ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
Subject(s)
Adult , Humans , Angiolymphoid Hyperplasia with Eosinophilia , Biopsy , Endothelial Cells , Eosinophils , Lymphocytes , Mouth , Plasma CellsABSTRACT
BACKGROUND: There has been considerable controversy about the relationship between angiolymphoid hyperplasia with eosinophilia(ALHE) and Kimura's disease. Recent reports have suggested that they are two different diseases. OBJECTIVE: The purposes of this study was aimed at evaluating the clinical and histopathological characteristics of ALHE and Kimura"s disease and differential points between them. METHODS: The hospital charts and histopathologic slides of 5 patients with ALHE and 2 patients with Kimura's disease diagnosed at Asan Medical Center from 1989 to 2000 were reviewed. We also reviewed the previously published reports of the patients with ALHE and Kimura's disease in Korea. In total, we studied 14 cases of ALHE and 29 of Kimura's disease. RESULTS: 1.Eight male and six female patients(M:F=1.3:1) were enrolled in ALHE group and twenty-four male and five female(M:F=4.8:1) in Kimura's disease. The mean age at diagnosis of ALHE was 25 years in men and 40 years in women. The mean age of Kimura's disease was 30 years in men and 33 years in women. 2.Head and neck were the most frequently involved sites in ALHE(92.9%) and Kimura's disease(89.7%). Especially, among the sites, the ear(57.1%) in ALHE and the mandible(44.8%) in Kimura's disease were the most commonly involved. One patient(7.1%) with ALHE and three(10.3%) with Kimura's disease had the lesions bilaterally. 3.Pruritus was the most common symptom in both the diseases. Lymphadenopathy was present in only one patient with ALHE, whereas 51.7% of patients with Kimura's disease had it. One patient with ALHE had underlying arteriovenous malformation. One case showing pregnancy-associated Kimura's disease was reported. Gastric lymphoma was followed in one case of Kimura's disease. 4.Laboratory investigation showed hypereosinophilia in 41.7% of patients with ALHE(5/12) and 96.4% of Kimura's disease(27/28). Elevated IgE was in 2 cases of ALHE(2/4) and 5 cases of Kimura's disease(5/6). 5.Histopathological examination revealed that angioproliferating lesion comprised of plump epithelioid or histiocytoid endothelial cells and accompanying eosinophils and lymphocytes characterized ALHE. In contrast, Kimura's disease was characterized by deeper lesions showing proliferation of vascular structure with less plump endothelial cells and more fibrotic stroma with many lymphoid follicular structures 6.ALHE was improved with CO2 laser, electrocautery, excision, embolization of underlying arteriovenous malformation and dapsone. However, Kimura's disease tended to recur despite various treatment modalities. CONCLUSIONS: In Korea, Kimura's disease was more common than ALHE. ALHE and Kimura's disease may be different diseases because they showed different clinical and histopathological features.