HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
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EtiopathogenesisThis section has been translated automatically.
ManifestationThis section has been translated automatically.
LocalizationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
On the neck, nape of the neck, face and capillitium localized, 2.0-10.0 cm in size, usually blurred, slightly reddish or reddish-brown or greyish-brown, clearly itchy (more rarely asymptomatic) plaques that hardly protrude from the level of the skin with mostly skin-coloured, 0.1-0.2 cm in size, always follicular, pointedly conical, rough horny papules (clinical picture of the so-called mucinosis follicularis). Often evidence of lesional comedones and cysts; in case of facial infestation also "acne-like" pictures. A lesionally limited alopecia can occur on the capillitium. It is not uncommon for alopecia of the eyebrows to occur in the early stages. In the advanced stage, development of clearly protruding plaques and nodules.
HistologyThis section has been translated automatically.
Histology: Follicular lymphocytic infiltrate from small to medium sized lymphocytes (these often have cerebriform nuclei). An epidermotropy is usually absent or only very focally detectable. Adnexal epithelia initially appear hyperplastic and later destroyed (image of mucinosis follicularis). Sometimes only individual epithelial bandages are detectable.
Some cases are characterized by a preferential syringotropy (older name: syringotropic MF).
DiagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
- S.u. Mycosis fungoides.
- Stage scheme:
- Initial strict local therapy with PUVA or narrow band UVB and glucocorticoid externa.
- In case of progression of MF: PUVA in combination with retinoids ( Acitretin: the effective dose is above 10 mg/day. The optimum effect is about 50 mg/day depending on body weight).
- In case of progression of MF: combination of PUVA and interferon alpha (dosage: 3.0-5.0 million IU, 3 times/week s.c.); in addition: local X-ray irradiation (3.0-5.0 Gy) by means of linear accelerators or conventional X-ray equipment.
- In stage IIb (see cutaneous T-cell lymphomas): chemotherapy (CHOP, doxorubicin, gemcitabine; see cytostatics below): poor response.
- Experimental: allogeneic stem cell transplantation.
Progression/forecastThis section has been translated automatically.
Approximately analogous prognosis in comparison to the "classical" Mycosis fungoides. The 5-year survival rate in stage IIA is 87%, in stage IIb 83%. In patients with more extensive plaques of folliculotropic MF, survival is expected to be analogous to patients with tunor stage MF. The 10 year survival rate for this group is 25% (van Santen S et al. 2016).
The therapeutic response to the usual standard therapies is rather worse than that of the "classic MF" (van Santen et al. 2017). Insofar an early "aggressive topical radiotherapy" is definitely recommended
Note(s)This section has been translated automatically.
LiteratureThis section has been translated automatically.
- Baratli J et al (2014) Folliculotropic Mycosis fungoides. Dermatologist 65: 1011-1013
- Boer A et al (2004) Alopecia mucinosa is mycosis fungoides. At J Dermatopathol 26: 33-52
- Geramy P et al (2008) Folliculotropic mycosis fungoides. Arch Dermatol 144: 738-746
- Kazakov DV et al (2004) Clinicopathological spectrum of mycosis fungoides. J Eur Acad Dermatol Venereol 18: 397-415
- Thein M et al (2004) Syringotropic cutaneous T-cell lymphoma: an immunophenotypic and genotypic study of five cases. Br J Dermatol 151: 216-226
- Vakilzadeh F, Bröcker EB (1984) Syringolymphoid hyperplasia with alopecia. Br J Dermatol 110: 95-101
- van Santen S et al (2016) Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides.
JAMA Dermatol 152:992-1000.
- van Santen S et al. (2017) Recommendations for treatment in folliculotropic mycosis fungoides: report of the Dutch Cutaneous Lymphoma Group. Br J Dermatol 177:223-228.
- Willemze R et al (1997) EORTC classification for primary cutaneous lymphomas: a proposal from cutaneous lymphoma study group of the European Organization for Research and Treatment of Cancer. Blood 90: 354-371
- Willemze R et al (2005) WHO-EORTC classification for cutaneous lymphomas. Blood 105: 3768-3785
- Zelger B, Sepp N, Weyrer K, Grunewald K, Zelger B (1994) Syringotropic cutaneous T-cell lymphoma: a variant of mycosis fungoides? Br J Dermatol 130: 765-769
Incoming links (4)Follicular mycosis fungoides; Folliculotropic t-cell lymphoma of the skin; Granulomatous slack skin; Syringotropic cutaneous t-cell-lymphoma;
Outgoing links (14)Acitretin; Cd3; Cd30; Cd4; Cd8; Cutaneous t-cell lymphomas; Cytostatics (overview); Doxorubicin; Follicular mucinosis; Gemcitabine; ... Show all
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