warty dyskeratomas are known to occur both on the skin and oral mucosa.¹³ Histologically, WD is characterized as a cup-shaped lesion with numerous villi at the base of the lesion along with acantholysis and dyskeratosis (Figure 4). The dyskeratotic cells in WD consist of corps ronds, which are cells with abundant pink cytoplasm, and small nuclei along with grains, which are flattened basophilic cells. These dyskeratotic cells help differentiate WD from IFK. Although they are endophytic neoplasms, WDs are well circumscribed and should not be confused with SCC. Despite this entity's name and histologic similarity to verrucae, no relationship with HPV has been established.¹⁴

The Diagnosis: Inverted Follicular Keratosis

The differential diagnosis for endophytic squamous neoplasms encompasses benign and malignant entities. The histologic findings of our patient's lesion were compatible with the diagnosis of inverted follicular keratosis (IFK), a benign neoplasm that usually presents as a keratotic papule on the head or neck. Histologically, IFK is characterized by an endophytic growth pattern with squamous eddies (quiz images). Inverted follicular keratosis may represent an irritated seborrheic keratosis or a distinct neoplasm derived from the infundibular portion of the hair follicle; the exact etiology is uncertain.^1,2 No relationship between IFK and human papillomavirus (HPV) has been established.³ Inverted follicular keratosis can mimic squamous cell carcinoma (SCC). Important clues to the diagnosis of IFK are the presence of squamous eddies and the lack of squamous pearls or cytologic atypia.⁴ Squamous eddies consist of whorled keratinocytes without keratinization or atypia. Superficial shave biopsies may fail to demonstrate the characteristic well-circumscribed architecture and may lead to an erroneous diagnosis.

Acantholytic SCC is characterized by atypical keratinocytes that have lost cohesive properties, resulting in acantholysis (Figure 1).⁵ This histologic variant was once categorized as an aggressive variant of SCC, but studies have failed to support this assertion.^5,6 Acantholytic SCC has a discohesive nature producing a pseudoglandular appearance sometimes mistaken for adenosquamous carcinoma or metastatic carcinoma. Recent literature has suggested that acantholytic SCCs, similar to IFKs, are derived from the follicular infundibulum.^5,6 Also similar to IFKs, acantholytic SCCs often are located on the face. The invasive architecture and atypical cytology of acantholytic SCCs can differentiate them from IFKs. Acantholytic SCCs can contain keratin pearls with concentric keratinocytes showing incomplete keratinization centrally, often with retained nuclei, but rare to no squamous eddies unless irritated.

Trichilemmoma is an endophytic benign neoplasm derived from the outer sheath of the pilosebaceous follicle characterized by lobules of clear cells hanging from the epidermis.⁷ A study investigating the relationship between HPV and trichilemmomas failed to definitively detect HPV in trichilemmomas and this relationship remains unclear.⁸ Desmoplastic trichilemmoma is a subtype histologically characterized by jagged islands of epithelial cells separated by dense pink stroma and encased in a glassy basement membrane (Figure 2). The presence of desmoplasia and a jagged growth pattern can mimic invasive SCC, but the absence of cytologic atypia and the surrounding basement membrane differs from SCC.^4,7 Trichilemmomas typically are solitary, but multiple lesions are associated with Cowden syndrome. Cowden syndrome is a rare autosomal-dominant condition characterized by the presence of benign hamartomas and a predisposition to the development of malignancies including breast, endometrial, and thyroid cancers.^9,10 There is no such association with desmoplastic trichilemmomas.¹¹

Pilar sheath acanthoma is a benign neoplasm that clinically presents as a solitary flesh-colored nodule with a central pore containing keratin.¹² Histologically, pilar sheath acanthoma is similar to a dilated pore of Winer with the addition of acanthotic epidermal projections (Figure 3).

Warty dyskeratoma (WD) is a benign endophytic neoplasm traditionally seen as a solitary lesion histologically similar to Darier disease. Warty dyskeratomas are known to occur both on the skin and oral mucosa.¹³ Histologically, WD is characterized as a cup-shaped lesion with numerous villi at the base of the lesion along with acantholysis and dyskeratosis (Figure 4). The dyskeratotic cells in WD consist of corps ronds, which are cells with abundant pink cytoplasm, and small nuclei along with grains, which are flattened basophilic cells. These dyskeratotic cells help differentiate WD from IFK. Although they are endophytic neoplasms, WDs are well circumscribed and should not be confused with SCC. Despite this entity's name and histologic similarity to verrucae, no relationship with HPV has been established.¹⁴