Familial atypical multiple mole melanoma…

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Familial atypical multiple mole melanoma syndrome

ORPHA:404560Disease

Also called B-K mole syndrome · FAMM-PC syndrome · FAMMM syndrome · Familial atypical mole syndrome · Familial atypical multiple mole melanoma-pancreatic carcinoma syndrome · Familial dysplastic nevus syndrome · Melanoma-pancreatic cancer syndrome

What it is

Familial atypical multiple mole melanoma (FAMMM) syndrome is an inherited genodermatosis characterized by the presence of multiple melanocytic nevi (often >50) and a family history of melanoma as well as, in a subset of patients, an increased risk of developing pancreatic cancer and other malignancies.

Key facts

Age of onset
All ages
Inheritance
Autosomal dominant
Classified as
Disease

Gene

CDKN2ADisease-causing germline mutation(s)

ICD-10 codes

C43.9filed under a broader ICD-10 category — shared with 2 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

GARD 9281MONDO 0018453OMIM 155600OMIM 606719UMLS C2314896

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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