Preferred Label : Xeroderma pigmentosum B;
ICD-11 definition : Xeroderma pigmentosum complementation group B (XPB) is an extremely rare subtype of
xeroderma pigmentosum (XP), a rare photodermatosis predisposing to skin cancers. Some
XPB patients present with classic XP features of varying severity (photosensitivity
of skin with burning and freckling, skin and eye tumors) and mild neurological abnormalities,
while others combine classical XP features with systemic and neurological manifestations
of Cockayne syndrome such as short stature, bilateral sensorineural hearing loss and
hyperreflexia (XP/CS complex).;
ICD-11 synonym : XPB - [xeroderma pigmentosum B];
ICD-11 acronym : XPB;
Origin ID : 1064918621;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
Xeroderma pigmentosum complementation group B (XPB) is an extremely rare subtype of
xeroderma pigmentosum (XP), a rare photodermatosis predisposing to skin cancers. Some
XPB patients present with classic XP features of varying severity (photosensitivity
of skin with burning and freckling, skin and eye tumors) and mild neurological abnormalities,
while others combine classical XP features with systemic and neurological manifestations
of Cockayne syndrome such as short stature, bilateral sensorineural hearing loss and
hyperreflexia (XP/CS complex).
