Make the Diagnosis

Diagnosis: Henoch-Schönlein purpura

Henoch-Schönlein purpura (HSP), also known as immunoglobulin A (IgA) vasculitis, is characterized by palpable purpura, abdominal pain, renal disease, arthritis and/or arthralgia. HSP affects all ages, but is most common in children (the mean age of onset is between six and seven years of age). It also has a slight predilection for males, as well as whites and Asians. Patients are more likely to present during the fall, winter, and spring. It is thought that HSP may be associated with infections that are more prevalent during these times of the year.

Patients typically have purpura and joint or abdominal pain, although some may present without significant skin manifestations or pain. The rash associated with HSP may appear as erythematous macules or papules that coalesce and evolve into palpable purpura with petechiae, ecchymoses, and/or subcutaneous edema. The skin is typically affected in areas that are more dependent or experience more pressure, such as the lower extremities or buttocks. A more diffuse distribution across the entire body can be seen in those who are nonambulatory.

The arthritis associated with HSP usually affects the lower extremities, and it is oligoarticular, nondeforming, and transient, with prominent swelling and tenderness. Gastrointestinal involvement may manifest as nausea, vomiting, abdominal pain, transient paralytic ileus, hemorrhage, bowel ischemia, intussusception (rare in adults), and/or bowel perforation. These symptoms tend to develop 1 week after the rash, but there are reports of gastrointestinal complaints preceding all other manifestations of HSP.

Additionally, there can be renal involvement, which is more common in adults. These patients may experience hematuria with or without red blood cell casts, nephrotic range proteinuria, and/or elevated serum creatinine. Other less common symptoms that have been reported in patients with HSP include scrotal pain and swelling, headaches, seizures, ataxia, central and peripheral neuropathies, impaired lung diffusion capacity, keratitis, and uveitis.

The diagnosis of HSP is classically based upon clinical presentation. The diagnosis may be complicated in cases in which patients do not present with classic signs, such as palpable purpura of the lower extremities and buttocks. In these situations, a biopsy of the skin or kidney may aid in the diagnosis.

The differential diagnosis for HSP includes acute hemorrhagic edema of infancy, coagulopathies, hemolytic uremic syndrome, hypersensitivity vasculitis, IgA nephropathy, immune thrombocytopenia purpura, juvenile idiopathic arthritis, reactive arthritis, rheumatic fever, small vessel vasculitis, and systemic lupus erythematosus.

The etiology of HSP is unclear. Most believe that it is an immune-mediated vasculitis with genetic and environmental influences. In HSP, there is leukocytoclastic vasculitis with deposition of IgA immune complexes, complement component 3 (C3), and fibrin in the affected vessel walls. Small vessels within the papillary dermis are usually present with an inflammatory infiltrate of neutrophils and monocytes.

The treatment of HSP usually consists of supportive care, since most patients will recover spontaneously. Therefore, hydration, rest, and pain relief are essential. Other treatment modalities may be required for more severe or extensive HSP and its complications.

Our patient’s biopsy results were consistent with a leukocytoclastic vasculitis. His biopsy revealed an inflammatory infiltrate of lymphocytes, neutrophils, and nuclear dust with extravasation of erythrocytes and fibrin in the walls of small blood vessels, along with prominent papillary dermal edema and subepidermal vesiculation. Direct immunofluorescence was positive for IgA, C3, and fibrinogen in upper dermal blood vessels. The patient also had renal involvement that was confirmed with a renal biopsy.

This case and photos are courtesy of Tanya Greywal, University of California, San Diego, and Dr. Brooke Resh Sateesh, San Diego Family Dermatology.

Dr. Bilu Martin is in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit your case for possible publication, send an email to [email protected].

Diagnosis: Henoch-Schönlein purpura

Henoch-Schönlein purpura (HSP), also known as immunoglobulin A (IgA) vasculitis, is characterized by palpable purpura, abdominal pain, renal disease, arthritis and/or arthralgia. HSP affects all ages, but is most common in children (the mean age of onset is between six and seven years of age). It also has a slight predilection for males, as well as whites and Asians. Patients are more likely to present during the fall, winter, and spring. It is thought that HSP may be associated with infections that are more prevalent during these times of the year.

Patients typically have purpura and joint or abdominal pain, although some may present without significant skin manifestations or pain. The rash associated with HSP may appear as erythematous macules or papules that coalesce and evolve into palpable purpura with petechiae, ecchymoses, and/or subcutaneous edema. The skin is typically affected in areas that are more dependent or experience more pressure, such as the lower extremities or buttocks. A more diffuse distribution across the entire body can be seen in those who are nonambulatory.

The arthritis associated with HSP usually affects the lower extremities, and it is oligoarticular, nondeforming, and transient, with prominent swelling and tenderness. Gastrointestinal involvement may manifest as nausea, vomiting, abdominal pain, transient paralytic ileus, hemorrhage, bowel ischemia, intussusception (rare in adults), and/or bowel perforation. These symptoms tend to develop 1 week after the rash, but there are reports of gastrointestinal complaints preceding all other manifestations of HSP.

Additionally, there can be renal involvement, which is more common in adults. These patients may experience hematuria with or without red blood cell casts, nephrotic range proteinuria, and/or elevated serum creatinine. Other less common symptoms that have been reported in patients with HSP include scrotal pain and swelling, headaches, seizures, ataxia, central and peripheral neuropathies, impaired lung diffusion capacity, keratitis, and uveitis.

The diagnosis of HSP is classically based upon clinical presentation. The diagnosis may be complicated in cases in which patients do not present with classic signs, such as palpable purpura of the lower extremities and buttocks. In these situations, a biopsy of the skin or kidney may aid in the diagnosis.

The differential diagnosis for HSP includes acute hemorrhagic edema of infancy, coagulopathies, hemolytic uremic syndrome, hypersensitivity vasculitis, IgA nephropathy, immune thrombocytopenia purpura, juvenile idiopathic arthritis, reactive arthritis, rheumatic fever, small vessel vasculitis, and systemic lupus erythematosus.

The etiology of HSP is unclear. Most believe that it is an immune-mediated vasculitis with genetic and environmental influences. In HSP, there is leukocytoclastic vasculitis with deposition of IgA immune complexes, complement component 3 (C3), and fibrin in the affected vessel walls. Small vessels within the papillary dermis are usually present with an inflammatory infiltrate of neutrophils and monocytes.

The treatment of HSP usually consists of supportive care, since most patients will recover spontaneously. Therefore, hydration, rest, and pain relief are essential. Other treatment modalities may be required for more severe or extensive HSP and its complications.

Our patient’s biopsy results were consistent with a leukocytoclastic vasculitis. His biopsy revealed an inflammatory infiltrate of lymphocytes, neutrophils, and nuclear dust with extravasation of erythrocytes and fibrin in the walls of small blood vessels, along with prominent papillary dermal edema and subepidermal vesiculation. Direct immunofluorescence was positive for IgA, C3, and fibrinogen in upper dermal blood vessels. The patient also had renal involvement that was confirmed with a renal biopsy.

This case and photos are courtesy of Tanya Greywal, University of California, San Diego, and Dr. Brooke Resh Sateesh, San Diego Family Dermatology.

Dr. Bilu Martin is in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit your case for possible publication, send an email to [email protected].

Diagnosis: Henoch-Schönlein purpura

Henoch-Schönlein purpura (HSP), also known as immunoglobulin A (IgA) vasculitis, is characterized by palpable purpura, abdominal pain, renal disease, arthritis and/or arthralgia. HSP affects all ages, but is most common in children (the mean age of onset is between six and seven years of age). It also has a slight predilection for males, as well as whites and Asians. Patients are more likely to present during the fall, winter, and spring. It is thought that HSP may be associated with infections that are more prevalent during these times of the year.

Patients typically have purpura and joint or abdominal pain, although some may present without significant skin manifestations or pain. The rash associated with HSP may appear as erythematous macules or papules that coalesce and evolve into palpable purpura with petechiae, ecchymoses, and/or subcutaneous edema. The skin is typically affected in areas that are more dependent or experience more pressure, such as the lower extremities or buttocks. A more diffuse distribution across the entire body can be seen in those who are nonambulatory.

