INTRODUCTION

Fox-Fordyce disease was first described in 1902 by George Henry Fox and John Addison Fordyce is an uncommon, inflammatory non-infectious dermatoses of apocrine glands¹. It is characterized by chronic processes and manifested by very itchy skin-colored papules, in parts of the body where the apocrine glands are concentrated². Its etiopathogenesis is little known and affects the areas with apocrine glands: the armpits, the groins, the pubic region, the perineum, the labia majora, the areolas and the umbilicus. It occurs mainly in postpubertal women, between 13 and 35 years old, although prepubertal and postmenopause^3,4cases have been reported; without racial predilection and with an incidence not yet established². It mainly affects women in a ratio of 9 to 1; occasionally some cases have been reported in men¹Due to emotional or physical stimulation of the apocrine glands, paroxysmal exacerbations are observed. Its treatment is discussed. Different therapeutic regimens have been used with disparate and not always satisfactory results^3,4,5.

The cause is an intraepidermal obstruction of the apocrine gland duct. The hypothesis is determined by hormonal and genetic factors, with histopathological criteria that include hyperkeratosis², obstruction of the follicular infundibulum², chronic inflammatory changes in the dermis1, and acinar dilation of the apocrine glands¹, which show a thick secretion of eosinophilic mucin in lumen¹. Treatment is not simple, as some treatment regimens can have annoying side effects and relapses are common². Other reviews do not specify a definitive treatment1, but describe some treatment options such as combined oral contraceptives to treat and reduce the symptoms of women of childbearing age¹. Even Isotretinoin has been used once in the treatment of this condition2, with variable results.

This aim of this study is to present a clinical case of Fordyce-Fox disease. It was decided to present this case because of the rare occurrence of this disease.

CASE PRESENTATION

It is presented the case of a postmenopausal 53-year-old female patient, with five years as date of her last period and negative Papanicolaou in 2019, which was evaluated in a gynaecological-obstetric consultation at the General Hospital Maria Auxiliadora, in November 2019, due to a papular eruption in the vulvar region, associated with sporadic itching that she had for 5 months. A biopsy of the lesion was taken and sent to the Pathological Anatomy Service, receiving a dark brown-colored tissue fragment, 0.3 x 0.2 x 0.1 cm, which was properly processed, obtaining a histological sheet, in which dilated apocrine glands were evidenced. Those showed a thick secretion constituted by mucin in lumen; associated with mild inflammatory changes in the dermis (Figure 1y 2). The patient met histopathological criteria for the diagnosis of Fordyce-Fox disease.

It was diagnosed as Fordyce-Fox disease. No medication has proven to be sufficiently effective, so it was decided to keep the patient on a symptomatic treatment.

Figure 1.  Mild inflammatory change in the dermis (H-E 40x) 

Figure 2.  Apocrine glands presence with mucin secretion in lumen (H-E 10x) 

DISCUSSION

The biopsy of the lesion exposed dilated apocrine glands, which showed a thick secretion of eosinophilic mucin in lumen. Histopathological criteria for the diagnosis of Fordyce-Fox disease include hyperkeratosis², obstruction of the follicular infundibulum², chronic inflammatory changes in the dermis¹, and acinar dilation of the apocrine glands¹, which show a thick eosinophilic secretion of mucin in its lumen¹. This case meets two of the four criteria mentioned, which in association with the clinical characteristics of the patient, allowed us to make the final diagnosis of Fordyce-Fox disease, discarding differential diagnoses such as lichen amyloidosis, lichen nitidus, eruptive syringomas, infectious folliculitis, follicular mucinosis, Darier's disease and so on^6,7.

Etiology has not yet been clarified. No genetic alterations or associated polymorphisms³have been described; however, Rubio and colleagues involve genetic, endocrine, metabolic or environmental elements with no evidence in favor of any of them^3,4,5.

As a first-line therapeutic option, some authors propose the use of calcineurin inhibitors, specifically pimecrolimus. This medication was developed to treat inflammatory processes, such as those characteristic of the disease after the rupture of the glandular duct, and by an unknown mechanism, it inhibits hyperkeratosis and follicular obstruction⁸. However, other literature does not specify a definitive treatment¹; but some treatment options are described, such as combined oral contraceptives to treat and reduce the symptoms of women of childbearing age¹, which together with a symptomatic one, is a proposal to start the patient's treatment. The patient received only symptomatic treatment, which was a restriction in the evolution of the clinical condition.

CONCLUSION

It is important to emphasize the use of vulvar biopsy in cases of pruritus vulvae of uncertain etiology and the report the first nationwide case of Fordyce-Fox disease in vulva.