Folliculotropic mycosis fungoides C84.0

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

Dieser Artikel auf Deutsch

Synonym(s)

FMF; follicular mycosis fungoides; Follicular mycosis fungoides; Folliculocentric mycosis fungoides; Folliculotropic cutaneous T-cell lymphoma; Folliculotropic mycosis fungoides; Folliculotropic Mycosis fungoides; Folliculotropic T-cell lymphoma of the skin; MF-associated follicular mucinosis; Mycosis fungoides folliculotropic; Pilotropic mycosis fungoides; SLHA; Syringotropic cutaneous T-cell-lymphoma

History
This section has been translated automatically.

Sarkany, 1969; Vakilzadeh, 1984

Definition
This section has been translated automatically.

Rare variant of a CD4-positive, cutaneous T-cell lymphoma of the mycosis fungoides type, which is characterized by a particular epitheliotropy (adnexotropy), often leaving out the epidermis (smooth epidermis)

Etiopathogenesis
This section has been translated automatically.

Manifestation
This section has been translated automatically.

Mainly occurring in adults (mean age of manifestation is 50-60 years), less frequently in children. Men are more frequently affected than women.

Localization
This section has been translated automatically.

Mainly localized on the trunk, capillitium (alopecic areas) and face, less frequently on the lower extremities.

Clinical features
This 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.

Histology
This 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).

Immunohistology: The tumor cells are CD3 positive, CD4 positive and CD8 negative as in classical MF. An admixture of CD30+ cells can be observed.

Diagnosis
This section has been translated automatically.

The clinical picture with the follicular, in the lateral view particularly prominent, pointed, skin-coloured, but little inflammatory, itchy horny papules (patient comes because of itching!) is characteristic. The histological pattern is characteristic. However, it takes an average of 3.9 years (!) until the diagnosis is made.

Therapy
This 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/forecast
This 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.

Similar cases have been described as "folliculocentric or pilotrope MF". It is likely that a relevant part of the patients classified under the clinical picture of mucinosis follicularis must indeed be considered as folliculotropic T cell lymphoma.

Literature
This section has been translated automatically.

  1. Baratli J et al (2014) Folliculotropic Mycosis fungoides. Dermatologist 65: 1011-1013
  2. Boer A et al (2004) Alopecia mucinosa is mycosis fungoides. At J Dermatopathol 26: 33-52
  3. Geramy P et al (2008) Folliculotropic mycosis fungoides. Arch Dermatol 144: 738-746
  4. Kazakov DV et al (2004) Clinicopathological spectrum of mycosis fungoides. J Eur Acad Dermatol Venereol 18: 397-415
  5. Thein M et al (2004) Syringotropic cutaneous T-cell lymphoma: an immunophenotypic and genotypic study of five cases. Br J Dermatol 151: 216-226
  6. Vakilzadeh F, Bröcker EB (1984) Syringolymphoid hyperplasia with alopecia. Br J Dermatol 110: 95-101
  7. van Santen S et al (2016) Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides.
    JAMA Dermatol 152:992-1000.
  8. 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.
  9. 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
  10. Willemze R et al (2005) WHO-EORTC classification for cutaneous lymphomas. Blood 105: 3768-3785
  11. 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

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 29.10.2020