Familial generalized lentiginosis

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Familial generalized lentiginosis

ORPHA:231040Disease

Also called Familial lentigines profusa · Familial multiple lentigines syndrome without systemic involvement

What it is

Familial generalized lentiginosis is a rare, inherited, skin hyperpigmentation disorder characterized by widespread lentigines without associated noncutaneous abnormalities. Patients present multiple brown to dark brown, non-elevated macula of 0.2 to 1 cm in diameter, spread over the entire body, sometimes including palms or soles, but never oral mucosa.

Key facts

Age of onset
All ages
Inheritance
Autosomal dominant, Unknown
Classified as
Disease

Gene

SASH1Disease-causing germline mutation(s)

ICD-10 codes

L81.4filed under a broader ICD-10 category — shared with 4 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MONDO 0007891OMIM 151001UMLS C4707357

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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