Angiolymphoid hyperplasia with eosinophilia: A report of a rare entity in an elderly male

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign proliferation of blood vessels of uncertain etiology. It primarily affects the head‑and‑neck region. Histologically, it is characterized by the prominent proliferation of plump endothelial cells, and accompanying eosinophilic and lymphocytic infiltration. Herein, we report the case of ALHE in a 65‑year‑old male.

Angiolymphoid hyperplasia: report of an unusual case in the oral cavity / Hiperplasia angiolinfoide: reporte de un caso inusual en cavidad oral

Abstract Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare inflammatory disease of unknown origin, which may be asymptomatic or produce symptoms like pruritus, pain, or spontaneous bleeding, and which has no sex predilection. The treatment options are cryosurgery, pulsed dye laser, topical tacrolimus, surgery, curettage, and oral retinoids. Below is a report of a clinical case of angiolymphoid hyperplasia with eosinophilia in the oral cavity, as well as the treatment performed. This was a female patient who attended a stomatology and oral surgery clinic due to a localized lesion at the level of the vestibule between dental organs 11 and 12. Clinically, there was a nodular lesion of about 1 cm in diameter, located at the level of attached gingiva; it was asymptomatic on palpation and of unknown etiology. Angiolymphoid hyperplasia is an unusual pathology in the oral cavity; its etiology is unknown, and a definitive diagnosis should be made through histopathological examination and clinical signs.

Resumen La hiperplasia angiolinfoide con eosinofilia (HACE) es una enfermedad rara de origen desconocido, de carácter inflamatorio, que puede ser asintomática o presentarse con prurito, dolor o sangrado espontáneo, sin predilección por género. Las opciones de tratamiento son la criocirugía, el láser de colorante pulsado, el tacrolimus tópico, la cirugía, el curetaje y los retinoides orales. Se reporta un caso clínico de hyperplasia angiolinfoide con eosinofilia en cavidad oral, así como el tratamiento realizado. Paciente de sexo femenino, que acudió a consulta de estomatología y cirugía oral por presentar lesión localizada a nivel de vestíbulo entre órganos dentarios 11 y 12. Clínicamente se observó lesión nodular localizada en encía adherida de aproximadamente 1 cm de diámetro, asintomático a la palpación y de etiología desconocida. La hyperplasia angiolinfoide es una patología inusual en cavidad oral, de etiología desconocida, en la que el diagnóstico definitivo debe hacerse a través del examen histopatológico y los signos clínicos.

Kimura disease with nephrotic syndrome in a child- A rare association

Lymphadenopathy along with various systemic manifestations is commonly encountered in pediatric patients, tuberculosis being the commonest etiology. Occasional patients may present a diagnostic conundrum. Here, the authors report an unusual manifestation of Kimura disease (KD) presenting as nephrotic syndrome associated with mesenteric lymphadenitis in an 11-year-old male child. KD is a chronic inflammatory disorder of unknown etiology. It typically affects young adult males in the age range of 27-40 years and usually presents as painless itchy nodular masses in the head and neck region. The involvement of mesenteric lymph nodes along with a very young age of presentation makes it a rare case, posing a diagnostic challenge for the unsuspecting physician.

Three Fibrotic Conditions Treated with 5% Topical Imiquimod Cream as an Antifibrotic Agent / 대한피부과학회지

Imiquimod, a toll-like receptor agonist, is a topical immunomodulator that induces the production of several cytokines including interferon-alpha, which shows antifibrotic properties. We hypothesized that the antifibrotic effect of imiquimod would soften fibrotic skin lesions. Therefore, we applied topical imiquimod with topical tacrolimus or systemic acitretin in patients with refractory lesions of myxedema, angiolymphoid hyperplasia, and generalized discoid lupus erythematosus and confirmed improvement in fibrotic lesions in these patients. Thus, we conclude that use of imiquimod improves fibrotic skin lesions. This report describes our experience with the treatment of this condition along with a review of the related literature.