The arthritis associated with HSP usually affects the lower extremities, and it is oligoarticular, nondeforming, and transient, with prominent swelling and tenderness. Gastrointestinal involvement may manifest as nausea, vomiting, abdominal pain, transient paralytic ileus, hemorrhage, bowel ischemia, intussusception (rare in adults), and/or bowel perforation. These symptoms tend to develop 1 week after the rash, but there are reports of gastrointestinal complaints preceding all other manifestations of HSP.

Additionally, there can be renal involvement, which is more common in adults. These patients may experience hematuria with or without red blood cell casts, nephrotic range proteinuria, and/or elevated serum creatinine. Other less common symptoms that have been reported in patients with HSP include scrotal pain and swelling, headaches, seizures, ataxia, central and peripheral neuropathies, impaired lung diffusion capacity, keratitis, and uveitis.

The diagnosis of HSP is classically based upon clinical presentation. The diagnosis may be complicated in cases in which patients do not present with classic signs, such as palpable purpura of the lower extremities and buttocks. In these situations, a biopsy of the skin or kidney may aid in the diagnosis.

The differential diagnosis for HSP includes acute hemorrhagic edema of infancy, coagulopathies, hemolytic uremic syndrome, hypersensitivity vasculitis, IgA nephropathy, immune thrombocytopenia purpura, juvenile idiopathic arthritis, reactive arthritis, rheumatic fever, small vessel vasculitis, and systemic lupus erythematosus.

The etiology of HSP is unclear. Most believe that it is an immune-mediated vasculitis with genetic and environmental influences. In HSP, there is leukocytoclastic vasculitis with deposition of IgA immune complexes, complement component 3 (C3), and fibrin in the affected vessel walls. Small vessels within the papillary dermis are usually present with an inflammatory infiltrate of neutrophils and monocytes.

The treatment of HSP usually consists of supportive care, since most patients will recover spontaneously. Therefore, hydration, rest, and pain relief are essential. Other treatment modalities may be required for more severe or extensive HSP and its complications.

Our patient’s biopsy results were consistent with a leukocytoclastic vasculitis. His biopsy revealed an inflammatory infiltrate of lymphocytes, neutrophils, and nuclear dust with extravasation of erythrocytes and fibrin in the walls of small blood vessels, along with prominent papillary dermal edema and subepidermal vesiculation. Direct immunofluorescence was positive for IgA, C3, and fibrinogen in upper dermal blood vessels. The patient also had renal involvement that was confirmed with a renal biopsy.

This case and photos are courtesy of Tanya Greywal, University of California, San Diego, and Dr. Brooke Resh Sateesh, San Diego Family Dermatology.

Dr. Bilu Martin is in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit your case for possible publication, send an email to [email protected].

Diagnosis: Basal cell carcinoma

Basal cell carcinoma (BCC) is the most common skin cancer diagnosed in the United States, with approximately 2.8 million cases diagnosed annually, according to the Skin Cancer Foundation. While a minority of cases are linked to genetic syndromes (e.g., basal cell nevus syndrome), most cases result from ultraviolet sun exposure. A power law model was recently described linking ultraviolet exposure and incidence of basal cell carcinoma.

Many diagnosed BCC cases are small (< 1cm in size) and easily treated in the clinic.  In cases where treatment is delayed for years due to financial, psychological, or psychiatric reasons, tumors can cause significant local tissue destruction and grow to alarming sizes.

The differential diagnosis of BCC may vary depending on the clinical sub-type (e.g., superficial, nodular, infiltrative, etc). Nodular BCC may mimic adnexal neoplasms, intradermal melanocytic nevi, Merkel cell carcinoma, or even amelanotic melanoma. Superficial BCC may mimic a lichenoid keratosis, Bowen’s disease, or other inflammatory conditions such as psoriasis and dermatitis. A larger lesion may mimic chronic infections such as mycetoma, or distant metastases from another primary carcinoma (breast or renal). Location and history can assist with teasing out the probable cause.

While diagnosis of this lesion can be made based on history and clinical appearance, this is best assisted with a biopsy, preferably a punch or incisional biopsy as chronic scarring and pseudoepitheliomatous hyperplasia may affect the pathologic diagnosis. Bacteria often colonize these large tumors and can lead to secondary infections. In this patient, maggots were noted in the skin.

While there are multiple modalities available for treating small tumors (e.g., topical imiquimod, 5-fluorouracil, electrodessication and curettage, excision, Mohs), larger tumors are often handled differently. While some might consider radiation therapy in this case, typically Mohs surgery is the treatment of choice. Oral vismodegib, a smoothened inhibitor, has been marketed for locally advanced basal cell carcinoma and has been used as a primary or adjunctive therapy with Mohs in tumors of this size. One important factor determining the choice of treatment is whether the primary tumor has spread. In tumors who have been left untreated for a long time, it is reasonable to image the patient to evaluate for metastases.

We present this case as an example where Mohs provided immediate definitive treatment. This tumor was cleared in 1 stage, with 67 slides read to evaluate the entire peripheral and deep margin. The Mohs surgery was performed under local anesthesia with no additional sedatives and 3-0 nylon sutures were used to approximate the wound. A two month follow-up photo is shown showing an almost healed surgical wound with an acceptable cosmetic result.

Diagnosis: Basal cell carcinoma

Basal cell carcinoma (BCC) is the most common skin cancer diagnosed in the United States, with approximately 2.8 million cases diagnosed annually, according to the Skin Cancer Foundation. While a minority of cases are linked to genetic syndromes (e.g., basal cell nevus syndrome), most cases result from ultraviolet sun exposure. A power law model was recently described linking ultraviolet exposure and incidence of basal cell carcinoma.

Many diagnosed BCC cases are small (< 1cm in size) and easily treated in the clinic.  In cases where treatment is delayed for years due to financial, psychological, or psychiatric reasons, tumors can cause significant local tissue destruction and grow to alarming sizes.

The differential diagnosis of BCC may vary depending on the clinical sub-type (e.g., superficial, nodular, infiltrative, etc). Nodular BCC may mimic adnexal neoplasms, intradermal melanocytic nevi, Merkel cell carcinoma, or even amelanotic melanoma. Superficial BCC may mimic a lichenoid keratosis, Bowen’s disease, or other inflammatory conditions such as psoriasis and dermatitis. A larger lesion may mimic chronic infections such as mycetoma, or distant metastases from another primary carcinoma (breast or renal). Location and history can assist with teasing out the probable cause.

While diagnosis of this lesion can be made based on history and clinical appearance, this is best assisted with a biopsy, preferably a punch or incisional biopsy as chronic scarring and pseudoepitheliomatous hyperplasia may affect the pathologic diagnosis. Bacteria often colonize these large tumors and can lead to secondary infections. In this patient, maggots were noted in the skin.

While there are multiple modalities available for treating small tumors (e.g., topical imiquimod, 5-fluorouracil, electrodessication and curettage, excision, Mohs), larger tumors are often handled differently. While some might consider radiation therapy in this case, typically Mohs surgery is the treatment of choice. Oral vismodegib, a smoothened inhibitor, has been marketed for locally advanced basal cell carcinoma and has been used as a primary or adjunctive therapy with Mohs in tumors of this size. One important factor determining the choice of treatment is whether the primary tumor has spread. In tumors who have been left untreated for a long time, it is reasonable to image the patient to evaluate for metastases.

We present this case as an example where Mohs provided immediate definitive treatment. This tumor was cleared in 1 stage, with 67 slides read to evaluate the entire peripheral and deep margin. The Mohs surgery was performed under local anesthesia with no additional sedatives and 3-0 nylon sutures were used to approximate the wound. A two month follow-up photo is shown showing an almost healed surgical wound with an acceptable cosmetic result.

Diagnosis: Basal cell carcinoma

Basal cell carcinoma (BCC) is the most common skin cancer diagnosed in the United States, with approximately 2.8 million cases diagnosed annually, according to the Skin Cancer Foundation. While a minority of cases are linked to genetic syndromes (e.g., basal cell nevus syndrome), most cases result from ultraviolet sun exposure. A power law model was recently described linking ultraviolet exposure and incidence of basal cell carcinoma.

Many diagnosed BCC cases are small (< 1cm in size) and easily treated in the clinic.  In cases where treatment is delayed for years due to financial, psychological, or psychiatric reasons, tumors can cause significant local tissue destruction and grow to alarming sizes.

