Fox-Fordyce disease (syn. apocrine miliaria)

Fox-Fordyce disease is a rare skin condition, usually affecting young women, and characterised by an itchy papular eruption in the axillae, ano‐genital area or on the areolae of the nipple.

This chapter is set out as follows:

• The cause is unknown

• The condition predominates in females and presents most commonly after puberty. Males, and older patients, are occasionally affected
• There can be very significant itch, although some cases are asymptomatic

Distribution

• The axillae, ano‐genital area or on the areolae of the nipple

Morphology

• Skin-coloured or light brown, dome-shaped, follicular papules
• The skin may become lichenified (thickened) as a result of scratching
• Hair loss often follows
• Sweating can be reduced or absent

• Provide a patient information leaflet on Fox-Fordyce disease

• Response to treatment is unsatisfactory. Treatments used include topical and intralesional steroids, topical clindamycin, an oral contraceptive pill, retinoids (topical and systemic), phototherapy, and in severe cases surgical therapies

• The condition runs a very prolonged course, and may persist until the menopause. Some remission may occur in pregnancy