Enfermedad de Fox-Fordyce: reporte de un caso / Fox-Fordyce disease: a case report

Resumen La enfermedad de Fox-Fordyce (EFF) es un trastorno inflamatorio crónicopoco frecuente de las glándulas apocrinas. Afecta comúnmente a mujeres entre los 13-35 años. Su etiopatogenia es multifactorial. Se caracteriza por pápulas foliculares pruriginosas dispuestas en zonas que contienen las glándulas mencionadas. Se presenta el caso clínico de una paciente de 30 años, con pápulas foliculares en axilas compatibles con enfermedad de Fox-Fordyce. El propósito deeste trabajo es presentar una entidad infrecuente y brindar una revisión bibliográfica repasando los diagnósticos diferenciales y las opciones terapéuticas disponibles.

Abstract Fox-Fordyce disease (FFD) is a rare chronic inflammatory disorder of the apocrine glands. It commonly affects women between 13-35 years. Its etiopathogenesis is multifactorial. This disorder is characterized by pruritic follicular papules arranged in areas containing the aforementioned glands. We reporta clinical case of a 30-year-old female patient, with follicular papules on armpits compatible with Fox-Fordyce disease. The purpose of this paper is to report a uncommon entity while providing a bibliographical revision highlighting the differential diagnoses and their available therapeutic options.

Nevus apocrino puro: una entidad raramente sospechada / Pure apocrine nevus: a rarely suspected entity

Los nevus apocrinos puros son hamartomas de las unidades pilosebáceas caracterizadas por proliferaciones benignas de glándulas apocrinas maduras, la cual es una descripción microscópica realizada en los reportes de patología sin que se nombre el diagnóstico exacto. Considerando además, los diagnósticos diferenciales clínicos y la baja frecuencia de este diagnóstico, presentamos un caso clínico y una revisión del tema

Pure apocrine nevi are hamartomas of the pilosebaceous units characterized by benign proliferations of mature apocrine glands, which is a microscopic description made in pathology reports without the exact diagnosis being named. Considering the clinical differential diagnoses and its low frequency, we present a case report and a review of the literature on this topic

¿Carcinoma de mama o carcinoma de glándula sudorípara? Presentación de dos casos y análisis de la literatura / Breast carcinoma or sweat gland carcinoma? A report of two cases and a comparison with the literature

Resumen El carcinoma apocrino primario de glándula sudorípara es una neoplasia con una muy baja incidencia, que puede representar un reto diagnóstico, clínico e histológico, y un reto terapéutico local, adyuvante y de la enfermedad avanzada. La edad media de los pacientes es de alrededor de 67 años, y no se ha observado preferencia según el sexo. Se presenta con mayor frecuencia en las axilas y en el cuero cabelludo. Se caracteriza clínicamente por un lento crecimiento, aunque puede progresar agresivamente, con compromiso local, ganglionar y metastásico, principalmente, pulmonar, hepático y óseo. El tratamiento recomendado -una vez establecida la histología- consiste en una resección local amplia con un margen claro de 1 a 2 cm y linfadenectomía regional si se detectan ganglios clínicamente positivos. El tratamiento adyuvante (radioterapia o quimioterapia) y de la enfermedad avanzada no está claramente establecido. Se presentan dos pacientes de sexo femenino con sospecha inicial de cáncer de mama, en quienes se diagnosticó finalmente un carcinoma apocrino de glándula sudorípara.

Abstract Primary apocrine carcinoma of the sweat gland is a neoplasm with a very low incidence that may represent a clinical and histological diagnostic challenge, as well as for adequate local, adjuvant, and advanced disease management. The average age of patients is around 67 years with no gender preference. This cancer develops primarily at the axillary and scalp levels and is clinically characterized by slow growth, but can progress aggressively with local, nodal, and metastatic involvement (primarily lung, liver, and bone). The recommended management, once the histology is established, consists of a wide local resection with a clear margin of 1 to 2 cm and regional lymphadenectomy if clinically positive nodes are detected. The adjuvant treatment (radiotherapy or chemotherapy) and for the advanced disease is not established. We report here the cases of two female patients initially diagnosed with breast cancer who were finally diagnosed with apocrine carcinoma of the sweat gland.