The differential diagnosis of BCC may vary depending on the clinical sub-type (e.g., superficial, nodular, infiltrative, etc). Nodular BCC may mimic adnexal neoplasms, intradermal melanocytic nevi, Merkel cell carcinoma, or even amelanotic melanoma. Superficial BCC may mimic a lichenoid keratosis, Bowen’s disease, or other inflammatory conditions such as psoriasis and dermatitis. A larger lesion may mimic chronic infections such as mycetoma, or distant metastases from another primary carcinoma (breast or renal). Location and history can assist with teasing out the probable cause.

While diagnosis of this lesion can be made based on history and clinical appearance, this is best assisted with a biopsy, preferably a punch or incisional biopsy as chronic scarring and pseudoepitheliomatous hyperplasia may affect the pathologic diagnosis. Bacteria often colonize these large tumors and can lead to secondary infections. In this patient, maggots were noted in the skin.

While there are multiple modalities available for treating small tumors (e.g., topical imiquimod, 5-fluorouracil, electrodessication and curettage, excision, Mohs), larger tumors are often handled differently. While some might consider radiation therapy in this case, typically Mohs surgery is the treatment of choice. Oral vismodegib, a smoothened inhibitor, has been marketed for locally advanced basal cell carcinoma and has been used as a primary or adjunctive therapy with Mohs in tumors of this size. One important factor determining the choice of treatment is whether the primary tumor has spread. In tumors who have been left untreated for a long time, it is reasonable to image the patient to evaluate for metastases.

We present this case as an example where Mohs provided immediate definitive treatment. This tumor was cleared in 1 stage, with 67 slides read to evaluate the entire peripheral and deep margin. The Mohs surgery was performed under local anesthesia with no additional sedatives and 3-0 nylon sutures were used to approximate the wound. A two month follow-up photo is shown showing an almost healed surgical wound with an acceptable cosmetic result.

Diagnosis: Systemic sclerosis

Systemic sclerosis, or scleroderma, is a rare connective tissue disorder in which excessive collagen is deposited in the skin and internal organs. This disease predominantly affects women (3-6:1) between the ages of 20 and 60 years with no apparent racial predominance. Effective treatment is critical, as scleroderma carries a poor prognosis, with a mortality rate of up to 50% at 5 years in severe cases. The pathogenesis of systemic sclerosis is unknown, but three pathways are implicated, including immune deregulation, vascular abnormalities, and abnormal fibroblast activation.

Clinical presentation is variable because of the involvement of multiple organ systems. Common features include cutaneous pruritus, skin thickening, Raynaud's phenomenon, difficulty swallowing, shortness of breath, palpitations, nonproductive cough, and joint pain and swelling, as well as muscle pain and weakness. Laboratory findings may include elevated erythrocyte sedimentation rate, thrombocytopenia, hypergammaglobulinemia, increased urea and creatinine levels, and elevated C-reactive protein. Antinuclear antibodies are usually elevated, especially Scl-70, antimitochondrial, and anticentromere antibodies. Cardiac and pulmonary function should be assessed upon diagnosis. A Doppler echocardiogram may detect cardiac abnormalities, and chest x-ray or high-resolution CT is used to assess for pulmonary fibrosis.

Despite the severity of the disease, there are no Food and Drug Administration-approved disease-modifying agents for the treatment of scleroderma, and management often focuses on symptom relief. For example, patients with kidney involvement should be placed on an ACE inhibitor or angiotensin II inhibitor therapy, and patients with gastrointestinal tract involvement should use proton pump inhibitors and H2 blockers to control reflux. Bosentan and pentoxifylline, which target vascular abnormalities, also may help improve skin fibrosis. Steroids show benefits in the early stages of the disease, but carry a risk of scleroderma renal crisis with doses greater than 15 mg of prednisone daily. Mycophenolate mofetil and sirolimus have immunomodulatory and antifibrotic properties, which may be of benefit in this disease.

Cyclophosphamide is reserved for more severe cases. Other treatment modalities include rituximab, intravenous immunoglobulin, and autologous stem cell transplantation.

Diagnosis: Systemic sclerosis

Systemic sclerosis, or scleroderma, is a rare connective tissue disorder in which excessive collagen is deposited in the skin and internal organs. This disease predominantly affects women (3-6:1) between the ages of 20 and 60 years with no apparent racial predominance. Effective treatment is critical, as scleroderma carries a poor prognosis, with a mortality rate of up to 50% at 5 years in severe cases. The pathogenesis of systemic sclerosis is unknown, but three pathways are implicated, including immune deregulation, vascular abnormalities, and abnormal fibroblast activation.

Clinical presentation is variable because of the involvement of multiple organ systems. Common features include cutaneous pruritus, skin thickening, Raynaud's phenomenon, difficulty swallowing, shortness of breath, palpitations, nonproductive cough, and joint pain and swelling, as well as muscle pain and weakness. Laboratory findings may include elevated erythrocyte sedimentation rate, thrombocytopenia, hypergammaglobulinemia, increased urea and creatinine levels, and elevated C-reactive protein. Antinuclear antibodies are usually elevated, especially Scl-70, antimitochondrial, and anticentromere antibodies. Cardiac and pulmonary function should be assessed upon diagnosis. A Doppler echocardiogram may detect cardiac abnormalities, and chest x-ray or high-resolution CT is used to assess for pulmonary fibrosis.

Despite the severity of the disease, there are no Food and Drug Administration-approved disease-modifying agents for the treatment of scleroderma, and management often focuses on symptom relief. For example, patients with kidney involvement should be placed on an ACE inhibitor or angiotensin II inhibitor therapy, and patients with gastrointestinal tract involvement should use proton pump inhibitors and H2 blockers to control reflux. Bosentan and pentoxifylline, which target vascular abnormalities, also may help improve skin fibrosis. Steroids show benefits in the early stages of the disease, but carry a risk of scleroderma renal crisis with doses greater than 15 mg of prednisone daily. Mycophenolate mofetil and sirolimus have immunomodulatory and antifibrotic properties, which may be of benefit in this disease.

Cyclophosphamide is reserved for more severe cases. Other treatment modalities include rituximab, intravenous immunoglobulin, and autologous stem cell transplantation.

Diagnosis: Systemic sclerosis

Systemic sclerosis, or scleroderma, is a rare connective tissue disorder in which excessive collagen is deposited in the skin and internal organs. This disease predominantly affects women (3-6:1) between the ages of 20 and 60 years with no apparent racial predominance. Effective treatment is critical, as scleroderma carries a poor prognosis, with a mortality rate of up to 50% at 5 years in severe cases. The pathogenesis of systemic sclerosis is unknown, but three pathways are implicated, including immune deregulation, vascular abnormalities, and abnormal fibroblast activation.

Clinical presentation is variable because of the involvement of multiple organ systems. Common features include cutaneous pruritus, skin thickening, Raynaud's phenomenon, difficulty swallowing, shortness of breath, palpitations, nonproductive cough, and joint pain and swelling, as well as muscle pain and weakness. Laboratory findings may include elevated erythrocyte sedimentation rate, thrombocytopenia, hypergammaglobulinemia, increased urea and creatinine levels, and elevated C-reactive protein. Antinuclear antibodies are usually elevated, especially Scl-70, antimitochondrial, and anticentromere antibodies. Cardiac and pulmonary function should be assessed upon diagnosis. A Doppler echocardiogram may detect cardiac abnormalities, and chest x-ray or high-resolution CT is used to assess for pulmonary fibrosis.

Despite the severity of the disease, there are no Food and Drug Administration-approved disease-modifying agents for the treatment of scleroderma, and management often focuses on symptom relief. For example, patients with kidney involvement should be placed on an ACE inhibitor or angiotensin II inhibitor therapy, and patients with gastrointestinal tract involvement should use proton pump inhibitors and H2 blockers to control reflux. Bosentan and pentoxifylline, which target vascular abnormalities, also may help improve skin fibrosis. Steroids show benefits in the early stages of the disease, but carry a risk of scleroderma renal crisis with doses greater than 15 mg of prednisone daily. Mycophenolate mofetil and sirolimus have immunomodulatory and antifibrotic properties, which may be of benefit in this disease.

Cyclophosphamide is reserved for more severe cases. Other treatment modalities include rituximab, intravenous immunoglobulin, and autologous stem cell transplantation.

