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.

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.

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)

Bilateral Involvement of Juvenile Temporal Arteritis Associated with Kimura Disease

Juvenile temporal arteritis (JTA) is a localized nodular arteritis confined to the temporal artery without evidence of systemic inflammation, and it occurs mainly in patients younger than 50 years. From the first case report, the pathological features of JTA have been suspected to be the morphological equivalent of Kimura disease (KD), which has been supported further by the concurrent cases of JTA with KD. We present the first case of bilateral JTA accompanying KD, which was confirmed by histological and ultrasound evaluations and supports the hypothesis that JTA is a manifestation of KD. The un-excised JTA lesion was resolved completely after corticosteroid therapy with no recurrence.

Anaplastic large cell lymphoma with marked peripheral eosinophilia misdiagnosed as Kimura disease

Hypereosinophilia, defined as an absolute eosinophil count of >1,500/μL, can be caused by a number of allergic, infectious, paraneoplastic and neoplastic disorders. In cases of hypereosinophilia with lymphoid proliferation, pathological confirmation is essential to exclude either myeloid or lymphoid malignancy. A 38-year-old woman with both cervical lymphadenopathies and peripheral blood eosinophilia visited our clinic. She had already performed core biopsy of lymph nodes and diagnosed as Kimura disease at a regional hospital. At the time of our clinic visit, there were no palpable cervical lymph nodes. The blood test showed hypereosinophilia with a high total IgE level. There was no evidence of tissue infiltration of eosinophils except for duodenitis with eosinophilic infiltration. Based on these findings, she was diagnosed as Kimura disease. She treated with high-dose systemic corticosteroid (1 mg/kg) and additional immunosuppressants sequentially used cyclophosphamide and cyclosporine. However, her eosinophilia waxed and waned, and a left inguinal mass was newly found. Excisional biopsy findings showed large atypical lymphoid cells with numerous eosinophilis, and immunohistochemistry showed CD3+, CD20−, CD30+ and anaplastic lymphoma kinase (ALK). The final diagnosis was ALK-negative anaplastic large cell lymphoma. We report a case of anaplastic large cell lymphoma with marked peripheral eosinophilia misdiagnosed as Kimura disease. In the case of hypereosinophilia with lymphadenopathy, it is necessary to differentiate hematologic diseases through immunochemical staining.

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.

Basophils are increased and express increased levels of interleukin-4 in the parotid lesions of Kimura disease

BACKGROUND: Kimura disease (KD) is a systemic soft-tissue disease that leads to formation of painless masses in lymph nodes, with the highest predilection for the head and neck and especially the parotid gland. KD lesions are characterized by marked eosinophil infiltration, production of IgE and increased expression of T-helper type 2 (Th2) cytokines (interleukin [IL]-4, IL-5, etc.). Skewing to a Th2 inflammation is also demonstrated in the peripheral blood, with elevated eosinophils and high IgE levels. It is thought that basophils may play important roles in orchestrating this Th2 inflammation via IL-4 production leading to the induction of IgE synthesis as well as eosinophil infiltration. However, there are no reports as yet on the role of basophils in KD. OBJECTIVE: The present study was performed to investigate the potential role of basophils in the pathogenesis of KD. In this context we also examined the expression of IL-4 in basophils in the KD lesions. METHODS: By immunohistochemistry using a monoclonal antibody against a basophil marker ProMBP1 we investigated the number and distribution of basophils in the KD lesions. By double immunohistochemistry we analyzed the colocalization of IL-4 in basophils. RESULTS: There was an increased number of basophils infiltrating the KD parotid gland lesions as compared to that in normal control parotid tissue. By double-immunofluorescence we found that approximately 7% of IL-4-positive cells in KD patients' parotid glands were basophils. CONCLUSION: Basophils may also play a role in the pathogenesis of KD, leading to the induction of IgE synthesis and eosinophil infiltration.

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.

Expressions of interleukin-21 and interleukin-22 in Kimura's disease / 中华口腔医学杂志

<p><b>OBJECTIVE</b>To investigate the expressions of interleukin (IL)-21 (IL-21) and IL-22 in patients with Kimura's disease (KD).</p><p><b>METHODS</b>Expressions of IL-21 and IL-22 were examined immunohistochemically in 36 patients with KD and 7 normal controls. The integral absorbance (IA) of the two groups was compared. Meanwhile, clinical data were reviewed.</p><p><b>RESULTS</b>The IA of IL-21 [M(Q): 1 373 418 (1 800 926)] and IL-22 [M(Q): 462 086(484 672)] in KD was significantly higher than those in normal controls [M(Q): 70 445(44 658), 51 599(71 241), P < 0.05]. The overexpression of IL-21 was significantly associated with pruritus (Z = -1.993, P < 0.05). Moreover, IL-21 was identified for disease recurrence (Z = -2.303, P < 0.05). There was a significant association between the expression of IL-22 and the number of affected sites (Z = -1.979, P < 0.05). In addition, IL-22 was significantly higher in the high-eosinophils group than in the low-eosinophils group (Z = -2.025, P < 0.05). There was no association between IL-21, IL-22 and age, gender, laterality, maximum size.</p><p><b>CONCLUSIONS</b>IL-21 and IL-22 may be involved in the pathogenesis of KD.</p>

Two cases of neck region Kimura's disease / 临床耳鼻咽喉头颈外科杂志

Kimura's disease is a rare, benign, slow-growing chronic inflammatory swelling with a predilection for the head and neck region and is almost always with peripheral blood eosinophilia and elevated serum IgE levels. It is endemic in Asian males and rare in Western people. Surgical excision of the lesion is the first line therapy. Drug and radiation therapy have to be considered for the refractory lesions.

