Rapid remission with calcipotriol betamethasone in refractory Fox-Fordyce disease.

Fox-Fordyce disease (FFD) is a rare pruritic dermatosis whose etiology has not been fully explored. It is mostly seen in women and presents as pruritic follicular papules at the apocrine (gland-bearing) regions, including the axilla, groins, perineum, and areola mammae, as well as the umbilicus. Treatment for FFD is extremely challenging in that there is no curative treatment for it. We report the case of a 26-year-old woman who was refractory to many treatments but who responded to calcipotriol betamethasone with rapid remission of her symptoms.

Clinical effects of topical pimecrolimus in a patient with Fox-Fordyce disease.

Fox-Fordyce disease (FFD) is characterized by a pruritic eruption of skin-coloured or yellowish papules in areas rich in apocrine glands. The histology comprises dilatation of follicular infundibula with hyperkeratosis, acanthosis, and spongiosis of the infundibular epithelium with perifollicular infiltration of lymphocytes and foamy histiocytes. We treated a 12-year-old girl with FFD with topical pimecrolimus for 12 weeks, this resulted in a complete clearance of lesions. After the therapy, the patient was followed for an additional 19 months without signs of relapse. The effects of pimecrolimus in FFD might imply that an inflammatory process inducing secondary reactive hyperkeratosis could be involved in the pathogenesis of FFD.

Fox-Fordyce disease: response to adapalene 0.1%.

The Fox-Fordyce disease is a rare inflammatory dermatosis that affects mainly young women and is characterized by multiple follicular papules, skin color or brownish, very itchy, localized in areas rich in apocrine glands. Histopathology shows focal spongiosis of the upper infundibulum with fibrosis and perifollicular lymphohistiocytic infiltrate. The diagnosis is based on clinical and histopathological examination. Many treatment options have been described; however none of them is excellent. We chose the topic adapalene 0.1% and a satisfactory improvement of the signs and symptoms of the disease was observed.

Fox-Fordyce disease.

Fox-Fordyce disease (FFD) is a rare inflammatory disorder that affects the apocrine sweat glands. Clinically, lesions are equidistant, smooth, uniform, firm, folliculocentric papules, which can range in color from flesh-colored to red-brown to slightly yellow. Whereas the axillae are most commonly involved, FFD also can involve the anogenital and periareolar areas, lips, umbilicus, sternum, perineum, and upper medial aspects of the thighs. The underlying etiology of FFD remains unclear although epidemiologic data support a hormonal component because women are more commonly affected than men. Moreover, symptoms initially present after the onset of puberty, flare perimenstrually, and often resolve during pregnancy and after menopause. Histopathologic findings include the obstruction of the apocrine duct by a hyperkeratotic plug in the follicular infundibulum, which is believed to represent the primary pathophysiologic process; subsequent ductal rupture and resulting inflammatory response produce the typical clinical picture. Treatment of FFD is difficult because no one agent has proven particularly effective. Topical and interlesional glucocorticoids are often considered the first-line pharmacologic agents, although use is often limited by concerns for atrophy. Other agents that have shown some success include topical and systemic retinoids, topical clindamycin, topical pimecrolimus cream, benzoyl peroxide, and oral contraceptives. For medication-refractory cases, mechanical destruction or removal of the apocrine glands has been efficacious in small case series.

Fox-Fordyce disease exacerbated by hyperhidrosis.

Fox-Fordyce disease is an uncommon disorder primarily affecting postpubertal females. It is characterized by intensely pruritic, papular eruptions in apocrine-gland bearing regions. Rarity and scant literature have resulted in a lack of definitive treatment options or pathognomonic diagnostic indicators. We report a 16-year-old boy with typical findings of Fox-Fordyce disease including numerous, grouped, rounded (1-3 mm), skin-colored papules confined to the axillary and periareolar areas, bilaterally. These lesions started before puberty, and were exacerbated by hyperhidrosis.

Enfermedad de Fox-Fordyce / Fox-Fordyce disease

La enfermedad de Fox-Fordyce es un trastorno inflamatorio poco frecuente caracterizado por la aparición de pápulas pruriginosas con distribución folicular localizadas en las áreas dotadas de glándulas apocrinas. La etiopatogenia no se conoce con claridad, involucrándose la obstrucción del conducto excretor apocrino como fenómeno precoz en el proceso. Se presenta un caso de enfermedad de Fox-Fordyce, confirmado histológicamente, en una mujer de 16 años, con gran expresividad clínica (AU)

Doença de Fox-Fordyce / Fox-Fordyce disease

Neste estudo relatamos o caso de uma paciente feminina de 12 anos de idade, com lesöes papulosas, pruriginosas, predominantes na regiäo das axilas. As lesöes eram típicas de doença de Fox-Fordyce e o exame anatomopatológico compatível com a hipótese clínica. A conduta terapêutica é abordada, observando-se remissäo das lesöes com o uso tópico de soluçäo de clindamicina em propilenoglicol.

Fox-Fordyce disease in a male patient--response to oral retinoid treatment.

Fox-Fordyce disease (apocrine milaria) is predominantly observed in women. A male patient with typical features of this disorder is described. Oral treatment with isotretinoin resulted in temporary relief.

[Fox-Fordyce disease (apocrine miliaria)]. / Morbus Fox-Fordyce (Apokrine Miliaria).

A 1-year follow-up in a 12 year old girl suffering from Fox-Fordyce disease is reported. Reddish papules were found in the typical locations in the regions with a high density of apocrine glands. A biopsy specimen showed keratin plugs in the infundibula of apocrine glands. Since hormone therapy could not yet be given, external therapy only was performed, with good results.