Diagnosis: Systemic sclerosis

Systemic sclerosis, or scleroderma, is a rare connective tissue disorder in which excessive collagen is deposited in the skin and internal organs. This disease predominantly affects women (3-6:1) between the ages of 20 and 60 years with no apparent racial predominance. Effective treatment is critical, as scleroderma carries a poor prognosis, with a mortality rate of up to 50% at 5 years in severe cases. The pathogenesis of systemic sclerosis is unknown, but three pathways are implicated, including immune deregulation, vascular abnormalities, and abnormal fibroblast activation.

Clinical presentation is variable because of the involvement of multiple organ systems. Common features include cutaneous pruritus, skin thickening, Raynaud's phenomenon, difficulty swallowing, shortness of breath, palpitations, nonproductive cough, and joint pain and swelling, as well as muscle pain and weakness. Laboratory findings may include elevated erythrocyte sedimentation rate, thrombocytopenia, hypergammaglobulinemia, increased urea and creatinine levels, and elevated C-reactive protein. Antinuclear antibodies are usually elevated, especially Scl-70, antimitochondrial, and anticentromere antibodies. Cardiac and pulmonary function should be assessed upon diagnosis. A Doppler echocardiogram may detect cardiac abnormalities, and chest x-ray or high-resolution CT is used to assess for pulmonary fibrosis.

Despite the severity of the disease, there are no Food and Drug Administration-approved disease-modifying agents for the treatment of scleroderma, and management often focuses on symptom relief. For example, patients with kidney involvement should be placed on an ACE inhibitor or angiotensin II inhibitor therapy, and patients with gastrointestinal tract involvement should use proton pump inhibitors and H2 blockers to control reflux. Bosentan and pentoxifylline, which target vascular abnormalities, also may help improve skin fibrosis. Steroids show benefits in the early stages of the disease, but carry a risk of scleroderma renal crisis with doses greater than 15 mg of prednisone daily. Mycophenolate mofetil and sirolimus have immunomodulatory and antifibrotic properties, which may be of benefit in this disease.

Cyclophosphamide is reserved for more severe cases. Other treatment modalities include rituximab, intravenous immunoglobulin, and autologous stem cell transplantation.

Diagnosis: Systemic sclerosis

Systemic sclerosis, or scleroderma, is a rare connective tissue disorder in which excessive collagen is deposited in the skin and internal organs. This disease predominantly affects women (3-6:1) between the ages of 20 and 60 years with no apparent racial predominance. Effective treatment is critical, as scleroderma carries a poor prognosis, with a mortality rate of up to 50% at 5 years in severe cases. The pathogenesis of systemic sclerosis is unknown, but three pathways are implicated, including immune deregulation, vascular abnormalities, and abnormal fibroblast activation.

Clinical presentation is variable because of the involvement of multiple organ systems. Common features include cutaneous pruritus, skin thickening, Raynaud's phenomenon, difficulty swallowing, shortness of breath, palpitations, nonproductive cough, and joint pain and swelling, as well as muscle pain and weakness. Laboratory findings may include elevated erythrocyte sedimentation rate, thrombocytopenia, hypergammaglobulinemia, increased urea and creatinine levels, and elevated C-reactive protein. Antinuclear antibodies are usually elevated, especially Scl-70, antimitochondrial, and anticentromere antibodies. Cardiac and pulmonary function should be assessed upon diagnosis. A Doppler echocardiogram may detect cardiac abnormalities, and chest x-ray or high-resolution CT is used to assess for pulmonary fibrosis.

Despite the severity of the disease, there are no Food and Drug Administration-approved disease-modifying agents for the treatment of scleroderma, and management often focuses on symptom relief. For example, patients with kidney involvement should be placed on an ACE inhibitor or angiotensin II inhibitor therapy, and patients with gastrointestinal tract involvement should use proton pump inhibitors and H2 blockers to control reflux. Bosentan and pentoxifylline, which target vascular abnormalities, also may help improve skin fibrosis. Steroids show benefits in the early stages of the disease, but carry a risk of scleroderma renal crisis with doses greater than 15 mg of prednisone daily. Mycophenolate mofetil and sirolimus have immunomodulatory and antifibrotic properties, which may be of benefit in this disease.

Cyclophosphamide is reserved for more severe cases. Other treatment modalities include rituximab, intravenous immunoglobulin, and autologous stem cell transplantation.

Diagnosis: Systemic sclerosis

Systemic sclerosis, or scleroderma, is a rare connective tissue disorder in which excessive collagen is deposited in the skin and internal organs. This disease predominantly affects women (3-6:1) between the ages of 20 and 60 years with no apparent racial predominance. Effective treatment is critical, as scleroderma carries a poor prognosis, with a mortality rate of up to 50% at 5 years in severe cases. The pathogenesis of systemic sclerosis is unknown, but three pathways are implicated, including immune deregulation, vascular abnormalities, and abnormal fibroblast activation.

Clinical presentation is variable because of the involvement of multiple organ systems. Common features include cutaneous pruritus, skin thickening, Raynaud's phenomenon, difficulty swallowing, shortness of breath, palpitations, nonproductive cough, and joint pain and swelling, as well as muscle pain and weakness. Laboratory findings may include elevated erythrocyte sedimentation rate, thrombocytopenia, hypergammaglobulinemia, increased urea and creatinine levels, and elevated C-reactive protein. Antinuclear antibodies are usually elevated, especially Scl-70, antimitochondrial, and anticentromere antibodies. Cardiac and pulmonary function should be assessed upon diagnosis. A Doppler echocardiogram may detect cardiac abnormalities, and chest x-ray or high-resolution CT is used to assess for pulmonary fibrosis.

Despite the severity of the disease, there are no Food and Drug Administration-approved disease-modifying agents for the treatment of scleroderma, and management often focuses on symptom relief. For example, patients with kidney involvement should be placed on an ACE inhibitor or angiotensin II inhibitor therapy, and patients with gastrointestinal tract involvement should use proton pump inhibitors and H2 blockers to control reflux. Bosentan and pentoxifylline, which target vascular abnormalities, also may help improve skin fibrosis. Steroids show benefits in the early stages of the disease, but carry a risk of scleroderma renal crisis with doses greater than 15 mg of prednisone daily. Mycophenolate mofetil and sirolimus have immunomodulatory and antifibrotic properties, which may be of benefit in this disease.

Cyclophosphamide is reserved for more severe cases. Other treatment modalities include rituximab, intravenous immunoglobulin, and autologous stem cell transplantation.

Diagnosis: Kaposi’s sarcoma

Kaposi's sarcoma is a vascular neoplasm with four principal clinical variants: HIV/AIDS-related Kaposi's sarcoma, classic Kaposi's sarcoma, African endemic Kaposi's sarcoma, and immunosuppression-associated Kaposi's sarcoma. The etiologic agent in all clinical variants is human herpes virus type 8 (HHV-8). HIV/AIDS-related Kaposi's sarcoma is primarily seen in men who have sex with men.

The four variants of Kaposi's sarcoma can have different clinical presentations. In HIV/AIDS-associated Kaposi's sarcoma, patients may present with a single lesion or with symmetric widespread lesions. Clinically, the lesions can range from faint erythematous macules, to small violaceous papules, to large plaques or ulcerated nodules. The lesions are generally asymptomatic.

Any mucocutaneous surface can be involved. Common body locations include the face (especially the nose), hard palate, trunk, penis, lower legs, and soles. The most common areas of internal involvement are the gastrointestinal system and lymphatics. Histologically, atypical, angular vessels with an associated inflammatory infiltrate containing plasma cells appear in the upper dermis in macular lesions. Nodules and tumors reveal a spindle cell neoplasm pattern. Lesions stain positive for human herpes virus 8 (HHV-8).

Since the introduction of highly active antiretroviral therapy (HAART), the incidence of Kaposi's sarcoma has greatly decreased. HAART is the most effective treatment method, and should be the initial therapy in most patients with mild to moderate disease.

However, some patients with HIV/AIDS-associated Kaposi's sarcoma require further treatment - those who have well-controlled HIV and undetectable viral loads. Other treatments include local destruction (cryotherapy), topical alitretinoin (9-cis-retinoic acid), intralesional interferon or vinblastine, superficial radiotherapy, liposomal doxorubicin, daunorubicin, or paclitaxel.

Diagnosis: Kaposi’s sarcoma

Kaposi's sarcoma is a vascular neoplasm with four principal clinical variants: HIV/AIDS-related Kaposi's sarcoma, classic Kaposi's sarcoma, African endemic Kaposi's sarcoma, and immunosuppression-associated Kaposi's sarcoma. The etiologic agent in all clinical variants is human herpes virus type 8 (HHV-8). HIV/AIDS-related Kaposi's sarcoma is primarily seen in men who have sex with men.

