Eccrine Angiomatous Hamartoma with Atypical Localization Treated by Mohs Micrographic Surgery.

ABSTRACT: Eccrine angiomatous hamartoma (EAH) is a rare hamartoma characterized by a benign proliferation of eccrine glands and vascular structures in the dermis. These tumors rarely regress spontaneously, so surgical excision of the involved tissue is required when pain or enlargement occurs. Here, the authors report the clinical case of a patient affected by an extremely painful EAH with the atypical localization at the last phalanx of the thumb of the right hand with involvement of nail matrix and nail bed. This report aims to emphasize the application of Mohs micrographic surgery for the treatment of painful EAH in a very difficult area at potential risk of amputation while preserving the maximum anatomical integrity and function of the damaged area. These results can pave the way for the use of Mohs micrographic surgery for very carefully selected benign neoplasms when their surgical removal is required.

A possible role for second-hit postzygotic GJB2 mutation in porokeratotic eccrine ostial and dermal duct nevus.

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare type of epidermal nevus involving the eccrine acrosyringia. It typically presents as asymptomatic linear keratotic papules and plaques along the lines of Blaschko and predominantly affects the extremities. This disease has recently been linked to somatic mutations within the GJB2 locus. Only four GJB2 mutations have been previously documented for PEODDN, and the underlying genetic basis remains inconclusive. Herein, we report an 18-year-old female with a hyperkeratotic plaque on the dorsa of the proximal interphalangeal joint of her right ring finger, as well as multiple small hyperkeratotic papules linearly distributed on the lateral sides of her fingers occurring since birth. Histopathological results revealed prominent parakeratotic cornoid lamella-like tiers at the opening of the eccrine secretory ducts. Whole-exome sequencing of the affected skin tissue revealed a heterozygous germline mutation and a postzygotic somatic mutation in GJB2. In summary, this study presents a case of PEODDN with compound heterozygous mutations in GJB2, which broadens the genetic spectrum of this disease entity and implies a possible role for second-hit mutations in the pathogenesis of PEODDN.

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

Pigmented apocrine hamartoma: A distinct and uncommon pigmented lesion with dendritic melanocytes and appraisal of terminology.

A 20-year-old female presented to a gynecologist with an irregular, darkly pigmented, vulvar lesion. Histopathologic sections of a biopsy specimen showed cystically dilated glands with apical snouts, pigmented secretion, and numerous dendritic melanocytes. The lesion was diagnosed as a pigmented apocrine hamartoma of the vulva. We report the fifth case of this uncommonly encountered entity and discuss the conflicting terminology in the literature of this rare, pigmented lesion.

Eccrine angiomatous hamartoma: First case in the cytology literature.

A 34-year-old male presented with a swelling on the volar surface of the third digit of his right hand. This swelling was associated with pain and erythema. Ultrasound-guided needle biopsy was performed. Cytologic and histologic preparations together confirmed the diagnosis of a rarely encountered mixed epithelial and mesenchymal proliferation, an eccrine angiomatous hamartoma. To our knowledge, this case is the first to illustrate the cytomorphologic features of this rare lesion.

Sweating Blood: A Case Series of 2 Siblings With Hematohidrosis.

Hematohidrosis is a condition that presents with the excretion of blood from intact skin. Reported cases suggest emotional stress reactions as the most common inciting events. The pathogenesis of the condition is not well understood. We describe a 9-year old boy and his 6-month old half-sister with a history of bleeding episodes from the ears, eyes, and scalp, as well as other sites. Symptoms in both children have shown a positive response to propranolol, with decreased frequency and severity of bleeding. There are no prior reports of siblings with hematohidrosis, suggesting a possible genetic predisposition.

Digital immunohistological dissection of immune privilege collapse in syringotropic autoimmune diseases: Implication for the pathogenesis.

