Eccrine Angiomatous Hamartoma Research Articles

Eccrine angiomatous hamartoma – A rare painful skin tumor

We report a 68-year-old male with a progressively enlarging, reddish raised painful lesion on the right arm for the previous 3 years. The starch iodine test was positive, dermoscopic findings were yellowish-brown globules on an erythematous background. Biopsy from the lesion showed the presence of eccrine glands, hair follicles, and lobules of capillary sized vessels surrounding hair follicles in the dermis. On immunohistochemistry, epithelial membrane antigen (a marker for eccrine gland) and CD31 (endothelial marker) were positive. A final diagnosis of eccrine angiomatous hamartoma (EAH) was made. There should be a high index of suspicion about EAH if the skin lesions are reddish and tender with associated localized sweating.

Somatic PIK3CA Variants Are Associated With Eccrine Angiomatous Hamartomas

Objectives: Eccrine angiomatous hamartoma (EAH) is a rare vascular anomaly with mixed eccrine and vascular components, typically identified in children. While benign, EAH can cause significant morbidity and be difficult to treat. The aims of this case series were to identify all patients with EAH that have been seen at the Queensland Children’s Hospital and describe their phenotypic and somatic genotypic details, in an effort to contribute to the limiting understanding and literature surrounding this condition. Methods: Individuals with EAH were retrospectively identified through engagement in a multidisciplinary vascular anomaly clinic in a tertiary Australian children’s hospital. All individuals had a previous histological diagnosis of EAH. High-read-depth sequencing of a panel of 27 genes known to be associated with vascular anomalies was undertaken on affected tissue. Samples were rereviewed by a senior pathologist and geneticist for this study. Results: Five cases of EAH were identified. All were associated with 1 of 3 somatic PIK3CA variants (c.1633G>A;p.Glu545Lys, c.1624G>A;p.Glu542Lys, and c.3140A>G;p.Histo1047Arg) in low allele fractions. These variants have previously been reported in a range of tumors and vascular anomalies, including PIK3CA-related overgrowth spectrum, but not in EAH. Conclusion: Occurrence of somatic PIK3CA variants in EAH provides evidence for a novel gene-disease association and is plausibly the cause of EAH in some individuals. This finding expands the phenotypic spectrum of PIK3CA, contributes to understanding of the pathophysiology of this rare condition, and may avail molecularly targeted therapy in the future.

A Case of Adult-Onset Eccrine Angiomatous Hamartoma—The Comparison with Epithelioid Hemangioma

Eccrine angiomatous hamartoma (EAH) is a relatively rare benign skin disease characterized by the proliferation of eccrine sweat glands associated with capillary hemangioma and the proliferation of other skin elements such as adipose tissue, hair, and epidermis. The onset of the disease is usually at birth or in childhood and tends to occur in the extremities of females, but it occurred in an adult male in this case. The patient was a 72-year-old man with a 12 × 12 mm light brown, elastic, slightly firm skin nodule on the flexor aspect of his right forearm. A biopsy revealed enlargement of blood vessels, sweat glands, sweat ducts, and erector spongiosum with both lumen dilation and narrowing, leading to the diagnosis of EAH. The histopathological features of EAH include a marked proliferation of microvessels, epithelial-like changes in vascular endothelial cells (such as enlarged nuclei), and infiltration of inflammatory cells, mainly lymphocytes and plasma cells. In adult-onset cases, EAH can be clinically difficult to distinguish from epithelioid hemangioma (EH), which differs in the predominance of microvascular proliferation and the presence of eosinophils in the infiltrating inflammatory cells. It can also be distinguished from EAH by the negative results of S100 and anti-EMA in immunohistological staining. In the current cases, we were able to differentiate the two cases from characteristic findings on HE staining.

Eccrine Angiomatous Hamartoma: A Rare Presentation in Late Adulthood

Eccrine angiomatous hamartoma is a tumor characterized by benign malformation of eccrine and vascular elements, occurring generally at birth or during early infancy and childhood. The rarity with which it is encountered in adults and the elderly makes it a diagnostic challenge in such age brackets. We report an interesting case of eccrine angiomatous hamartoma presenting in late adulthood confirmed by histopathology.
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Painful nail: What’s is your diagnosis

A glomus tumor is a rare benign neoplasia; which involves a thermoregulatory microvascular system, the glomus body. It is often found in the subungual tissue. Eccrine angiomatous Hamartoma (EAH) is a rare proliferation of vascular and eccrine structures. We present an original case by its location, clinical examination (pain is the main symptom with onycholysis) and its misleading appearance on MRI. The treatment consists of surgical excision of the tumor with good evolution. We will insist on the histological exam of the piece, which is the key to the diagnosis.