The four variants of Kaposi's sarcoma can have different clinical presentations. In HIV/AIDS-associated Kaposi's sarcoma, patients may present with a single lesion or with symmetric widespread lesions. Clinically, the lesions can range from faint erythematous macules, to small violaceous papules, to large plaques or ulcerated nodules. The lesions are generally asymptomatic.

Any mucocutaneous surface can be involved. Common body locations include the face (especially the nose), hard palate, trunk, penis, lower legs, and soles. The most common areas of internal involvement are the gastrointestinal system and lymphatics. Histologically, atypical, angular vessels with an associated inflammatory infiltrate containing plasma cells appear in the upper dermis in macular lesions. Nodules and tumors reveal a spindle cell neoplasm pattern. Lesions stain positive for human herpes virus 8 (HHV-8).

Since the introduction of highly active antiretroviral therapy (HAART), the incidence of Kaposi's sarcoma has greatly decreased. HAART is the most effective treatment method, and should be the initial therapy in most patients with mild to moderate disease.

However, some patients with HIV/AIDS-associated Kaposi's sarcoma require further treatment - those who have well-controlled HIV and undetectable viral loads. Other treatments include local destruction (cryotherapy), topical alitretinoin (9-cis-retinoic acid), intralesional interferon or vinblastine, superficial radiotherapy, liposomal doxorubicin, daunorubicin, or paclitaxel.

Diagnosis: Kaposi’s sarcoma

Kaposi's sarcoma is a vascular neoplasm with four principal clinical variants: HIV/AIDS-related Kaposi's sarcoma, classic Kaposi's sarcoma, African endemic Kaposi's sarcoma, and immunosuppression-associated Kaposi's sarcoma. The etiologic agent in all clinical variants is human herpes virus type 8 (HHV-8). HIV/AIDS-related Kaposi's sarcoma is primarily seen in men who have sex with men.

The four variants of Kaposi's sarcoma can have different clinical presentations. In HIV/AIDS-associated Kaposi's sarcoma, patients may present with a single lesion or with symmetric widespread lesions. Clinically, the lesions can range from faint erythematous macules, to small violaceous papules, to large plaques or ulcerated nodules. The lesions are generally asymptomatic.

Any mucocutaneous surface can be involved. Common body locations include the face (especially the nose), hard palate, trunk, penis, lower legs, and soles. The most common areas of internal involvement are the gastrointestinal system and lymphatics. Histologically, atypical, angular vessels with an associated inflammatory infiltrate containing plasma cells appear in the upper dermis in macular lesions. Nodules and tumors reveal a spindle cell neoplasm pattern. Lesions stain positive for human herpes virus 8 (HHV-8).

Since the introduction of highly active antiretroviral therapy (HAART), the incidence of Kaposi's sarcoma has greatly decreased. HAART is the most effective treatment method, and should be the initial therapy in most patients with mild to moderate disease.

However, some patients with HIV/AIDS-associated Kaposi's sarcoma require further treatment - those who have well-controlled HIV and undetectable viral loads. Other treatments include local destruction (cryotherapy), topical alitretinoin (9-cis-retinoic acid), intralesional interferon or vinblastine, superficial radiotherapy, liposomal doxorubicin, daunorubicin, or paclitaxel.

Diagnosis: Granuloma annulare

Granuloma annulare (GA) is a self-limited cutaneous disorder predominantly seen in women that affects children and adults. The cause is unknown. Inciting factors can include herpes zoster infection, sun exposure, medications, and trauma.

Several clinical variants exist. Localized GA is the most common form, often presenting in the first three decades of life as an asymptomatic, erythematous, annular plaque with a firm border and central clearing localized to the wrists, ankles, and dorsal hands or feet. Generalized GA accounts for 15% of reported cases and presents in the fourth to seventh decades of life as multiple asymptomatic or pruritic skin-colored or erythematous papules and plaques on the trunk and extremities. Subcutaneous GA is more common in children and presents as multiple painless nodules on the scalp or extremities. Patch GA can be localized or generalized. Perforating GA presents as asymptomatic erythematous papules that evolve into yellow, umbilicated papules with a clear-to-white discharge.

Histopathologically, an interstitial or palisading pattern is seen with a dermal lymphohistiocytic infiltrate, degenerated collagen, and mucin deposition (visualized with alcian blue or colloidal iron stains). The interstitial pattern presents in the majority of cases.

Diagnosis of GA is predominantly clinically based. When the diagnosis is questionable or the presentation is atypical, biopsy is useful. Granuloma annulare is often self-limiting and resolves within 2 years, although recurrence is possible. First-line therapy for localized GA includes high-potency topical corticosteroids or intralesional corticosteroids. Other treatments include cryotherapy, phototherapy, and topical tacrolimus. For generalized GA, topical or intralesional corticosteroids may be used for select lesions. Topical calcineurin inhibitors, light therapy, hydroxychloroquine, isotretinion, and dapsone also have been reported as treatments in the literature.

Diagnosis: Granuloma annulare

Granuloma annulare (GA) is a self-limited cutaneous disorder predominantly seen in women that affects children and adults. The cause is unknown. Inciting factors can include herpes zoster infection, sun exposure, medications, and trauma.

Several clinical variants exist. Localized GA is the most common form, often presenting in the first three decades of life as an asymptomatic, erythematous, annular plaque with a firm border and central clearing localized to the wrists, ankles, and dorsal hands or feet. Generalized GA accounts for 15% of reported cases and presents in the fourth to seventh decades of life as multiple asymptomatic or pruritic skin-colored or erythematous papules and plaques on the trunk and extremities. Subcutaneous GA is more common in children and presents as multiple painless nodules on the scalp or extremities. Patch GA can be localized or generalized. Perforating GA presents as asymptomatic erythematous papules that evolve into yellow, umbilicated papules with a clear-to-white discharge.

Histopathologically, an interstitial or palisading pattern is seen with a dermal lymphohistiocytic infiltrate, degenerated collagen, and mucin deposition (visualized with alcian blue or colloidal iron stains). The interstitial pattern presents in the majority of cases.

Diagnosis of GA is predominantly clinically based. When the diagnosis is questionable or the presentation is atypical, biopsy is useful. Granuloma annulare is often self-limiting and resolves within 2 years, although recurrence is possible. First-line therapy for localized GA includes high-potency topical corticosteroids or intralesional corticosteroids. Other treatments include cryotherapy, phototherapy, and topical tacrolimus. For generalized GA, topical or intralesional corticosteroids may be used for select lesions. Topical calcineurin inhibitors, light therapy, hydroxychloroquine, isotretinion, and dapsone also have been reported as treatments in the literature.

Diagnosis: Granuloma annulare

Granuloma annulare (GA) is a self-limited cutaneous disorder predominantly seen in women that affects children and adults. The cause is unknown. Inciting factors can include herpes zoster infection, sun exposure, medications, and trauma.

Several clinical variants exist. Localized GA is the most common form, often presenting in the first three decades of life as an asymptomatic, erythematous, annular plaque with a firm border and central clearing localized to the wrists, ankles, and dorsal hands or feet. Generalized GA accounts for 15% of reported cases and presents in the fourth to seventh decades of life as multiple asymptomatic or pruritic skin-colored or erythematous papules and plaques on the trunk and extremities. Subcutaneous GA is more common in children and presents as multiple painless nodules on the scalp or extremities. Patch GA can be localized or generalized. Perforating GA presents as asymptomatic erythematous papules that evolve into yellow, umbilicated papules with a clear-to-white discharge.

Histopathologically, an interstitial or palisading pattern is seen with a dermal lymphohistiocytic infiltrate, degenerated collagen, and mucin deposition (visualized with alcian blue or colloidal iron stains). The interstitial pattern presents in the majority of cases.

Diagnosis of GA is predominantly clinically based. When the diagnosis is questionable or the presentation is atypical, biopsy is useful. Granuloma annulare is often self-limiting and resolves within 2 years, although recurrence is possible. First-line therapy for localized GA includes high-potency topical corticosteroids or intralesional corticosteroids. Other treatments include cryotherapy, phototherapy, and topical tacrolimus. For generalized GA, topical or intralesional corticosteroids may be used for select lesions. Topical calcineurin inhibitors, light therapy, hydroxychloroquine, isotretinion, and dapsone also have been reported as treatments in the literature.