Apocrine Hidrocystoma on the Penis: Report of a Case and Review of the Previous Cases

Apocrine hidrocystoma, also called apocrine cystadenoma, is a benign cystic tumor-like lesion that arises from the proliferation of apocrine glands. Clinically, it usually occurs singly as a unilocular or multilocular, dome-shaped translucent cyst. Histologically, it appears as unilocular or multilocular cysts composed of an inner layer of single or double layer of secretory columnar epithelium with decapitation secretion lying above an outer myoepithelial cell layer. Apocrine hidrocystomas mostly occur within the head and neck region and involvement of genitalia is extremely rare. This paper emphasizes the importance of considering the differential diagnosis of a genital cystic lesion. Herein, we report a case of apocrine hidrocystoma occurring in the penis and compare the clinicopathological characteristics of apocrine hidrocystoma in genitalia with the previous cases.

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.

Combination of topical agents and oxybutynin as a therapeutic modality for patients with both osmidrosis and hyperhidrosis / Uso combinado de agentes tópicos e oxibutinina para tratamento de pacientes com osmidrose e hiper-hidrose

Summary Introduction: The association of osmidrosis and hyperhidrosis often causes emotional and social problems that may impair the patients' quality of life. The purpose of our study was to analyze the therapeutic results of oxybutynin and topical agents in 89 patients with both osmidrosis and hyperhidrosis. Method: We conducted an observational study at two specialized centers of hyperhidrosis between April 2007 and August 2013. Eighty-nine (89) patients with both osmidrosis and hyperhidrosis were treated with oxybutynin and topical agents. Patients were evaluated before treatment and at 3 and 6 weeks after treatment started, by using the Quality of Life Questionnaire and the Sweating Evolution Scale. Results: Before treatment, 98% of the patients presented with poor or very poor quality of life. After six weeks of treatment, 70% stated their quality of life as being slightly better or much better (p<0.001) and nearly 70% of the patients experienced a moderate or great improvement in sweating and malodor. Improvement in osmidrosis was significantly greater when the axillary region was the first most disturbing site of hyperhidrosis. Conclusion: There was a significant improvement in quality of life and a reduction in sweating and malodor after six weeks of treatment with topical agents and oxybutynin in patients with both hyperhidrosis and osmidrosis. Therefore, clinical treatment should be considered before invasive techniques.

Resumo Introdução: A associação entre osmidrose e hiper-hidrose com frequência causa problemas emocionais e sociais que podem deteriorar a qualidade de vida dos pacientes. O objetivo deste estudo foi analisar os resultados terapêuticos do uso de oxibutinina associada a agentes tópicos em 89 pacientes com osmidrose e hiper-hidrose. Método: Nós conduzimos um estudo observacional em dois centros especializados em hiper-hidrose entre abril de 2007 e agosto de 2013. Oitenta e nove (89) pacientes com osmidrose associada a hiper-hidrose foram tratados com oxibutinina e agentes tópicos. Os pacientes foram avaliados antes do tratamento e após 3 e 6 semanas do início do tratamento, por meio do Questionário de Qualidade de Vida e da Escala de Evolução da Sudorese. Resultados: Antes do tratamento, 98% dos pacientes apresentavam qualidade de vida ruim ou muito ruim. Após seis semanas de tratamento, 70% classificou sua qualidade de vida como sendo pouco ou muito melhor (p<0.001) e aproximadamente 70% dos pacientes relataram melhora moderada ou grande de sudorese e odor. Houve melhora significativamente maior da osmidrose quando a região axilar era o sítio em que a hiper-hidrose mais incomodava. Conclusão: Houve melhora significativa da qualidade de vida e uma redução da sudorese e do odor após seis semanas de tratamento com agentes tópicos e oxibutinina em pacientes com hiper-hidrose associada a osmidrose. Dessa maneira, a terapia clínica deve ser considerada antes de técnicas invasivas.

