Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma

Orpha code: 178528OMIM code:

Definition

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma is a rare form of primary cutaneous T-cell lymphoma characterized by rapidly progressing, localized or disseminated nodules, tumors or eczematous skin lesions. It has a particularly aggressive clinical course with a high tendency to spread, in advanced stages, to extracutaneous locations (the central nervous system, lung, testes). Lymph nodes are often spared.

Disease data
Classification

Disease

Synonyms
Berti lymphoma
Chłoniak Bertiego
Pierwotny skórny chłoniak epidermotropowy cytotoksyczny z komórek T CD8+
Primary cutaneous epidermotropic cytotoxic CD8+ T-cell lymphoma
ORPHA code
178528
OMIM code
-
ICD10 code
C84.5
ICD11 code
-

No additional description.

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