Diagnosis: Lichen Planus

Lichen planus is a common inflammatory condition involving the skin, nails, mucous membranes, and hair follicles. It has no racial predilection, and often affects men and women aged 20-60 years. It is less common in children, who account for only 4% of cases. The lesions are often atypical.

Clinically, patients often present with erythematous to violaceous small, flat-topped, polygonal papules that may coalesce into plaques. Lesions are generally pruritic, and may be tender or painful. Older lesions may be hyperpigmented. White streaks known as Wickham striae can cross the surface of lesions. These striae also can be present orally, such as in the patient described here. Oral lesions also may be atrophic or erosive.

Common body locations with involvement are the inner wrists, legs, torso, or genitals (glans penis). The face is rarely involved. Nail changes, such as longitudinal ridging and splitting, onycholysis, red lunula, yellow nail syndrome, and pterygium formation, can occur.

Lichen planus often spontaneously resolves on its own, with 2/3 of patients resolving in a year. Mucous membrane disease tends to be more chronic. The etiology of lichen planus is unknown. It may have an autoimmune mechanism in which T cells induce keratinocytes to undergo apoptosis. Between 4% and 60% of lichen planus patients also have hepatitis C infections. The differential diagnosis for cutaneous lesions include lichenoid drug eruption, guttate psoriasis, syphilis, and pityriasis lichenoides et varioliformis acuta. Oral lesions may resemble candidiasis, leukoplakia, malignancies, and bullous disease.

Topical and intralesional steroids are often effective for localized disease. Systemic corticosteroids can be useful when lesions are widespread. Phototherapy, isotretinoin, acitretin, hydroxychloroquine, and oral immunosuppressive agents (such as cyclosporine and mycophenolate mofetil) all have been described in the treatment of lichen planus.

Diagnosis: Lichen Planus

Lichen planus is a common inflammatory condition involving the skin, nails, mucous membranes, and hair follicles. It has no racial predilection, and often affects men and women aged 20-60 years. It is less common in children, who account for only 4% of cases. The lesions are often atypical.

Clinically, patients often present with erythematous to violaceous small, flat-topped, polygonal papules that may coalesce into plaques. Lesions are generally pruritic, and may be tender or painful. Older lesions may be hyperpigmented. White streaks known as Wickham striae can cross the surface of lesions. These striae also can be present orally, such as in the patient described here. Oral lesions also may be atrophic or erosive.

Common body locations with involvement are the inner wrists, legs, torso, or genitals (glans penis). The face is rarely involved. Nail changes, such as longitudinal ridging and splitting, onycholysis, red lunula, yellow nail syndrome, and pterygium formation, can occur.

Lichen planus often spontaneously resolves on its own, with 2/3 of patients resolving in a year. Mucous membrane disease tends to be more chronic. The etiology of lichen planus is unknown. It may have an autoimmune mechanism in which T cells induce keratinocytes to undergo apoptosis. Between 4% and 60% of lichen planus patients also have hepatitis C infections. The differential diagnosis for cutaneous lesions include lichenoid drug eruption, guttate psoriasis, syphilis, and pityriasis lichenoides et varioliformis acuta. Oral lesions may resemble candidiasis, leukoplakia, malignancies, and bullous disease.

Topical and intralesional steroids are often effective for localized disease. Systemic corticosteroids can be useful when lesions are widespread. Phototherapy, isotretinoin, acitretin, hydroxychloroquine, and oral immunosuppressive agents (such as cyclosporine and mycophenolate mofetil) all have been described in the treatment of lichen planus.

Diagnosis: Lichen Planus

Lichen planus is a common inflammatory condition involving the skin, nails, mucous membranes, and hair follicles. It has no racial predilection, and often affects men and women aged 20-60 years. It is less common in children, who account for only 4% of cases. The lesions are often atypical.

Clinically, patients often present with erythematous to violaceous small, flat-topped, polygonal papules that may coalesce into plaques. Lesions are generally pruritic, and may be tender or painful. Older lesions may be hyperpigmented. White streaks known as Wickham striae can cross the surface of lesions. These striae also can be present orally, such as in the patient described here. Oral lesions also may be atrophic or erosive.

Common body locations with involvement are the inner wrists, legs, torso, or genitals (glans penis). The face is rarely involved. Nail changes, such as longitudinal ridging and splitting, onycholysis, red lunula, yellow nail syndrome, and pterygium formation, can occur.

Lichen planus often spontaneously resolves on its own, with 2/3 of patients resolving in a year. Mucous membrane disease tends to be more chronic. The etiology of lichen planus is unknown. It may have an autoimmune mechanism in which T cells induce keratinocytes to undergo apoptosis. Between 4% and 60% of lichen planus patients also have hepatitis C infections. The differential diagnosis for cutaneous lesions include lichenoid drug eruption, guttate psoriasis, syphilis, and pityriasis lichenoides et varioliformis acuta. Oral lesions may resemble candidiasis, leukoplakia, malignancies, and bullous disease.

Topical and intralesional steroids are often effective for localized disease. Systemic corticosteroids can be useful when lesions are widespread. Phototherapy, isotretinoin, acitretin, hydroxychloroquine, and oral immunosuppressive agents (such as cyclosporine and mycophenolate mofetil) all have been described in the treatment of lichen planus.

Diagnosis: Bleomycin-induced flagellate hyperpigmentation

Bleomycin is an antineoplastic agent. Most reported side effects involve the lung and skin since these organs have lower concentrations of the enzyme that detoxifies bleomycin. Other dermatologic side effects of bleomycin include Raynaud's phenomenon, hyperkeratosis, nailbed changes, and peeling of the skin on the palmar and plantar surfaces.

Flagellate erythema has been described in relation to bleomycin treatment along with other reported associations with peplomycin (a bleomycin derivative), docetaxel, dermatomyositis, adult-onset Still's disease, and shiitake mushroom dermatitis. The rash may appear following administration of bleomycin by any route and has been shown to be dose independent. Onset occurs anytime from 1 day to several months after exposure to the offending agent. Patients typically present with history of itching followed by the appearance of red linear streaks, which are most commonly found on the trunk. Over time, the erythema will resolve to postinflammatory hyperpigmentation.

The exact cause is unknown, but it is thought that scratching causes vasodilation with bleomycin accumulating in the skin. Diagnosis is made by physical examination of the characteristic appearance of the rash along with the history of chemotherapy. A skin biopsy may be performed. In most cases, the rash resolves spontaneously within 6-8 months. Severe rashes may warrant discontinuation of bleomycin. Using antihistamines and topical and oral corticosteroids in conjunction with bleomycin may reduce the incidence of flagellate erythema/hyperpigmentation.

Diagnosis: Bleomycin-induced flagellate hyperpigmentation

Bleomycin is an antineoplastic agent. Most reported side effects involve the lung and skin since these organs have lower concentrations of the enzyme that detoxifies bleomycin. Other dermatologic side effects of bleomycin include Raynaud's phenomenon, hyperkeratosis, nailbed changes, and peeling of the skin on the palmar and plantar surfaces.

Flagellate erythema has been described in relation to bleomycin treatment along with other reported associations with peplomycin (a bleomycin derivative), docetaxel, dermatomyositis, adult-onset Still's disease, and shiitake mushroom dermatitis. The rash may appear following administration of bleomycin by any route and has been shown to be dose independent. Onset occurs anytime from 1 day to several months after exposure to the offending agent. Patients typically present with history of itching followed by the appearance of red linear streaks, which are most commonly found on the trunk. Over time, the erythema will resolve to postinflammatory hyperpigmentation.

The exact cause is unknown, but it is thought that scratching causes vasodilation with bleomycin accumulating in the skin. Diagnosis is made by physical examination of the characteristic appearance of the rash along with the history of chemotherapy. A skin biopsy may be performed. In most cases, the rash resolves spontaneously within 6-8 months. Severe rashes may warrant discontinuation of bleomycin. Using antihistamines and topical and oral corticosteroids in conjunction with bleomycin may reduce the incidence of flagellate erythema/hyperpigmentation.

Diagnosis: Bleomycin-induced flagellate hyperpigmentation

Bleomycin is an antineoplastic agent. Most reported side effects involve the lung and skin since these organs have lower concentrations of the enzyme that detoxifies bleomycin. Other dermatologic side effects of bleomycin include Raynaud's phenomenon, hyperkeratosis, nailbed changes, and peeling of the skin on the palmar and plantar surfaces.