Apocrine Encapsulated Papillary Carcinoma of the Breast: The First Reported Case with an Infiltrative Component / 한국유방암학회지

Apocrine encapsulated papillary carcinoma (EPC) of the breast is a rare neoplasm, and only 10 cases have been reported in the literature to date. Although EPC by definition lacks a peripheral myoepithelial layer, all previously published apocrine EPC cases were clinically indolent and lacked a conventional invasive component. Herein, we report the 11th case of apocrine EPC, which had a conventional invasive carcinoma component and provides evidence of the malignant potential of this entity. We postulate that apocrine EPC is most likely a morphological variant of conventional EPC, with the same unpredictable malignant potential as non-apocrine cases.

Enfermedad de paget extramamaria: A propósito de un caso / Extramammary paget´s disease: A case report

La enfermedad de Paget extramamaria es un adenocarcinoma intraepitelial, que ocurre en sitios ricos en glándulas apocrinas, como: vulva, axila, región anogenital y escroto en el varón. Presentamos el caso de una paciente, con una lesión eritemato-descamativa en región perianal, de seis meses de evolución, con buena respuesta al tratamiento y favorable evolución.

Extramammry Paget's disease is an intraepithelial adenocarcinoma that occurs in areas rich apocrine glands, such as the: vulva, axilla, anogenital area and scrotum in man. We present the clinical case in a patient with an erythematous exudative lesión in perineal area over the last six months with good response to treatment. The patient evolved favorably.

Syringocystadenoma papilliferum in an unusual location

Abstract: Syringocystadenoma papilliferum is a rare benign hamartomatous adnexal tumor of the apocrine or eccrine sweat glands. Most patients present a solitary lesion in the head and neck region. Presentation outside the head and neck region is even more uncommon. We present a case of Syringocystadenoma papilliferum with papulonodular lesion located on the vulva of an infant girl. This case illustrates the atypical location of this rare disease and adds to the differential diagnosis of lesions on the vulva.

Large cutaneous apocrine carcinoma occurring on right thigh aggravated after moxa treatment

Primary cutaneous apocrine carcinoma is a rare adnexal tumor of the skin that occurs mainly in the axilla, anogenital area while the scalp and the lower extremities, especially the thigh, are very unusual sites. However, clinical or pathologic characteristics have not been well established due to a paucity of this tumor. Herein, we report very unusual case of apocrine carcinoma present as a huge mass in the lateral thigh of 77-year-old woman, which was aggravated abruptly after an irritation by moxa treatment, with a brief review of the literature.

Distribution of apocrine sweat glands in axillary region of patients with axillary osmidrosis / 中华皮肤科杂志

Objective To determine the distribution profile of apocrine sweat glands in axillary region of patients with axillary osmidrosis,and to compare their distribution at different sites.Methods Fifteen patients with axillary osmidrosis were enrolled in this study from September to December 2010.All the patients underwent surgical removal of apocrine sweat glands under direct vision.Full-thickness skin tissue measuring 2 mm in width was excised down to the axillary superficial fascia at the incisional surgical sites from five patients.Five points,which were at the center of axillary region (point 1),1 cm away from the center of axillary region (point 2),1 cm inside the edge of axillary hair-bearing area (point 3),the edge of axillary hair-bearing area (point 4),and 1 cm outside the edge of axillary hair-bearing area (point 5),were marked,and dark red,rough granular subcutaneous tissue was obtained at these points in 10 patients with axillary osmidrosis.Results The secretory portion of apocrine sweat glands was mainly distributed in the reticular dennis and superficial subcutaneous adipose tissue,but no apocrine sweat glands were obs erred in the epidermis,dermal papilla or axillary superficial fascia.The distribution profile of apocrine sweat glands was consistent with that of axillary hairs.There were numerous apocrine sweat glands in the center of axillary region,but only a small number at the edge of axillary hair-bearing area,and no apocrine sweat gland was observed at 1 cm outside the edge.The percentage of apocrine sweat gland area at point 1-5 was 74.1％,46.6％,25.3％,2.1％,and 0 respectively,with significant differences between point 1 and 2 (t--29.78,P＜ 0.01),point 2 and 3 (t--9.76,P＜ 0.01),point 3 and 4 (t =20.83,P＜ 0.01),but not between point 4 and 5 (t =1.96,P ＞ 0.05).Conclusions During the surgical treatment of axillary osmidrosis,the removal of apocrine sweat glands should be extended to the reticular dennis and superficial subcutaneous adipose tissue in depth and the edge of axillary hair-bearing area in width,and there is no need to blindly increase the extent of removal.

