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.     

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.     

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: 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.     

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 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 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.     

Successful treatment of angiolymphoid hyperplasia with eosinophilia and Kimura's disease in the same patient with surgery

Until now, there have only been two cases describing the coexistence of angiolymphoid hyperplasia with eosinophilia (ALHE) and Kimura's disease in one patient. Herein, we report the previous case of recurrent ALHE and Kimura's disease that was successfully treated with surgery.     

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.     

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.     

CARBON DIOXIDE LASER TREATMENT OF PERIUNGUAL AND SUBUNGUAL VIRAL WARTS

This is a retrospective study of the cure rates of forty patients with subungual and periungual viral warts treated with carbon dioxide laser vapourisation (total of 69 lesions). 70.6% (48/68) lesions had failed treatment with cryotherapy and/or electrocautery treatment previously. 20/68 were treated with CO2 laser vapourisation as a first line treatment. The overall cure rate over 10 months follow-up period was 57.4% (39/68). Most recurrences (24/25) occurred within the first 3 months of vapourisation. The carbon dioxide laser vapourisation cure rate for warts in which previous cryotherapy and/or electrocautery had failed was 47.9% whereas those treated with carbon dioxide laser vapourisation as a first line treatment had a cure rate of 80% (p = 0.043). Subungual warts responded slightly better with a cure rate of 64.7% compared with periungual warts (54.9%) (n.s.). The carbon dioxide laser vapourisation cure rate for recurrent subungual and periungual warts (which failed previous carbon dioxide laser vapourisation) was 73.3% (11/15). Our findings appeared to indicate that periungual and subungual warts can be eradicated by CO2 laser vapourisation. Recurrent warts can be effectively eradicated by further vapourisation. Recalcitrant periungual and subungual warts which have previously failed to respond to cryotherapy and/or electrocautery can be effectively eradicated with CO2 laser vapourisation.     

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.     

Granuloma faciale: efficacy of cryosurgery in 2 cases

BACKGROUND: Granuloma faciale is a rare condition of unknown pathogenesis. Treatment often gives less than satisfactory results. We report two cases successfully treated with cryosurgery. CASE REPORT: A 42-year-old woman had a round 65 x 40 mm erythematous violet-colored papulous plaque on the right cheek. Histology reported a dense inflammatory infiltration of the superficial and mid derma around dilated vessels under a normal epidermis. The infiltration was composed of histiocytes, lymphocytes, plasma cells, neutrophils and eosinophils and was compatible with granuloma facial. Surgical treatment was performed but was followed by recurrence within a few months. Class I local steroids, cotton swab cryotherapy and oral dapsone remained ineffective. Cryosurgery however led to cure without recurrence at 1 year follow-up. The second patient was a 52-year old man with an oval reddish-brown papulous plaque on the right preauricular area. The plaque measured 40 mm in diameter and histology favored granuloma facial. Three cotton swab cryotherapy sessions and one surgical excision procedure were followed by recurrence. Cryosurgery provided successful cure without recurrence at 3.5 years follow-up. DISCUSSION: Most destructive treatments for granuloma facial, including pulverization cryotherapy, surgery, and CO(2) or argon laser are unsuccessful in preventing recurrence. Cryosurgery was proposed in 1977 but few cases have been reported since. Medical treatments have been variously successful with most authors reporting unsatisfactory results. In our two cases, surgical excision was rapidly followed by recurrence and in one case dapsone was ineffective. Cryosurgery provided rapid regression of the lesion and recurrence-free cure at 1 and 3.5 years follow-up respectively. Cryosurgery is an effective treatment for readily recurrent eosinophilic granuloma facial and should be proposed as first intention therapy.     

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 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.     

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 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.     

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.     

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.