Keratoma hereditaria mutilans (Vohwinkel). Differentiating features of conditions with constriction of digits

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Abstract
A patient with keratoma hereditaria multilans (KHM), a young white girl, presented the following classic features of this rare disease: familial palmar and plantar hyperkeratosis beginning early in life, starfish-like kera-toses of the dorsa of hands and feet, linear keratoses of elbows and knees, and constriction of digits beginning in the teens. A classification of the hereditary and nonhereditary diseases which exhibit digital constriction is briefly presented. The majority of the hereditary diseases with digital constriction have palmar and plantar hyperkerato-sis, whereas the nonhereditary conditions are usually not keratotic. In addition to the usual KHM findings the patient presents a high-frequency hearing loss; an unusual, pseudopelade-type alopecia; and transient, plantar bullae. The disease is probably a variant of mal de Meleda.

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