A Case of Tubular Adenoma in the External Auditory Canal / 대한이비인후과학회지

Tumors occurring in the external auditory canal (EAC) are rare. In particular, tubular adenoma (TA) is an extremely rare sweat gland neoplasm occurring in the EAC and is generally considered as benign. Up to now, less than one hundred cases of TA have been reported in the English literature. In fact, a comprehensive review of literature shows that TA in the EAC has seldom been reported. Herein, we report a case of a 35-year-old man with TA that occured in the EAC, together with characteristic histopathological findings.

A Case of Tubular Apocrine Adenoma with Syringocystadenoma Papilliferum of External Auditory Canal Removed by Postauricular Approach / 대한이비인후과학회지

Tubular apocrine adenoma (TAA) and syringocystadenoma papilliferum (SCAP) are uncommon cutaneous adnexal neoplasms often found in the head, perianal area, but rarely in external auditory canal (EAC). SCAP in EAC generally presents itself with a mass in the EAC along with conductive hearing loss. We present here a case of a 50-year-old man presenting TAA with SCAP confined to EAC, which was surgically removed. The clinical and histopathological features were described. The patient has been well without relapse or metastasis.

Comparison of efficacy and complications among three small incision procedures in treatment of axillary osmidrosis / 中华医学美学美容杂志

Objective To treat axillary osmidrosis by using three procedures (small incision apocrine curettage,cut-off operation and aspiration surgery),to observe the postoperative complications,efficacy and the pathological changes of skin,and to explore the advantages of these three procedures.Methods Seventy-five cases of evere bilateral axillary osmidrosis were involved in this study,totally 150 axillary surgery area,which was randomly divided into different group:Group A (curettage group); Group B (cut-off group) and Group C (aspiration group).Different surgical treatment was conducted in the three groups of operation area.Results Incidence of complications was 28.30 ％ and total effective rate was 94.34 ％ in Group A; in Group B,incidence of complications was 6.38 ％,and total effective rate was 97.87 ％ ; in Group C,incidence of complications was 4.00 ％ and total effective rate was 78.00 ％.The incidence of complications was significantly different between Groups A and B and between Groups A and C,but not significant difference between Groups B and C.The cure rate and efficiency were not significant difference between Groups A and B groups,but significant difference was found between Groups A and C and Groups B and C.HE staining results showed that the dermal tissues were partially destroyed in Group A and B,and no apocrine sweat glands were not seen within the dermis and subcutaneous tissues; in Group C,the dermal tissue was rarely destroyed,and a lot of apocrine sweat glands were reserved within the dermis and subcutaneous tissues.Conclusions Efficacy of curettage and cut-off procedures is better,but that of aspiration is worse in the treatment of axillary asmidrosis.The complications of cut-off surgery and aspiration are less,but urettage has more complications.Therefore,the efficacy of cut-off operation is the best with least complications among the three procedures.