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.

Adult-onset eccrineangiomatous hamartoma treated with excision and skin grafting: a case report

<p>Eccrine angiomatous hamartoma is a rare benign tumour like malformation characterized by intradermal proliferation of eccrine and vascular components. It usually arises before puberty but can occur at any age. It typically presents as a solitary or multiple reddish or brownish plaque or nodule that progressively enlarges in size. Symptoms such as pain and hyperhidrosis may be associated. Lesions are commonly located over distal extremities. We report a case of a 45-year-old lady who presented with a gradually enlarging solitary reddish plaque over the left retro-auricular region of the scalp since the last 12 years and associated with occasional pain. Histopathology revealed pproliferation of eccrine sweat glands and thin-walled blood vessels in the dermis. Additional ultrasound and MRI contrast studies were done to assess the vascular component and involvement of underlying structures. The patient was treated successfully with complete excision of the lesion followed by split thickness skin grafting.</p><p><strong> </strong></p>

Hairless hyperhidrotic plaque in a young male

An 18-year-old male presented with an erythematous plaque on the left side of face and neck since 4 years. The lesion was devoid of hair, had follicular prominences, and showed increased localized sweating. Histopathology revealed an increased number of eccrine glands in the myxoid stroma with multiple vascular elements in the dermis, favoring the diagnosis of eccrine angiomatous hamartoma. This case illustrates a relatively uncommon clinical presentation of eccrine angiomatous hamartoma as a hairless, hyperhidrotic plaque in the head and neck region.

A Fast and Effective Surgical Method for a Painful Skin Lesion

ABSTRACT Background Eccrineangiomatoushamartoma (EAH) is a rare benign disease that comprises vascular and eccrine malformations. Pain is one of the most common symptoms and surgical resection is generally required. Objective To report a novel surgical technique that can alleviate severe pain caused by a large lesion in an EAH patient. Methods On magnetic resonance imaging (MRI), a subcutaneous abnormal signal was detected in the right hip and groin area of an 18-year-old male. The lesion was carefully elevated from the normal tissue above the plane of the deep fascia and was sutured in situ. Additionally, a small sample of the central lesion tissue was collected for histological examination. Results The visual analog scale score of pain decreased from 9 points to 2 points at 1week and to 0 points at 2 weeks after the surgery. The only complication observed was local numbness, which recovered spontaneously. No recurrence of pain was observed after 1 year of surgery. Histological findings demonstrated that the numbers of eccrine sweat glands and abnormal vessels decreased post-surgery. Conclusion The surgical procedure used in our case may represent a novel, easy, safe, and effective option for the treatment of a large, severely painful EAH.

Painful tumors of the skin: "CALM HOG FLED PEN AND GETS BACK" [Corrigendum

Painful tumors of the skin: "CALM HOG FLED PEN AND GETS BACK" [Corrigendum] Cohen PR, Erickson CP, Calame A. Clin Cosmet Investig Dermatol. 2019;12:123–132. On page 123, Abstract section, line 7, “Based on our clinical experience and review of the literature, 25 painful skin tumors were identified: chondrodermatitis nodularis helicis, angioendotheliomatosis, leiomyoma, metastases, hidradenoma, osteoma cutis, glomus tumor, fibromyxoma, leiomyosarcoma, eccrine angiomatous hamartoma, Dercum’s disease, peizogenic pedal papule, neurilemmoma, angiolipoma, neuroma, dermatofibroma, granular cell tumor, endometriosis, thrombus, scar, blue rubber bleb nevus, angioma, calcinosis cutis, and keloid.” should read “Based on our clinical experience and review of the literature, 25 painful skin tumors were identified: chondrodermatitis nodularis helicis, angioendotheliomatosis, leiomyoma, metastases, hidradenoma, osteoma cutis, glomus tumor, fibromyxoma, leiomyosarcoma, eccrine angiomatous hamartoma, Dercum’s disease, peizogenic pedal papule, eccrine spiradenoma, neurilemmoma, angiolipoma, neuroma, dermatofibroma, granular cell tumor, endometriosis, thrombus, scar, blue rubber bleb nevus, angioma, calcinosis cutis, and keloid.”Read the original article

