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.     

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.     

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 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.     

Kimura's disease and angiolymphoid hyperplasia with eosinophilia: New observations from immunohistochemical studies of lymphocyte markers, endothelial antigens, and granulocyte proteins

Kimura's disease (KD) typically presents as large subcutaneous masses in young Oriental males. It is characterized by deep inflammation with vascular proliferation, lymphocytic nodules with subcutaneous germinal centers, fibrosis, and edema. In comparison, angiolymphoid hyperplasia with eosinophilia (ABLE) occurs in all races and the lesions usually are smaller and more superficial. The causes of diese two diseases are debated.
We compared histologic features of 4 cases of KD with 22 cases of ALHE and studied expression of endothelial antigens and lymphocyte markers as well as localization of eosinophil, mast cell, and neutrophil granule proteins in lesional tissue. T-cell lymphoid aggregates with well-formed B-cell germinal centers occurred in KD, and nodular and diffuse T-cell infiltration with small B-cell clusters occurred in ALHE. Endothelial proliferation was more pronounced in KD, lacking the atypical histiocytoid endothelial cells characteristic of ALHE. Many intact eosinophils infiltrated lesions in both diseases, although KD had less extracellular granule protein deposition than ALHE. Intact mast cells were seen in both diseases. There was neutrophil elastase staining of occasional scattered intact cells but no extracellular deposition. Compared with KD, ALHE is more varied in its clinical, histopathologic, and immunohistochemical features.     

Angiolymphoid hyperplasia with eosinophilia--report of a case with reference to the vascular changes and cell infiltrates in this disease

We reported angiolymphoid hyperplasia with eosinophilia (ALHE) in a 47-year-old woman. The most characteristic histological findings were epithelioid endothelial cells with intracytoplasmic vacuoles, and remarkable arterio-venous (A-V) shunts. Mast cells, seen in the lesions, had no special relationship with eosinophils in their distribution. Degranulation of mast cells was not seen. Electron microscopic study revealed abundant cytoplasmic organellae and fine filaments in epithelioid endothelial cells. Immunohistochemical study revealed mixed infiltration of T and B lymphocytes, and polymorphous T-cell population in the lesions. These findings suggest that active vascular proliferations take place in the lesions of ALHE, that the eosinophilic infiltrate and mast cells may be independent of each other, and that the lymphocytic infiltrate probably is a reactive process. The vascular changes seen in Kimura's disease are characterized by a proliferation of capillaries with swollen, but not epithelioid, endothelial cells and vessels with lamellated adventitia. A-V shunts are not observed in its lesions. Therefore ALHE and Kimura's disease should be considered different entities.     

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 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.     

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. 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.     

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.     

Further cases of Kimura's disease in the USSR

The authors review literature on the clinical picture and possible pathogenesis of Kimura's disease, as well as on therapy of the condition. They describe a case of this disease. A woman aged 37 has developed this condition with typical clinical and histological signs.     

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.     

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.     

Reactive perforating collagenosis

Five cases of reactive perforating collagenosis (RPC) are reported. Four of these cases were in a father and his three children. This is the first reported instance of RPC occurring in more than one generation. The clinical and histological features of the disease are described and compared with other disorders showing perforating skin lesions, such as Kyrle's disease, perforating folliculitis and elastosis perforans serpiginosa. Evidence is presented to show that RPC is a genetically determined disorder with an inherited predisposition to an unusual skin reaction to superficial trauma.     

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.     

Localized soft tissue angiomatosis with subsequent development of angiolymphoid hyperplasia with eosinophilia

We report an unusual case of arteriovenous (AV) malformation (localized form of soft tissue angiomatosis): a reaction of angiolymphoid hyperplasia with eosinophilia (ALHE) developed over the lesion of this pre-existing AV malformation. To the best of our knowledge, there is no other report of ALHE in association with a pre-existing bona fide AV malformation. A 71-year-old woman consulted our clinic because a reddish, dome-shaped tumor on her preauricular region, present as a slightly elevated, skin-colored tumor since her childhood, had rapidly enlarged and become more elevated with color change during the preceding two months. The histological features of most of the lesion corresponded to those of soft tissue angiomatosis (localized form). The proliferative vessels underlying the soft tissue angiomatosis streamed out and welled into the dermis with an accompanying prominent inflammatory infiltrate composed of lymphoid cells and eosinophils: these histological features were characteristic of ALHE. The recent abrupt clinical changes in the tumor were considered to represent a reaction to the angiomatosis with development of ALHE.     

Two cases of Kimura's disease with unusual clinical manifestations

We report 2 cases of Kimura's disease with unusual clinical presentation. Lesions were itching nodules located at the elbow and the dorsum of the hand, and were associated with unusual laboratory values. These features may lead to problems in clinical differential diagnosis.     

Treatment of Kimura's disease with cyclosporine

Kimura's disease is a rare, chronic inflammatory disease of unknown cause. It is characterized by painless subcutaneous swellings and lymphadenopathy, commonly affecting the head and neck region. This is associated with peripheral blood eosinophilia and raised serum IgE. It has distinct histological features of lymphoid follicles, eosinophilic infiltrate, fibrosis and vascular proliferation. The disease usually has a benign, indolent course. Traditionally, therapeutic options have included surgery, radiotherapy and steroids but response has been less than satisfactory. Recently, cyclosporine has been reported to be effective in the treatment of Kimura's disease. In this article, we present a middle-aged Chinese female with Kimura's disease for 20 years and her favourable response to cyclosporine.     

Transitorische akantholytische Dermatose (M. Grover) Eine Analyse des klinischen Spektrums anhand von 21 histologisch erfassten Fällen

BACKGROUND AND OBJECTIVE: In 1970 Ralph W. Grover described a clinicopathologic entity characterized by pruritic keratotic papules and papulovesicles predominantly on the trunk, disappearing spontaneously after a few weeks or months and demonstrating the histological features of epidermal acantholysis. The etiology remains unknown; sweating, heat and sunlight are suspected trigger factors. In our survey the clinical spectrum of the disease will be examined. PATIENTS AND METHODS: We have analyzed 21 cases of Grover's disease histologically diagnosed in Berne in 1997 and 1998. RESULTS: In most cases we observed isolated papules disseminated on the trunk. The predominant histological type was the Darier-type; presumably there is no correlation between histological type and clinical features. At the time of diagnosis the skin disorder had been present on average for 83 months. There was a strong association with sweating and heat. Topical steroids were successful in 50% of patients. CONCLUSIONS: Our 21 cases reflect in general the literature. Nevertheless, the long persistence of the skin lesions was striking perhaps making the term transient somewhat inaccurate.