Fox Fordyce disease: a side effect of laser therapy.

Fox-Fordyce disease (FFD) is an uncommon apocrine disorder. We reported a 26-year-old woman with bilateral axillary pruritic papular lesions following 2 sessions of treatment with Diode (800 nm) laser for axillary hair removal. No lesions were developed in the other treated areas. Topical 0.1% tacrolimus gel was used for her treatment.

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.

Inflammatory and glandular skin disease in pregnancy.

A switch from cell-mediated to humoral immunity (helper T 1 [Th1] to helper T 2 [Th2] shift) during gestation plays a key role in placental immune tolerance. As a result, skin diseases that are Th2 mediated often worsen, whereas skin diseases that are Th1 mediated often improve during gestation. Also, due to fluctuations in glandular activity, skin diseases involving sebaceous and eccrine glands may flare, whereas those involving apocrine glands may improve during pregnancy. Despite these trends, inflammatory and glandular skin diseases do not always follow the predicted pattern, and courses are often diverse. We review the gestational course of inflammatory skin diseases, such as atopic dermatitis (atopic eruption of pregnancy), psoriasis, impetigo herpetiformis, urticaria, erythema annulare centrifugum, pityriasis rosea, sarcoidosis, Sweet syndrome, and erythema nodosum, as well as glandular skin diseases, including acne vulgaris, acne rosacea, perioral dermatitis, hidradenitis suppurativa, Fox-Fordyce disease, hyperhidrosis, and miliaria. For each of these diseases, we discuss the pathogenesis, clinical presentation, and management with special consideration for maternal and fetal safety.

Fox-Fordyce Disease: An under-diagnosed adverse event of laser hair removal?

BACKGROUND: Fox-Fordyce Disease (FFD) is a rare chronic inflammatory skin disease of the apocrine glands, mainly affecting post-pubertal women. It involves apocrine gland-bearing areas including the axilla, areola, anogenital area and umbilicus. FFD induced by laser hair removal is a newly reported entity of unknown pathogenesis. To the best of our knowledge, there are only four reported cases in the literature describing a total of four patients. OBJECTIVES: We are reporting the first case series of laser-induced FFD with a histopathological and clinical evaluation of the reported cases. METHODS: A review of the available literature to date about laser-induced FFD was performed. Clinical and histopathological features were reviewed. RESULTS: In our case series, the clinical and histological appearance of FFD following laser hair removal is similar to that of classic FFD. Exacerbating factors were present in two patients, and were similar to that of classic FFD. The main histopathological features included dilatation of the follicular infundibulum, follicular hyperkeratosis, dyskeratosis in the follicular infundibulum, periductal lymphohistiocytic infiltrate and perifollicular fibrosis. Two of the four reported cases as well as one of our patients had no perifollicular xanthomatous inflammation, a hallmark feature of classic FFD. Mast cells were absent in all reported cases, as well as in our patients. CONCLUSION: We believe that laser-induced FFD is under-diagnosed as a newly reported adverse event of laser hair removal. It is clinically and histopathologically similar to classic FFD.

Successful treatment of areolar Fox-Fordyce disease with surgical excision and 1550-nm fractionated erbium glass laser.

Fox-Fordyce disease (FFD) is a rare chronic disorder characterised by persistent inflammation because of the obstruction of apocrine sweat glands, which is a key factor of pathogenesis. The treatment of FFD is known to be difficult, and the modalities of treatment have not yet been widely studied. We report the successful treatment of a case of bilateral areolar FFD by a combination of surgical excision and 1550-nm fractionated erbium glass laser in an 18-year-old woman. The patient presented with a bilateral areolar eruption of multiple, severely pruritic, 3-4 mm skin- to grey-coloured folliculocentric dome-shaped papules. The initial treatment plan was for bilateral surgical excision of the larger and more highly elevated papules via circumferential dermal excision, which was intended to maintain the areolar contour and minimise distortion. A 1550-nm fractional erbium glass laser was then used to control the remnant lesions. The patient was recurrence-free at 14 months after the final laser treatment, and she was fully satisfied with the treatment results.

[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.

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.

