Cutis

Pyogenic granuloma, often referred to by many other names, including lobular capillary hemangioma, is a benign vascular hyperplasia that tends to occur on the gingiva, lips, nasal mucosa, face, and hands.1,2 The lesion often is preceded by minor trauma, has a history of bleeding easily, and appears as a pedunculated or sessile papule with a rough red surface. There is a well-known tendency toward local recurrence after treatment.3 Results of a histopathologic examination shows lobular aggregates of capillaries and venules with plump endothelial cells.4 The pathogenesis of this lesion is poorly understood.

Port-wine stains are a type of nevus flammeus that present as a deep red or purple macule and are typically unilateral.4 Unlike salmon patches, the other type of nevus flammeus, port-wine stains tend to persist into adulthood.5 These congenital malformations are composed of capillary and venule ectasia in the dermis, and the ectasias progress over the course of a lifetime with increasing erythrocyte stasis.6-8 As Finley6 pointed out, in time, the port-wine stain can develop a thickened "cobblestone pattern," as well as nodules that are thought to be arteriovenous malformations or "localized tumor formations." Klapman and Yao8 recently noted that the thickening and nodules are most likely to occur in the areas of the face innervated by the second and third branches of the trigeminal nerve than in other parts of the body. Port-wine stains have been associated with other abnormalities such as nevus anemicus, glaucoma, choroidal angiomas, and Sturge-Weber syndrome.4 The pathogenesis of the port-wine stain has been a controversial issue, but one possibility is that it represents a deficiency of sympathetic innervation of blood vessels.4

The occurrence of vascular neoplasms arising in capillary malformations is a rare, but documented, event.9 Pyogenic granulomas infrequently have been reported to occur in association with other vascular abnormalities such as port-wine stains or hemangiomas, particularly after laser treatment.10,11 A number of other vascular neoplasms, such as capillary hemangiomas, tufted angiomas, and cavernous hemangiomas, have been reported in association with port-wine stains, as well.6,12,13 Very few pyogenic granulomas have arisen in port-wine stains without a history of trauma, laser treatment, or pregnancy. We now report a pyogenic granuloma occurring within a port-wine stain without a history of trauma or laser treatment in a man, and we contend that this occurrence offers unique insight into the pathogenesis of these poorly understood but commonly encountered entities. 

Case Report

A 35-year-old Asian man with a history of a port-wine stain on his left upper back presented for evaluation of a "mushroom" growing within his birthmark (Figure 1). He noted that some lesion had been present in this location for several months but that it had grown rapidly in the previous 3 weeks and begun to bleed easily. There was no history of trauma or treatment of the area. On physical examination, the patient had a 1.4-cm pedunculated, moist tumor with a rough surface within a port-wine stain with surrounding contact dermatitis from the bandaging he had used to protect the friable papule. Saucerization was performed, and the extremely vascularized base was cauterized. Results of histopathologic examination revealed lobules of vascular hyperplasia consistent with pyogenic granuloma (Figure 2). Vascular ectasia was seen below the pyogenic granuloma that represented the underling port-wine stain.

Comment

The Table documents the reported cases of pyogenic granulomas arising within port-wine stains.3,10,11,13-23 Many have occurred after laser treatment of port-wine stains.11,15-17,24,25 Several cases have occurred after other manipulation of port-wine stains, such as grenz-ray therapy15 or local trauma.20 Three cases occurred during pregnancy, which is a hormonal state that is known to predispose the patient to the formation of pyogenic granulomas, including one patient who previously was treated with the 577-nm pulsed dye laser and subsequently became pregnant.14,15,21 Holloway et al18 noted the development of a proliferation that had a superficial component of pyogenic granuloma and a deeper component of cavernous hemangioma. Other authors have seen the occasional association of port-wine stains and cavernous hemangiomas.6 Not surprisingly, pyogenic granulomas have occurred within port-wine stains in the setting of other syndromes, such as phacomatosis pigmentovascularis and Sturge-Weber syndrome.19

Only rarely does the pyogenic granuloma seem to emerge from the port-wine stain without a history of manipulation or pregnancy, as it did in the present case. When there is no history of predisposing factors, the presentation can be very alarming, simulating other processes such as amelanotic melanoma.22,23 Interestingly, as opposed to the thickening and nodules that have shown to be more likely to develop in port-wine stains in the distribution of the trigeminal nerve, most of the pyogenic granulomas that have arisen in port-wine stains did so outside of this distribution, occurring more often on the neck, trunk, or extremities.

Clearly, the co-occurrence of these 2 vascular abnormalities must offer some information on the pathogenesis of these poorly understood conditions. Several years ago, Swerlick and Cooper10 speculated that the co-occurrence supported the possibility of pyogenic granulomas developing at the sites of microscopic arteriovenous anastomoses. More recently, as discussed by Garzon et al,9 a number of studies indicate that abnormalities in embryonic vasculogenesis are involved in forming vascular malformations such as port-wine stains, and these same abnormalities may predispose patients toward forming vascular hyperplasia or tumors. The well-known hormonally driven vascular changes that happen during pregnancy also may be able to generate pyogenic granulomas in the setting of port-wine stains.21

On a basic science level, chemical mediators such as inducible nitric oxide synthase have been implicated in the formation of pyogenic granulomas, and mast cells have been found to be dramatically increased in the settings of both pyogenic granulomas and port-wine stains, though this may not be evidence of a cause-and-effect relationship.26,27 With regard to laser-induced pyogenic granulomas in port-wine stains, speculation has involved several factors including local tissue trauma in an area of increased microscopic arteriovenous anastomoses, retention of abnormal vasculature, and nonspecific laser interaction with tissue.15,16

Pyogenic granulomas tend to develop on the fingers, lips, face, and hands (places known to be rich in arteriovenous anastomoses), suggesting that this vascular phenomenon tends to occur with minor trauma in the setting of such anastomoses.13,20 This conclusion is consistent with the available data. The present case of a pyogenic granuloma arising without recognized trauma in a port-wine stain also supports this hypothesis.

