HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
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EtiopathogenesisThis section has been translated automatically.
See below Cutaneous T-cell lymphomas. This cutaneous T-cell variant was described after organ transplantation (Spence-Shishido A et al. 2015).
ManifestationThis section has been translated automatically.
LocalizationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
On neck, nape, face and capillitium localized, 2.0-10.0 cm large, mostly blurred, slightly reddened or reddish-brown or grayish-brown, clearly pruritic (rarely asymptomatic), little protruding from the skin level plaques with mostly skin-colored, 0.1-0.2 cm large, always follicular, pointed conical, rough horny papules (clinical picture of the so-called mucinosis follicularis). Not infrequently, evidence of lesional comedones and cysts; when affecting the face, also "acne-like" pictures (Shamim H et al. 2021). Lesional alopecia may occur at the capillitium, which may be reminiscent of alopecia areata.
Not infrequently, alopecia of the eyebrows may occur in the early stages.
In the advanced stage of the disease, the plaques and nodules emerge more clearly from the skin level.
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 compared to "classic" 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 tumor-stage MF. The 10-year survival rate for this collective is 25% (van Santen S et al. 2016).
The therapeutic response to the usual standard therapies is considered to be somewhat worse compared to "classic MF" (van Santen et al. 2017). In this respect, early "aggressive topical radiotherapy" is recommended in case of localized manifestation .
Note(s)This section has been translated automatically.
Similar cases have been described as "folliculocentric or pilotropic MF." It is likely that a relevant proportion of patients classified under the clinical picture of mucinosis follicularis must actually be evaluated as folliculotropic T-cell lymphoma.
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. Am J Dermatopathol 26: 33-52.
- Geramy P et al (2008) Folliculotropic mycosis fungoides. Arch Dermatol 144: 738-746
- Gu AK et al (2022) Folliculotropic mycosis fungoides. JAMA Dermatol 158: 316.
- Kazakov DV et al (2004) Clinicopathological spectrum of mycosis fungoides. J Eur Acad Dermatol Venereol 18: 397-415.
- Shamim H et al (2021) Acneiform presentations of folliculotropic mycosis fungoides. Am J Dermatopathol 43: 85-92.
- Spence-Shishido A et al (2015) Folliculotropic mycosis fungoides as a posttransplant lymphoproliferative disorder. Pediatrics 136: e701-705.
- 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 (5)Follicular mycosis fungoides; Folliculotropic t-cell lymphoma of the skin; Granulomatous slack skin; Keratosis pilaris; Syringotropic cutaneous t-cell-lymphoma;
Outgoing links (15)Acitretin; Alopecia areata (overview); Cd3; Cd30; Cd4; Cd8; Cutaneous t-cell lymphomas; Cytostatics (overview); Doxorubicin; Follicular mucinosis; ... Show all
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