Use of Various Treatment Modalities for Autism Spectrum Disorder and Mental Retardation

OBJECTIVES: The purpose of this study was to investigate the use of various treatment modalities including pharmacotherapy, education-behavior therapy, and complementary alternative treatment for children with mental retardation (MR) or autism spectrum disorder (ASD) in Korea. METHODS: The sample consisted of 50 parents who have children with MR (N=28) or ASD (N=22), 38 boys, 12 girls ; mean age 14.06 (4.14) years old. A questionnaire was composed of the experienced modality, duration, cost, satisfaction etc. RESULTS: According to the results, 56.0%, 100.0%, and 36.0% of children with MR or ASD have experienced pharmacological treatment, educational-behavioral therapy and complementary alternative medicine (CAM), respectively. Children who experienced education-behavioral therapy and CAM experienced 3.52 kinds of education-behavioral therapy and 2.78 kinds of CAM, respectively. Monthly cost of pharmacological treatment was lowest among three modality categories. Regarding treatment satisfaction by parental report, the lowest score was recorded fir CAM. CONCLUSION: Parents who have a child with MR or ASD are trying many treatment modalities and feeling the burden of their treatment.

A Case of Neutrophilic Eccrine Hidradenitis in an Infant

Neutrophilic eccrine hidradenitis (NEH) is a rare neutrophilic dermatosis which is characterized by the erythematous papules or plaques with a neutrophilic infiltrates around eccrine glands and coils. In 90% of cases, NEH develops in patients who have received chemotherapy for malignant diseases, but other drugs, infections, and paraneoplastic phenomenons are considered as possible etiologic factors. Furthermore, NEH is known to be extremely rare in infants. Herein we report a case of NEH in nine-month-old infant with acute myelogenous leukemia (AML), which lesions appeared on the extremities including palms and soles before starting chemotherapy.

A Case of Epithelioid Sarcoma in a Child

Epithelioid sarcoma is a rare distinctive malignant neoplasm of soft tissue with frequent recurrences and late metastases. It typically affects young adult males, and epithelioid sarcoma arising in the first decade of life is very rare. Epithelioid sarcoma may be confused with a wide range of benign, especially granulomatous, diseases and other malignancies, but the differentiation can be made, in general, by the characteristic clinical, histopathologic, and immunohistochemical features. We report herein a case of epithelioid sarcoma arising on the volar side of the right 5th finger in an 8-year-old boy. To our knowledge, this is the first report of epithelioid sarcoma in a Korean child.

Angiolymphoid Hyperplasia with Eosinophilia and Kimura's Disease: a Clinico-Pathologic Study in Korea / 대한피부과학회지

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.

A Case of lupus vulgaris diagnosed with polymerase chain reaction / 대한피부과학회지

Lupus vulgaris is the most common form of cutaneous tuberculosis showing various clinical manifestations. Because AFB staining and culture are often negative, diagnosis can be difficult. Polymerase chain reaction with high sensitivity and specificity may improve the diagnostic panel for cutaneous tuberculosis. We report a case of lupus vulgaris in a 5 year-old girl. The presence of Mycobaterium tuberculosis DNA in a skin biopsy demonstrated by polymerase chain reaction led to the diagnosis of lupus vulgaris.

Porokeratosis: Clinical Observation of 29 Cases

BACKGROUND: Porokeratosis is a disorder of epidermal keratinization which is characterized by the presence of cornoid lamellae. Since the original description of the plaque form of Mibelli, various types of porokeratosis have been reported. OBJECTIVES: The purposes of this study were aimed at evaluating the clinical and biological features of porokeraosis. METHODS: The hospital charts and slides of 29 patients with porokeratosis were reviewed. Only the cases showing characteristic cornoid lamellae by histopathological examinations were included. RESULTS: Female patients outnumbered the male ones (M:F = 1:2.2). Ages of the onset of porokeratosis varied from infancy to 68 years. Disseminated superficial actinic porokeratosis (DSAP) was the predominant type (22 cases). The Mibelli type was seen in 3 cases and the linear type in 4 cases. Only one case had a family history of porokeratosis. Two patients had complained of pruritus in the lesions, which were the DSAP and linear type, respectively. In that linear type, squamous cell carcinoma developed. Eight patients had other associated diseases. Although 6 patients were treated with CO₂ laser, topical isotretinoin or cryotherapy, the lesions tended to recur, persist or even progress. CONCLUSIONS: Porokeratosis showed various clinical features. DSAP was the predominant type. Two patients complained of itching of the lesions, which were the DSAP and linear types, respeciively. Within that linear type, squamous cell carcinoma developed. We speculate that pruritus might be an ominous sign of malignant transformation of the porokeratosis. We suggest that the Mibelli type and linear type of porokeratosis should be either excised or destroyed, or in widespread cases close follow-up should be mandatory.