Conclusion

Pyogenic granuloma is a well-known and probably underreported complication of port-wine stains. Unlike thickening and nodule formation that tend to occur in port-wine stains in the trigeminal nerve distribution, pyogenic granulomas arising in port-wine stains tend to occur more commonly on the neck, trunk, or extremities. The pyogenic granuloma should not be confused with a true malignancy because it represents hyperplasia of vascular tissue in response to trauma, but the performance of an excisional biopsy is warranted.

Pyogenic granuloma, often referred to by many other names, including lobular capillary hemangioma, is a benign vascular hyperplasia that tends to occur on the gingiva, lips, nasal mucosa, face, and hands.1,2 The lesion often is preceded by minor trauma, has a history of bleeding easily, and appears as a pedunculated or sessile papule with a rough red surface. There is a well-known tendency toward local recurrence after treatment.3 Results of a histopathologic examination shows lobular aggregates of capillaries and venules with plump endothelial cells.4 The pathogenesis of this lesion is poorly understood.

Port-wine stains are a type of nevus flammeus that present as a deep red or purple macule and are typically unilateral.4 Unlike salmon patches, the other type of nevus flammeus, port-wine stains tend to persist into adulthood.5 These congenital malformations are composed of capillary and venule ectasia in the dermis, and the ectasias progress over the course of a lifetime with increasing erythrocyte stasis.6-8 As Finley6 pointed out, in time, the port-wine stain can develop a thickened "cobblestone pattern," as well as nodules that are thought to be arteriovenous malformations or "localized tumor formations." Klapman and Yao8 recently noted that the thickening and nodules are most likely to occur in the areas of the face innervated by the second and third branches of the trigeminal nerve than in other parts of the body. Port-wine stains have been associated with other abnormalities such as nevus anemicus, glaucoma, choroidal angiomas, and Sturge-Weber syndrome.4 The pathogenesis of the port-wine stain has been a controversial issue, but one possibility is that it represents a deficiency of sympathetic innervation of blood vessels.4

The occurrence of vascular neoplasms arising in capillary malformations is a rare, but documented, event.9 Pyogenic granulomas infrequently have been reported to occur in association with other vascular abnormalities such as port-wine stains or hemangiomas, particularly after laser treatment.10,11 A number of other vascular neoplasms, such as capillary hemangiomas, tufted angiomas, and cavernous hemangiomas, have been reported in association with port-wine stains, as well.6,12,13 Very few pyogenic granulomas have arisen in port-wine stains without a history of trauma, laser treatment, or pregnancy. We now report a pyogenic granuloma occurring within a port-wine stain without a history of trauma or laser treatment in a man, and we contend that this occurrence offers unique insight into the pathogenesis of these poorly understood but commonly encountered entities. 

Case Report

A 35-year-old Asian man with a history of a port-wine stain on his left upper back presented for evaluation of a "mushroom" growing within his birthmark (Figure 1). He noted that some lesion had been present in this location for several months but that it had grown rapidly in the previous 3 weeks and begun to bleed easily. There was no history of trauma or treatment of the area. On physical examination, the patient had a 1.4-cm pedunculated, moist tumor with a rough surface within a port-wine stain with surrounding contact dermatitis from the bandaging he had used to protect the friable papule. Saucerization was performed, and the extremely vascularized base was cauterized. Results of histopathologic examination revealed lobules of vascular hyperplasia consistent with pyogenic granuloma (Figure 2). Vascular ectasia was seen below the pyogenic granuloma that represented the underling port-wine stain.

Comment

The Table documents the reported cases of pyogenic granulomas arising within port-wine stains.3,10,11,13-23 Many have occurred after laser treatment of port-wine stains.11,15-17,24,25 Several cases have occurred after other manipulation of port-wine stains, such as grenz-ray therapy15 or local trauma.20 Three cases occurred during pregnancy, which is a hormonal state that is known to predispose the patient to the formation of pyogenic granulomas, including one patient who previously was treated with the 577-nm pulsed dye laser and subsequently became pregnant.14,15,21 Holloway et al18 noted the development of a proliferation that had a superficial component of pyogenic granuloma and a deeper component of cavernous hemangioma. Other authors have seen the occasional association of port-wine stains and cavernous hemangiomas.6 Not surprisingly, pyogenic granulomas have occurred within port-wine stains in the setting of other syndromes, such as phacomatosis pigmentovascularis and Sturge-Weber syndrome.19

Only rarely does the pyogenic granuloma seem to emerge from the port-wine stain without a history of manipulation or pregnancy, as it did in the present case. When there is no history of predisposing factors, the presentation can be very alarming, simulating other processes such as amelanotic melanoma.22,23 Interestingly, as opposed to the thickening and nodules that have shown to be more likely to develop in port-wine stains in the distribution of the trigeminal nerve, most of the pyogenic granulomas that have arisen in port-wine stains did so outside of this distribution, occurring more often on the neck, trunk, or extremities.