A case of angiolymphoid hyperplasia with eosinophilia treated with Nd:YAG laser / 中华皮肤科杂志

A 38-year-old male patient presented with multiple papules on the scalp with itching and tingling for 3 years. Skin examination showed multiple millet- to soybean-sized red papules on the scalp. Histopathological examination showed epidermal hyperkeratosis, mildly thickened prickle cell layer, proliferation of a large number of capillaries in the dermis, and perivascular infiltration of closely distributed lymphocytes and scattered eosinophils. The blood vessels were lined by hyperplastic and hypertrophic endothelial cells, some of which projected into the vascular cavity. The patient was diagnosed with angiolymphoid hyperplasia with eosinophilia. After 1 session of Nd:YAG laser therapy, all the skin lesions were removed, and no obvious adverse reactions were observed. No relapse occurred during 5 months of follow up.

A case of angiolymphoid hyperplasia with eosinophilia treated with Nd:YAG laser / 中华皮肤科杂志

A 38-year-old male patient presented with multiple papules on the scalp with itching and tingling for 3 years. Skin examination showed multiple millet- to soybean-sized red papules on the scalp. Histopathological examination showed epidermal hyperkeratosis, mildly thickened prickle cell layer, proliferation of a large number of capillaries in the dermis, and perivascular infiltration of closely distributed lymphocytes and scattered eosinophils. The blood vessels were lined by hyperplastic and hypertrophic endothelial cells, some of which projected into the vascular cavity. The patient was diagnosed with angiolymphoid hyperplasia with eosinophilia. After 1 session of Nd:YAG laser therapy, all the skin lesions were removed, and no obvious adverse reactions were observed. No relapse occurred during 5 months of follow up.

Surgical resection and skin graft repair for a case of angiolymphoid hyperplasia with eosinophilia of the posterior ear / 中华皮肤科杂志

A 34-year-old female patient presented with itchy nodules on the left posterior ear for 7 years.Skin examination showed clustered light-red or brown-black nodules measuring about 4 cm × 5 cm in size on the left posterior auricle,as well as from the posterior sulcus of the auricle to the anterior border of the hair line on the posterior auricle.Histopathological examination of the skin lesion revealed epidermal hyperkeratosis,proliferation of a lot of small blood vessels in the dermis,swelling vascular endothelial cells with enlarged nuclei and eosinophilic cytoplasm bulging into vascular cavity,and perivascular infiltration of lymphocytes and many eosinophils.The patient was diagnosed with angiolymphoid hyperplasia with eosinophilia.After extended surgical resection,a skin graft from the left lower abdomen was used to repair the defect.After the surgery,the flap survived well,and the appearance of the auricle was improved.No recurrence was observed during 1 year of follow-up.

Hiperplasia angiolinfoide con eosinofilia / Angiolymphoid Hyperplasia with eosinophilia

La hiperplasia angiolinfoide con eosinofilia es una enfermedad vascular o tumor vascular, infrecuente, tumoral o reactiva, con carácter inflamatorio, benigna y de etiología desconocida. Los hallazgos histológicos consisten en la proliferación vascular de células endoteliales prominentes y un infiltrado inflamatorio intersticial crónico de leucocitos, histiocitos y eosinófilos. Afecta frecuentemente a mujeres de la tercera y cuarta década de la vida. Se han descrito múltiples variantes de tratamiento pero es frecuente su recurrencia. Clínicamente se presenta como pápulas o nódulos subcutáneos, limitados, únicos o múltiples, color rojo, marrón o violáceo, que se localizan con frecuencia a nivel de la cabeza, cuello; en ocasiones se asocia a prurito y dolor. El interés en presentar este caso radica en su rara localización, su presentación en el sexo masculino; el tamaño, la extensión y la infrecuencia de la lesión. Se presenta el caso de un paciente masculino de 42 años de edad con múltiples lesiones en el eje longitudinal del miembro superior izquierdo, la mayor de 15 x 15 cm, localizada en la cara póstero-interna del brazo en sus dos tercios dístales, limitada, móvil, no dolorosa, con desplazamiento del tejido muscular, y deformidad estructural, con diagnóstico, después de unabiopsia posquirúrgica,de hiperplasia angiolinfoide con eosinofilia según histología La evolución del paciente fue satisfactoria y se mantiene seguimiento médico para comprobarposibles recidivas(AU)

