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.
Bergman
R
R
Department of Dermatology, Rambam Medical Center, Haifa, Israel.
Bitterman-Deutsch
O
O
Fartasch
M
M
Gershoni-Baruch
R
R
Friedman-Birnbaum
R
R
D000387:Ainhum / Q000188:drug therapy / Q000209:etiology*; D002648:Child; D005050:Etretinate / Q000627:therapeutic use; D005260:Female; D005533:Foot Dermatoses / Q000150:complications* / Q000235:genetics / Q000473:pathology; D006229:Hand Dermatoses / Q000150:complications* / Q000235:genetics / Q000473:pathology; D006801:Humans; D007642:Keratosis / Q000150:complications* / Q000235:genetics / Q000473:pathology; D008854:Microscopy, Electron; D010375:Pedigree; D012867:Skin / Q000648:ultrastructure
D005050:Etretinate