Mycosis fungoides granulomatousC84.0
Synonym(s)
DefinitionThis section has been translated automatically.
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 featuresThis section has been translated automatically.
In larger (partly retrospective) studies, there was no clinical difference between the classical mycosis fungoides and the granulomatous variant.
HistologyThis section has been translated automatically.
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 therapyThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
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.
LiteratureThis section has been translated automatically.
- Gammon B et al (2014) CD8(+) granulomatous cutaneous T-cell lymphoma: a potential association with immunodeficiency. J Am Acad Dermatol 71:555-560.
- Kogut M et al (2015) Granulomatous mycosis fungoides, a rare subtype of cutaneous T-cell lymphoma. JAAD Case Rep 1: 298-302.
- 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.
- Mattox AR et al (2014) Granulomatous mycosis fungoides with clinical features of granulomatous slack skin. Cutis 93:E4-5.
- Pousa CM et al. (20159 Granulomatous mycosis fungoides--a diagnostic challenge. To Bras Dermatol 90:554-556.
- Wirtz M et al (2014) Granulomatous mycosis fungoides: combination therapy with bexarotene and PUVA. Dermatologist 65:274-276.