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Fox-Fordyce disease: causes, symptoms, diagnosis, treatment
Last reviewed: 04.07.2025
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Fox-Fordyce disease usually develops in women of young or middle age, but can also occur during menopause and in children in the post-pubertal period.
The cause of Fox-Fordyce disease is unknown.
Symptoms of Fox-Fordyce disease. The localization of apocrine sweat glands is predominantly affected, primarily the armpits, pubic area, and perineum. The rash is small-papular, tends to be follicular and parafollicular. Papules are hemispherical, sometimes conical, round, dense to the touch, shiny, reddish-bluish in color or the color of normal skin, in most cases accompanied by intense itching, increasing before menstruation.
Pathomorphology of Fox-Fordyce disease. Initially, a keratotic plug is formed in the infundibulum of the hair follicle, blocking the excretory duct of the apocrine gland, which opens into the infundibulum. As a result, the gland duct sharply expands and ruptures, which leads to the formation of a spongiotic vesicle in the external root sheath of the hair follicle. The formed retention cyst is surrounded by thickened epithelium and perifollicular inflammatory infiltrate.
Histogenesis of Fox-Fordyce disease. In the development of the disease, great importance is attached to the dysfunction of the apocrine sweat glands caused by a violation of the neurohumoral regulation of the menstrual cycle, manifested by an excess of estrogens. At the same time, the main disorders leading to the development of clinical manifestations consist of blockage of the upper part of the apocrine gland duct with keratotic masses with subsequent rupture and the occurrence of an inflammatory reaction around, metabolic changes in the form of deposits of glycosaminoglycans. According to JH Graham et al. (1960), the presence of the disease in identical twins may be evidence of its nevoid nature or a predisposition to its development. The presence of ovarian dysfunction in this disease and an improvement in its course during pregnancy, as well as increased itching in the premenstrual period and a positive effect from taking estrogen drugs, support the hypothesis of a causal relationship between Fox-Fordyce disease and ovarian insufficiency.
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