The angiolymphoid hyperplasia with eosinophilia is a vascular disease (or vascular tumor) uncommon, tumoral or reactive, with inflammatory characteristics, benign of unknown etiologic. The histological finds consist on the vascular proliferation of endotelial prominent cells and a chronic inflammatory interstitial infiltrator of leukocytes, histiocytes and eosinophils. It frequently affect to elder women in the third and fourth ages. Clinically, it is presented as papules or subcutaneous nodules, limited, only one or multiple nodules; in red, brown or purplish color that are frequently located at the level of the head, neck; in occasions, it is associated with pruritus and pain. The interest in presenting this case is due to its rare localization, its appearance in the male sex, the size, extension and infrequency of the lesion. The case consists in a 42 year-old masculine patient with multiple lesions in the left superior member's longitudinal axis. The biggest lesion is of 15 x 15 cm, located in the postero-internal part of the arm in its distals two thirds, limited, movable, not painful, with displacement of the muscular tissue, and structural deformity, with a diagnostic of angiolymphoid hyperplasia with eosinophilia according to histology (post-surgical biopsy).The evolution of the patient was satisfactory, and remains medically followed-up in case of possible relapses(AU)

Clinicopathological analysis of 22 cases of cutaneous epithelioid hemangioma / 中华皮肤科杂志

Objective To investigate clinical and histopathological features of cutaneous epithelioid hemangioma (EH).Methods Clinical and histological data were collected from 22 patients with EH and analyzed.Results Of the 22 EH patients,10 were male,and 12 were female.The age at onset ranged from 16 to 62 years,with an average age of 45.91 years.The duration of disease varied from 1 month to 20 years,with an average disease duration of 37 months.Skin lesions most frequently occurred on the scalp in 14 cases (63.6％),followed by the ear in 6 cases (27.3％),the forehead in 2 cases (9.1％),and the thigh in 1 case (4.5％).Lesions affected both the scalp and forehead in 1 case.Histopathological examination of the 22 cases revealed vascular proliferation.The blood vessels were lined with epithelioid endothelial cells,and a large number of lymphocytes and eosinophils infiltrated around the vessels.The dermis were involved in all cases,and subcutaneous tissues were involved in 8 cases (36.4％).Immunohistochemical examination of 6 cases showed positive staining for CD31,CD34 and factor Ⅷ in blood vessel walls.Conclusion EH is an uncommon disease characterized by vascular proliferation and inflammatory infiltration,and clinical manifestations in combination with histopathological examination facilitate its diagnosis.

Angiolymphoid hyperplasia with eosinophilia versus Kimura's disease: a case report and a clinical and histopathological comparison

Abstract Angiolymphoid hyperplasia with eosinophilia is a rare and benign vascular tumor whose etiology remains uncertain. It clinically presents itself by angiomatous papules or nodules located on the head and neck. Many controversies in the literature are found in relation to angiolymphoid hyperplasia with eosinophilia and Kimura's disease - its main differential diagnosis - due to their clinical and histopathological similarities. However, currently, most studies agree that they are distinct diseases. The present case illustrates a characteristic description of angiolymphoid hyperplasia with eosinophilia and also highlights the main differences with Kimura's disease.