Flagellate erythema has been described in relation to bleomycin treatment along with other reported associations with peplomycin (a bleomycin derivative), docetaxel, dermatomyositis, adult-onset Still's disease, and shiitake mushroom dermatitis. The rash may appear following administration of bleomycin by any route and has been shown to be dose independent. Onset occurs anytime from 1 day to several months after exposure to the offending agent. Patients typically present with history of itching followed by the appearance of red linear streaks, which are most commonly found on the trunk. Over time, the erythema will resolve to postinflammatory hyperpigmentation.

The exact cause is unknown, but it is thought that scratching causes vasodilation with bleomycin accumulating in the skin. Diagnosis is made by physical examination of the characteristic appearance of the rash along with the history of chemotherapy. A skin biopsy may be performed. In most cases, the rash resolves spontaneously within 6-8 months. Severe rashes may warrant discontinuation of bleomycin. Using antihistamines and topical and oral corticosteroids in conjunction with bleomycin may reduce the incidence of flagellate erythema/hyperpigmentation.

Diagnosis: Bullous impetigo

Bullous impetigo is a superficial skin infection that is most commonly seen in children (especially under the age of 2 years). It accounts for 10% of all pediatric skin disease and 25% of all impetigo.

Bullous impetigo is a variant of impetigo that produces exfoliative or epidermolytic toxins (ETA and ETB) in response to a staphylococcal infection. ETA and ETB are glutamate-specific serine proteases that bind and cleave desmoglein-1, a glycoprotein integral to intraepidermal keratinocyte adhesion. ETA and ETB produced at the site of a staphylococcal infection drive a cutaneous blistering response caused by intraepidermal cleavage below or within the stratum granulosum.  While toxins typically act locally, they can spread systemically and produce generalized blistering. Therefore, lesions may be few and localized, or numerous and widespread.

A patient with bullous impetigo typically presents with a history of vesicular lesions that progress to flaccid bullae with little/no surrounding erythema. The color of the fluid contents can change from clear to cloudy/yellow. These bullae can easily rupture and leave a moist, erythematous base with surrounding honey-colored crust. Sometimes only the central portion of the bullae will drain, leaving a surrounding rim that retains fluid for days. Bullous impetigo most commonly affects moist, intertriginous areas. Most bullae heal without scarring, although hyperpigmentation has been reported in patients with darker skin types. Fever and other constitutional symptoms are uncommon.

The differential diagnosis of bullous impetigo includes contact dermatitis, bullous insect bites, thermal burns, pemphigus vulgaris, bullous pemphigoid, erythema multiforme, and dermatitis herpetiformis. It is especially important to rule out herpes simplex, varicella, bullous tinea, bullous fixed drug reaction, bullous drug eruption, and staphylococcal scalded skin syndrome.

The diagnosis of bullous impetigo is usually based upon clinical presentation. However, the diagnosis can easily be confirmed through bacterial culture of blister fluid that isolates Staphylococcus. Nikolsky’s sign is usually negative.

Bullous impetigo is self-limiting and, if left untreated, will typically resolve in weeks to months. The treatment of choice includes topical mupirocin ointment, along with local cleansing and crust removal. Oral antistaphylococcal drugs for 7-10 days are prescribed in the setting of widespread or complicated disease. Penicillinase-resistant penicillins and cephalosporins are effective against methicillin-susceptible Staphylococcus aureus. It is also important to consider methicillin-resistant S. aureus  and treat with trimethoprim-sulfamethoxazole, clindamycin, or linezolid. Intravenous vancomycin should be reserved for patients with severe, widespread disease.

This patient was treated with cephalexin and mupirocin. Her lesions began drying up 2 days after the initiation of antibiotics. Bacterial culture results were positive for Staphylococcus. 

Diagnosis: Bullous impetigo

Bullous impetigo is a superficial skin infection that is most commonly seen in children (especially under the age of 2 years). It accounts for 10% of all pediatric skin disease and 25% of all impetigo.

Bullous impetigo is a variant of impetigo that produces exfoliative or epidermolytic toxins (ETA and ETB) in response to a staphylococcal infection. ETA and ETB are glutamate-specific serine proteases that bind and cleave desmoglein-1, a glycoprotein integral to intraepidermal keratinocyte adhesion. ETA and ETB produced at the site of a staphylococcal infection drive a cutaneous blistering response caused by intraepidermal cleavage below or within the stratum granulosum.  While toxins typically act locally, they can spread systemically and produce generalized blistering. Therefore, lesions may be few and localized, or numerous and widespread.

A patient with bullous impetigo typically presents with a history of vesicular lesions that progress to flaccid bullae with little/no surrounding erythema. The color of the fluid contents can change from clear to cloudy/yellow. These bullae can easily rupture and leave a moist, erythematous base with surrounding honey-colored crust. Sometimes only the central portion of the bullae will drain, leaving a surrounding rim that retains fluid for days. Bullous impetigo most commonly affects moist, intertriginous areas. Most bullae heal without scarring, although hyperpigmentation has been reported in patients with darker skin types. Fever and other constitutional symptoms are uncommon.

The differential diagnosis of bullous impetigo includes contact dermatitis, bullous insect bites, thermal burns, pemphigus vulgaris, bullous pemphigoid, erythema multiforme, and dermatitis herpetiformis. It is especially important to rule out herpes simplex, varicella, bullous tinea, bullous fixed drug reaction, bullous drug eruption, and staphylococcal scalded skin syndrome.

The diagnosis of bullous impetigo is usually based upon clinical presentation. However, the diagnosis can easily be confirmed through bacterial culture of blister fluid that isolates Staphylococcus. Nikolsky’s sign is usually negative.

Bullous impetigo is self-limiting and, if left untreated, will typically resolve in weeks to months. The treatment of choice includes topical mupirocin ointment, along with local cleansing and crust removal. Oral antistaphylococcal drugs for 7-10 days are prescribed in the setting of widespread or complicated disease. Penicillinase-resistant penicillins and cephalosporins are effective against methicillin-susceptible Staphylococcus aureus. It is also important to consider methicillin-resistant S. aureus  and treat with trimethoprim-sulfamethoxazole, clindamycin, or linezolid. Intravenous vancomycin should be reserved for patients with severe, widespread disease.

This patient was treated with cephalexin and mupirocin. Her lesions began drying up 2 days after the initiation of antibiotics. Bacterial culture results were positive for Staphylococcus. 

Diagnosis: Bullous impetigo

Bullous impetigo is a superficial skin infection that is most commonly seen in children (especially under the age of 2 years). It accounts for 10% of all pediatric skin disease and 25% of all impetigo.

Bullous impetigo is a variant of impetigo that produces exfoliative or epidermolytic toxins (ETA and ETB) in response to a staphylococcal infection. ETA and ETB are glutamate-specific serine proteases that bind and cleave desmoglein-1, a glycoprotein integral to intraepidermal keratinocyte adhesion. ETA and ETB produced at the site of a staphylococcal infection drive a cutaneous blistering response caused by intraepidermal cleavage below or within the stratum granulosum.  While toxins typically act locally, they can spread systemically and produce generalized blistering. Therefore, lesions may be few and localized, or numerous and widespread.

A patient with bullous impetigo typically presents with a history of vesicular lesions that progress to flaccid bullae with little/no surrounding erythema. The color of the fluid contents can change from clear to cloudy/yellow. These bullae can easily rupture and leave a moist, erythematous base with surrounding honey-colored crust. Sometimes only the central portion of the bullae will drain, leaving a surrounding rim that retains fluid for days. Bullous impetigo most commonly affects moist, intertriginous areas. Most bullae heal without scarring, although hyperpigmentation has been reported in patients with darker skin types. Fever and other constitutional symptoms are uncommon.

The differential diagnosis of bullous impetigo includes contact dermatitis, bullous insect bites, thermal burns, pemphigus vulgaris, bullous pemphigoid, erythema multiforme, and dermatitis herpetiformis. It is especially important to rule out herpes simplex, varicella, bullous tinea, bullous fixed drug reaction, bullous drug eruption, and staphylococcal scalded skin syndrome.

The diagnosis of bullous impetigo is usually based upon clinical presentation. However, the diagnosis can easily be confirmed through bacterial culture of blister fluid that isolates Staphylococcus. Nikolsky’s sign is usually negative.