Clearly, the co-occurrence of these 2 vascular abnormalities must offer some information on the pathogenesis of these poorly understood conditions. Several years ago, Swerlick and Cooper10 speculated that the co-occurrence supported the possibility of pyogenic granulomas developing at the sites of microscopic arteriovenous anastomoses. More recently, as discussed by Garzon et al,9 a number of studies indicate that abnormalities in embryonic vasculogenesis are involved in forming vascular malformations such as port-wine stains, and these same abnormalities may predispose patients toward forming vascular hyperplasia or tumors. The well-known hormonally driven vascular changes that happen during pregnancy also may be able to generate pyogenic granulomas in the setting of port-wine stains.21

On a basic science level, chemical mediators such as inducible nitric oxide synthase have been implicated in the formation of pyogenic granulomas, and mast cells have been found to be dramatically increased in the settings of both pyogenic granulomas and port-wine stains, though this may not be evidence of a cause-and-effect relationship.26,27 With regard to laser-induced pyogenic granulomas in port-wine stains, speculation has involved several factors including local tissue trauma in an area of increased microscopic arteriovenous anastomoses, retention of abnormal vasculature, and nonspecific laser interaction with tissue.15,16

Pyogenic granulomas tend to develop on the fingers, lips, face, and hands (places known to be rich in arteriovenous anastomoses), suggesting that this vascular phenomenon tends to occur with minor trauma in the setting of such anastomoses.13,20 This conclusion is consistent with the available data. The present case of a pyogenic granuloma arising without recognized trauma in a port-wine stain also supports this hypothesis.

Conclusion

Pyogenic granuloma is a well-known and probably underreported complication of port-wine stains. Unlike thickening and nodule formation that tend to occur in port-wine stains in the trigeminal nerve distribution, pyogenic granulomas arising in port-wine stains tend to occur more commonly on the neck, trunk, or extremities. The pyogenic granuloma should not be confused with a true malignancy because it represents hyperplasia of vascular tissue in response to trauma, but the performance of an excisional biopsy is warranted.

Pyogenic granuloma, often referred to by many other names, including lobular capillary hemangioma, is a benign vascular hyperplasia that tends to occur on the gingiva, lips, nasal mucosa, face, and hands.1,2 The lesion often is preceded by minor trauma, has a history of bleeding easily, and appears as a pedunculated or sessile papule with a rough red surface. There is a well-known tendency toward local recurrence after treatment.3 Results of a histopathologic examination shows lobular aggregates of capillaries and venules with plump endothelial cells.4 The pathogenesis of this lesion is poorly understood.

Port-wine stains are a type of nevus flammeus that present as a deep red or purple macule and are typically unilateral.4 Unlike salmon patches, the other type of nevus flammeus, port-wine stains tend to persist into adulthood.5 These congenital malformations are composed of capillary and venule ectasia in the dermis, and the ectasias progress over the course of a lifetime with increasing erythrocyte stasis.6-8 As Finley6 pointed out, in time, the port-wine stain can develop a thickened "cobblestone pattern," as well as nodules that are thought to be arteriovenous malformations or "localized tumor formations." Klapman and Yao8 recently noted that the thickening and nodules are most likely to occur in the areas of the face innervated by the second and third branches of the trigeminal nerve than in other parts of the body. Port-wine stains have been associated with other abnormalities such as nevus anemicus, glaucoma, choroidal angiomas, and Sturge-Weber syndrome.4 The pathogenesis of the port-wine stain has been a controversial issue, but one possibility is that it represents a deficiency of sympathetic innervation of blood vessels.4

The occurrence of vascular neoplasms arising in capillary malformations is a rare, but documented, event.9 Pyogenic granulomas infrequently have been reported to occur in association with other vascular abnormalities such as port-wine stains or hemangiomas, particularly after laser treatment.10,11 A number of other vascular neoplasms, such as capillary hemangiomas, tufted angiomas, and cavernous hemangiomas, have been reported in association with port-wine stains, as well.6,12,13 Very few pyogenic granulomas have arisen in port-wine stains without a history of trauma, laser treatment, or pregnancy. We now report a pyogenic granuloma occurring within a port-wine stain without a history of trauma or laser treatment in a man, and we contend that this occurrence offers unique insight into the pathogenesis of these poorly understood but commonly encountered entities. 

Case Report

A 35-year-old Asian man with a history of a port-wine stain on his left upper back presented for evaluation of a "mushroom" growing within his birthmark (Figure 1). He noted that some lesion had been present in this location for several months but that it had grown rapidly in the previous 3 weeks and begun to bleed easily. There was no history of trauma or treatment of the area. On physical examination, the patient had a 1.4-cm pedunculated, moist tumor with a rough surface within a port-wine stain with surrounding contact dermatitis from the bandaging he had used to protect the friable papule. Saucerization was performed, and the extremely vascularized base was cauterized. Results of histopathologic examination revealed lobules of vascular hyperplasia consistent with pyogenic granuloma (Figure 2). Vascular ectasia was seen below the pyogenic granuloma that represented the underling port-wine stain.

