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Eccrine angiomatous hamartoma (EAH) is a rare benign skin neoplasm characterized by an increased size and number of eccrine glands or ducts, along with proliferation of vascular structures in the dermis. This case is unique in its presentation of bilateral symmetrical nodules on both hands and the development of new nodules during puberty. It highlights the need for further research and understanding of this rare condition and its potential progression over time.

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., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

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., (© 2022 Japanese Dermatological Association.)

Chromhidrosis is a rare disorder, which can have significant psychosocial consequences for patients, particularly when it affects the face, as it can be mistaken for scarring, dirt or erroneous make-up application. Chromhidrosis can be divided into apocrine, eccrine and pseudo subtypes. We present a case of apocrine chromhidrosis localized to the bilateral cheek area. Click https://www.wileyhealthlearning.com/#/online-courses/9c761d0a-20de-4709-a57d-36be93cf64e1 for the corresponding questions to this CME article., (© 2021 British Association of Dermatologists.)

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., (© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

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., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: This study aimed to assess the use of 33 MHz ultra-high-frequency ultrasonography (33MHz-UHFUS) for evaluating axillary sweat glands with osmidrosis in comparison with histological techniques. Axillary osmidrosis is a common problem in Asian societies, and the number and size of apocrine sweat glands have a strong relationship with osmidrosis severity. Currently, there are no methods to evaluate sweat gland distribution non-invasively., Methods: In this study, 35 skin specimens from 10 fresh human cadavers without osmidrosis and retrospective ultrasonographic images from 20 patients with osmidrosis were used. Skin specimens were embedded in paraffin, thinly sliced, and finally stained with hematoxylin and eosin. Histologically, the apocrine and eccrine glands were evaluated, and the top and bottom depths of follicles were measured from the skin surface. In 33 MHz ultrasonography images, the depths of sweat glands were measured, and the mean grey value was calculated using Image J., Results: Compared to histological data, 33MHz-UHFUS could be used to identify sweat glands as a hyperechoic structure between the dermis and fat layer. Furthermore, it could evaluate sweat gland distribution but could not distinguish between types of sweat glands., Conclusions: The distribution of sweat glands in the axilla can be non-invasively evaluated via 33MHz-UHFUS., Competing Interests: The authors have declared that no competing interests exist.

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., Competing Interests: The authors declare no conflict of interest., (Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.)

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., Competing Interests: Declaration of Competing Interest The authors have no conflict of interest to declare., (Copyright © 2020 Japanese Society for Investigative Dermatology. Published by Elsevier B.V. All rights reserved.)

Apocrine chromhidrosis is a rare diagnosis that occurs due to colored sweat being secreted from the apocrine glands, which are located in the axillae, anogenital skin, and areolae and over the skin of the trunk, face, and scalp. We present the case of a 65-year-old woman who presented with a sudden onset of pink sweating affecting mainly her axillae but also her pelvis, causing staining of clothing and bed sheets. There was nil to note on examination and histology with immunostaining demonstrated focally prominent yellow-brown lipofuscin granules in the cytoplasm of the apocrine secretory cells confirming the diagnosis. The disease can have a significant psychosocial impact, and treatment remains challenging. Our case is unique because the red and pink coloring of sweat is less common in cases of apocrine chromhidrosis, which is often in favor of darker colored sweat, and the distribution involved the inguinal canal, which is also less often seen.

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.

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.

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.

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.

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., (© 2017 Wiley Periodicals, Inc.)

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.

Objective: To investigate the clinical and histopathologic features, diagnosis and differential diagnosis of eccrine angiomatous hamartoma(EAH)., Methods: Four cases of eccrine angiomatous hamartoma were studied by light microscopy and immunohistochemical staining along with review of the literature., Results: There were 3 male and 1 female patients at diagnosis in age of 4 months, 3.5 years, 5.5 years and 14.0 years, respectively. Tumor sites included the left little finger (1 case), right index finger (1 case), lower back (1 case) and knee (1 case). Clinically, most cases presented as a solitary, flesh or reddish papule, plaque or nodule with size of 0.4-6.0 cm in diameter. The skin lesions in 3 cases enlarged commensurate with the growth of the patients, and local hyperhidrosis in one case. Histologically, EAH was characterized by proliferation of well-differentiated eccrine secretory and ductal elements closely associated with thin-walled angiomatous channels in the middle or deep dermis and subcutaneous tissue. By immunohistochemistry, the vascular components were positive for CD31, CD34 and factor Ⅷ related antigen while the eccrine glands were positive for S-100 protein, CEA, EMA, CAM5.2 and GCDFP15. Local surgical resection was performed in 4 cases and follow-up data (0.5 to 4.5 years) showed no recurrence., Conclusion: EAH is a rare, benign cutaneous hamartoma. Combination of clinical manifestations, histological changes and immunohistochemical findings is useful for the diagnosis and differential diagnosis.

