ICD-11 classes
03 Diseases of the blood or blood-forming organs
Neoplasms of haematopoietic or lymphoid tissues
Myeloproliferative neoplasms
2A21 — Mastocytosis
2A21.1 — Cutaneous mastocytosis

ICD-11 2A21.1 — Cutaneous mastocytosis

Cutaneous mastocytosis is characterised by abnormal accumulation and proliferation of cutaneous mast cells. Most types are isolated but cutaneous mastocytosis can occur in association with systemic disease. Clinical forms include cutaneous mastocytoma, urticaria pigmentosa (the most frequent form), pseudoxanthomatous nodular cutaneous mastocytosis, telangiectasia macularis eruptiva perstans and diffuse cutaneous mastocytosis.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 2A21.1 contains 2 clarifying diagnoses:

  1. 2A21.10 — Urticaria pigmentosa
  2. 2A21.1Y — Other specified cutaneous mastocytosis

The diagnosis is coded elsewhere:

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