Efficacy of Pimecrolimus in Fox-Fordyce Disease~!2010-01-31~!2010-05-10~!2010-06-17~!

Abstract

Fox-Fordyce disease (FFD) or 'apocrine miliaria' is a rare benign skin condition characterized by obstruction of the apocrine sweat gland. Treatment is generally unsatisfactory. A 15-year-old girl presented with intensely pruritic lesions on her axillae, chest and groin. Histology from an axillary skin biopsy confirmed FFD. The patient was commenced on pimecrolimus cream twice daily for three months and then once daily for a further three months. After a month she had marked improvement of her symptoms. By three months, the papules had flattened. The lesions remained healed at follow up nine months after completing therapy. Pimecrolimus is a safe and easy to use option and we suggest that it may be considered as first-line therapy for FFD.Keywords: Fox-Fordyce disease, apocrine miliaria, pimecrolimus, Elidel, treatment.Fox-Fordyce disease (FFD), or 'apocrine miliaria' is a rare benign skin condition characterized by obstruction of the apocrine sweat gland [1]. Treatment is generally unsatisfactory. We report a patient with FFD successfully treated with pimecrolimus.The pathogenesis of FFD is unclear but involves an initial keratin plug in the infundibulum of the hair follicle that then extends into the apocrine sweat duct. Intraepidermal parts of the apocrine duct rupture and microvesicles form. Acanthosis, spongiosis, dermal mucin and an inflammatory infiltrate result [2].A 15-year-old girl presented with intensely pruritic lesions on her axillae, chest and groin. On examination, she had follicular papules and pigmentation confined to these sites (Fig. 1). She had been previously unsuccessfully treated with moderate potency topical steroids, antifungals, tretinoin and oral antibiotics prescribed by her GP. Histology from an axillary skin biopsy confirmed FFD. The patient was commenced on pimecrolimus cream twice daily for three months and then once daily for a further three months. After a month she had marked improvement of her symptoms. By three months, the papules had flattened, the pruritus had lessened and the postinflammatory hyperpigmentation was not as prominent (Fig. 2). The lesions remained healed at follow up nine months after completing therapy.Fox-Fordyce disease runs a prolonged course. As well as the discomfort associated with pruritus, the lesions are cosmetically disfiguring. Case reports describe treatments with limited efficacy or associated with side effects that outweigh the benefits of treatment. Treatments include topical clindamycin, corticosteroids, tretinoin, benzoyl peroxide, oral isotretinoin, oral contraceptives, ultraviolet *Address correspondence to this author at the Department of Dermatology, Chelsea and Westminster Hospital, 369 Fulham Road, London, SW10 9NH, UK; E-mail: nilesh.morar@chelwest.nhs.uk Fig. (1). Follicular papules and pigmentation seen in the left axilla of the patient pre-treatment.

Prurito Vulvar y Enfermedad de Fox – Fordyce, un reporte de caso

“…Como opción terapéutica de primera línea, algunos autores proponen el uso de inhibidores de la calcineurina, específicamente pimecrolimus, ya que este medicamento fue desarrollado para tratar procesos inflamatorios como los que caracterizan la enfermedad luego de la ruptura del conducto glandular y también porque, mediante un mecanismo desconocido, inhibe la hiperqueratosis y la obstrucción folicular (8) . No obstante, en otras literaturas no se especifica un tratamiento definitivo (1) ; pero se describen algunas opciones de tratamiento como los anticonceptivos orales combinados para tratar y disminuir los síntomas de mujeres en edad fértil (1) , lo cual actualmente, junto con tratamiento sintomático, es lo que se plantea iniciar como tratamiento en la paciente.…”