BACKGROUND: Syringotropic cell infiltration is a histological hallmark of some autoimmune diseases. However, its underlying mechanism remains unclear. OBJECTIVES: To assess the immune privilege (IP) of the human sweat gland (SwG) in homeostasis and in syringotropic autoimmune diseases. METHODS: We combined quantitative digital image microdissection with immunohistochemisty to analyze IP molecule expression in SwG of normal and diseased skin. The human skin organ culture model was used to examine the influence of proinflammatory conditions on IP in SwG. RESULTS: In the normal subjects (n = 10), major histocompatibility complex (MHC) class І expression was significantly reduced in SwGs compared to the epidermis. In contrast, IP-guardians, macrophage migration inhibitory factor (MIF) and alpha-melanocyte stimulating hormone (α-MSH) were upregulated in SwGs. MHC class І was upregulated in whole SwGs in lupus erythematosus (LE; n = 7) and scleroderma/morphea (Scl; n = 9), whereas differential expression was noted only in the secretory portion in Sjögren's syndrome (SjS) (n = 4). MIF expression level inversely correlated with that of MHC class I in all samples tested, and downregulation of α-MSH was detected in LE SwGs alone. The severity of inflammatory changes and MIF and ⍺-MSH expression were inversely correlated in LE. CD200 expression was decreased exclusively in atrophic stage of Scl. In a human skin organ culture model, intratissue injection of interferon-gamma up-regulated MHC class I and downregulated MIF and α-MSH. CONCLUSIONS: These findings indicate that SwGs enjoy IP. Dysregulated IP molecule expression may lead to SwG IP collapse and contribute to distinct inflammatory cell distribution in syringotropic autoimmune disorders.

Adult-onset porokeratotic eccrine ostial and dermal duct nevus:dermatoscopic findings and treatment with tazarotene.

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare dermatosis initially described as 'comedo nevus' and renamed 'PEODDN'; it has also been referred to as linear eccrine nevus with comedones, porokeratotic eccrine ostial and hair follicle nevus, and porokeratotic adnexal ostial nevus. PEODDN is usually present at birth or develops early in life. Rarely, PEODDN can develop in adults. The treatment of this puzzling condition is not standardized. We report herein a new case of adultonset PEODDN with dermatoscopic images. Our patient responded favorably to topical tazarotene.

Cutaneous apocrine cystomatosis in three slaughter-aged pigs.

Apocrine cystomatosis is a rare condition characterized by clusters of cystically dilated sweat glands or other specialized apocrine glands. Cystic dilation of cutaneous sweat glands has been described in humans, dogs, and cats, but not in pigs, to our knowledge. We describe herein linear, brown, colloid-filled cavities < 1 cm diameter within the subcutaneous fat of the entire dorsal region of carcasses of three 6-mo-old pigs. These incidental findings were detected during meat inspection in 3 different slaughterhouses in Catalonia, Spain. Histopathology revealed multiple cystic cavities lined by flattened glandular epithelium, filled with proteinaceous material, and corresponding to cystic hyperplasia of sweat glands.

Bilateral Facial Apocrine Fibrosing Hamartoma Mimicking Microcystic Adnexal Carcinoma.

An otherwise healthy 50-year-old woman was evaluated for the presence of 2 erythematous, and slightly pruritic plaques, involving both cheeks for 30 years. Left-side skin biopsy showed a diffuse proliferation of ductal structures horizontally arranged and involving the reticular dermis that resembled tubular adenoma embedded in a sclerotic stroma and surrounded by a peculiar periductal desmoplasia. Nuclear atypia or mitosis was not found. Contralateral biopsy showed identical findings. Differential diagnosis included microcystic adnexal carcinoma (MAC) and plaque-like syringoma and a peculiarly horizontally arranged tubular adenoma. We ruled out MAC as the lesions were long-standing, without infundibular cysts, solid strands, or perineural infiltration. Our case closely resembled those previously described as sweat duct proliferation associated with aggregates of elastic tissue and atrophoderma vermiculatum, although striking differences were observed, as our case did not present aggregates of elastic tissue, did not involve the papillary and superficial reticular dermis, and presented evidences of decapitation secretion as a sign of apocrine differentiation. We consider our case as a MAC simulator and we propose the descriptive name of bilateral facial apocrine fibrosing hamartoma.

Liposuction-assisted circumferential trimming in treatment of axillary osmidrosis (AO).

BACKGROUND: The study is to investigate the effectiveness and safety of the minimally invasive treatment for axillary osmidrosis by liposuction assisted circumferential trimming. METHODS: It was a retrospective study. From July 2014 to July 2017, 79 patients underwent superficial liposuction and circumferential trimming for bilateral axillary osmidrosis. The preoperative and postoperative degree of axillary malodor was measured by doctors and the patients themselves. In the doctor's evaluation, the odor levels were scored by the sniffing method before and 1 year after treatment. In the patient's self-assessment, each patient selected a scale value to convey his/her satisfaction during the visits after 1 year. The complications were recorded. RESULTS: The follow-up period ranged from 12 to 24 months after surgery, and the mean follow-up period was 16 months. 75 (94.9%) had good results, four (5.1%) had moderate malodor or recurrence after one year. When considering patient's own satisfaction, 93.7% (74/79) of patients were satisfied with the outcomes after one year. The partial epidermis necrosis was observed in four patients, it healed spontaneously without scarring. Three patients had a small amount of hematoma which was easily evacuated through the central primary incision. There was no other serious side effect. CONCLUSIONS: The liposuction assisted circumferential trimming technique is proved reliable and safe in treatment for axillary osmidrosis.

Chemotherapy-induced eccrine squamous syringometaplasia in an infant.

There are few reports of chemotherapy-induced eccrine squamous syringometaplasia in children. We report the first case of an infant developing this condition after treatment with busulfan, fludarabine, and antithymocyte globulin in preparation for bone marrow transplantation. Twenty-eight days after transplantation, the infant developed faintly erythematous papules and plaques on the bilateral axillae, inguinal folds, and sites of adhesives. Punch biopsy revealed eccrine glands with dyskeratotic cells and focal squamous metaplasia consistent with chemotherapy-induced eccrine squamous syringometaplasia.

Porokeratotic eccrine and hair follicle nevus: a report of two cases and review of the literature.

Porokeratotic eccrine and hair follicle nevus is a very rare non-hereditary disorder of keratinization with eccrine and hair follicle involvement with only 9 cases described in the literature. In 2009 the term porokeratotic anexial ostial nevus was proposed to comprehend porokeratotic eccrine and hair follicle nevus and a related and more common process without follicular involvement: porokeratotic eccrine ostial and dermal duct nevus Recent findings suggest that both entities may be produced by a mutation in GJB2 gene, which is associated to KID syndrome. Herein we report 2 cases of porokeratotic eccrine and hair follicle nevus and review the existing cases in the Spanish and English literature.

Tratamento cirúrgico da bromidrose / Surgical treatment of bromhidrosis

INTRODUÇÃO:A bromidrose ou osmidrose é um problema que leva inúmeros pacientes a procurar tratamento médico especializado. A remoção das glândulas sudoríparas da região axilar por meio de exérese e lipoaspiração complementar é um procedimento de pequeno porte, tecnicamente simples e com poucas complicações. O objetivo deste trabalho é mostrar a aplicação da cirurgia neste problema, suas complicações e o grau de satisfação dos pacientes. MÉTODO: Trinta e dois pacientes foram submetidos à lipoaspiração e retirada dos tecidos da axila, sob anestesia local e sedação. Acompanhou-se por no mínimo 6 meses estes pacientes no pós-operatório, avaliando a evolução e possíveis complicações e aplicou-se o questionário CSQ-8 para o grau de satisfação no sexto mês. RESULTADOS: Após 6 meses de acompanhamento, poucas foram as complicações e as respostas ao questionário demonstraram alto grau de satisfação. CONCLUSÃO: Além de ser facilmente exequível, o procedimento se mostrou seguro e com poucas complicações.

INTRODUCTION: Bromhidrosis or osmidrosis causes many patients to seek specialized medical treatment. Removal of the sweat glands from the axillary region through excision and complementary liposuction is a minor, technically simple procedure, with few complications. The objective of this study is to review the role of surgery in bromhidrosis, complications of treatment, and the degree of patient satisfaction. METHOD: Thirty-two patients underwent liposuction and removal of axillary tissue under local anesthesia and sedation. The patients were followed up for at least 6 months postoperatively, to evaluate the outcome and possible complications. The Client Satisfaction Questionnaire was completed after 6 months. RESULTS: After 6 months of follow-up, there were few complications and the questionnaire revealed a high degree of satisfaction. CONCLUSION: In addition to being easily performed, the procedure was safe, with few complications.

Porokeratotic eccrine and hair follicle nevus: a report of two cases and review of the literature

Abstract: Porokeratotic eccrine and hair follicle nevus is a very rare non-hereditary disorder of keratinization with eccrine and hair follicle involvement with only 9 cases described in the literature. In 2009 the term porokeratotic anexial ostial nevus was proposed to comprehend porokeratotic eccrine and hair follicle nevus and a related and more common process without follicular involvement: porokeratotic eccrine ostial and dermal duct nevus Recent findings suggest that both entities may be produced by a mutation in GJB2 gene, which is associated to KID syndrome. Herein we report 2 cases of porokeratotic eccrine and hair follicle nevus and review the existing cases in the Spanish and English literature.