Background: Eccrine angiomatous hamartoma (EAH) is a benign cutaneous lesion defined by the proliferation of hamartomatous eccrine and capillary-like vascular elements in the dermis. However, the epidemiologic, morphologic, and histopathologic aspects of this uncommon disorder have yet to be fully delineated., Methods: The authors retrospectively reviewed 18 EAH cases (including 14 accompanying skin biopsy specimens) diagnosed at 4 American university hospitals from 1996 to 2014., Results: Patients ranged from 3 days to 84 years at time of diagnosis with a median age of 15 years. A male:female ratio of 11:7 was observed. Sixty-seven percent of cases presented in the extremities, but lesions in the trunk and head/neck regions also occurred. Four patients had multiple lesions, and 2 displayed a segmental pattern. Histologically, dermal vascular dilatation and acanthosis often accompanied EAH's typical eccrine and vascular comingling. One individual developed EAH at the site of a recurrent squamous cell carcinoma after previous excision., Conclusions: Although previously thought to occur primarily as a solitary angiomatous-appearing malformation on the extremities of children, EAH may develop with some frequency in adults and may manifest in a multifocal linear distribution. The authors also raise additional histopathologic consideration in support of the vascular theory of histogenesis for this condition.

The expression of p16INK4a has been reported to be a significant marker for malignant transformation of epidermal tumors. However, little is known about sweat gland tumors. We examined the immunohistological expression of p16INK4a in benign and malignant sweat gland tumors. The ductal and acrosyringial portion of normal eccrine glands were positively stained with p16INK4a while it was negative in the normal epidermis. Moderate to strong expression of p16INK4a was found in 16 of 17 eccrine poromas, 4 of 5 hidradenomas, 3 of 3 syringocystadenoma papilliferums, 2 of 2 mixed tumors, and 3 of 3 syringomas. The p16INK4a expression was observed focally or diffusely in 4 of 4 porocarcinomas, 4 of 4 apocrine carcinomas and 12 of 17 extramammary Paget's diseases. We conclude that the p16INK4a expression is not a good marker for dictating malignant transformation of sweat gland tumors.

Introduction: Chromhidrosis is a rare sweat gland disorder characterized by the excretion of colored sweat. It can be classified as apocrine, true eccrine, and pseudochromhidrosis. Amongst the different types of chromhidrosis, green chromhidrosis is extremely rare. We describe herein a case of blue green chromhidrosis induced by ingestion of homeopathic medicine., Case Report: A middle aged man presented to us with blue green discoloration of hands and feet. There was a preceding history of ingestion of homeopathic medication. Histopathology from the involved skin showed greenish particles within eccrine glands. Initial blood copper level was high which returned to normal level after discontinuation of the homeopathic medicine. Spectrophotometry revealed high copper content of the green sweat., Conclusion: Our case emphasizes the importance of considering any type of ingested medicine, including homeopathic medicine, as a probable cause of chromhidrosis.

It has been suggested that the adenosine triphosphate‑binding cassette sub‑family C member 11 (ABCC11) gene polymorphism and apolipoprotein D (ApoD), an odor precursor carrier, may be important in the formation of axillary odor. To date, few studies have examined the potential correlation between these two factors. The present study aimed to investigate the association between a 538 G>A single‑nucleotide polymorphism (SNP) of the ABCC11 gene and the mRNA expression levels of ApoD in the apocrine gland of patients with osmidrosis. The 538 G>A polymorphism genotypes of 33 patients with a clinical diagnosis of osmidrosis were analyzed by polymerase chain reaction (PCR) and a base‑quenched probe method, and they were divided into two groups according to the results. The G allele functions as a dominant gene; therefore, patients with the GG or GA genotype were allocated to Group I (n=28) and patients with the AA genotype to Group II (n=5). The mRNA expression levels of ApoD in the apocrine glands were determined by reverse transcription‑PCR. The results indicated that the mRNA expression levels of ApoD were significantly higher in the apocrine glands of patients in Group I compared with those in Group II (P<0.01). In conclusion, the results indicated that the ABCC11 gene SNP of the 538 G>A allele was associated with a downregulation of the mRNA expression of ApoD in the apocrine glands, which may indicate a role for the ABCC11 gene in the mediation of osmidrosis by enhancing the transition of odor precursors via the ApoD pathway.

