Mycosis fungoides granulomatous C84.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

granulomatous mycosis fungoides; Granulomatous Mycosis fungoides

Definition
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Histological variant of mycosis fungoides (about 6% of all patients with mycosis finmgoides) with formation of epitheloid cell granulomas reminiscent of sarcoidosis or anular granuloma.

Clinical features
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In larger (partly retrospective) studies, there was no clinical difference between the classical mycosis fungoides and the granulomatous variant.

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Histology
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There is an atypical lichenoid CD4(+) CD8(-) lymphocytic infiltrate with interstitial histiocytes and/or perivascular epithelial cell granulomas with giant cells (partly sarcoid aspect). Eosinophilic granulocytes and plasma cells are added to the infiltrate. Nuclear atypia and the detection of clonality of the T cell population consolidate the diagnosis.

General therapy
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Progression/forecast
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Apparently, this subtype of mycosis fungoides is associated with a worse prognosis than the classical form, both with regard to the response to therapy and the 5- and 10-year progression-free survival rate. However, the overall survival rate is the same between both groups.

Literature
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  1. Gammon B et al (2014) CD8(+) granulomatous cutaneous T-cell lymphoma: a potential association with immunodeficiency. J Am Acad Dermatol 71:555-560.
  2. Kogut M et al (2015) Granulomatous mycosis fungoides, a rare subtype of cutaneous T-cell lymphoma. JAAD Case Rep 1: 298-302.
  3. Li JY et al (2013) A case-control study of clinicopathologic features, prognosis, and therapeutic responses in patients with granulomatous mycosis fungoides. J Am Acad Dermatol 69:366-374.
  4. Mattox AR et al (2014) Granulomatous mycosis fungoides with clinical features of granulomatous slack skin. Cutis 93:E4-5.
  5. Pousa CM et al. (20159 Granulomatous mycosis fungoides--a diagnostic challenge. To Bras Dermatol 90:554-556.
  6. Wirtz M et al (2014) Granulomatous mycosis fungoides: combination therapy with bexarotene and PUVA. Dermatologist 65:274-276.

Outgoing links (1)

Mycosis fungoides;

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Last updated on: 29.10.2020