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syndroom van keratosis palmoplantaris en oesofageaal carcinoom (aandoening)
syndroom van keratosis palmoplantaris en oesofageaal carcinoom
Howell-Evans-syndroom
syndroom van palmoplantaire hyperkeratose en oesofageaal carcinoom
syndroom van palmoplantaire keratodermie en oesofageaal carcinoom
Palmoplantar keratoderma esophageal carcinoma syndrome
Palmoplantar hyperkeratosis esophageal carcinoma syndrome
Howel Evans syndrome
Keratosis palmoplantaris esophageal carcinoma syndrome
Howel-Evans-Clark syndrome
Howel-Evans' syndrome
Keratoderma with carcinoma of esophagus
Bennion Patterson syndrome
Tylosis esophageal carcinoma syndrome
A rare genetic disease characterized by thickening of the skin on palms and soles restricted to areas of weight bearing and/or friction (focal, non-epidermolytic palmoplantar keratoderma) and oral and esophageal leukokeratosis, associated with a very high lifetime risk of developing squamous cell carcinoma of the esophagus. The skin lesions appear in childhood and can be complicated by fissuring and infection.
Id111030006
StatusPrimitive
SNOMED CT to Orphanet simple map2198
SNOMED CT to ICD-10 extended map
TargetQ82.8
RuleTRUE
AdviceALWAYS Q82.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified