Enfermedad de Fox-Fordyce en edad infantil / Fox-Fordyce Disease in a Pediatric Patient

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

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.

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.

Foxc1 Ablated Mice Are Anhidrotic and Recapitulate Features of Human Miliaria Sweat Retention Disorder.

Sweat glands are critical for thermoregulation. The single tubular structure of sweat glands has a lower secretory portion and an upper reabsorptive duct leading to the secretory pore in the skin. Genes that determine sweat gland structure and function are largely unidentified. Here we report that a Fox family transcription factor, Foxc1, is obligate for appreciable sweat duct activity in mice. When Foxc1 was specifically ablated in skin, sweat glands appeared mature, but the mice were severely hypohidrotic. Morphologic analysis revealed that sweat ducts were blocked by hyperkeratotic or parakeratotic plugs. Consequently, lumens in ducts and secretory portions were dilated, and blisters and papules formed on the skin surface in the knockout mice. The phenotype was strikingly similar to the human sweat retention disorder miliaria. We further show that Foxc1 deficiency ectopically induces the expression of keratinocyte terminal differentiation markers in the duct luminal cells, which most likely contribute to keratotic plug formation. Among those differentiation markers, we show that Sprr2a transcription is directly repressed by overexpressed Foxc1 in keratinocytes. In summary, Foxc1 regulates sweat duct luminal cell differentiation, and mutant mice mimic miliaria and provide a possible animal model for its study.

[UROLOGICAL ASPECTS OF THE FOX - FORDYCE DISEASE].

The article presents the description of the clinical observation of the patient 28 years old with multiple granulomatous eruptions on the skin of the scrotum, accompanied by itching and pain when walking. Surgical treatment consisted of excising the skin of the scrotum and substitution dermatoplasty was performed. Fox - Fordyce disease was confirmed by histological examination of tissue removed: advanced cystic sebaceous gland duct with calcifications in its lumen was revealed. The were no relapses during follow-up of 15 months.

Enfermedad de Fox-Fordyce con presentación clínica atípica / Fox-Fordyce Disease With an Atypical Clinical Presentation

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Fox-Fordyce-like disease following laser hair removal appearing on all treated areas.

Fox-Fordyce disease is an uncommon inflammatory disease of the apocrine sweat glands. Two recent reports indicated laser hair removal as a novel cause of axillary Fox-Fordyce disease. We report the first case of Fox-Fordyce disease developing in women after completing treatment with a depilatory hair laser appearing in the axillae, umbilicus, and pubis. We describe a case of Fox-Fordyce disease that developed in a 27-year-old woman 3 months after she had completed two LightSheer Diode laser treatments of her axilla, periumbilical region, and bikini area. Clinical and histopathological changes are as well detailed. Laser therapy induces damage to follicular infundibulum, resulting in altered maturation of keratinocytes which led to keratin plugging causing the common pathologic features in Fox-Fordyce disease. Differences in the physiologic features of the anatomic sites, in the susceptibility to laser-induced injury among these areas, or additional factors may contribute to Fox-Fordyce disease.

Histopathology attributes of Fox-Fordyce disease.

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.

Clinical effects of topical pimecrolimus in a patient with Fox-Fordyce disease.

Fox-Fordyce disease (FFD) is characterized by a pruritic eruption of skin-coloured or yellowish papules in areas rich in apocrine glands. The histology comprises dilatation of follicular infundibula with hyperkeratosis, acanthosis, and spongiosis of the infundibular epithelium with perifollicular infiltration of lymphocytes and foamy histiocytes. We treated a 12-year-old girl with FFD with topical pimecrolimus for 12 weeks, this resulted in a complete clearance of lesions. After the therapy, the patient was followed for an additional 19 months without signs of relapse. The effects of pimecrolimus in FFD might imply that an inflammatory process inducing secondary reactive hyperkeratosis could be involved in the pathogenesis of FFD.

Fox-Fordyce disease: response to adapalene 0.1%.

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.

Fox-Fordyce disease.

Fox-Fordyce disease (FFD) is a rare inflammatory disorder that affects the apocrine sweat glands. Clinically, lesions are equidistant, smooth, uniform, firm, folliculocentric papules, which can range in color from flesh-colored to red-brown to slightly yellow. Whereas the axillae are most commonly involved, FFD also can involve the anogenital and periareolar areas, lips, umbilicus, sternum, perineum, and upper medial aspects of the thighs. The underlying etiology of FFD remains unclear although epidemiologic data support a hormonal component because women are more commonly affected than men. Moreover, symptoms initially present after the onset of puberty, flare perimenstrually, and often resolve during pregnancy and after menopause. Histopathologic findings include the obstruction of the apocrine duct by a hyperkeratotic plug in the follicular infundibulum, which is believed to represent the primary pathophysiologic process; subsequent ductal rupture and resulting inflammatory response produce the typical clinical picture. Treatment of FFD is difficult because no one agent has proven particularly effective. Topical and interlesional glucocorticoids are often considered the first-line pharmacologic agents, although use is often limited by concerns for atrophy. Other agents that have shown some success include topical and systemic retinoids, topical clindamycin, topical pimecrolimus cream, benzoyl peroxide, and oral contraceptives. For medication-refractory cases, mechanical destruction or removal of the apocrine glands has been efficacious in small case series.