Fox-Fordyce disease: causes, symptoms, diagnosis, treatment
Last reviewed: 23.04.2024
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The disease of Fox-Fordis develops, as a rule, in women in young or middle age, but can occur in the menopausal period, as well as in children in the post-pubertal period.
The cause of Fox-Fordis disease is unknown.
Symptoms of Fox-Fordis disease. The sites of localization of the apocrine sweat glands, primarily the axillary cavities, the pubic region, the perineum are affected. The rash is small, has a tendency to follicular and parafollicular position. Papules hemispherical, sometimes conical, rounded, dense to the touch, shiny, reddish-cyanotic in color or color of normal skin, in most cases accompanied by intense itching, increasing before menstruation.
Pathomorphology of Fox-Fordis disease. First, a keratotic plug is formed in the funnel of the hair follicle, which clogs the excretory duct of the apocrine gland, which opens into the funnel area. As a result, the duct of the gland sharply expands and ruptures, which leads to the formation of a spongiotic vesicle in the outer root vagina of the hair follicle. The formed retention cyst is surrounded by a thickened epithelium and a perifollicular inflammatory infiltrate.
Histogenesis of Fox-Fordis disease. In the development of the disease, great importance is attached to the dysfunction of the apocrine sweat glands caused by the violation of the neurohumoral regulation of the menstrual cycle, which is manifested by an excess of estrogens. In this case, the main disorders leading to the development of clinical manifestations are the occlusion of the upper part of the duct of the apocrine glands by keratotic masses, followed by rupture and the occurrence of an inflammatory reaction around, metabolic changes in the form of glycosaminoglycan deposits. According to JH Graham et al. (1960), the presence of the disease in identical twins may be evidence of its non-void nature or predisposition to its development. In favor of the hypothesis of a causal relationship between Fox-Fordis disease and ovarian insufficiency, the presence of ovarian dysfunction in this disease and the improvement of its course during pregnancy, as well as the intensification of itching in the premenstrual period and the positive effect of taking estrogen preparations, are indicative.
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