Fox-Fordyce disease: report of two cases with perifollicular xanthomatosis on histological image

Abstract

Fox-Fordyce disease is a relatively infrequent pathology of the apocrine glands that affects almost exclusively young women. The disease is characterized by the presence of pruritic follicular papules mainly in the armpits that respond poorly to treatment and severely affect the patient’s quality of life. We report two cases with clinical diagnosis and histopathological confirmation, presenting perifollicular xanthomatosis on histological examination, recently described as a distinctive, consistent, and specific feature of this disease.

Pruritic axillary papules in a 14‐year‐old girl

“…The lesions tend to display chronic relapsing and remitting evolution, described as lasting weeks to years. Differential diagnoses include Graham‐Little‐Piccardi‐Lasseur syndrome, trichostasis spinulosa, Darier's disease, syringomas, lichen nitidus, lichen amyloid and papular mucinosis . However, patients with FFD do not have cicatricial alopecia or lesions elsewhere on the body or mucosa.…”

“…The pathogenesis remains unknown, although hormonal factors, laser hair removal and inheritance may be involved . Clinically, FFD is characterised by numerous pruritic, uniform, 1–3 mm, flesh‐coloured to light brown, dome‐shaped papules with a smooth surface . Anhidrosis and hypotrichosis are often seen.…”

“…However, patients with FFD do not have cicatricial alopecia or lesions elsewhere on the body or mucosa. The diagnosis of FFD disease is based on characteristic clinical and histopathological features . Non‐specific findings such as intrafollicular corneal plug, hyperkeratosis, spongiosis, retention vesicles, glandular dilation with mucin deposits and perifollicular lymphohistiocytic inflammatory infiltrate can be observed in histopathological examination.…”

“…FFD, also known as apocrine miliaria, is a rare chronic condition characterised by a pruritic symmetric papular eruption in areas where apocrine glands are found predominantly, such as axillae, areolae and anogenital region. 1,2 The disease occurs mainly in women (female-to-male ratio is 9:1), aged 15-35 years and usually remits in menopause. 2 The pathogenesis remains unknown, although hormonal factors, laser hair removal and inheritance may be involved.…”

“…Differential diagnoses include Graham-Little-Piccardi-Lasseur syndrome, trichostasis spinulosa, Darier's disease, syringomas, lichen nitidus, lichen amyloid and papular mucinosis. 1,2 However, patients with FFD do not have cicatricial alopecia or lesions elsewhere on the body or mucosa. The diagnosis of FFD disease is based on characteristic clinical and histopathological features.…”