Bullous impetigo is self-limiting and, if left untreated, will typically resolve in weeks to months. The treatment of choice includes topical mupirocin ointment, along with local cleansing and crust removal. Oral antistaphylococcal drugs for 7-10 days are prescribed in the setting of widespread or complicated disease. Penicillinase-resistant penicillins and cephalosporins are effective against methicillin-susceptible Staphylococcus aureus. It is also important to consider methicillin-resistant S. aureus  and treat with trimethoprim-sulfamethoxazole, clindamycin, or linezolid. Intravenous vancomycin should be reserved for patients with severe, widespread disease.

This patient was treated with cephalexin and mupirocin. Her lesions began drying up 2 days after the initiation of antibiotics. Bacterial culture results were positive for Staphylococcus. 

Diagnosis: Roseola

Roseola, also known as exanthema subitum or sixth disease, is a common viral infection that affects children by the age of 2 years, most commonly between ages 6 and 15 months. Two strains of viruses, human herpes virus 6 and 7, are the most common causes. Roseola spreads from person to person through respiratory secretions. Symptoms can vary in severity and can start with a sudden high fever, often greater than 103° F, that can last several days and may be associated with a febrile seizure. Associated symptoms may include sore throat, runny nose, cough, and lymphadenopathy.

After the fever resolves, erythematous macules and papules develop, starting on the chest, back, or abdomen and subsequently spreading to the neck and arms. The rash is not typically pruritic, is not contagious, and can last anywhere from several hours to several days. It is important to note that a person with roseola is contagious even if no rash is present. 

Roseola can be difficult to diagnose given the nonspecific symptoms that are very similar to those of other childhood illnesses. Diagnosis is often clinical, based on history and physical, but can be confirmed by the characteristic rash or by detecting antibodies to roseola. 

Treatment of roseola is supportive, with most children recovering within a week. Over-the-counter medications to treat fever, such as acetaminophen and ibuprofen, can be used, but aspirin should be avoided to treat fever in children younger than 2 years given the risk of Reye's syndrome. Antibodies developed against roseola are protective against future infections. Measures should be taken to avoid contact with those infected and hands should be washed frequently to avoid spreading the virus. Adults can contract roseola if they did not have it as a child; the disease is milder in adults but they can spread it to children. 

Another common viral childhood illness is hand, foot, and mouth disease, caused by an enterovirus (coxsackievirus) that leads to sores and blisters on the hands, feet, and buttocks/legs that last for a week. The virus is spread through coughing and sneezing and through fecal-to-oral transmission. Symptoms include sore throat and fever with the rash. Diagnosis is clinical, based on history and physical exam, and the condition is treated supportively similar to roseola. 

Another common illness is erythema infectiosum, also known as fifth disease, and is caused by parvovirus B19. The virus is common among school-aged children. The rash typically starts as erythematous patches on the cheeks giving the characteristic "slapped cheek" appearance. By the time the rash appears, the child is no longer contagious. The virus is spread through respiratory secretions.

Adults whose occupations put them in close contact with children can become infected with symptoms, including joint pain, rather than the rash. Infection in pregnant women and patients with sickle cell disease carries an increased risk of miscarriage and severe anemia, respectively. Similar to roseola and hand, foot, and mouth disease, diagnosis of fifth disease is clinical, and treatment is largely supportive. 

Diagnosis: Roseola

Roseola, also known as exanthema subitum or sixth disease, is a common viral infection that affects children by the age of 2 years, most commonly between ages 6 and 15 months. Two strains of viruses, human herpes virus 6 and 7, are the most common causes. Roseola spreads from person to person through respiratory secretions. Symptoms can vary in severity and can start with a sudden high fever, often greater than 103° F, that can last several days and may be associated with a febrile seizure. Associated symptoms may include sore throat, runny nose, cough, and lymphadenopathy.

After the fever resolves, erythematous macules and papules develop, starting on the chest, back, or abdomen and subsequently spreading to the neck and arms. The rash is not typically pruritic, is not contagious, and can last anywhere from several hours to several days. It is important to note that a person with roseola is contagious even if no rash is present. 

Roseola can be difficult to diagnose given the nonspecific symptoms that are very similar to those of other childhood illnesses. Diagnosis is often clinical, based on history and physical, but can be confirmed by the characteristic rash or by detecting antibodies to roseola. 

Treatment of roseola is supportive, with most children recovering within a week. Over-the-counter medications to treat fever, such as acetaminophen and ibuprofen, can be used, but aspirin should be avoided to treat fever in children younger than 2 years given the risk of Reye's syndrome. Antibodies developed against roseola are protective against future infections. Measures should be taken to avoid contact with those infected and hands should be washed frequently to avoid spreading the virus. Adults can contract roseola if they did not have it as a child; the disease is milder in adults but they can spread it to children. 

Another common viral childhood illness is hand, foot, and mouth disease, caused by an enterovirus (coxsackievirus) that leads to sores and blisters on the hands, feet, and buttocks/legs that last for a week. The virus is spread through coughing and sneezing and through fecal-to-oral transmission. Symptoms include sore throat and fever with the rash. Diagnosis is clinical, based on history and physical exam, and the condition is treated supportively similar to roseola. 

Another common illness is erythema infectiosum, also known as fifth disease, and is caused by parvovirus B19. The virus is common among school-aged children. The rash typically starts as erythematous patches on the cheeks giving the characteristic "slapped cheek" appearance. By the time the rash appears, the child is no longer contagious. The virus is spread through respiratory secretions.

Adults whose occupations put them in close contact with children can become infected with symptoms, including joint pain, rather than the rash. Infection in pregnant women and patients with sickle cell disease carries an increased risk of miscarriage and severe anemia, respectively. Similar to roseola and hand, foot, and mouth disease, diagnosis of fifth disease is clinical, and treatment is largely supportive. 

Diagnosis: Roseola

Roseola, also known as exanthema subitum or sixth disease, is a common viral infection that affects children by the age of 2 years, most commonly between ages 6 and 15 months. Two strains of viruses, human herpes virus 6 and 7, are the most common causes. Roseola spreads from person to person through respiratory secretions. Symptoms can vary in severity and can start with a sudden high fever, often greater than 103° F, that can last several days and may be associated with a febrile seizure. Associated symptoms may include sore throat, runny nose, cough, and lymphadenopathy.

After the fever resolves, erythematous macules and papules develop, starting on the chest, back, or abdomen and subsequently spreading to the neck and arms. The rash is not typically pruritic, is not contagious, and can last anywhere from several hours to several days. It is important to note that a person with roseola is contagious even if no rash is present. 

Roseola can be difficult to diagnose given the nonspecific symptoms that are very similar to those of other childhood illnesses. Diagnosis is often clinical, based on history and physical, but can be confirmed by the characteristic rash or by detecting antibodies to roseola. 

Treatment of roseola is supportive, with most children recovering within a week. Over-the-counter medications to treat fever, such as acetaminophen and ibuprofen, can be used, but aspirin should be avoided to treat fever in children younger than 2 years given the risk of Reye's syndrome. Antibodies developed against roseola are protective against future infections. Measures should be taken to avoid contact with those infected and hands should be washed frequently to avoid spreading the virus. Adults can contract roseola if they did not have it as a child; the disease is milder in adults but they can spread it to children. 

Another common viral childhood illness is hand, foot, and mouth disease, caused by an enterovirus (coxsackievirus) that leads to sores and blisters on the hands, feet, and buttocks/legs that last for a week. The virus is spread through coughing and sneezing and through fecal-to-oral transmission. Symptoms include sore throat and fever with the rash. Diagnosis is clinical, based on history and physical exam, and the condition is treated supportively similar to roseola. 

Another common illness is erythema infectiosum, also known as fifth disease, and is caused by parvovirus B19. The virus is common among school-aged children. The rash typically starts as erythematous patches on the cheeks giving the characteristic "slapped cheek" appearance. By the time the rash appears, the child is no longer contagious. The virus is spread through respiratory secretions.

Adults whose occupations put them in close contact with children can become infected with symptoms, including joint pain, rather than the rash. Infection in pregnant women and patients with sickle cell disease carries an increased risk of miscarriage and severe anemia, respectively. Similar to roseola and hand, foot, and mouth disease, diagnosis of fifth disease is clinical, and treatment is largely supportive.