Generalised recessive dystrophic epidermolysis bullosa in two sisters.
Two female siblings of a family presented with a mechanobullous disorder since birth. Both had retarded physical development, flaccid bullac, extensive cutaneous erosions and scars, mucosal erosions, milia, corneal haziness, deformed and carious teeth, dystrophic nails, cicatricial alopecia of scalp and positive Nikolsky's sign. Skin biopsy revealed subepidermal bulla. They were diagnosed as generalized recessive dystrophic epidermolysis bullosa. They were given oral phenytoin but failed to show significant response.
Department of Dermatology, 5 Air Force Hospital, C/o 99 APO, India.
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Grover