Comment

The Table documents the reported cases of pyogenic granulomas arising within port-wine stains.3,10,11,13-23 Many have occurred after laser treatment of port-wine stains.11,15-17,24,25 Several cases have occurred after other manipulation of port-wine stains, such as grenz-ray therapy15 or local trauma.20 Three cases occurred during pregnancy, which is a hormonal state that is known to predispose the patient to the formation of pyogenic granulomas, including one patient who previously was treated with the 577-nm pulsed dye laser and subsequently became pregnant.14,15,21 Holloway et al18 noted the development of a proliferation that had a superficial component of pyogenic granuloma and a deeper component of cavernous hemangioma. Other authors have seen the occasional association of port-wine stains and cavernous hemangiomas.6 Not surprisingly, pyogenic granulomas have occurred within port-wine stains in the setting of other syndromes, such as phacomatosis pigmentovascularis and Sturge-Weber syndrome.19

Only rarely does the pyogenic granuloma seem to emerge from the port-wine stain without a history of manipulation or pregnancy, as it did in the present case. When there is no history of predisposing factors, the presentation can be very alarming, simulating other processes such as amelanotic melanoma.22,23 Interestingly, as opposed to the thickening and nodules that have shown to be more likely to develop in port-wine stains in the distribution of the trigeminal nerve, most of the pyogenic granulomas that have arisen in port-wine stains did so outside of this distribution, occurring more often on the neck, trunk, or extremities.

Clearly, the co-occurrence of these 2 vascular abnormalities must offer some information on the pathogenesis of these poorly understood conditions. Several years ago, Swerlick and Cooper10 speculated that the co-occurrence supported the possibility of pyogenic granulomas developing at the sites of microscopic arteriovenous anastomoses. More recently, as discussed by Garzon et al,9 a number of studies indicate that abnormalities in embryonic vasculogenesis are involved in forming vascular malformations such as port-wine stains, and these same abnormalities may predispose patients toward forming vascular hyperplasia or tumors. The well-known hormonally driven vascular changes that happen during pregnancy also may be able to generate pyogenic granulomas in the setting of port-wine stains.21

On a basic science level, chemical mediators such as inducible nitric oxide synthase have been implicated in the formation of pyogenic granulomas, and mast cells have been found to be dramatically increased in the settings of both pyogenic granulomas and port-wine stains, though this may not be evidence of a cause-and-effect relationship.26,27 With regard to laser-induced pyogenic granulomas in port-wine stains, speculation has involved several factors including local tissue trauma in an area of increased microscopic arteriovenous anastomoses, retention of abnormal vasculature, and nonspecific laser interaction with tissue.15,16

Pyogenic granulomas tend to develop on the fingers, lips, face, and hands (places known to be rich in arteriovenous anastomoses), suggesting that this vascular phenomenon tends to occur with minor trauma in the setting of such anastomoses.13,20 This conclusion is consistent with the available data. The present case of a pyogenic granuloma arising without recognized trauma in a port-wine stain also supports this hypothesis.

Conclusion

Pyogenic granuloma is a well-known and probably underreported complication of port-wine stains. Unlike thickening and nodule formation that tend to occur in port-wine stains in the trigeminal nerve distribution, pyogenic granulomas arising in port-wine stains tend to occur more commonly on the neck, trunk, or extremities. The pyogenic granuloma should not be confused with a true malignancy because it represents hyperplasia of vascular tissue in response to trauma, but the performance of an excisional biopsy is warranted.

Onychotillomania is an uncommon condition characterized by self-destruction of the fingernails and/or toenails by compulsive manipulation. We report 2 cases of onychotillomania with differing presentations in a young man and in an older man. Onychotillomania may be a form of obsessive-compulsive disorder (OCD), and we discuss the psychologic factors and current treatments for this condition.

Emotional and psychologic factors have the ability to influence the underlying disease process in at least 33% of patients with a dermatologic condition.1 In some cases, such as onychotillomania, a psychiatric condition can be the underlining cause. Onychotillomania, a condition whose true incidence is unknown, is characterized by the compulsive or irresistible urge in patients to pick at, pull off, or harmfully bite or chew their nails. This urge may be conscious or unconscious. The word onychotillomania is derived from the Greek onycho, nail; tillo, to pull; and mania, madness or frenzy. In psychiatry, onychotillomania has been classified as an impulse control disorder that includes conditions such as compulsive gambling, kleptomania, pyromania, habit-tic deformity, and obsessive-compulsive disorder (OCD). The more well-documented trichotillomania, or pulling of the hair, is estimated to occur in up to 1 in 200 individuals.2 The incidence of onychotillomania is thought to be much lower and widely underreported. However, the incidence may surpass that of trichotillomania when nail biting, nail chewing, or habit-tic deformity is included, though this thought is controversial. In this report, we document 2 patients with slightly different presentations of onychotillomania and the approaches to their therapy.

Case Reports

Patient 1—A 72-year-old white man was referred to the dermatology clinic with an 8-month history of fingernail loss and pain. On physical examination, he was missing 2 nails on the left hand. Prominent longitudinal ridging was observed on the remaining nails, which were thick and yellow and showed some loss of the distal nail plate. All nails on the right hand were normal. Results of a biopsy showed epithelial necrosis with no evidence of lichen planus or inflammation. Fungal culture results were negative.

