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.
Subject(s)
Fox-Fordyce Disease/pathology , Xanthomatosis/pathology , Adult , Female , Fox-Fordyce Disease/complications , Humans , Immunohistochemistry , Xanthomatosis/etiology , Young AdultABSTRACT
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.
Subject(s)
Humans , Female , Adult , Young Adult , Xanthomatosis/pathology , Fox-Fordyce Disease/pathology , Immunohistochemistry , Xanthomatosis/etiology , Fox-Fordyce Disease/complicationsSubject(s)
Fox-Fordyce Disease/pathology , Adult , Axilla/pathology , Biopsy , Dermis/pathology , Female , Hair Follicle/pathology , Humans , Vulva/pathologySubject(s)
Humans , Female , Adult , Fox-Fordyce Disease/pathology , Axilla/pathology , Vulva/pathology , Biopsy , Hair Follicle/pathology , Dermis/pathologyABSTRACT
BACKGROUND: Fox-Fordyce disease is a rare chronic papular condition with a very characteristic clinical presentation but a nonspecific histopathology. Its traditionally described histopathologic features have been criticized as variable and indistinct. Recently, a perifollicular infiltrate of histiocytes with foamy cytoplasm has been described as a consistent and reliable diagnostic finding. METHODS: To evaluate the traditional and most recently described histopathologic and immunohistochemical attributes of Fox-Fordyce disease, we performed a computerized search of specimens in two dermatopathologic databases in Puerto Rico from the years 2000-2010. An additional specimen was donated by a dermatopathologist from an outside institution. Three cases were evaluated using hematoxylin-eosin-stained sections. The tip of an axillary lipoma excision specimen was used as the control tissue. Periodic acid-Schiff, colloidal iron, and immunoperoxidase staining for CD68 and c-kit (CD117) were performed in all specimens. RESULTS: We were able to verify traditionally described histological features such as infundibulum dilation, hyperkeratosis, plugging, acanthosis, and lymphohistiocytic infiltrate. Infundibular spongiosis was also common. A perifollicular foam cell infiltrate was the most distinct pathologic feature among our cases. The periodic acid-Schiff staining patterns suggested that the foam cell cytoplasm material might be similar in nature to the apocrine gland secretion content. CONCLUSIONS: Our results confirmed that a perifollicular foam cell infiltrate is the most distinct histopathologic feature. In addition, findings suggest that the intracytoplasmic foam cell material may be similar in nature to the apocrine gland secretion.
Subject(s)
Fox-Fordyce Disease/pathology , Adolescent , Adult , Female , Humans , Young AdultABSTRACT
The Fox-Fordyce disease is a rare inflammatory dermatosis that affects mainly young women and is characterized by multiple follicular papules, skin color or brownish, very itchy, localized in areas rich in apocrine glands. Histopathology shows focal spongiosis of the upper infundibulum with fibrosis and perifollicular lymphohistiocytic infiltrate. The diagnosis is based on clinical and histopathological examination. Many treatment options have been described; however none of them is excellent. We chose the topic adapalene 0.1% and a satisfactory improvement of the signs and symptoms of the disease was observed.
Subject(s)
Dermatologic Agents/therapeutic use , Fox-Fordyce Disease/drug therapy , Naphthalenes/therapeutic use , Adapalene , Child , Female , Fox-Fordyce Disease/pathology , Humans , Treatment OutcomeABSTRACT
Fox-Fordyce disease is a condition with protean histopathological alterations whose pathogenesis remains a mystery. Although recent studies have addressed histological changes specific of this disease, including perifollicular xanthomatosis, no attention has been given to apocrine acini dilation as an adjunct histopathological finding to the diagnosis. Moreover, although previous efforts were done to demonstrate that perifollicular foamy histiocytes harbor apocrine secretion content, this concept has not been proved to date. In this study, we report 2 cases harboring prominent dilation of apocrine coils with mucinous content. Such mucinous content showed mucin profile identical to the dermal mucin deposits in both cases. Of note, perifollicular foamy histiocytes demonstrated cytoplasmic mucin, supporting the suggestion that these cells phagocytose apocrine secretion. Although not specific, apocrine coil dilation is another histopathological feature of Fox-Fordyce disease and it may be used as a low-power magnification clue for the correct diagnosis. We also propose that the so-called perifollicular xanthomatosis may be composed of muciphages or mixed cell (muciphages/xanthomatous) population, an issue that should be further investigated in future studies.