Report of one case Kimura's disease / 临床耳鼻咽喉头颈外科杂志

A 48-year-old middle aged male presented swelling lymph nodes and mass in neck for 5 years. Physical examination shows swollen mass in head and neck regions. The masses could be touched in bilateral parotids and neck with a little movement and moderate tenderness. The level of IgG was normal, but Eosinophi count was high. The function of heart liver and kidney was normal. The result of B-mode ultrasonography reveals bilateral parotids and subcutaneous near parotids were widely swollen and several swollen lymph nodes in neck. pathological examination displays features of a large number of lymph follicles hyperplasia, acidophilic granulocyte infiltration, capillary hyperplasia and fibrosis of different level. The disease were eventually diagnosed by pathological examination. Method of treatment includes glucocorticoid drug therapy, surgical resection and local radiotherapy. The last treatment of patients with Kimura's disease should be combined with the clinical manifestation of them to determine the individualized treatment, so as to improve the quality of life of patients.

Intralesional radiofrequency ablation for nodular angiolymphoid hyperplasia on forehead: A minimally invasive approach.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an idiopathic acquired condition characterized by erythematous papulo-nodular lesions with a predilection for the head and neck. The lesions are cosmetically disfiguring, resistant to most medical and surgical therapies and tend to recur. We report the novel use of radiofrequency equipment in the management of nodular ALHE on forehead of a 53-year-old man. Intra-lesional radiofrequency ablation was done using a modified 18 gauge intravenous cannula and three sittings over a period of four years yielded cosmetically acceptable results with no recurrence and minimal side effects.

Glomus carotídeo bilateral. Reporte de un caso / Bilateral glomus caroticum. case report

Los glomus o paragangliomas carotídeos son tumores de crecimiento lento, hipervascularizados, poco frecuentes, derivados del cuerpo carotídeo. El objetivo es presentar un caso poco frecuente de tumores glómicos carotídeos bilaterales. Para su diagnóstico se realizó angiorresonancia magnética y angiografía de cuello. Se encontró una tumoración bilateral hipervascularizada a nivel de la división de la arteria carótida común, con signo de la lira...

Carotid glomus or paragangliomas are slow growing tumors, highly vascularized, rare, originated from the carotid body. The objective is to present a very rare case of bilateral carotid glomic tumors. Neck angioresonance was performed for diagnosis. A highly vascularized tumor was found bilaterally in the bifurcation of the common carotid artery, showing the lyre sign...

Remission of secondary membranous nephropathy in a patient with Kimura disease after surgical resection

Kimura disease (KD) is an eosinophilic, granulomatous, benign, chronic inflammatory disease with an unknown etiology. A 33-year-old woman visited our hospital because of a palpable, left subclavian mass, a left scapulo-anterior pseudoaneurysm, and nephrotic syndrome. Her subclavian lymph node biopsy examination result was consistent with KD, and results of a renal biopsy indicated secondary membranous nephropathy. After renal histological examination confirmed nephropathy, treatment with prednisolone and cyclosporine was initiated, which was maintained for over 1 year. However, this therapy only provided a transient improvement in proteinuria. One year after commencing the treatment, both proteinuria and azotemia aggravated as the left axillary mass doubled in size. Finally, the mass was surgically excised, following which the azotemia rapidly normalized and proteinuria resolved within 1 month. This case shows that tumor resection in a patient with KD with secondary nephropathy may resolve secondary renal manifestations. Furthermore, reversible renal dysfunction may be caused by unknown secreted molecules.

Granuloma piógeno como diagnóstico diferencial de las masas del vestíbulo nasal / Pyogenic granuloma as differential diagnosis of nasal mass the hall: case report

Las masas del vestíbulo nasal son tumores poco frecuentes. Pueden dividirse en dos grupos; benignos como el papiloma invertido, angiofibroma juvenil y malignos como el carcinoma escamolacular. El granuloma piógeno corresponde a una hiperplasia inflamatoria con tejido de granulación de muy baja aparición. Se presenta el caso de una mujer de 34 años con masa nasal diagnosticada como carcinoma escamolacular del vestíbulo nasal, a quien se le realizo recesión amplia de la lesión...

The masses of the nasal vestibule are rare tumors. They can be divided into two groups: benign such as Inverted Papilloma and Juvenile Angiofibroma and malign as Escamolacular Carcinoma. Pyogenic granuloma corresponds to an inflammatory granulation tissue hyperplasia with very poor appearance. It is presented the case of a 34 year old woman diagnosed with nasal mass as Escamolacular Carcinoma of the nasal vestibule, a wide recession of the lesson was performed...