The patient was confrontational during the visit, protesting the nail examination. He continually drew back in protest, not wanting his nails examined. His wife reported the same, stating that he would slap her hands away anytime she tried to look at his nails. He also reported that there was a "clear goo" under his nails that he thought was necessary to remove by picking. Past medical history was significant for essential tremors, chronic obstructive pulmonary disease, and congestive heart failure. Medications included primidone and gabapentin for essential tremor and alprazolam at night for insomnia.

Based on examination findings and the patient's own admission, the diagnosis of onychotillomania was made. Results of plain film radiographs that were obtained to rule out osteomyelitis were negative. Attempts to use occlusive dressings were made, but the patient refused to keep the nails covered because he was unable to manipulate the remaining nails or nail beds. Also, referral for psychiatric evaluation was vehemently refused. The patient did not return for follow-up.

Patient 2—A 22-year-old white man presented to the dermatology clinic with a several-month history of pain in his toenails. On physical examination, he was missing all nails on his right foot. He had blood on and under all the remaining toenails, blood on all nail beds, and blood under most of his fingernails and on his fingertips. On questioning, the patient adamantly denied pulling his nails, even after confronted with the blood evidence on his fingers and fingernails. His mother reported that he constantly picked at his toenails.

Due to our suspicion of onychotillomania with secondary infection, the patient was treated initially with oral cephalexin followed by placement of an Unna boot on the involved foot with modifications to cover the entire foot and digits. This was changed once weekly. After one month, new healthy nail was noted, but the patient refused our recommendation for psychiatric evaluation and did not follow-up with us as recommended.

Comment

In both of our cases, the diagnosis of onychotillomania was made by the obvious physical signs on examination, as well as by self-admission in patient 1. Although we believed a psychiatric evaluation was necessary for proper treatment, it was refused by both patients.

Psychodermatologic problems can be grouped into 3 categories. Psychophysiologic disorders are exacerbated by emotional stress and include atopic dermatitis and psoriasis.3 Primary psychiatric disorders (anxiety, depression, delusion, and OCD) may present as delusions of parasitosis, neurotic excoriations, trichotillomania, and onychotillomania. In secondary psychiatric disorders, patients endure psychologic or emotional distress as a result of physical or visual deformity caused by primary skin disorders such as acne, leprosy, psoriasis, and vitiligo.4

The term onychotillomania previously has been used to include nail biting in addition to physical deformities caused by self-induced damage to the nails or periungual tissues by picking or pulling. However, it is generally reserved for the manual removal of the nail plate. Examination of individuals with onychotillomania may show periungual erosions and crusts associated with nail-plate surface abnormalities.5 The damage simply may be diminished or missing nails. The matrix melanocytes can be stimulated by chronic trauma, which may result in longitudinal melanonychia.6

Onychotillomania has been classified as a habit-tic deformity that may occur after psychological and emotional stress or as a form of OCD.7 However, the habit-tic deformity may not fit the true definition of onychotillomania, though pharmacologic treatment is similar. Paranoid delusions and psychoses also have been associated with onychotillomania,8 as has Smith-Magenis syndrome. This congenital anomaly associated with mental retardation is estimated to occur in 1 in 25,000 individuals. Self-mutilatory behavior is seen in 70% of patients and includes onychotillomania.9 The differential diagnosis also should include Lesch-Nyhan syndrome.

Treatment of the underlying psychologic disorders should be considered in those with onychotillomania. In addition to onychotillomania, the more common manifestations of OCD in dermatology include trichotillomania, onychophagia, acne excoriee, and neurotic excoriations.10 OCD most frequently is manifested in childhood, though behavior such as obsessive hand washing, AIDS phobia, and other psychosomatic dermatoses can be observed in all age groups.

However, not every patient with onychotillomania has OCD as the underlying psychopathology. Before coming to a conclusion that a patient with onychotillomania has OCD, one must rule out other psychiatric diagnostic possibilities, mainly delusion and simple habit disorder (habit-tic deformity). The key distinction between obsession and delusion is the presence or absence of insight on the part of the patient. Obsessive patients have more insight than delusional patients do. Often, patients with OCD are apologetic for their behavior.10 Patients with habit-tic deformity may be differentiated from true onychotillomania in that they may only rub their nails unconsciously and not actually pick off their nails.

It is important for the underlying psychiatric disorder to be defined by psychiatric evaluation and subsequent treatment with psychoactive medications.11 Common treatments for OCD include individual psychotherapy and behavioral therapy. In addition, there are 3 oral medications commonly used for their anti–obsessive-compulsive effect, namely, clomipramine, fluoxetine, and fluvoxamine.9 Paroxetine, sertraline, the mixed uptake inhibitor venlafaxine, and citalopram are the latest additions for the treatment of OCD.12 Fluoxetine hydrochloride also has been found helpful, specifically in the treatment of onychotillomania.12 In addition, pimozide has been used specifically to treat onychotillomania.8 Topical treatments also have been approached using distasteful preparations applied to the nails to discourage nail biting and chewing.5 The physical barrier method appeared to work quite well in our younger patient, though it was not effective in the treatment of our older patient.

