Fox-Fordyce disease is a specific skin disease that has a gender coloring, affecting the localization areas of the glandular appendages of the dermis, usually combined with dysfunction of the thyroid and genital glands. The leading symptom of dermatosis is attacks of severe itching in combination with a small-nodular rash in the armpits, around the breast nipples, navel, pubis, genitals. The nodules are oval, flesh-colored, matte-shiny, tightly surround the follicles of the apocrine glands and sebaceous ducts, closing their openings with a cluster of horny scales. Diagnosis is based on a typical clinic. Treatment is aimed at relieving itching and suppressing the activity of the skin glands.
General information
Fox-Fordyce disease is a chronic dermatosis of unknown origin, characterized by benign hyperplasia of the ducts of the sebaceous glands (Fordyce granules), blockage of the excretory ducts of the apocrine sweat glands. Women get sick more often. Starting during puberty, the disease can accompany patients throughout their lives, significantly disrupting its quality. The pathology is named after two American dermatologists: John Fordyce, head of the Department of Dermatology and Syphilidology at New York University and George Fox, one of the founders of the American Dermatological Association. They were the first to describe an unusual skin pathology in 1902 during a joint dermatological practice at Bellevue Hospital Medical College. A separate merit of J. Fordyce is a description of specific granules – pseudoseborrheic cysts of the sebaceous glands as the primary element of the rash. In addition, scientists were able to link the localization of rashes with somatic pathology (urological, endocrine, infectious). This approach to understanding the disease is still relevant today.
Causes
The etiology and pathogenesis of the disease have not been fully studied. In modern dermatology, the trigger (trigger) of the disease is called injuries during laser hair removal. It is believed that dermatosis occurs as a result of metabolic disorders that increase the production of secretions of the skin glands. The composition of the secret affects the physiological, biochemical, immune functions of the skin. Hypersecretion in combination with blockage of the gland as a result of localization of primary nodules around the hair follicle causes a toxic effect, accompanied by a sharp itching.
Some dermatologists tend to associate the development of Fox-Fordyce disease with dysfunction of the genital glands, others with endocrine disorders (thyroid gland, ovaries, adrenal glands), which is confirmed by spontaneous improvement of the clinic in the third trimester of pregnancy, increased itching before menstruation. There is an opinion about an individual predisposition to the occurrence of the disease.
Symptoms
The main clinical symptom is severe itching in the area of skin rashes. The primary element of the rash is a dense, shiny, spherical or cone–shaped nodule the size of a millet grain of all shades of flesh color, having no tendency to merge, localized at the mouth of the glandular formations of the skin. Unbearable itching eventually leads to skin lichenification, combs with hemorrhagic crusts, pigmentation, hair damage.
Rashes on the skin of the genitals (scrotum, foreskin – in men; labia, vagina – in women) are possible in two variants: in the form of a nodular perifollicular rash leading to the development of toxic skin damage, disharmony of sexual relations; in the form of seborrheic granules Fordyce – a cosmetic defect resulting from violations of the rules of intimate hygiene. Foci in the pubic area, navel, breast nipples, perineum, armpits are often considered as a symptom accompanying dysfunction of the endocrine glands or hormonal changes. The course is long, recurrent. Spontaneous self-resolution of the process is possible.
Diagnostics
Diagnosis is based on determining the typical type and localization of skin rash, the intensity of itching attacks. Histology is not specific: dilated ducts of glands with corneal plugs in the mouths of follicles, hyperkeratosis, parakeratosis, acanthosis, lymphocytic perivascular infiltration of the epidermis. Differentiate dermatosis with the following conditions:
- limited neurodermatitis (foci of rashes are not associated with the zones of apocrine glands, the primary elements merge with each other, forming plaques with a lichenized surface and white dermographism – atypical skin coloration with mechanical irritation);
- red lichen planus (histologically); papillary-pigmented skin dystrophy (“shagreen skin”, combined with malignant neoplasms);
- elastic pseudoxanthoma (there are changes in the fundus and degeneration of elastic fibers);
- dry eczema;
- a contagious mollusk (clinically and laboratory).
Fox-Fordyce disease treatment
The main objective of therapy is to neutralize itching attacks: antihistamines (chloropyramine), sedatives (diazepam) are used for this purpose. According to indications, after consultation with an endocrinologist, corticosteroids, hormones of the genital glands, oral contraceptives are prescribed. To restore harmony in the intimate sphere, consultations of a urologist and a sexologist are necessary.
In order to suppress the activity of the skin glands, radiation therapy is performed (hard rays with aluminum, zinc filters). In resistant cases, skin grafting is indicated after consultation with a dermatologist and a plastic surgeon. Limited rashes (up to 5 cm) are removed using a laser. Curettage is possible – curettage of sweat glands, electrocoagulation. Background therapy includes taking vitamins A, B, C, E; external use of drugs with anesthetic, menthol, retinol, corticosteroids, jojoba oil. The prognosis is favorable for life.
