Published on 19/03/2015 by admin
Filed under Dermatology
Last modified 19/03/2015
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Justine Kluk and Malcolm H.A. Rustin
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Follicular mucinosis is characterized histologically by mucinous degeneration of the follicular outer root sheaths and sebaceous glands with an inflammatory infiltrate composed of lymphocytes, histiocytes, and eosinophils. Lesions consist of erythematous, scaly, and infiltrated plaques with follicular papules or prominent follicular orifices, and may demonstrate alopecia (alopecia mucinosa). Benign follicular mucinosis tends to affect younger patients (under 40 years), with fewer lesions, usually situated on the head and neck. Although lesions may resolve spontaneously within 2 years, a more generalized form, with lesions on the trunk and extremities, may run a chronic relapsing course over many years. Follicular mucinosis is associated with lymphoma, particularly mycosis fungoides, in 15–30% of cases. It is still unclear whether follicular mucinosis is a transitional state evolving into mycosis fungoides in these cases. No single clinical or histological feature predicts which patients will have a benign course although those found to have mycosis fungoides rarely had initial lesions on the head and neck. Associated lymphoma tends (although not invariably) to be associated with age over 30 years, a wider distribution of lesions, and possibly systemic features such as night sweats, weight loss, or lymphadenopathy.
There is no standard therapy for follicular mucinosis. As spontaneous resolution occurs in the benign forms, observation alone is certainly justified, particularly in the younger patient with limited disease. However, the need for follow-up and evaluation to exclude lymphoma must be emphasized. Follicular mucinosis associated with mycosis fungoides or other neoplastic or inflammatory disorders is managed by treating the underlying associated condition.
Skin biopsy
Immunohistochemistry and T-cell gene receptor analysis may be helpful adjuncts
Consider investigations to rule out lymphoma or other underlying disorders, depending on the presenting clinical features (general examination, plain radiology, and CT scans)
Truhan AP, Roenigk HH. J Am Acad Dermatol 1986; 14: 1–18.
Rongioletti F, De Lucchi D, Meyes D, Mora M, Rebora A, Zupo S, et al. J Cutan Pathol 2010; 37: 15–19.
Two excellent reviews of the follicular mucinosis literature, including histopathology and investigation.
Emmerson RW. Br J Dermatol 1969; 81: 395–413.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
Topical and intralesional corticosteroids
Dapsone
Mepacrine
Tetracycline