Onychotillomania has been cited in the literature to include both nail pulling and nail biting, but our 2 patients exhibited the most classic form of onychotillomania of picking and pulling of the nails, as the term was originally coined. Neither of our patients had what would be classified as habit-tic deformity. We speculate that onychotillomania can be divided into a nail-pulling/picking group, a nail-biting group, and a combination of the 2. In any case, it is advisable to explore etiologies other than self-inflicted trauma, such as mechanical or friction trauma, fungal infection, or another form of nail dystrophy. However, when the diagnosis of onychotillomania is reached, in addition to occlusion to prevent the self-induced damage, referral to and treatment by a psychiatrist is warranted because of the strong association with underlying psychiatric conditions, namely, OCD. 

Onychotillomania is an uncommon condition characterized by self-destruction of the fingernails and/or toenails by compulsive manipulation. We report 2 cases of onychotillomania with differing presentations in a young man and in an older man. Onychotillomania may be a form of obsessive-compulsive disorder (OCD), and we discuss the psychologic factors and current treatments for this condition.

Emotional and psychologic factors have the ability to influence the underlying disease process in at least 33% of patients with a dermatologic condition.1 In some cases, such as onychotillomania, a psychiatric condition can be the underlining cause. Onychotillomania, a condition whose true incidence is unknown, is characterized by the compulsive or irresistible urge in patients to pick at, pull off, or harmfully bite or chew their nails. This urge may be conscious or unconscious. The word onychotillomania is derived from the Greek onycho, nail; tillo, to pull; and mania, madness or frenzy. In psychiatry, onychotillomania has been classified as an impulse control disorder that includes conditions such as compulsive gambling, kleptomania, pyromania, habit-tic deformity, and obsessive-compulsive disorder (OCD). The more well-documented trichotillomania, or pulling of the hair, is estimated to occur in up to 1 in 200 individuals.2 The incidence of onychotillomania is thought to be much lower and widely underreported. However, the incidence may surpass that of trichotillomania when nail biting, nail chewing, or habit-tic deformity is included, though this thought is controversial. In this report, we document 2 patients with slightly different presentations of onychotillomania and the approaches to their therapy.

Case Reports

Patient 1—A 72-year-old white man was referred to the dermatology clinic with an 8-month history of fingernail loss and pain. On physical examination, he was missing 2 nails on the left hand. Prominent longitudinal ridging was observed on the remaining nails, which were thick and yellow and showed some loss of the distal nail plate. All nails on the right hand were normal. Results of a biopsy showed epithelial necrosis with no evidence of lichen planus or inflammation. Fungal culture results were negative.

The patient was confrontational during the visit, protesting the nail examination. He continually drew back in protest, not wanting his nails examined. His wife reported the same, stating that he would slap her hands away anytime she tried to look at his nails. He also reported that there was a "clear goo" under his nails that he thought was necessary to remove by picking. Past medical history was significant for essential tremors, chronic obstructive pulmonary disease, and congestive heart failure. Medications included primidone and gabapentin for essential tremor and alprazolam at night for insomnia.

Based on examination findings and the patient's own admission, the diagnosis of onychotillomania was made. Results of plain film radiographs that were obtained to rule out osteomyelitis were negative. Attempts to use occlusive dressings were made, but the patient refused to keep the nails covered because he was unable to manipulate the remaining nails or nail beds. Also, referral for psychiatric evaluation was vehemently refused. The patient did not return for follow-up.

Patient 2—A 22-year-old white man presented to the dermatology clinic with a several-month history of pain in his toenails. On physical examination, he was missing all nails on his right foot. He had blood on and under all the remaining toenails, blood on all nail beds, and blood under most of his fingernails and on his fingertips. On questioning, the patient adamantly denied pulling his nails, even after confronted with the blood evidence on his fingers and fingernails. His mother reported that he constantly picked at his toenails.

Due to our suspicion of onychotillomania with secondary infection, the patient was treated initially with oral cephalexin followed by placement of an Unna boot on the involved foot with modifications to cover the entire foot and digits. This was changed once weekly. After one month, new healthy nail was noted, but the patient refused our recommendation for psychiatric evaluation and did not follow-up with us as recommended.

Comment

In both of our cases, the diagnosis of onychotillomania was made by the obvious physical signs on examination, as well as by self-admission in patient 1. Although we believed a psychiatric evaluation was necessary for proper treatment, it was refused by both patients.

Psychodermatologic problems can be grouped into 3 categories. Psychophysiologic disorders are exacerbated by emotional stress and include atopic dermatitis and psoriasis.3 Primary psychiatric disorders (anxiety, depression, delusion, and OCD) may present as delusions of parasitosis, neurotic excoriations, trichotillomania, and onychotillomania. In secondary psychiatric disorders, patients endure psychologic or emotional distress as a result of physical or visual deformity caused by primary skin disorders such as acne, leprosy, psoriasis, and vitiligo.4

The term onychotillomania previously has been used to include nail biting in addition to physical deformities caused by self-induced damage to the nails or periungual tissues by picking or pulling. However, it is generally reserved for the manual removal of the nail plate. Examination of individuals with onychotillomania may show periungual erosions and crusts associated with nail-plate surface abnormalities.5 The damage simply may be diminished or missing nails. The matrix melanocytes can be stimulated by chronic trauma, which may result in longitudinal melanonychia.6

Onychotillomania has been classified as a habit-tic deformity that may occur after psychological and emotional stress or as a form of OCD.7 However, the habit-tic deformity may not fit the true definition of onychotillomania, though pharmacologic treatment is similar. Paranoid delusions and psychoses also have been associated with onychotillomania,8 as has Smith-Magenis syndrome. This congenital anomaly associated with mental retardation is estimated to occur in 1 in 25,000 individuals. Self-mutilatory behavior is seen in 70% of patients and includes onychotillomania.9 The differential diagnosis also should include Lesch-Nyhan syndrome.

