Biomaterial Database

Successful treatment of keratoderma hereditaria mutilans with an aromatic retinoid. 1981

A F Chang Sing Pang, and A P Oranje, and V D Vuzevki, and E Stolz

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.

UI	MeSH Term	Description	Entries
D007645	Keratoderma, Palmoplantar	Group of mostly hereditary disorders characterized by thickening of the palms and soles as a result of excessive keratin formation leading to hypertrophy of the stratum corneum (hyperkeratosis).	Hyperkeratosis Palmaris et Plantaris,Keratosis Palmaris et Plantaris,Keratosis, Palmoplantar,Meleda Disease,Palmoplantar Keratoderma,Keratosis Palmoplantaris Transgradiens of Siemens,Mal de Meleda,Disease, Meleda,Keratodermas, Palmoplantar,Keratoses, Palmoplantar,Meleda, Mal de,Palmoplantar Keratodermas,Palmoplantar Keratoses,Palmoplantar Keratosis,de Meleda, Mal
D008297	Male		Males
D010859	Pigmentation Disorders	Diseases affecting PIGMENTATION, including SKIN PIGMENTATION.	Incontinentia Pigmenti Achromians,Ito Syndrome,Schamberg's Disease,Schamberg Disease,Disease, Schamberg,Disease, Schamberg's,Disorder, Pigmentation,Disorders, Pigmentation,Pigmentation Disorder,Schambergs Disease,Syndrome, Ito
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D003251	Constriction, Pathologic	The condition of an anatomical structure's being constricted beyond normal dimensions.	Stenosis,Stricture,Constriction, Pathological,Pathologic Constriction,Constrictions, Pathologic,Pathologic Constrictions,Pathological Constriction,Stenoses,Strictures
D004487	Edema	Abnormal fluid accumulation in TISSUES or body cavities. Most cases of edema are present under the SKIN in SUBCUTANEOUS TISSUE.	Dropsy,Hydrops,Anasarca
D005050	Etretinate	An oral retinoid used in the treatment of keratotic genodermatosis, lichen planus, and psoriasis. Beneficial effects have also been claimed in the prophylaxis of epithelial neoplasia. The compound may be teratogenic.	B10-9359,Ethyl Etrinoate,Ro 10-9359,Ro-10-9359,Tigason,Tigazon,B10 9359,B109359,Etrinoate, Ethyl,Ro 10 9359,Ro 109359,Ro109359
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000284	Administration, Oral	The giving of drugs, chemicals, or other substances by mouth.	Drug Administration, Oral,Administration, Oral Drug,Oral Administration,Oral Drug Administration,Administrations, Oral,Administrations, Oral Drug,Drug Administrations, Oral,Oral Administrations,Oral Drug Administrations
D000387	Ainhum	Spontaneous autoamputation of the fourth or fifth toe.	Dactylolysis Spontanea,Spontaneous Dactylolysis,Dactylolyses, Spontaneous,Dactylolysis, Spontaneous,Spontaneous Dactylolyses