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Ocular ectodermal syndrome of epibulbar dermoid and cutaneous myxovascular hamartoma
  1. KAAN GÜNDÜZ,
  2. CAROL L SHIELDS,
  3. YELENA DOYCH
  1. Oncology Service, Wills Eye Hospital, Philadelphia, PA, USA
  2. Pediatric Ophthalmology Service
  3. Oncology Service
  1. BRUCE SCHNALL
  1. Oncology Service, Wills Eye Hospital, Philadelphia, PA, USA
  2. Pediatric Ophthalmology Service
  3. Oncology Service
  1. JERRY A SHIELDS
  1. Oncology Service, Wills Eye Hospital, Philadelphia, PA, USA
  2. Pediatric Ophthalmology Service
  3. Oncology Service
  1. Carol L Shields, MD, Oncology Service, Wills Eye Hospital, 900 Walnut Street, Philadelphia, PA 19107, USA

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Editor,—Ocular ectodermal syndrome is a congenital disorder manifesting classically with epibulbar dermoids and aplasia cutis congenita. Systemic abnormalities may also accompany ocular ectodermal syndrome.1-4 We describe the case of a newborn boy who presented with unilateral epibulbar dermoid and myxovascular hamartomas of the scalp. We believe that the association of epibulbar dermoid and myxovascular hamartoma of the skin represents an unusual variant of ocular ectodermal syndrome.

CASE REPORT

A newborn boy was noted at birth to have multiple serous purple blisters on the left scalp region, measuring 0.5 cm to 2.0 cm in base (Fig 1). Anterior fontanelle asymmetry was also recognised. The skin lesions were thought to be aplasia cutis congenita and a skin biopsy was performed. Histopathological examination of the biopsied skin revealed loose connective tissue in the dermis with an apparent absence …

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