Treatment of the underlying psychologic disorders should be considered in those with onychotillomania. In addition to onychotillomania, the more common manifestations of OCD in dermatology include trichotillomania, onychophagia, acne excoriee, and neurotic excoriations.10 OCD most frequently is manifested in childhood, though behavior such as obsessive hand washing, AIDS phobia, and other psychosomatic dermatoses can be observed in all age groups.

However, not every patient with onychotillomania has OCD as the underlying psychopathology. Before coming to a conclusion that a patient with onychotillomania has OCD, one must rule out other psychiatric diagnostic possibilities, mainly delusion and simple habit disorder (habit-tic deformity). The key distinction between obsession and delusion is the presence or absence of insight on the part of the patient. Obsessive patients have more insight than delusional patients do. Often, patients with OCD are apologetic for their behavior.10 Patients with habit-tic deformity may be differentiated from true onychotillomania in that they may only rub their nails unconsciously and not actually pick off their nails.

It is important for the underlying psychiatric disorder to be defined by psychiatric evaluation and subsequent treatment with psychoactive medications.11 Common treatments for OCD include individual psychotherapy and behavioral therapy. In addition, there are 3 oral medications commonly used for their anti–obsessive-compulsive effect, namely, clomipramine, fluoxetine, and fluvoxamine.9 Paroxetine, sertraline, the mixed uptake inhibitor venlafaxine, and citalopram are the latest additions for the treatment of OCD.12 Fluoxetine hydrochloride also has been found helpful, specifically in the treatment of onychotillomania.12 In addition, pimozide has been used specifically to treat onychotillomania.8 Topical treatments also have been approached using distasteful preparations applied to the nails to discourage nail biting and chewing.5 The physical barrier method appeared to work quite well in our younger patient, though it was not effective in the treatment of our older patient.

Onychotillomania has been cited in the literature to include both nail pulling and nail biting, but our 2 patients exhibited the most classic form of onychotillomania of picking and pulling of the nails, as the term was originally coined. Neither of our patients had what would be classified as habit-tic deformity. We speculate that onychotillomania can be divided into a nail-pulling/picking group, a nail-biting group, and a combination of the 2. In any case, it is advisable to explore etiologies other than self-inflicted trauma, such as mechanical or friction trauma, fungal infection, or another form of nail dystrophy. However, when the diagnosis of onychotillomania is reached, in addition to occlusion to prevent the self-induced damage, referral to and treatment by a psychiatrist is warranted because of the strong association with underlying psychiatric conditions, namely, OCD. 

Onychotillomania is an uncommon condition characterized by self-destruction of the fingernails and/or toenails by compulsive manipulation. We report 2 cases of onychotillomania with differing presentations in a young man and in an older man. Onychotillomania may be a form of obsessive-compulsive disorder (OCD), and we discuss the psychologic factors and current treatments for this condition.

Emotional and psychologic factors have the ability to influence the underlying disease process in at least 33% of patients with a dermatologic condition.1 In some cases, such as onychotillomania, a psychiatric condition can be the underlining cause. Onychotillomania, a condition whose true incidence is unknown, is characterized by the compulsive or irresistible urge in patients to pick at, pull off, or harmfully bite or chew their nails. This urge may be conscious or unconscious. The word onychotillomania is derived from the Greek onycho, nail; tillo, to pull; and mania, madness or frenzy. In psychiatry, onychotillomania has been classified as an impulse control disorder that includes conditions such as compulsive gambling, kleptomania, pyromania, habit-tic deformity, and obsessive-compulsive disorder (OCD). The more well-documented trichotillomania, or pulling of the hair, is estimated to occur in up to 1 in 200 individuals.2 The incidence of onychotillomania is thought to be much lower and widely underreported. However, the incidence may surpass that of trichotillomania when nail biting, nail chewing, or habit-tic deformity is included, though this thought is controversial. In this report, we document 2 patients with slightly different presentations of onychotillomania and the approaches to their therapy.

Case Reports

Patient 1—A 72-year-old white man was referred to the dermatology clinic with an 8-month history of fingernail loss and pain. On physical examination, he was missing 2 nails on the left hand. Prominent longitudinal ridging was observed on the remaining nails, which were thick and yellow and showed some loss of the distal nail plate. All nails on the right hand were normal. Results of a biopsy showed epithelial necrosis with no evidence of lichen planus or inflammation. Fungal culture results were negative.

The patient was confrontational during the visit, protesting the nail examination. He continually drew back in protest, not wanting his nails examined. His wife reported the same, stating that he would slap her hands away anytime she tried to look at his nails. He also reported that there was a "clear goo" under his nails that he thought was necessary to remove by picking. Past medical history was significant for essential tremors, chronic obstructive pulmonary disease, and congestive heart failure. Medications included primidone and gabapentin for essential tremor and alprazolam at night for insomnia.