Subject(s)
Apocrine Glands/pathology , Axilla/pathology , Fox-Fordyce Disease/pathology , Adult , Biopsy , Female , Humans , Pruritus/pathology , Xanthomatosis/pathologyABSTRACT
PURPOSE: To present a case of epibulbar Fordyce nodules, with a referral diagnosis of primary tumor. METHODS: Case report. RESULTS: A 38-year-old woman was referred for ocular oncology consultation because of a conjunctival lesion in the right eye. She had had a buccal mucous graft to treat recurrent pterygium 18 years earlier. The lesion consisted of multiple small, yellow granules over a pink, thickened mucosa from the 12 to 3 o'clock meridians. Excisional biopsy revealed multiple subepithelial sebaceous glands consistent with Fordyce nodules. CONCLUSIONS: Fordyce nodules are a possible late benign complication of buccal mucous grafts.
Subject(s)
Fox-Fordyce Disease/etiology , Mouth Mucosa/transplantation , Pterygium/surgery , Sebaceous Glands/pathology , Adult , Female , Fox-Fordyce Disease/pathology , Humans , Pterygium/pathologyABSTRACT
La patología blanca de la mucosa oral comprende una constelación de enfermedades cuyos cuadros clínicos se asemejan mucho entre sí y se caracterizan por la presentación de una lesión de aspecto blanquesino en la cavidad bucal. Con el fin de remarcar las diferencias entre cada afección, sus diversas evoluciones y pronósticos y alertar sobre aquellas con tendencia cancerígena y pronóstico más ominoso es que se realiza este trabajo. Es también nuestro interés señalar que no debemos olvidar a la cavidad bucal en el examen dermatológico de rutina (AU)
Subject(s)
Humans , Dyskeratosis Congenita/diagnosis , Fox-Fordyce Disease/diagnosis , Leukoplakia, Hairy/diagnosis , Nevus/diagnosis , Mouth Mucosa/pathology , Glossitis, Benign Migratory/diagnosis , Candidiasis, Oral/diagnosis , Syphilis/diagnosis , Glossitis, Benign Migratory/pathology , Fox-Fordyce Disease/pathology , Candidiasis, Oral/pathology , Leukoplakia, Hairy/pathology , Syphilis/pathology , Measles/diagnosis , Measles/pathology , Condylomata Acuminata/diagnosis , Condylomata Acuminata/pathology , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/pathology , Stomatitis/diagnosis , Stomatitis/etiology , Psoriasis/diagnosis , Psoriasis/pathology , Focal Epithelial Hyperplasia/diagnosis , Focal Epithelial Hyperplasia/pathology , Leukoplakia, Oral/diagnosis , Leukoplakia, Oral/pathology , Papilloma/diagnosis , Papilloma/pathology , Mouth Neoplasms/diagnosis , Mouth Neoplasms/pathology , Leukoedema, Oral/diagnosis , Leukoedema, Oral/pathology , Mouth Diseases , Diagnosis, DifferentialABSTRACT
La patología blanca de la mucosa oral comprende una constelación de enfermedades cuyos cuadros clínicos se asemejan mucho entre sí y se caracterizan por la presentación de una lesión de aspecto blanquesino en la cavidad bucal. Con el fin de remarcar las diferencias entre cada afección, sus diversas evoluciones y pronósticos y alertar sobre aquellas con tendencia cancerígena y pronóstico más ominoso es que se realiza este trabajo. Es también nuestro interés señalar que no debemos olvidar a la cavidad bucal en el examen dermatológico de rutina
Subject(s)
Humans , Candidiasis, Oral/diagnosis , Dyskeratosis Congenita/diagnosis , Fox-Fordyce Disease/diagnosis , Glossitis, Benign Migratory/diagnosis , Leukoplakia, Hairy/diagnosis , Mouth Mucosa/pathology , Nevus/diagnosis , Syphilis/diagnosis , Candidiasis, Oral/pathology , Condylomata Acuminata/diagnosis , Condylomata Acuminata/pathology , Diagnosis, Differential , Focal Epithelial Hyperplasia/diagnosis , Focal Epithelial Hyperplasia/pathology , Fox-Fordyce Disease/pathology , Glossitis, Benign Migratory/pathology , Leukoedema, Oral/diagnosis , Leukoedema, Oral/pathology , Leukoplakia, Hairy/pathology , Leukoplakia, Oral/diagnosis , Leukoplakia, Oral/pathology , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/pathology , Mouth Diseases , Mouth Neoplasms/diagnosis , Mouth Neoplasms/pathology , Papilloma/diagnosis , Papilloma/pathology , Psoriasis/diagnosis , Psoriasis/pathology , Measles/diagnosis , Measles/pathology , Syphilis/pathology , Stomatitis/diagnosis , Stomatitis/etiologyABSTRACT
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.