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OpenAthens Shibboleth
Special Feature
September 2010

Picture of the Month—Quiz Case

Author Affiliations

SAMIR SSHAHMD, MSCEAuthor Affiliations:Department of Pediatric Dermatology, Hospital Sant Joan de Déu, Esplugues de Llobregat (Drs Leal, Vicente, and González-Enseñat) and Departments of Dermatology (Dr Mascaró) and Pathology (Dr Bombí), Hospital Clinic i Provincial, Barcelona, Spain.


Copyright 2010 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2010

Arch Pediatr Adolesc Med. 2010;164(9):875. doi:10.1001/archpediatrics.2010.150-a

A 3-year-old adopted girl was referred for evaluation of a history of skin fragility and blister formation since adoption 2 years earlier. Although the lesions appeared widespread, they were mainly located in sites of trauma. They rapidly evolved to crusts, leaving some pigmentary changes.

She was a native of the tribe of Ngöbe-Buglé. The adoptive parents were unaware of the medical history of the patient or her family.

Skin examination revealed erosions and crusts over the face and fingers. There was dyspigmentation with hyperpigmented and hypopigmented macules in both sun-exposed and non–sun-exposed areas. The skin was thin and atrophic, with cigarette-paper–like wrinkling, especially over the dorsa of the hands and feet (Figure 1). Proximal webbing was present between the middle and ring fingers (Figure 2). There were no scars and no milia (Figure 3). Nails, gingiva, and mucous membranes were normal.

Figure 1.
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Close-up of the feet demonstrates cigarette-paper–like wrinkling.

Figure 2.
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The patient showed interdigital webbing of the fingers. There also were healing blisters and crusts over the dorsum of the hand.

Figure 3.
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Picture showing pigmentary changes over the legs. Note the absence of scars or milia.

What is your diagnosis?