A 9‐Year‐Old Girl with Pearly Skin Colored Papules with Palmar Pits

Abstract

A 9-year-old girl with a history of developmental delay and postnatal hydrocephalus was referred to the dermatology clinic for evaluation. Her past medical history was significant for macrocephaly, an intracerebral arachnoid cyst, and postaxial polydactyly. Family history was unremarkable.On physical examination, the patient had macrocephaly but no frontal bossing or hypertelorism. She had mild scoliosis. Cutaneous examination revealed palmar pitting (Fig. 1) and two skin-colored papules over the right chest and right axillae (Fig. 2) that had a pearly appearance with overlying telangiectasia on dermoscopy (Fig. 3). She had postaxial polydactyly of the left fifth finger.A shave biopsy was done of one of the pearly papules (Fig. 4).