MULTIPLE ANGIOKERATOMAS WITH ZOSTERIFORM DISTRIBUTION IN TWO SISTERS

Abstract

Case 1: At the age of 12 years a 28 year‐old woman noticed multiple reddish papular lesions on the left buttock and thigh. The lesions gradually spread to involve her left thigh towards the knee. The patient had no systemic symptoms, Examination of the skin revealed 23 well‐circumscribed, purplish verrucous lesions each of 2–3 mm diameter, located over the left buttock and extending in a linear fashion to the popliteal flexure (Fig. 1). General examination and routine laboratory studies were normal. Case 2: A 27‐year‐old woman, sister of the previous patient, noticed at the age of 10 years a steadily increasing number of purple lesions on her left buttock that gradually extended to the left posterior thigh. The lesions were asymptomatic, although the patient noticed occasional bleeding with trauma. The lesions of the thigh had disappeared spontaneously without scarring during the previous 4 years. When we first examined this patient, at the age of 27, she had eight reddish papular hyperkeratotic lesions, 2–3 mm in size, with a linear zosteriform distribution over the left buttock. She was healthy and symptom free. Routine laboratory studies were normal.An excision biopsy was taken from each sister. The histopathologic examination revealed similar findings in both lesions. Hyperkeratosis with slight acanthosis and papillomatosis was present. Greatly dilated capillaries were seen in the upper and middermis. The capillary walls were lined by flattened endothelium. In one area of the lesion from the second patient there was intravascular thombosis (Fig. 2).