Fox-Fordyce disease of the vulva.

Fox-Fordyce disease is a rare, chronic skin disorder which affects the apocrine areas. This disease is due to the obstruction of the apocrine sweat duct. Extragenital regions are commonly affected than the genital region. We, herein, report a case of Fox-Fordyce in a female, with onset in the fifth decade and involving only the genital region.

INTRODUCTION

Fox–Fordyce disease is a rare, pruritic skin disorder which affects the areas bearing apocrine sweat glands. This disease occurs commonly in females after adolescence. The pathogenesis appears to be related to the blockage of apocrine sweat duct. There is severe itching present over the lesions. Infection or folliculitis may develop secondary to trauma due to scratching. Fox–Fordyce disease is a chronic condition with intermittent flares and treatment is often unsatisfactory.

CASE REPORT

A 45-year-old female patient presented with complaints of skin lesions in the vulva for past 6 months with a history of intermittent exacerbations. Intense itching of the lesions was also present. At the time of presentation, she had developed oozing from the lesions of 1-week duration. There was no history of developing similar lesions in the past. She also had not taken any specific treatment for the lesions. She had a history of undergoing hysterectomy with bilateral salpingo-oophorectomy 2 years back. She was married for 20 years and had no previous history of exposure to sexually transmitted diseases.

General examination of the patient was normal. Multiple, firm, discrete, dome-shaped, skin-colored, follicular papules were seen over the pubic area and labia majora with few areas of oozing and crusting [Figure 1]. Regional lymphadenopathy was absent. There were no other specific skin lesions elsewhere on the body. Skin biopsy taken from the lesions showed features of spongiotic dermatitis with periadnexal and perivascular lymphocytic infiltrate [Figure 2].

Figure 1

Multiple, firm, discrete, dome-shaped, skin-colored, follicular papules seen over the pubic area and labia majora with few areas of oozing and crusting

Figure 2

Skin biopsy taken from the lesions showed features of spongiotic dermatitis with periadnexal and perivascular lymphocytic infiltrate

A diagnosis of Fox–Fordyce disease with secondary infection was made based on the typical history, clinical picture, and histopathology. The oozing and crusting over the lesions were due to secondary bacterial infection as a result of scratching.

The patient was initially started on antibiotics and antihistamines. After a week, the secondary infection had subsided, and the patient was started on topical corticosteroids and topical tretinoin 0.025% cream. The itching subsided, and partial improvement of lesions was noted after 2 weeks of treatment.

Genital involvement is rare in Fox–Fordyce disease. We report this case, with onset in the fifth decade and isolated involvement of the genital region.

DISCUSSION

Fox–Fordyce disease is an uncommon eruption which affects the sites bearing apocrine sweat glands. George Henry Fox and John Addison Fordyce described the disease in 1902. The term apocrine miliaria is used as a synonym for this disease.

Exact etiology is unclear. It is speculated that keratinous obstruction of the intraepidermal apocrine gland ducts is necessary for the development of the disease. Rupture of the blocked ducts leads to seepage of sweat from the ruptured duct and produces inflammation around the hair follicles. Genetics likely has a role in the development of the disease. The disease has been described in identical twins, and familial occurrence has occasionally been reported. Epidemiologic data support a hormonal component. More than 90% of affected are females. Symptoms initially present after puberty, flare perimenstrually and mostly resolve during pregnancy and after menopause. Men, children, and postmenopausal women, however, can also be affected. There is no racial or ethnic predilection.

Clinically, the disease manifests as smooth, uniform, firm, dome-shaped, equidistant, skin-colored to slightly yellow, folliculocentric pustules associated with intense pruritus. Axillae are most commonly affected. Other less commonly involved sites are the pubic region, perineum, mammary areola, presternal region, periumbilical region, and upper inner aspect of thighs. Heat, humidity, friction, and stress exacerbate the symptoms. Itching is paroxysmal and precipitated by emotional or other stimuli which induce apocrine sweating. Excoriations and lichenification may be seen as a consequence of scratching. As a result, skin near the affected area may become darkened, and hair may become sparse in the affected areas.[1] Apocrine anhidrosis may result eventually.

Clinically, folliculitis, miliaria, syringomas, hidradenitis suppurativa, lichen nitidus, and acneiform eruptions can be considered in the differential diagnoses of Fox–Fordyce disease. The diagnosis is mainly clinical based on the typical distribution and morphology of the eruption and the characteristic pruritus.

Histopathological examination is seldom required to diagnose the condition as the findings vary considerably. Transverse histological section is considered to be most effective way to diagnose Fox–Fordyce disease on biopsy.[2] Dilatation and hyperkeratosis of the follicular infundibulum are seen. A spongiotic vesicle occurs in the follicular infundibulum. The apocrine duct behind the obstructing keratin plug becomes dilated. Lymphocyte exocytosis is seen in the area of spongiosis. There may also be infundibular acanthosis and mild perivascular and periadnexal lymphocytic inflammation in the adjacent upper dermis. Perifollicular foam cells are a specific histologic feature.[3]

Fox–Fordyce disease is a chronic disorder with intermittent flares and treatment is often difficult. Avoidance of excessive sweating may minimize the flares.[4] Topical clindamycin in alcoholic propylene glycol solution is effective. Topical adapalene and tretinoin have demonstrated efficacy. Other medical therapies tried are oral contraceptive pills, oral isotretinoin, topical pimecrolimus, topical benzoyl peroxide, and topical corticosteroids. Intralesional triamcinolone 5 mg/ml may offer benefit in early cases. Surgical options include electrocoagulation, liposuction assisted curettage, and surgical excision of the affected glands.

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Conflicts of interest

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