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.     

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.     

Propranolol: a novel treatment for angiolymphoid hyperplasia with eosinophilia

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon, idiopathic disease that manifests as dermal or subcutaneous red or brown papules or nodules, most commonly on the head and neck. Histologically, ALHE is characterized by vascular proliferation with epithelioid endothelial cells with surrounding lymphocytic and eosinophilic infiltrate. There may be an accompanying serum eosinophilia and local lymphadenopathy. We report a case of a 32‐year‐old woman who presented with multiple erythematous nodules in the periauricular area and the vertex of her scalp. The nodules had been present for several months. Surgical removal of one of the lesions confirmed the histological diagnosis of ALHE. The patient was started on oral propranolol (40 mg once daily) in an effort to reduce the vascular component of the lesions. Within 6 weeks, the patient noted that several of the lesions had decreased in size, and all were less erythematous. Propranolol was subsequently stopped within a few months of initiating treatment. One lesion recurred over a year later, and propranolol was then restarted. No new lesions occurred after 2 years of follow‐up.     

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.     

Angiolymphoid hyperplasia with eosinophilia: good response to photodynamic therapy

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign vascular disease of unknown pathogenesis, that occurs as solitary or multiple nodules or papules. Lesions are located mainly on the head, forehead and neck, and may be persistent and difficult to eradicate. We report a case of ALHE treated with aminolaevulinic acid photodynamic therapy (ALA‐PDT). Treatment consisted of two ALA‐PDT sessions with a 2‐week interval. Clinical evaluation, 8 weeks after treatment, showed marked improvement though not complete regression. The treatment was well tolerated. At follow‐up 4 months after treatment, the lesions were stable. We believe that PDT could be an alternative therapeutic approach for ALHE or could be used as a neoadjuvant treatment to reduce lesion size especially where size or site of lesions limits the efficacy or acceptability of other treatments. The lack of cumulative effects allows repeated treatments with ALA‐PDT, but long‐term follow‐up is advised for assessment of recurrence.     

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.     

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 eosinophilia developing within a port wine stain

A 19‐year‐old male with a port wine stain on the base of his neck presented with a 5‐month history of gradual thickening of the involved skin which interfered with clothing and caused repeated bleeding. The lesion was excised and histopathologic examination revealed angiolymphoid hyperplasia with eosinophilia (ALHE) arising from the pre‐existing port wine stain – a rare finding with only one previously reported case. Additionally the lesion was associated with elevated serum renin levels which virtually normalized following excision of the lesion. We further demonstrated the expression of angiotensin converting enzyme and angiotensin II receptors 1 and 2 by the lesion and discuss the possible role of the renin–angiotensin system in this condition.     

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 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.     

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.     

Rapid remission of severe pruritus from angiolymphoid hyperplasia with eosinophilia by pulsed dye laser therapy

A 48-year-old Japanese woman with angiolymphoid hyperplasia with eosinophilia (ALHE) was successfully treated with a flashlamp pulsed dye laser (585 nm, 450 micros pulse duration). The lesion was severely pruritic and had been enlarging slowly for 2 years but was resistant to conventional therapies, including topical, intralesional, and systemic corticosteroid, and cryotherapy. The severe pruritus immediately improved after the first treatment using the pulsed dye laser. The erythema and papules gradually improved without scarring and this was followed by further five treatments over approximately a 4-month interval. No clinical recurrences have been observed 1 year after completion of the treatment. We think that pulsed dye laser therapy is an effective treatment for ALHE in both Japanese as well as Caucasian patients. Pulsed dye laser therapy is also helpful in reducing the pruritus in ALHE patients.     

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 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 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.     

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.     

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 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.     

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.