Angioqueratomas: parte 1: [revisión] / Angiokeratoma: part 1: [review]

Los angioqueratomas son lesiones vasculares relativamente infrecuentes que consisten en pápulas hiperqueratósicas rojo-violáceas. Éstas pueden ser únicas o múltiples, estar localizadas en un solo segmento corporal o ser generalizadas y estar o no asociadas a otras enfermedades subyacentes. El presente trabajo abordará en profundidad los angioqueratomas localizados y se complementará con una segunda parte en la que se tratarán los angioqueratomas generalizados.

Angiokeratomas are a relatively non-frequent group of vascular lesions that consist on hyperkeratotic red-violaceous papules. Lesions can be solitary or multiple, localized or generalized and may be associated or not with a systemic disease. The present work is a throughout review on localized angiokeratomas and will be complemented with a second part in which generalized angiokeratomas will be discussed.

Clinicopathological study of Fox-Fordyce disease.

Fox-Fordyce disease (FFD) is a rare skin disease manifesting as multiple pruritic follicular papules involving the skin-bearing apocrine glands. Reports of FFD in Asian people are scant. In this retrospective study, we describe the clinicopathological findings of five cases of FFD affecting Taiwanese subjects. Clinically, all patients presented with numerous uniform, 2-3-mm, skin-colored to light brown, dome-shaped papules with smooth surface, which were distributed in the apocrine gland-containing areas. Pruritus varied from mild to severe. The histopathology is characterized by focal spongiosis in the upper infundibulum with perifollicular fibrosis and lymphohistiocytic infiltrate. FFD needs to be differentiated from lichen amyloidosis, Darier's disease, syringoma, lichen simplex chronicus and spongiotic dermatitis clinically or pathologically. The findings of focal spongiosis in upper infundibulum associated with a perifollicular lymphohistiocytic infiltrate can facilitate the diagnosis of FFD.

Dilation of apocrine glands. A forgotten but helpful histopathological clue to the diagnosis of axillary Fox-Fordyce disease.

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.

Perifollicular xanthomatosis as the hallmark of axillary Fox-Fordyce disease: an evaluation of histopathologic features of 7 cases.

BACKGROUND: Fox-Fordyce disease (FFD) or apocrine miliaria is a rare condition with features that are characteristic clinically but not histopathologically. It is traditionally described as a condition that shows infundibular plugging, acanthosis, parakeratosis, spongiosis, and a nonspecific infiltrate. The so-called retention vesicle, which reputedly involves the apocrine duct, is often difficult to find. Recently, 4 uncontrolled observations were described (infundibular dyskeratotic cells, vacuolar alteration, cornoid lamella-like parakeratosis, and perifollicular foamy macrophages). In this study, we evaluated both established and new histopathologic criteria for the diagnosis of FFD and searched for other meaningful findings. OBSERVATIONS: Most established features were observed in both patients with FFD and control patients. All cases occurred during 1995 through 2005. No unequivocal retention vesicle was identifiable in any case. Infundibular vacuolar change and cornoid lamella-like parakeratosis were not corroborated as being diagnostically meaningful. Few dyskeratotic cells were seen in some patients with FFD and in control patients. Perifollicular foam cells were noted in most patients with FFD but not among control patients. These cells expressed CD68 but lacked expression of carcinoembryonic antigen, gross cystic disease fluid protein 15, and periodic acid-Schiff with diastase digestion. Perifollicular mucin, fibrosis, and mast cells in the infiltrate were also observed. CONCLUSIONS: The established histopathologic attributes of FFD are nonspecific, and a retention vesicle is difficult to find even in level sections. In contrast, perifollicular foam cells are a distinct, relatively consistent, and specific feature of FFD. We contend that perifollicular foam cells represent a useful hallmark of FFD.

[Perifollicular xanthomatosis as a key histological finding in Fox-Fordyce disease]. / Xantomatosis perifolicular: hallazgo histológico clave en la enfermedad de Fox-Fordyce.

Fox-Fordyce disease is a rare skin condition characterized by the presence of multiple pruritic follicular papules in areas rich in apocrine glands, such as the axillae, mammary areolae, or genital regions. There is a high degree of variability in the histological findings seen in Fox-Fordyce disease. In addition to those described as typical of this entity, such as dilation of the infundibulum and hyperkeratosis and spongiosis of the infundibular epithelium, many other histological changes can be observed. We report the case of a 21-year-old woman with Fox-Fordyce disease and highlight the importance of perifollicular xanthomatosis as a key histological finding in the diagnosis of the disease.