Preferred Label : Dowling-Degos disease;
ICD-11 definition : Dowling-Degos disease is a rare autosomal dominant condition which usually presents
post-pubertally with multiple small, round pigmented freckle-like macules. They become
progressively more numerous and may become reticulate with time. The axillae and groins
are the usual sites, although other areas may be involved, including the intergluteal
and inframammary folds, neck, scalp, trunk, arms and genitalia. Pigmentation is symmetrical
and progressive, but is otherwise asymptomatic.;
ICD-11 synonym : Reticulate pigmented anomaly of the flexures;
Origin ID : 15123132;
Automatic exact mappings (from CISMeF team)
Currated CISMeF NLP mapping
Validated automatic mappings to BTNT
Dowling-Degos disease is a rare autosomal dominant condition which usually presents
post-pubertally with multiple small, round pigmented freckle-like macules. They become
progressively more numerous and may become reticulate with time. The axillae and groins
are the usual sites, although other areas may be involved, including the intergluteal
and inframammary folds, neck, scalp, trunk, arms and genitalia. Pigmentation is symmetrical
and progressive, but is otherwise asymptomatic.