Minimal Subdermal Shaving by Means of Sclerotherapy Using Absolute Ethanol: A New Method for the Treatment of Axillary Osmidrosis

BACKGROUND: Axillary osmidrosis is characterized by unpleasant odors originating from the axillary apocrine glands, resulting in psychosocial stress. The main treatment modality is apocrine gland removal. Until now, of the various surgical techniques have sometimes caused serious complications. We describe herein the favorable outcomes of a new method for ablating apocrine glands by minimal subdermal shaving using sclerotherapy with absolute ethanol. METHODS: A total of 12 patients underwent the procedure. The severity of osmidrosis was evaluated before surgery. Conventional subdermal shaving was performed on one side (control group) and ablation by means of minimal subdermal shaving and absolute ethanol on the other side (study group). Postoperative outcomes were compared between the study and control groups. RESULTS: The length of time to removal of the drain was 1 day shorter in the study group than in the control group. There were no serious complications, such as hematoma or seroma, in either group, but flap margin necrosis and flap desquamation occurred in the control group, and were successfully managed with conservative treatment. Six months after surgery, we and our patients were satisfied with the outcomes. CONCLUSIONS: Sclerotherapy using absolute ethanol combined with minimal subdermal shaving may be useful for the treatment of axillary osmidrosis. It can reduce the incidence of seroma and hematoma and allow the skin flap to adhere to its recipient site. It can degrade and ablate the remaining apocrine glands and eliminate causative organisms. Furthermore, since this technique is relatively simple, it takes less time than the conventional method.

Axillary Keloid Formation after Osmidrosis Surgery

Keloid is a scar tissue that undergoes recovery and excessive growth over the original wounds when the collagen is excessively accumulated in the dermis during the wound healing process. The common sites of keloid occurrence include the anterior thorax, shoulder, upper extremities and ear. To our knowledge, however, there are few cases of keloid that occurs in the axilla. In addition, there are fewer cases of keloid that bilaterally occurs at the operated sites postoperatively in individuals with no past or family history. 21-year-old male had undergone subdermal shaving for the management of osmidrosis but had not received appropriate scar management during the military service. He visited us with a keloid-like scar formed in the bilateral axillae. We excised the scar tissue and treated it with local steroid injection and postoperative axilla compression. In histopathological report, no flattening of the overlying epidermis, and presence of keloid collagen which confirms diagnosis of keloid. We report our clinical experience with a rare case of keloid occurring in the axilla and treatment process.

Retalho fasciocutâneo toracodorsal no tratamento cirúrgico da hidradenite supurativa: relato de caso e revisão de literatura / Thoracodorsal fasciocutaneous flap in the treatment of hidradenitis suppurativa: a case report and literature review

A hidradenite supurativa, também conhecida como doença de Verneuil, é uma inflamação crônica e recorrente das glândulas apócrinas, sem etiologia conhecida, que evolui frequentemente com saída de secreção purulenta, formação de fístulas, cicatrizes inestéticas e retráteis, podendo acometer região inguinal, axilar, aréolas mamárias e umbigo. Neste trabalho, é apresentado um caso grave de doença de Verneuil em região axilar, em que foi realizada exérese da lesão e reconstrução com retalho fasciocutâneo toracodorsal bilateral como uma opção de reconstrução da região axilar, com boa resolubilidade e resultado estético.

Hidradenitis suppurativa also known as Verneuil's disease is a chronic inflammatory and recurrent disease that occurs in the apocrine glands. It results in recurrent abscesses with purulent secretion, fistulae formation, unaesthetic and retractable scars. The most common sites involved include the axilla, inguinoperineal regions, nipple-areolar complex, and umbilicus. Here, we report a case of severe Verneuil's disease of the axillary region managed with wide excision surgery, and thoracodorsal fasciocutaneous flap and bilateral coverage. This method provides good results for reconstruction of the axillary region.

