Cases reported "Ainhum"

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1/2. Successful treatment of keratoderma hereditaria mutilans with an aromatic retinoid.

    Clinical features and treatment of keratoderma hereditaria mutilans (Vohwinkel's syndrome) are described in an 11-year-old boy. The disease, in particular the mutilating complications (pseudo-ainhum), responded satisfactorily to the oral administration of etretinate, an aromatic retinoid.
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ranking = 1
keywords = keratoderma
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2/2. Mal de Meleda keratoderma with pseudoainhum.

    Pseudoainhum is an infrequent complication in the autosomal-recessive keratodermas. We describe two related families in which the diagnosis of mal de Meleda keratoderma has been confirmed by mode of inheritance and ultrastructural findings. One family member, a 9-year-old girl, developed pseudoainhum which threatened the viability of her little fingers. This responded to treatment with etretinate. The treatment dilemma posed by keratoderma-induced pseudoainhum in children, i.e. the concern over the possible skeletal toxic effects of long-term etretinate treatment vs. the risks and outcome of surgery, is discussed.
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ranking = 1.4
keywords = keratoderma
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