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.     

Kimura's disease. A case report

Subcutaneous masses removed from periauricular lesions showed histologic features of Kimura's disease. These consist of remarkable vascular proliferation and lymphoid follicles in the subcutis rather than lower dermis, with numerous infiltrations of tissue eosinophils. Moderate eosinophilia and high IgE levels in the peripheral blood, and positive intradermal test and RAST with candida antigen were observed, which are also characteristic of Kimura's disease. However, the patient is a middle-aged female without any swollen lymph nodes or systemic involvement. Moreover, a few vessels with prompt endothelial cells were recognized histologically. This resembles subcutaneous angiolymphoid hyperplasia with eosinophilia and its allied diseases. Review of the relevant literature and study of our own case suggested that Kimura's disease is highly similar to subcutaneous angiolymphoid hyperplasia with eosinophilia and its related diseases. We surmise that Kimura's disease belongs to this group.     

Kimura's disease: immunohistochemistry of infiltrating cells using monoclonal and polyclonal antibodies

This study reports immunohistochemical findings with polyclonal and monoclonal antibodies in a case of Kimura's disease, angiolymphoid hyperplasia with eosinophilia, which showed a histopathology similar to that of follicular lymphoma in the initial biopsy. Major findings in our case were the variations in distribution of eosinophils, mast cells and IgE producing plasma cells between the biopsies. No proliferation of eosinophils was noted in the initial biopsy. The follicular structures in Kimura's disease appeared immunohistochemically to have derived from a reactive inflammatory process. It is suggested that the frequency of eosinophils in Kimura's disease varies with the course of the disease and that there is no direct relationship between the distributions of mast cells and eosinophils.     

An Unusual Clinical Presentation of Kimura's Disease Occurring on the Buttock of a Five-year-old Boy

Kimura''s disease is a rare inflammatory disorder of unknown etiology primarily seen in young Asian males. It is characterized by painless subcutaneous masses, blood and tissue eosinophilia, and markedly elevated serum immunoglobulin E (IgE) levels. Clinically, the subcutaneous nodules occur predominantly in the head and neck. However, we report the case of a five-year-old boy presenting with an asymptomatic solitary brown pigmented nodule on the left buttock diagnosed with Kimura''s disease. Here we describe the diagnosis and treatment of this unusual case.     

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.     

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.     

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.     

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.     

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 eosinophils. Case report and differentiation from Kimura disease

We report a 40-year-old patient, who presented with a nodular tumor at the scalp. The histological diagnosis was angiolymphoid hyperplasia with eosinophils (ALHE). Immunohistochemical staining of the proliferating vessels was characteristic for endothelial cells. The perivascular inflammatory infiltrate mainly consisted of CD4-positive lymphocytes. Recently it has become clear that Kimura disease, which was thought to be a variant of ALHE, can be distinguished from ALHE by clinical, histological and immunohistochemical criteria and constitutes its own clinical identity. While ALHE is a localized proliferation of endothelial cells, Kimura disease is a proliferation of lymphoid cells characterized by invasive growth and possibly lymphadenopathy.     

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).     

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 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.     

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.     

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.     

Transdermal nicotine patches for eosinophilic pustular folliculitis

We previously reported the clinical effectiveness of transdermal nicotine patches for the treatment of skin disorders with eosinophilic infiltration such as Kimura's disease, erythema nodosum and eosinophilic pustular folliculitis (EPF). We assessed the efficacy and safety of transdermal nicotine patches for EPF. We treated eight patients with EPF with transdermal nicotine patches and evaluated the treatment response by performing overall lesional assessment. Excellent 77and good responses were obtained in five and one patient(s), respectively. In the other two patients, the lesions remained unchanged. No severe adverse effects were observed. Our results suggest that transdermal nicotine patches may be useful and safe in the treatment of EPF.     

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. The spectrum from Kimura disease to atypical pyogenic granuloma

A patient showed the "atypical pyogenic granuloma" of the head in combination with atopic dermatitis and proceeding pyogenic granuloma of the back. The diagnostic aspects of angiolymphoid hyperplasia with eosinophilia (ALHE) including Kimura's disease, subcutaneous ALHE, and atypical pyogenic granuloma are discussed.     

Human herpesvirus-8 is not associated with angiolymphoid hyperplasia with eosinophilia

BACKGROUND: Angiolymphoid hyperplasia with eosinophilia (ALHE) is an angioproliferative lesion, typically consisting of single or multiple red papules or nodules in the head and neck region. The etiology of ALHE, whether reactive or neoplastic, is unclear. It has been well documented in the literature that human herpesvirus-8 (HHV-8) DNA is present in the majority of cases of Kaposi's sarcoma; however, there is contradictory data regarding the association of this virus with ALHE. METHODS: We performed immunohistochemical studies for HHV-8 on paraffin-embedded tissue from 23 cases of histologically confirmed ALHE. Polymerase chain reaction (PCR) analysis for HHV-8 DNA was performed on 14 of the 23 cases that had adequate remaining tissue for the procedure. The results of the immunohistochemical studies and PCR analysis were compared. RESULTS: HHV-8 immunohistochemical studies were negative in all 23 cases of ALHE. PCR-based analysis on 14 cases failed to identify HHV-8 DNA. CONCLUSIONS: Combined data from several, small published studies are equivocal for an association between HHV-8 and ALHE. The results of our large study show no association between HHV-8 and ALHE.