Carcinoma apócrino de mama: relato de caso e revisão de literatura / Apocrine breast carcinoma: a case report and literature review

O carcinoma apócrino de mama é raro e, por esse motivo, permanece com origem e definição controversas. Há quem acredite que se trate de uma variante do carcinoma invasivo. Apresenta-se em cerca de 0,5 a 8% dos casos de carcinoma mamário, não sendo possível sua diferenciação em relação aos carcinomas usuais da mama por meio de exames radiológicos. O diagnóstico pode ser realizado pela citologia, que é típica desse tumor. Geralmente é positivo para a GCDFP-15(gross cystic disease fluid protein-15), marcador de diferenciação apócrina mais confiável do que amorfologia. A imunoistoquímica costuma ser negativa para estrógeno e progesterona e positiva para o receptor de andrógeno. A taxa de positividade para a p53, HER-2 e BCL-2 em carcinomas apócrinos é quase a mesma que para os carcinomas não apócrinos. O tratamento é cirúrgico; a adjuvância costuma ser a mesma do carcinoma ductal invasor e, ao que tudo indica, também tem o mesmo prognóstico.

Apocrine carcinoma of the breast is a rare condition, and for this reason its origin and definition remains controversial. Some believe that is a variant of invasive carcinoma. The incidence of this disease is about 0.5 to 8% of the new diagnosed cases of breast carcinoma. Since it is not possible to differentiate the apocrine carcinoma from the usual breast carcinomas by using radiological images, diagnosis can be made by cytology showing typical alterations of this tumor. It is usually positive for GCDFP-15 (gross cystic disease fluid protein-15), a marker of apocrine differentiation more reliable than morphology. This type of tumor is often negative for estrogen and progesterone and positive for the androgen receptor. The rate of positivity for p53, HER-2 and BCL-2 in apocrine carcinomas is almost the same as for non-apocrine carcinomas. The surgical and adjuvant treatment is normally the same as invasive ductal carcinoma, and both seem also to have the same prognosis.

Apocrine Carcinoma of the Axilla with Predominant Signet Ring Cell Features A Case Report

Apocrine carcinoma arising from the apocrine sweat glands is a rare cutaneous malignant tumor which occurs predominantly in the axilla of elderly individuals. The typical histologic features of apocrine carcinoma is within a well developed glandular lumina with abundant eosinophilic cytoplasm and evidence of decapitation secretion. In rare instances, predominant signet ring cell features in apocrine carcinoma has been reported. We experienced a case that occured in the right axilla of a 59-year-old. Histopathologic examination showed a solid tumor that extended from the upper dermis into the subcutis, with a delicate infiltrate of epithelial cells. The cells had granular amphophilic cytoplasm, predominantly showed distinct signet ring cell morphology, and were strongly positive for epithelial mucin. Both lysozyme and gross cystic disease fluid protein-15 were identified in the tumor cells. We diagnosed this to be a case of primary signet ring cell apocrine carcinoma of the axilla after several immunohistochemical and clinical evaluations.

Hidrocistomas múltiplos apócrinos: um diagnóstico clínico confuso / Multiple apocrine hidrocystomas: a confusing clinical diagnosis

This is a case report followed by a short review of literature of apocrine hidrocystomas. A 67 year old lady came to this clinic with multiple translucent, asymptomatic papulocystic lesions on the face which got worse in summer. A biopsy showed a single cystic cavity lined by two layers of small cuboial epithelial cells was seen. Apocrine differentiation characterised by decapitation secretion was seen. A diagnosis of multiple apocrine hidrocystomas was made. Treatment can be attempted with individual excision of lesions or lasers. Topical atropine and scopolamine too have been tried with variable success. Our patient did not wish any treatment once the benign nature of the condition was confirmed.

Este é um relato de caso seguido por uma breve revisão da literatura sobre hidrocistomas apócrinos. Uma senhora de 67 anos de idade foi à clínica anteriormente citada apresentando lesões papulocísticas múltiplas, translúcidas e assintomáticas na face que pioravam durante o verão. Uma biopsia mostrou uma cavidade cística única contornada por duas camadas de pequenas células epiteliais cubóides. Diferenciação apócrina caracterizada por remoção de secreção foi observada. Foi feito um diagnóstico de hidrocistomas múltiplos apócrinos. Tratamento pode ser tentado com excisão individual de lesões ou laser. Atropina tópica e escopolamina também foram tentadas com sucesso variável. Nossa paciente não quis nenhum tratamento uma vez que a natureza benigna da condição foi confirmada.