Profound proliferating angiolymphoid hyperplasia with eosinophilia of pregnancy mimicking angiosarcoma

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign vascular proliferation that clinically manifests as nodules and papules of the head and neck region. We report a profound, rapidly proliferating case of ALHE in a 3-week postpartum woman that clinically mimicked angiosarcoma. The clinical and histologic features of ALHE, Kitamura disease, and cutaneous angiosarcoma are reviewed, and the relationship between ALHE and pregnancy is discussed.     

Angiolymphoid hyperplasia with eosinophilia showing characteristics of Kimura's disease

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign condition affecting principally the head and neck region of young females. We describe a 42-year-old female patient of ALHE showing the typical changes of endothelial cells and features similar to Kimura's disease in histologic and immunohistochemical findings.     

Angiolymphoid Hyperplasia with Eosinophilia Mimicking Multiple Cylindromas: A Rare Case Report

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare and idiopathic vascular disorder. It is characterized by red to brown papules and nodules, typically localized on the head and neck, particularly around the ear as singular or multiple lesions. Although ALHE is a benign disease, lesions are often persistent and difficult to eradicate. Young to middle age women are more commonly affected. The histological examination corresponds to a florid vascular proliferation with atypical endothelial cells surrounded by a lymphocytic and eosinophilic infiltrate. We describe an elderly male with multiple nodular lesions over the scalp mimicking cylindromas; the histological examination was consistent with ALHE.     

Angiolymphoid hyperplasia with eosinophilia and Kimura disease overlap,with evidence of diffuse visceral involvement

A relationship between Kimura disease (KD) and angiolymphoid hyperplasia with eosinophilia (ALHE) has been debated. Given substantial clinical and histological overlap, these entities were once considered to represent a disease spectrum; however, they are now widely considered to be nosologically distinct. A diagnosis of either condition is further complicated by resemblance to various malignancies, which must be carefully excluded. Coexistence of ALHE and KD in a patient is extremely rare, with only four cases reported in the English literature. We report what is to our knowledge the first case of ALHE and KD overlap with evidence of diffuse visceral involvement.     

Intra-arterial angiolymphoid hyperplasia with eosinophilia

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare skin disorder of unknown etiology. This paper describes the case of a 34-year-old man with a lesion located on the right part of his forehead. Microscopically, it displayed a typical morphology of ALHE. However, it was exceptional by its intra-arterial location and its prominent lymphocytic rim-like component. Differential diagnoses, with special emphasis to organized thrombosis and juvenile arteritis, are discussed here.     

Angiolymphoid hyperplasia with eosinophilia affecting the oral mucosa: report of a case and a review of the literature

Summary Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon disorder of unknown aetiology. Clinically, it is characterized by cutaneous papules or nodules on the head and neck and extra cutaneous involvement is rare. We report a 30-year-old woman who had an asymptomatic submucosal nodule on the upper lip, which histopathological showed features of ALHK and review the previously described 15 cases of oral ALHE.     

Angiolymphoid hyperplasia with eosinophilia of the nail bed and bone: successful treatment with radiation therapy

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare entity characterized by the presence of one or several papules or nodules in the skin. Histopathology of ALHE shows a marked proliferation of blood vessels with distinctive large endothelial cells and variable inflammatory infiltrates with eosinophils. We report a 32-year-old Caucasian woman with multiple nodules involving the skin, subcutaneous tissue and bone of the distal phalanx of the fingers that were treated successfully with orthovoltage radiation therapy (40 Gy/20 fractions) and without any side-effects after 9 years of follow-up.     

Angiolymphoid hyperplasia with eosinophilia affecting the scrotum: A rare case report with molecular evidence of T‐cell clonality

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign entity of unknown pathogenesis. It often presents as painful or pruritic intradermal or subcutaneous red to brown papules or nodules on the head and neck of young adults. A 38‐year‐old man had a gradually enlarging and mild pruritic plaque on the scrotum for half a year. Pathological findings showed dermal proliferation of anomalous blood vessels lined by plump endothelium with a significant perivascular inflammatory infiltrate composed of lymphocytes, histiocytes, scattered plasma cells and many eosinophils. They were consistent with the diagnosis of ALHE. In addition, the inflammatory infiltrate was analyzed by immunohistochemistry and T‐cell receptor (TCR) gene rearrangement. They were mostly CD3⁺ T cells and a monoclonal T‐cell population. To the best of our knowledge, this is the first case of ALHE affecting the scrotum to be reported in the published work. We present this case to expand the anatomical distribution of this rare tumor. The molecular study of our case supports that ALHE might be a low‐grade T‐cell lymphoproliferative disorder.     