Based on examination findings and the patient's own admission, the diagnosis of onychotillomania was made. Results of plain film radiographs that were obtained to rule out osteomyelitis were negative. Attempts to use occlusive dressings were made, but the patient refused to keep the nails covered because he was unable to manipulate the remaining nails or nail beds. Also, referral for psychiatric evaluation was vehemently refused. The patient did not return for follow-up.

Patient 2—A 22-year-old white man presented to the dermatology clinic with a several-month history of pain in his toenails. On physical examination, he was missing all nails on his right foot. He had blood on and under all the remaining toenails, blood on all nail beds, and blood under most of his fingernails and on his fingertips. On questioning, the patient adamantly denied pulling his nails, even after confronted with the blood evidence on his fingers and fingernails. His mother reported that he constantly picked at his toenails.

Due to our suspicion of onychotillomania with secondary infection, the patient was treated initially with oral cephalexin followed by placement of an Unna boot on the involved foot with modifications to cover the entire foot and digits. This was changed once weekly. After one month, new healthy nail was noted, but the patient refused our recommendation for psychiatric evaluation and did not follow-up with us as recommended.

Comment

In both of our cases, the diagnosis of onychotillomania was made by the obvious physical signs on examination, as well as by self-admission in patient 1. Although we believed a psychiatric evaluation was necessary for proper treatment, it was refused by both patients.

Psychodermatologic problems can be grouped into 3 categories. Psychophysiologic disorders are exacerbated by emotional stress and include atopic dermatitis and psoriasis.3 Primary psychiatric disorders (anxiety, depression, delusion, and OCD) may present as delusions of parasitosis, neurotic excoriations, trichotillomania, and onychotillomania. In secondary psychiatric disorders, patients endure psychologic or emotional distress as a result of physical or visual deformity caused by primary skin disorders such as acne, leprosy, psoriasis, and vitiligo.4

The term onychotillomania previously has been used to include nail biting in addition to physical deformities caused by self-induced damage to the nails or periungual tissues by picking or pulling. However, it is generally reserved for the manual removal of the nail plate. Examination of individuals with onychotillomania may show periungual erosions and crusts associated with nail-plate surface abnormalities.5 The damage simply may be diminished or missing nails. The matrix melanocytes can be stimulated by chronic trauma, which may result in longitudinal melanonychia.6

Onychotillomania has been classified as a habit-tic deformity that may occur after psychological and emotional stress or as a form of OCD.7 However, the habit-tic deformity may not fit the true definition of onychotillomania, though pharmacologic treatment is similar. Paranoid delusions and psychoses also have been associated with onychotillomania,8 as has Smith-Magenis syndrome. This congenital anomaly associated with mental retardation is estimated to occur in 1 in 25,000 individuals. Self-mutilatory behavior is seen in 70% of patients and includes onychotillomania.9 The differential diagnosis also should include Lesch-Nyhan syndrome.

Treatment of the underlying psychologic disorders should be considered in those with onychotillomania. In addition to onychotillomania, the more common manifestations of OCD in dermatology include trichotillomania, onychophagia, acne excoriee, and neurotic excoriations.10 OCD most frequently is manifested in childhood, though behavior such as obsessive hand washing, AIDS phobia, and other psychosomatic dermatoses can be observed in all age groups.

However, not every patient with onychotillomania has OCD as the underlying psychopathology. Before coming to a conclusion that a patient with onychotillomania has OCD, one must rule out other psychiatric diagnostic possibilities, mainly delusion and simple habit disorder (habit-tic deformity). The key distinction between obsession and delusion is the presence or absence of insight on the part of the patient. Obsessive patients have more insight than delusional patients do. Often, patients with OCD are apologetic for their behavior.10 Patients with habit-tic deformity may be differentiated from true onychotillomania in that they may only rub their nails unconsciously and not actually pick off their nails.

It is important for the underlying psychiatric disorder to be defined by psychiatric evaluation and subsequent treatment with psychoactive medications.11 Common treatments for OCD include individual psychotherapy and behavioral therapy. In addition, there are 3 oral medications commonly used for their anti–obsessive-compulsive effect, namely, clomipramine, fluoxetine, and fluvoxamine.9 Paroxetine, sertraline, the mixed uptake inhibitor venlafaxine, and citalopram are the latest additions for the treatment of OCD.12 Fluoxetine hydrochloride also has been found helpful, specifically in the treatment of onychotillomania.12 In addition, pimozide has been used specifically to treat onychotillomania.8 Topical treatments also have been approached using distasteful preparations applied to the nails to discourage nail biting and chewing.5 The physical barrier method appeared to work quite well in our younger patient, though it was not effective in the treatment of our older patient.

Onychotillomania has been cited in the literature to include both nail pulling and nail biting, but our 2 patients exhibited the most classic form of onychotillomania of picking and pulling of the nails, as the term was originally coined. Neither of our patients had what would be classified as habit-tic deformity. We speculate that onychotillomania can be divided into a nail-pulling/picking group, a nail-biting group, and a combination of the 2. In any case, it is advisable to explore etiologies other than self-inflicted trauma, such as mechanical or friction trauma, fungal infection, or another form of nail dystrophy. However, when the diagnosis of onychotillomania is reached, in addition to occlusion to prevent the self-induced damage, referral to and treatment by a psychiatrist is warranted because of the strong association with underlying psychiatric conditions, namely, OCD.