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An Indian Case of Keratoderma Hereditarium Mutilans (Vohwinkel's Syndrome) Associated with Ichthyosiform Dermatosis
Author(s) -
Vijaikumar Marykolandainathen,
Thappa Devinder Mohan,
Jeevankumar Balasubramanian
Publication year - 2001
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.2001.tb00031.x
Subject(s) - palmoplantar keratoderma , acanthosis , hyperkeratosis , parakeratosis , keratoderma , dermatology , epidermolytic hyperkeratosis , medicine , dyskeratosis , pathology , ichthyosis
Keratoderma hereditarium mutilans (KHM) or Vohwinkel's syndrome (VS) is usually inherited as an autosomal dominant disease, but a recessive type has occasionally been described. We report a 19‐year‐old male born to consanguineous parents, who presented with KHM associated with generalized ichthyosiform dermatosis. On examination, his generalized ichthyosiform dermatosis was accompanied by severe ‘honey comb’ like palmoplantar keratoderma and marked hyperhidrosis. Bilateral fifth toes showed complete constricting bands just distal to the metatarsophalangeal joints leading to pseudoainhum. The histopathologic examination of a biopsy from the sole showed hyperkeratosis, marked parakeratosis, hypergranulosis, acanthosis, and a few mononuclear cells in the papillary dermis, consistent with the diagnosis of KHM. Several hereditary disorders may show varying degrees of hyperkeratosis and constriction of digits; their differentiating features are discussed. This case is reported for its rarity and interesting associated findings.