A case of angiolymphoid hyperplasia with eosinophilia (ALHE) of the upper lip

Angiolymphoid hyperplasia with eosinophilia (ALHE) is clinically characterized by intradermal or subcutaneous papules and/or nodules usually occurring in young adults. Lesions in the oral mucosa are extremely rare. We report a case and review the literature of ALHE cases involving the oral mucosa. A 40-year-old man presented with a painless, 20 x 20 mm, submucosal nodule on the upper lip. Histological examination of lip biopsy specimens revealed an increase in many small vessels. The vascular walls consisted of prominent endothelial cells with a histiocytoid appearance, which protruded into the lumen. Many eosinophils and lymphocytes were also seen around the vessels. The diagnosis of ALHE was made from the above findings.     

Angiolymphoid Hyperplasia with Eosinophilia That Was Possibly Induced by Vaccination in a Child

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign vasoproliferative disease of an unknown cause involving the skin or subcutaneous tissue of the head and neck, and particularly around the ear. It predominantly affects Caucasian adults during the third and fourth decades and it very rarely occurs in children. We experienced a case of ALHE in a 2-year-old Korean boy who had a firm, pruritic, skin-colored, subcutaneous nodule on his right upper arm. The histopathological findings were compatible with ALHE and they showed prominent vascular changes with epitheloid or histiocytoid endothelial cells surrounded by inflammatory cells, including a large proportion of eosinophils. This unusual distribution of the lesion and the young age of the patient may be associated with vaccination.     

Treatment of angiolymphoid hyperplasia with eosinophilia with the carbon dioxide laser

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon, idiopathic condition that presents with isolated or grouped plaques or nodules in the periauricular region, forehead, or scalp. ALHE is marked by a proliferation of blood vessels with distinctive large endothelial cells accompanied by a characteristic inflammatory infiltrate that includes eosinophils. The lesion is benign but may be persistent and difficult to eradicate. Various therapeutic modalities that have been tried for its treatment include intralesional and oral corticosteroids, cryotherapy, oral retinoids, vinblastine, surgical excision, laser therapy, and INFalpha2a. We report two cases with this rare condition: one patient, treated with cryotherapy, did not improve, while the second patient was successfully treated with the CO(2) laser.     

Unilateral angiolymphoid hyperplasia with eosinophilia involving the left arm and hand

A case report of recurrent angiolymphoid hyperplasia with eosinophilia (ALHE) in an otherwise healthy 20-year-old female with manifestation of the disease limited to the left arm and hand is presented together with brief evaluation of the literature as well as the features distinguishing ALHE and Kimura's disease. Immunohistochemical investigations support the hypothesis that ALHE represents a reactive inflammatory lesion rather than a benign vascular neoplasm. A viral cause of ALHE (e.g., HHV8 or Epstein-Barr virus (EBV)) could not be demonstrated. The recurrent nature of the disease is shown by this case, which also demonstrates the need for frequent medical and surgical management.     

Three unusual histopathological presentations of angiolymphoid hyperplasia with eosinophilia

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a poorly understood benign vasculoproliferative disorder. Histopathologically, the lesions are composed of a dermal or subcutaneous proliferation of blood vessels of varying sizes with plump and bland endothelial cells, which often show vacuolization and protrusion of the cytoplasm into the lumen. The vascular proliferation is admixed with a diffuse inflammatory infiltrate composed of lymphocytes, eosinophils and mast cells. Lymphoid follicles with germinal centers can sometimes be seen. In addition, fibrosis is a common accompanying feature. Some clinical and histopathological variants of ALHE have already been described in the literature. In this report, we present 3 rare associations of ALHE that have not been previously described. Case 1 was a 73‐year‐old woman with a lesion on her right medial thigh. Examination showed ALHE admixed with a chronic lymphocytic leukemic (CLL) infiltrate. Case 2 was a 55‐year‐old woman with a lesion on her right anterior ankle, which was a syringocystadenoma papilliferum co‐existing with an ALHE. Case 3 was a 54‐year‐old man with a lesion on the left medial thigh, which showed overlapping features of IgG4‐related disease associated with areas of ALHE. Given these multiple and diverse associations, it seems likely that ALHE may be a reactive rather than neoplastic process.     

Angiolymphoid hyperplasia with eosinophilia and Kimura's disease – a clinical and histopathological comparison

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular neoplasm mainly affecting middle‐aged women. Lesions typically affect the head and neck region. ALHE is considered a distinct disease entity different from Kimura's disease, a benign reactive lymphoid proliferation that is predominantly seen in young Asian men although it can affect all ethnic groups. In contrast to ALHE, Kimura's disease is typically associated with peripheral blood eosinophilia, increased serum IgE and lymphadenopathy. Several case reports suggest an overlap between ALHE and Kimura's disease. We review the current literature and discuss whether AHLE and Kimura's disease might represent two extreme variants of the same disease entity.     

Co-existence of lichen amyloidosus and angiolymphoid hyperplasia with eosinophilia

Summary Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign vascular tumour. It presents with small, dull red papules or nodules usually on the ears and preauricular areas and only 20% of lesions are multiple. We report a case of multiple scattered lesions of ALHE in a patient who subsequently developed lichen amyloidosus. Cases of lichen amyloidosus in association with Kimura's disease have been reported previously, but there are no reports of lichen amyloidosus with ALHE. The coexistence of these two conditions implies that ALHE is an inflammatory disorder, as an inflammatory process resulting in basal layer damage is necessary for the occurrence of lichen amyloidosus.     

