Case Reports
Apoeccrine sweat duct obstruction as a cause for Fox-Fordyce disease,☆☆,,★★

https://doi.org/10.1067/mjd.2003.93Get rights and content

Abstract

The pathogenesis of Fox-Fordyce disease has been reported to be hyperkeratosis and obstruction of the upper hair follicle, where the duct of the apocrine sweat gland opens. We report a case of Fox-Fordyce disease with full clinical manifestation. It appeared to be caused by the obstruction of intraepidermal apoeccrine sweat ducts by apoeccrine secretory cells detached and released from the secretory epithelium. A 24-year-old woman visited our clinic with intensely pruritic papules on axillae, mammary areolae, and pubic areas. Histopathologic examination revealed an obstruction of the sweat duct in the epidermis, which opened directly to the skin surface. The closing substance of the duct was an aggregate of epithelial cells, probably derived from the secretory portion. In the dermis, the secretory cells of apocrinelike sweat glands had been detaching from the secretory epithelia. These findings suggest that Fox-Fordyce disease can occur by the mechanism in which apoeccrine secretory cells obstruct sweat ducts. (J Am Acad Dermatol 2003;48:453-5.)

Section snippets

Case report

A 24-year-old otherwise healthy woman presented with light-brown papules involving both axillae (Fig 1), mammary areolae, and pubes, which invoked intense itching.

. Slightly brownish, solid papules on axilla.

She had experienced this disorder for several years. Previous therapies, including very strong-class corticosteroid ointment and oral antihistaminics, were not effective. The axillae, mammary areolae, and pubic area revealed slightly brownish, solid papules ranging from 2 to 4 mm in

Discussion

Fox-Fordyce disease shows pruritic or tingling skin-colored papules on the axilla, areola of the nipple, and periumbilic area.2 Shelley and Levy4 reported that obstruction of the apocrine sweat duct induces destruction of the duct and the secretory portion of apocrine sweat gland. Hyperkeratosis of the duct causes the obstruction of the apocrine duct that opens to hair follicles. Therefore, it had been thought Fox-Fordyce disease was essentially apocrine miliaria.4 This thesis was supported by

References (8)

There are more references available in the full text version of this article.

Cited by (35)

  • Skin diseases of the breast and nipple: Inflammatory and infectious diseases

    2019, Journal of the American Academy of Dermatology
    Citation Excerpt :

    Fox-Fordyce disease (FFD) is a rare disease characterized by the development of intensely pruritic papules on apocrine sweat gland–bearing skin caused by the obstruction of apocrine sweat glands (Fig 8).87-89 The pathogenesis of FFD is multifactorial, although it is thought to have a hormonal component because it primarily occurs in postpubertal females and usually resolves during pregnancy and after menopause.89 The condition flares during periods of increased apocrine sweating (eg, stress).

  • Common spongiotic dermatoses

    2017, Seminars in Diagnostic Pathology
    Citation Excerpt :

    Histologically it manifests with spongiosis of the hair follicle infundibulum, adjacent to the point of entry of the apocrine duct. Few neutrophils can sometimes be present.37–39 Occasionally, periductal xanthomatized cells are seen (Fig. 4).

  • Diagnostic Pathology: Nonneoplastic Dermatopathology

    2016, Diagnostic Pathology: Nonneoplastic Dermatopathology
  • Diseases of cutaneous appendages

    2009, Weedon's Skin Pathology: Third Edition
View all citing articles on Scopus

Funding sources: None.

☆☆

Conflict of interest: None identified.

Reprint requests: Kenji Saga, MD, PhD, Department of Dermatology, Sapporo Medical University School of Medicine, Minami 1 Nishi 16, Chyuo-ku, 060-8543, Sapporo, Japan. E-mail: [email protected].

★★

0190-9622/2003/$30.00 + 0

View full text