A case report of adult onset eccrine angiomatous hamartoma: A rare entity

Abstract Intoduction: Eccrine angiomatous hamartoma (EAH) is an extremely rare and benign malformation in adults which presents with single or multiple nodules or single large plaque over the extremities at birth with pain and marked sweating. On histopathological examination,there is increased proliferation of eccrine sweat glands and capillary vessels in the dermis. Herein we report a 43 year-old male who presented with a single hyperpigmented plaque over the hypothenar eminence of right palm associated with excessive sweating for 3 months duration.Biopsy from the lesion was suggestive of eccrine angiomatous hamartoma.This case has been reported due to it’s unusual adult onset. Keywords: Eccrine angiomatous hamartoma, EAH, Benign tumor, Adult onset. Introduction Eccrine angiomatous hamartoma (EAH) is a rare and benign tumor, histologically characterized by increased number of dilated eccrine glands and capillary vessels. It was first described in 1859 by Lotzbeck as an angiomatous lesion on the cheek of a child and later EAH was coined in 1968 by Hyman and coworkers.[1] [2]EAH may appear as flesh colored, blue brown, yellow or reddish macule, plaque or nodule associated with exquisite pain on palpation and hyperhidrosis. Asymptomatic lesions do not require any treatment. It is important to recognize this benign clinical entity, for which aggressive treatment is not necessary.?1; Case Report A 43 year old male, working as a supervisor in a hotel came with complaints of hyperpigmented plaque over right palm for 3 months duration which started as a small red itchy plaque. It was associated with excessive sweating over the plaque and pain following local pressure and physical exertion. No history of trauma or similar lesions in other parts of the body. No history of atopy. No history of hypopigmented patches or altered sensations over the extremities. Clinical Examination A solitary, well defined, hyperpigmented plaque of 5x3cm

Painful tumors of the skin: "CALM HOG FLED PEN AND GETS BACK".

Painful tumors of the skin present as dermal or subcutaneous nodules. They can originate from several sources: adipose tissue, cartilage degeneration, deposition of bone or calcium, eccrine glands, fibrous tissue, infiltration of benign (endometrium) or malignant (metastatic neoplasm) tissue, muscle, nerve, or vascular structures. Although pathologic evaluation of the lesion is necessary to determine the diagnosis, it is possible to make a reasonable differential diagnosis based on knowledge of prior tumors that have appeared as tender lesions. Two women with painful skin tumors – either osteoma cutis or an organizing thrombus – are described. Based on our clinical experience and review of the literature, 25 painful skin tumors were identified: chondrodermatitis nodularis helicis, angioendotheliomatosis, leiomyoma, metastases, hidradenoma, osteoma cutis, glomus tumor, fibromyxoma, leiomyosarcoma, eccrine angiomatous hamartoma, Dercum’s disease, peizogenic pedal papule, neurilemmoma, angiolipoma, neuroma, dermatofibroma, granular cell tumor, endometriosis, thrombus, scar, blue rubber bleb nevus, angioma, calcinosis cutis, and keloid. An acronym – inspired by Charlotte’s Web, a book that many children have read – that can be used as a memory aid for recalling the list of painful skin tumors is introduced: “CALM HOG FLED PEN AND GETS BACK”.

The non-neuronal and nonmuscular effects of botulinum toxin: an opportunity for a deadly molecule to treat disease in the skin and beyond.

There is growing evidence that botulinum neurotoxins (BoNTs) exhibit biological effects on various human cell types with a host of associated clinical implications. This review aims to provide an update on the non-neuronal and nonmuscular effects of botulinum toxin. We critically analysed recent reports on the structure and function of cellular signalling systems subserving biological effects of BoNTs. The BoNT receptors and intracellular targets are not unique for neurotransmission. They have been found in both neuronal and non-neuronal cells, but there are differences in how BoNT binds to, and acts on, neuronal vs. non-neuronal cells. The non-neuronal cells that express one or more BoNT/A-binding proteins, and/or cleavage target synaptosomal-associated protein 25, include: epidermal keratinocytes; mesenchymal stem cells from subcutaneous adipose; nasal mucosal cells; urothelial cells; intestinal, prostate and alveolar epithelial cells; breast cell lines; neutrophils; and macrophages. Serotype BoNT/A can also elicit specific biological effects in dermal fibroblasts, sebocytes and vascular endothelial cells. Nontraditional applications of BoNT have been reported for the treatment of the following dermatological conditions: hyperhidrosis, Hailey-Hailey disease, Darier disease, inversed psoriasis, aquagenic palmoplantar keratoderma, pachyonychia congenita, multiple eccrine hydrocystomas, eccrine angiomatous hamartoma, eccrine sweat gland naevi, congenital eccrine naevus, Raynaud phenomenon and cutaneous leiomyomas. Experimental studies have demonstrated the ability of BoNT/A to protect skin flaps, facilitate wound healing, decrease thickness of hypertrophic scars, produce an anti-ageing effect, improve a mouse model of psoriasiform dermatitis, and have also revealed extracutaneous effects of BoNT arising from its anti-inflammatory and anticancer properties. BoNTs have a much wider range of applications than originally understood, and the individual cellular responses to the cholinergic impacts of BoNTs could provide fertile ground for future studies.

