Fox‐Fordyce Disease in Two Prepubertal Girls: Histopathologic Demonstration of Eecrine Sweat Gland Involvement

Abstract

This report presents two prepubertal girls with Fox-Fordyce disease. The pruritic papules extensively affected the areas where apocrine glands are distributed (axillae, periareolar and intermammary zones, pubes, infraumbilical midline), and also extended to the neck and face near the external angle of the eyes in one child. Analyses of several biopsy specimens showed that the main lesion was a spongiotic vesicle containing inflammatory cells and keratinocytes affecting the hair infundibula and acrosyringia, together, with hyperkeratosis of both adnexa. The cause of the disease remains elusive, but the microscopic findings may explain the good results obtained with keratolytic agents.

Pathophysiology, clinical findings, and management of Fox‐Fordyce disease: A systematic review

“…Only two cases were associated with anhidrosis that is caused by gland destruction 9,12 . Scratching may cause lesions to become erythematous and may lead to lichenification of the skin in case of chronic disease 1,5,7,13–18 . No cases of FFD were associated with systemic symptoms in reviewed articles.…”

“…9,12 Scratching may cause lesions to become erythematous and may lead to lichenification of the skin in case of chronic disease. 1,5,7,[13][14][15][16][17][18] No cases of FFD were associated with systemic symptoms in reviewed articles.…”

“…While six cases occurred in people above the age of 40, only two cases were reported in people below the age of 10, both occurring in females aged 8 and 9, respectively. 1,[5][6][7][8][9] Additionally, three cases of FFD occurred in families. 3,10,11…”

“…In very rare cases, FFD was found to involve the thoracic area (around the sternum) the abdominal area (around the umbilicus), and the face, including the eyes, cheeks, lips, and neck. 5,16,[19][20][21]…”

A novel modality using microwave technology for the treatment of Fox-Fordyce disease (FFD)

“… 1 The lymphohistiocytic infiltrate may be the cause of the intense pruritus that often accompanies the lesions. 3 , 4 , 8 The precise pathogenesis of FFD remains unknown but hormonal factors, genetics, and stress are all thought to be contributors. 1 …”