Christ-Siemens-Touraine syndrome


An X-linked [MIM 305100] ectodermal dysplasia with anhidrosis, heat intolerance, and hypoplasia of sebaceous and sweat glands, resulting in dry, smooth and glossy skin; CSTS is accompanied by gonadal hypoplasia, ageusia, anosmia, URIs, absent nipples, partial anodontia or peg teeth, hypotrichosis, saddle nose, dysphagia, physical and mental retardation, feminine appearance and cleft palate; almost all patients are male

Christ-Siemens-Touraine syndrome

(krĭst′sē′mĭns-too-rĕn′) A rare congenital disease characterized by hairlessness, inability to sweat, and abnormal tooth formation.

Christ,

Josef, German physician and dentist, 1871-1948. Christ-Siemens-Touraine syndrome - congenitally defective or absent sweat glands, smooth, finely wrinkled skin, sunken nose, malformed and missing teeth, sparse fragile hair, and associated with deformed nails, absent breast tissue, mental retardation, or syndactyly. Synonym(s): anhidrotic ectodermal dysplasia

Siemens,

Hermann Werner, German dermatologist, 1891-1969. Christ-Siemens-Touraine syndrome - see under ChristHallopeau-Siemens syndrome

Touraine,

Albert, French dermatologist, 1883-1961. Christ-Siemens-Touraine syndrome - see under ChristTouraine-Solente-Golé syndrome - primary form of osteoarthropathy marked by clubbing of fingers, new bone formation, and synovial effusions.