85 - Follicular mucinosis

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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. Clinically it presents as erythematous, scaly, and infiltrated plaques with follicular prominence, acneiform lesions, or focal alopecia (alopecia mucinosa). The disorder may be considered as primary follicular mucinosis, an uncommon benign idiopathic form of the disease affecting younger patients with fewer lesions, usually situated on the head and neck. Secondary follicular mucinosis occurs in older patients with more generalized lesions and a chronic course associated with other disorders, particularly cutaneous T-cell lymphoma (15–30% of cases) and folliculotropic mycosis fungoides. No single clinical or histologic feature predicts a benign course, although an isolated patch in the head and neck region is more likely to be primary follicular mucinosis. Follicular mucinosis associated with hematological malignancies tends (not invariably) to be associated with age over 30 years, a wider distribution of lesions, and systemic features such as night sweats, weight loss, or lymphadenopathy. There are also increasing reports of drug-induced follicular mucinosis. There is no standard therapy, and because 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, medications, or other neoplastic or inflammatory disorders is managed by treating the underlying associated condition.