Palmar pit-like lesions as a rare presentation in epidermodysplasia verruciformis: case report.

Epidermodysplasia verruciformis (EV) is a rare inherited syndrome characterized by early onset of numerous plane, vulgaris and versicolor-like warts especially on the face, neck, trunk and extremities with lifelong course and occasional cutaneous squamous cell carcinomas (SCCs) (1,2). Palmar pit-like lesions have been reported in one case of EV from United Arab Emirates (3) and our cases share the same features including their histologic findings, supporting the probable relationship between the pit-like lesions and at least some EV human papilloma virus (HPV) types. Here we report on a 17-year-old girl with EV, presenting with palmar pit-like lesions in addition to otherwise typical clinical presentation. A 17-year-old girl with 11-year history of refractory eruption as shiny pink to skin colored plane papules on her hands, face and chin and perioral areas was referred to the skin clinic in our hospital. Clinical examination revealed hypopigmented maculae and patches on her neck, forearms, several verruca vulgaris on extremities and multiple asymptomatic well-defined pin-point to 2-3 mm pink to brown pit-like lesions on her palms (Fig. 1). She was otherwise healthy. Two biopsy specimens were obtained from her body and palmar lesions for histologic examination. Histologic analysis showed hyperkeratosis, acanthosis with prominent vacuolar changes, especially in the granular layer of the epidermis, and scattered large atypical nuclei, compatible with HPV infection (Fig. 2). All family members were examined to detect the possible evidence of EV in her relatives. Her parents had no symptoms, but three siblings had evidence of EV with the same palmar pit-like lesions and warts on other boy parts (Fig. 3). These features were consistent with EV. Epidermodysplasia verruciformis was first described in 1922 (4). It is a very rare, chronic, mostly autosomal recessive syndrome characterized by early onset of widespread persistent warts (plane, vulgaris, versicolor-like) on the face, neck, trunk, hands and feet, with lifelong course and susceptibility to cutaneous SCC (1,2). The 5, 8 and 47 EV HPV types are found in more than 90% of EV skin cancers (5-7). Figure 1. Several pin-points to 2-3 mm brownish pit-like lesions on the palms of the patient.