Diagnosis of Parotid Eosinophilic Lymphogranuloma Characterized by Multiple Nodules and Masses by Using Multi-slice Spiral CT / 中国医学影像学杂志

Purpose To explore the clinical features and multi-slice spiral CT (MSCT) imaging characteristics of nodule-type and mass-type parotid eosinophilic lymphogranuloma,in order to improve the understanding of the disease.Materials and Methods The clinical and imaging data of 6 patients with nodule-type or mass-type parotid eosinophilic lymphogranuloma confirmed by surgery pathology in Chongqing Jiulongpo Chinese Medicine Hospital from June 2007 to March 2016 (experimental group) were retrospectively analyzed.Another 32 synchronous patients with Warthin tumor confirmed by surgery pathology were enrolled as the control group.MSCT conventional and enhanced scanning were performed on all these patients,and the clinical features and MSCT characteristics were compared between the two groups.Results Compared with patients with Warthin tumor,the value and percentage of peripheral eosinophil and serum IgE level increased in all the 6 patients (age ＜ 50) with eosinophilic lymphogranuloma,and the difference was statistically significant (P＜0.01).Compared with Warthin tumor,eosinophilic lymphogranuloma was often accompanied by ipsilateral parotid enlargement,neck deep fascia thickening and edge blurring,lesions reducing,and neck lymph node enlargement.The enhanced scanning of eosinophilic lymphogranuloma showed delayed enhancement,and the enhancement in venous phase and delayed-phase was significant;while Warthin tumor showed significant enhancement in arterial phase and contrast clearing features in delayed-phase.Conclusion The MSCT findings of eosinophilic lymphogranuloma characterized by nodules or masses shows features of inflammatory lesions,in which mass density is relatively uniform and presents delayed enhancement.Combining clinical manifestation with laboratory tests will contribute to distinguish eosinophilic lymphogranuloma and Warthin tumor.

Acute Limb Ischemia and Coronary Artery Disease in a Case of Kimura's Disease

Kimura disease (KD) is an immune-mediated chronic inflammatory disease of unknown etiology. KD has many complications associated with hypereosinophilia, including various forms of allergic reactions and eosinophilic lung disease. Additionally, hypereosinophilia is associated with hypercoagulability, which may lead to thromboembolic events. A 36-year-old man with KD presented with acute limb ischemia and coronary artery occlusion. He underwent thrombectomy, partial endarterectomy of both popliteal arteries, and coronary artery stent insertion. KD is a systemic disease that affects many organs and presents with thromboembolism and vasculitis. In a patient with KD, physicians should evaluate the vascular system, including the coronary arteries.

A Rare Case of Kimura Disease with Bilateral Parotid Involvement

Kimura disease is a rare idiopathic chronic inflammatory disorder. It typically presents in the head and neck area, whereas bilateral involvement is unusual. Its diagnosis requires it to be differentiated from other inflammatory diseases and from head and neck tumors. Treatment methods include conservative management, steroid administration, radiotherapy, and surgery; however, no single treatment of choice has been established. Herein, we report an unusual presentation of Kimura disease with bilateral parotid involvement. This case was treated by surgical excision.

A Rare Case of Kimura Disease with Bilateral Parotid Involvement

Kimura disease is a rare idiopathic chronic inflammatory disorder. It typically presents in the head and neck area, whereas bilateral involvement is unusual. Its diagnosis requires it to be differentiated from other inflammatory diseases and from head and neck tumors. Treatment methods include conservative management, steroid administration, radiotherapy, and surgery; however, no single treatment of choice has been established. Herein, we report an unusual presentation of Kimura disease with bilateral parotid involvement. This case was treated by surgical excision.

Bilateral extraocular muscles enlargement from Kimura’s disease of the orbit.

Kimura’s disease (KD) is a rare chronic inflammatory disease of unclear etiology, characterized by subcutaneous nodules, mainly in the head and neck region, frequently associated with regional lymphadenopathy. Orbital involvement is infrequent and when it occurs, usually affects the eyelid or the lacrimal gland. We report a case of a 44-year-old man that presented with bilateral slowly progressive proptosis that was initially misdiagnosed as Graves’ Ophthalmopathy. 15 months of worsening proptosis and the development of facial and temporal swelling led to further investigation. Computed tomography and magnetic resonance imaging showed enlargement of all recti muscles and diffuse orbital infiltration. An orbital biopsy was performed and was consistent with the diagnosis of KD. Long term oral corticosteroid showed marked improvement of proptosis and facial swelling. This case serves to emphasize that KD should be included in the differential diagnosis of inflammatory diseases of the orbit, even when characterized by predominant involvement of the extraocular muscles.

