Patterns Histopathologic of Fox-Fordyce Disease

Abstract

Fox-Fordyce disease is an uncommon condition that is biopsied even less commonly. Since the publication of Shelley and Levy, in 1956, the disease is regarded widely to be "apocrine miliaria", an analogue of eccrine miliaria. Histopathologically, an "intraepidermal sweat retention vesicle" is considered the only feature diagnostic of the condition. In this article, the entire literature devoted to the subject and reviewed features clinically and histopathologically as they were presented in those publications. Furthermore, observations in sections of tissue cut from 8 biopsy specimens taken from 4 patients with Fox-Fordyce disease are presented including correlation clinicopathologic. Changes histopathologically in Fox-Fordyce disease vary greatly. Apart from findings described previously as being typical (e.g., dilation, hyperkeratosis, and spongiosis of infundibula), there were vacuolar alteration at the dermo-epithelial junction of infundibula, dyskeratotic cells scattered in infundibula, and parakeratosis in the manner of cornoid lamellation within an orthokeratotic plug filling a dilated infundibulum. Infundibula and apocrine ducts were surrounded by numerous foamy macrophages. Despite that variation of features histopathologically, the appearance clinically, was similar in all patients, namely small infundibulocentric papules, sometimes with a keratotic plug in the center of it, and sometimes with a yellow cast. In conclusion, current concept of Fox-Fordyce disease is too restricted. All the different patterns histopathologically observed by us are manifestations of one single pathologic process named for Fox and Fordyce.