Hypopigmented, Hyperkeratotic Papules in Two Siblings

Abstract

An 18-year-old black woman presented with asymptomatic disseminated hypopigmented and hyperpigmented papules on her trunk and extremities. She had a history of asymptomatic keratoses since childhood as well as a history ofcystic acne and hidradenitis suppurativa. Her family history was significant for a brother and mother with similar hypopigmented and hyperpigmented lesions in the same distribution. On physical examination, she had numerous 1-to 3-mm hypopigmented, hyperkeratotic, flat-topped, nonfollicular papules involving the extremities and trunk with extension to the neck and ears.The hypopigmented papules appeared to be unrelated to any previous lesions. She also had hyperpigmented papules on the distal extremities and the dorsa of her hands and feet (Fig 1).Skin markings were preserved in these lesions. Hyperpigmented macules with central pitting were also observed on her palms and soles. No oral lesions were present. A biopsy specimen was taken from one of the hypopigmented papules (Fig 2) as well as from one of the hyperpigmented papules (Fig 3).Her 22-year-old brother was seen 2 years later in the derma¬ tology clinic. His medical history was significant for a history of widespread kératoses since the age of 6 years. On physical examination, two types of discrete, round papules were ob¬ served. Hypopigmented, hyperkeratotic, flat-topped, nonfol¬ licular 1-to 3-mm papules were seen on the trunk and extrem¬ ities. As with his sister, these papules did not seem to be associated with any previous lesions. Dispersed among these were 2-to 4-mm hyperpigmented, round papules. Both types of lesions were asymptomatic, unaffected by sunlight, and present in approximately equal numbers on the trunk and extremities. No oral lesions were noted.His sister was seen in follow-up 14 years later at which time she reported an increase in the number of hypopigmented and hyperpigmented papules with no change in the distribution.The lesions had remained asymptomatic and were still unaf¬ fected by sunlight. She stated that her brother, who was unavailable for follow-up, had also noted a slow increase in the number of lesions over the years. Findings from her physical examination were essentially unchanged, except for the great¬ er number of hypopigmented and hyperpigmented papules.