Angiolymphoid hyperplasia with arterial ectasia and arteriovenous malformation: Response to intra-arterial embolization.

Angiolymphoid hyperplasia with eosinophilia is a rare disease characterized by vascular proliferation of unknown origin. The lesions are mostly seen in the head and neck region and are characterized by papules or nodules. A 20-year-old man presented with a 1 year history of reddish papulo-nodular lesions overlying a pulsatile swelling on the left auricular area. Histopathology was suggestive of angiolymphoid hyperplasia with eosinophilia. B-mode ultrasonography, color Doppler and angiography revealed arterial ectasia and arteriovenous malformation of the left auricular artery. Such malformations have been reported previously, in association with angiolymphoid hyperplasia with eosinophilia. The cutaneous lesions responded well to transarterial embolization of the nidus.

Kimura’s Disease of Face: A Clinicopathological Presentation and Review.

Kimura’s disease is an extremely unusual disorder of benign nature affecting the connective tissue, and primarily seen in young males in countries of Asian pacifi c region particularly in China and Japan. This is characterized by three essential components of diagnostic importance. (a) Painless benign subcutaneous masses and rarely lymph nodes most predominantly in the head and neck region and usually solitary, (b) Gross eosinophilia in the blood and also in the affected tissue, (c) Markedly elevated serum immunoglobulin E levels. We are reporting this extensive case in a male aged 28 years, and the lesion started when he was 15 years old, affecting the cheeks and submental region, right parotid gland, and associated lymph nodes. Since we operated all the three masses, we present our operative and clinical experiences along with histopathological fi ndings. We are also reviewing the cases already reported earlier and the problems of clinical diagnosis and to differentiate from other diseases which may simulate this disease. Relative merits of all the treatment protocols and the outcome of the disease is also discussed in the light of our experience with this patient.

Cutaneous epithelioid hemangioma of atypical location: a case report / Hemangioma epitelioide cutâneo de localização atípica: relato de caso

The epithelioid hemangioma, also called angiolymphoid hyperplasia with eosinophilia, is a rare benign vascular tumor that manifests as nodules. Histopathological examination shows vascular spaces of various sizes lined by prominent endothelium, and inflammatory infiltrates composed of eosinophils, histiocytes, mast cells and lymphocytes. We report a case of epithelioid hemangioma characterized by skin lesion in the left upper limb. This is an unusual location, since the lesions occur most often in the head and neck.

O hemangioma epitelioide, também denominado hiperplasia angiolinfoide com eosinofilia, é um tumor vascular raro, de caráter benigno, que se manifesta como nódulos. O exame histopatológico evidencia espaços vasculares de diversos calibres, revestidos por endotélio proeminente e infiltrado inflamatório composto de eosinófilos, histiócitos, mastócitos e linfócitos. Relatamos um caso de hemangioma epitelioide caracterizado por lesão de pele no membro superior esquerdo. Essa é uma localização incomum, uma vez que as lesões ocorrem mais frequentemente na cabeça e no pescoço.

Epithelioid Haemangioma – A Diagnostic Dilemma.

Epithelioid haemangioma (EH), synonymously termed as angiolymphoid hyperplasia with eosinophilia, is a rare lesion whose etiopathogenesis is unclear. Clinically it manifests as solitary or multiple nodules in the skin and subcutaneous tissues of the head and neck region. It can also arise in the deep tissues such as muscle, bone and lymphnode. Intra oral involvement is less common and very few cases have been reported in the literature. Common intra oral sites include lip followed by buccal mucosa and tongue. Epithelioid Haemangioma affecting alveolar mucosa is very rare. We report one such case of epithelioid hemangioma of size 4x4 cm on lower right alveolar mucosa in 38 year old female patient.