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.     

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.     

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.     

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).     

Angiolymphoid Hyperplasia with Eosinophilia on the Palm

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon dermal angioproliferating tumor, characterized by red to brown papules or nodules on the head and neck, though also occurring in the mouth, trunk, extremities and inguinal area. The palm is a very unusual site for ALHE, and there have been very few cases reported globally thus far. ALHE can be pruritic and painful and histopathologic findings show vascular proliferation with infiltration of eosinophils and lymphocytes in the dermis. Plump endothelial cells protrude into the lumen. We report a case of ALHE occurring at an unusual site, the right palm, in a 62-year-old man, who had suffered from a solitary pinkish-colored, central depressed round hyperkeratotic plaque on his palm for 4 years. On the basis of clinical and histopathologic data, a diagnosis of ALHE was made. To our knowledge, this is the first report of ALHE on the palm in Korean dermatologic literature.     

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.     

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.     

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.     

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.     

Morfología ecográfica en placa pseudonodular hipervascular en hiperplasia angiolinfoide

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular proliferation characterized by solitary or multiple angiomatous lesions. It is most common in young or middle-aged women, and the lesions typically affect the head and neck, showing a particular predilection for the periauricular region. The differential diagnosis in patients with ALHE is broad and includes both benign and malignant conditions. We report on a series of cases of periauricular ALHE in which ultrasound imaging revealed an hypervascular, pseudonodular and plaque-like morphology with clinical and histologic correlations. It also evidenced vascular communication between lesions that appeared to be separate on clinical examination. Familiarity with such ultrasound presentations could help to improve diagnostic accuracy and facilitate disease monitoring in patients with ALHE.     

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.     

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.     

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.     

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.     

Intra-arterial angiolymphoid hyperplasia with eosinophilia of the temporal artery: Report of two cases and review of the literature

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a relatively common benign vascular disorder of uncertain etiology and has many synonyms, such as pseudopyogenic granuloma, atypical pyogenic granuloma, epithelioid hemangioma, and histiocytoid hemangioma. It usually develops on the head and neck, manifesting as reddish-to-dark purpuric papules or nodules. In this article, we report two patients who had uncommon intra-arterial ALHE that occurred in the temporal artery. In both cases, the ALHE presented as skin-colored subcutaneous nodules over the forehead, mimicking temporal arteritis. Histopathologically, intravascular epithelioid endothelial cell proliferation occurred, with lymphocyte and eosinophil infiltration in the stroma. One patient also showed typical findings of ALHE in the adjacent soft tissue. We know of seven similar cases that have previously been reported in the literature to date.     

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: successful treatment with the Nd:YAG laser.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon idiopathic proliferation of blood vessels that manifests in adults as isolated or grouped papules, plaques, or nodules in the skin of the head and neck. We describe the case of a 31-year-old woman with a 3-year history of persistent ALHE, located on the tragus of her right ear, with no sign of spontaneous regression over a period of 3-6 months and refractory to intralesional corticosteroids. We report the successful use of the Nd:YAG laser for this condition, which offered excellent symptomatic and cosmetic results and suggests the consideration of this modality in the treatment of ALHE.     

D2-40 highlights lymphatic vessel proliferation of angiolymphoid hyperplasia with eosinophilia

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign, uncommon idiopathic condition, characterized by cutaneous papules or nodules, whose etiopathogenesis is still unclear. It has been considered an angioproliferating lesion (epithelioid hemangioma) since histologically it is marked by a proliferation of blood vessels, accompanied by an inflammatory infiltrate, consisting mainly of lymphocytes and eosinophils.
We present a case of ALHE assessed immunohistochemically for D2-40—a new marker for lymphatic endothelial cells. A biopsy specimen obtained from the same anatomical area of a healthy individual served as a normal control. The ALHE specimen showed increased number of lymphatic vessels when stained for D2-40, whereas the endothelial cells lining blood vessels were negative. The specificity of D2-40 for lymphatic vessels was further substantiated by studying Factor VIII-related antigen expression in consecutive sections of both ALHE and the control specimen. A reverse pattern was appreciated—blood vessels showed Factor VIII positive labeling, whereas lymphatic endothelial cells remained unlabeled.
We therefore assume that apart from the lymphocytic infiltrate in the lesion, the recognized lymphoid component in ALHE is due to lymphatic vessel proliferation as well. Hence, this condition may be considered as possibly derived from lymphatic endothelium.     

Angiolymphoid hyperplasia with eosinophilia successfully treated with imiquimod. A case report

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare disorder characterized by vascular proliferation, eosinophilia in peripheral blood samples and lymphocytosis. The etiology is unknown; this rare condition is generally seen in women. It often presents as multiple, grouped red nodules in the periauricular region, scalp line and neck. Numerous therapeutic approaches have been tried for ALHE including destructive techniques such as electrodesiccation, cryotherapy, surgical excision, radiotherapy or laser (argon, CO(2), pulse dye). We present an ALHE case that was treated successfully by using topical imiquimod cream.     

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.