Eccrine angiomatous hamartoma: a clinicalopathologic analysis of 4 cases

To investigate the clinical and histopathologic features, diagnosis and differential diagnosis of eccrine angiomatous hamartoma(EAH). Four cases of eccrine angiomatous hamartoma were studied by light microscopy and immunohistochemical staining along with review of the literature. 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. EAH is a rare, benign cutaneous hamartoma. Combination of clinical manifestations, histological changes and immunohistochemical findings is useful for the diagnosis and differential diagnosis.

Eccrine Angiomatous Hamartoma: A Clinicopathologic Review of 18 Cases.

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. The authors retrospectively reviewed 18 EAH cases (including 14 accompanying skin biopsy specimens) diagnosed at 4 American university hospitals from 1996 to 2014. 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. 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.

Angiomatous Hamartoma - A Rare Presentation

Eccrine Angiomatous Hamartoma (EAH) is a benign rare skin neoplasm characterised histologically by abnormal proliferation of sweat glands and surrounding capillaries and other dermal elements like fatty lobules and hair. It usually presents at birth or in early childhood in the form of solitary nodules mostly affecting the extremities. Here, we report a case of angiomatous hamartoma over the face which presented as a cystic swelling in preauricular region in a 55-year-old man. The late onset and a rare site for presentation of EAH prompted us to report the case. There is not even a single case of EAH arising in the "preauricular" region, reported.

Multiple painful brownish plaques associated with local hyperhidrosis.

Eccrine angiomatous hamartoma is a rare hamartomatous lesion characterized by proliferation of eccrine glands and small blood vessels, and occasionally other elements. It generally arises congenitally or later in childhood, as solitary or multiple lesions on the distal extremities. Adult-onset multiple lesions are very rare. Herein we describe a 33-year-old male with symptomatic multiple eccrine angiomatous hamartoma lesions.

HAMARTOMA ANGIOMATOSO ÉCRINO

O hamartoma angiomatoso écrino é uma malformação benigna rara, de etiologia desconhecida, caracterizada histologicamente por numerosos capilares e estruturas glandulares écrinas. Surge mais frequentemente ao nascimento ou na infância, manifestando-se por mancha, placa ou nódulo com crescimento proporcional ao da criança. Pode apresentar hiperhidrose, hipertricose e dor. Descreve-se o caso de uma criança do sexo masculino, de 3 anos, referenciada por 3 manchas discretamente hiperpigmentadas de tonalidade castanha clara, com contornos irregulares e hipertricose, com mais de 20 cm de maior eixo cada, localizadas à área toracolombar esquerda. À fricção as lesões tornavam-se mais evidentes. Evoluíam desde os 6 meses de idade com hiperhidrose local. A biopsia confirmou o diagnóstico de hamartoma angiomatoso écrino. Face à benignidade da lesão e à fruste sintomatologia optou-se por não realizar tratamento. O hamartoma angiomatoso écrino é uma entidade rara destacando-se neste caso, as lesões de grande dimensão, segundo o nosso conhecimento, não referidas anteriormente na literatura.

Eccrine angiomatous hamartoma: late onset facial presentation.

Eccrine angiomatous hamartoma (EAH) is a very rare benign neoplastic condition characterized by hamartomatous proliferation of eccrine glands and accompanying blood vessels and lymphatics. These lesions are more often present at birth or appear during early infancy and childhood and present as solitary nodule or plaque with occasional pain and sweating. They are generally present on the extremities, mostly the palms and soles. We report here a case of EAH in a 20-year-old female who presented with a solitary angiomatous plaque close to the lower eyelid of the right eye with occasional pain and sweating for its rarity.

Adult‐onset eccrine angiomatous hamartoma: report of a rare entity with unusual histological features

Adult‐onset eccrine angiomatous hamartoma: report of a rare entity with unusual histological features