We report a case of a 70-year-old man with a 5-year history of red pigmented sweating on the axillae, groin, forearms, trunk, and and peri-nuchal sites. No identifiable cause of the pigmented sweat was identified. Biopsy revealed lipofuscin pigment leading to the diagnosis of idiopathic chromhidrosis. This case represents an unusual presentation of chromhidrosis that was not limited to the apocrine sweat gland distribution and ultimately revealed no identifiable cause. After failing multiple treatment options, the patient elected for a trial of treatment with 20% aluminum chloride solution.

Background: Eccrine chromhidrosis, or colored eccrine sweating, may be caused by contamination of sweat by dyes, pigmentation from microorganisms, or more rarely, hyperbilirubinemia. Pigment usually affects the palms and soles, where abundant sweat glands are found.Purpose, Material and Methods: We report a unique case of eccrine chromhidrosis in the setting of hyperbilirubinemia and review the current literature available on PubMed of previously reported cases., Results: Six patients with chromhidrosis have been previously reported in the setting of significant hyperbilirubinemia, in association with fever and thickened stratum corneum., Conclusions: Eccrine chromhidrosis secondary to hyperbilirubinemia is very rare, but can be diagnosed on the basis of classic clinical findings, dermoscopic examination, and negative tissue cultures.

Chromhidrosis is a rare idiopathic disorder characterized by colored secretions most typically from the malar cheeks, axilla, or areolar regions. Histologically, chromhidrosis is notable for glandular structures with decapitation secretion indicating ectopic apocrine glands in the dermis, and the presence of lipofuscin pigments under ultraviolet fluorescence and in cytology smears. This case report describes a 26-year-old man who presented with a 2- to 3-year history of black-colored secretions on the bilateral malar cheeks, present on exertion or with squeezing of the cheeks. A 3-mm punch biopsy of the left cheek demonstrated histopathologic findings characteristic of chromhidrosis under hematoxylin and eosin staining and ultraviolet fluorescence. To our best knowledge, this is the second case report in the literature of an adult male being affected by chromhidrosis, and the first of an adult male with black-colored malar cheek secretions in chromhidrosis.

Background: Eccrine angiomatous hamartoma (EAH) is recognized as a vascular hamartoma composed of abnormal proliferation of blood vessels and eccrine glands., Objective: We sought to investigate the immunohistochemical results of D2-40 and Prox1 in EAH in order to gain further insight into its histogenesis., Materials and Methods: We collected 21 cases of EAH diagnosed in a dermatology department. Immunohistochemical staining of D2-40 and Prox1 was performed on all cases., Results: Prox1 was universally positive in the endothelial cells of proliferated vessels in all cases. D2-40 was universally positive in the endothelial cells of proliferated vessels in 3 cases, focally positive in 18 cases., Conclusion: EAH is a lymphatic proliferation and therefore we suggest the name of eccrine lymphangiomatous hamartoma.

Eccrine angiomatous hamartoma (EAH) is a benign hamartomatous tumour characterized by a combination of proliferative eccrine and vascular elements. Papillary haemangioma (PH) is a distinct cutaneous haemangioma with predominantly intravascular capillary proliferation within dilated thin-walled blood vessels. EAHs have been reported in combination with various vascular components. We present the first case in the dermatological literature, to our knowledge, of a patient with congenital EAH with the histopathological features of a PH., (© 2013 British Association of Dermatologists.)

Eccrine angiomatous hamartoma (EAH) is a rare tumoral lesion that consists of vascular and eccrine malformation and often occurs in the distal extremities of children. Although EAH is benign, a therapeutic excision may be required for symptomatic or cosmetic considerations. We hereby report a typical case of EAH presenting as a painful and rapidly growing plaque on the right thigh. The associated symptoms of pain, hyperhidrosis and local hypertrichosis caused walking difficulties until the patient was cured by two excisional surgeries., (© 2011 The Authors. Australasian Journal of Dermatology © 2011 The Australasian College of Dermatologists.)

Eccrine squamous syringometaplasia is characterized by the metaplasia of cuboidal epithelial cells of the eccrine sweat ducts into squamous epithelial cells. It has been associated with several conditions including chemotherapy-related bilateral dermatitis, an entity that can take place in body areas rich in eccrine glands, as well as in acral erythema related to chemotherapy. Only a few cases because of cutaneous extravasation of chemotherapy have been previously reported. We report three cases of eccrine squamous syringometaplasia secondary to extravasation of docetaxel., (© 2012 John Wiley & Sons A/S. Published by Blackwell Publishing Ltd.)