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 keratoses 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 hyperkeratosis, whereas the nonhereditary conditions are usually not keratotic. In addition to the usual KHM findings our 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.