Angiolymphoid hyperplasia with eosinophilia associated with arteriovenous malformation: a clinicopathological correlation with angiography and serial estimation of serum levels of renin, eosinophil cationic protein and interleukin 5

We present a case of angiolymphoid hyperplasia with eosinophilia (ALHE) affecting the auricular area of a 31-year-old man, which clinically mimicked arteriovenous malformation (AVM). The histology and laboratory data distinctively revealed ALHE, while angiography demonstrated typical findings of AVM. Although several reports have hitherto mentioned the relationship between ALHE and AVM, the aetiology of the disease remains unknown. During the 3 years treatment course, we performed angiography several times to assess the efficacy of the treatments and compared the clinical and pathological findings, based on the hypothesis that AVM might be a cause of ALHE. This study showed first, that the clinicopathological findings of ALHE correlated with the extent of AVM shown by angiography, so that AVM could be a primary cause of ALHE. Secondly, systemic corticosteroids and local irradiation therapy produced only a temporary effect on the inflammatory changes of ALHE; therefore, surgical resection is recommended as a curative treatment. Thirdly, the patient's serum levels of renin, eosinophil cationic protein and interleukin 5 corresponded closely with the clinical course of ALHE.     

Epithelioid Hemangioma (Angiolymphoid Hyperplasia with Eosinophilia) in Zosteriform Distribution

Epithelioid hemangioma (EH) or angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign disease. We report an unusual case of EH (ALHE) that arose on the lower back in a zosteriform array. The presence of the characteristic histological appearance of plump endothelial cells with hobnail-like protrusions led to the diagnosis of EH (ALHE). Histological examination of the lesion also revealed the existence of arteriovenous shunts, the possible factor contributing to the pathogenesis of EH (ALHE).     

Polymerase chain reaction (PCR) for human herpesvirus 8 and heteroduplex PCR for clonality assessment in angiolymphoid hyperplasia with eosinophilia and Kimura's disease

BACKGROUND: Recently, human herpesvirus 8 (HHV-8) has been isolated from almost all cases of Kaposi's sarcoma. It has not been found in most cutaneous hemangioproliferative disorders other than Kaposi's sarcoma. Benign vascular lesions including Kimura's disease were not found to contain the HHV-8 DNA sequence. However, there has been contradictory data concerning the presence of HHV-8 in angiolymphoid hyperplasia with eosinophilia (ALHE). Clonality studies in ALHE and Kimura's disease were rare. METHODS: We performed polymerase chain reaction (PCR)-based analysis to determine whether HHV-8 is present and heteroduplex analysis of rearranged T-cell receptor (TCR) gene for clonality assessment in paraffin-embedded skin biopsy samples of 7 ALHE and 2 Kimura's disease, taken from immunocompetent patients. RESULTS: HHV-8 could not be identified in all the cases of ALHE and Kimura's disease. Although 2 cases (2/7) of ALHE and 2 cases (2/2) of Kimura's disease showed positive result for PCR analysis of TCR, all the cases were negative for heteroduplex-PCR. CONCLUSIONS: We suggest that HHV-8 may not involve in a pathogenetic role in ALHE and Kimura's disease and the failure to demonstrate clonality may be consistent with the reactive nature of these diseases and lack of malignant transformation. In addition, heteroduplex-PCR can be applied to confirm doubtful cases of lymphoma in that heteroduplex-PCR is more specific than PCR as seen in our study.     

Hiperplasia angiolinfoide con eosinofilia. Estudio clínico patológico de 9 casos

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare disease characterized by single or multiple angiomatous lesions typically located on the scalp and the face. We present a retrospective analysis of 9 cases of ALHE. The lesions appeared largely as multiple grouped papules or, in some cases, subcutaneous nodules, located mainly on the scalp, particularly around the ear. We also observed lesions in atypical locations, such as areas of the head other than the scalp, and the shoulder, neck, and forearm. At these sites the lesions had an atypical clinical appearance that made diagnosis difficult; this should be borne in mind in patients with single, well-delimited lesions with a vascular appearance and superficial ulceration or crusting. Surgery was the most common treatment in our series, and even though ALHE is considered a benign condition, recurrence was common.     

Angiolymphoid hyperplasia with eosinophilia and Kimura's disease coexisting in the same patient: Evidence for a spectrum of disease

The relationship between angiolymphoid hyperplasia with eosinophilia (ALHE) and Kimura's disease has always been contentious. Initially, ALHE and Kimura's disease were thought to be conditions within the same disease spectrum, but it is now widely accepted that they are two separate disease entities. The two lesions may coexist in one patient. Thus, ALHE and Kimura's disease may be different manifestations of the one disease.