Urticaria-like follicular mucinosis L98.5

Exceptionally rare, localized, cyclic inflammatory clinical picture with passive, not infrequently pruritic, inflammatory papules but also transient urticae. The cutaneous disease may be associated with autoimmune diseases (Hashimoto's thyroiditis, lupus erythematosus) (O'Reilly K et al. 2010). In this rare constellation, urticarial lesions have been reported in the setting of the disease, as well as blistering (Schuppli R et al 1981).

Follicular (primary) mucinosis may present as a benign idiopathic form that is not associated with other cutaneous or extracutaneous diseases. The primary form is usually confined to the face and scalp and is often associated with alopecia (alopecia mucinosa).

In contrast, secondary follicular mucinosis is associated with benign inflammatory diseases such as atopic dermatitis, lupus erythematosus, Graves' disease, or malignancies such as cutaneous T-cell lymphoma (O'Reilly K et al. 2010). In 1997, the association of follicular mucinosis and systemic lupus erythematosus was first reported (Dawn G, et al.1997; Rongioletti F et al. 1986).

Middle aged men

Face (forehead, cheeks, nose, chin) , front neck areas.

Treatment of secondary follicular mucinosis requires treatment of the underlying disease. Because cases of follicular mucinosis progressing to cutaneous T-cell lymphoma have been reported, patients with persistent or extensive disease should be monitored long-term (O'Reilly K et al (2010).

The rare follicular mucinosis was first described by Pinkus in 1957. At that time he reported six cases of alopecia mucinosa, a focal alopecia with mucin deposits in hair follicles . In 1959, the disease was renamed follicular mucinosis because of the frequent absence of alopecia.

The entity of "urticarial follicular mucinosis" is controversial. The efflorescent type described is an inflammatory follicular papule. This description is not compatible with the title "urticarial".

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13. Tupker RA et al (1997) Urticaria-like follicular mucinosis in a young female patient. Acta Derm Venereol 77:323-324.