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Follicular mucinosis
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. 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.
Management Strategy
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.
Specific Investigations
The cutaneous mucinoses
Truhan AP, Roenigk HH. J Am Acad Dermatol 1986; 14: 1–18.
Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides-associated follicular mucinosis
Rongioletti F, De Lucchi D, Meyes D, et al. J Cutan Pathol 2010; 37: 15–9.
Two excellent reviews of the follicular mucinosis literature, including histopathology and investigation .
Pediatric follicular mucinosis: presentation, histopathology, molecular genetics, treatment and outcomes over an 11-year period at the Mayo Clinic
Alikhan A, Griffin J, Nguyen N, et al. Pediatr Dermatol 2013; 30: 192–8.
A review of the clinical and histopathologic findings as well as the treatment and clinical outcomes in children with follicular mucinosis with and without mycosis fungoides .
Follicular mucinosis in patients with hematologic malignancies other than mycosis fungoides: a clinicopathologic study
Geller S, Gomez CJ, Myskowski PL, et al. J Am Acad Dermatol 2019; 80: 1704–11.
A review of the clinicopathological characteristics of follicular mucinosis associated with systemic hematologic malignancies versus those that are cutaneous lymphoma-associated.
Follicular mucinosis: a study of 47 patients
Emmerson RW. Br J Dermatol 1969; 81: 395–413.
Topical or intralesional corticosteroids improved surface eczematous change in eight of 22 patients with benign disease whose lesions resolved spontaneously, independently of treatment, within 2 years. Six of 10 patients with benign chronic disease of more than 2 years’ duration showed slight improvement with topical or intralesional corticosteroids.
Neonatal follicular mucinosis
Dalle S, Marrou K, Balme B, et al. Br J Dermatol 2007; 157: 609–10.
A solitary plaque on the scalp of a 21-day-old newborn responded to mild-potency topical corticosteroid with resolution within 2 months.
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