A case of Wegener's granulomatosis is described, with special attention focused on the typical histologic findings and involvement of both middle ear and lung. The patient is a 37-year-old man presented with four-month history of cough and sputum. He had a past history of surgery of both ears because of otitis media followed by left facial palsy. Chest radiographs showed variable sized ill defined nodules in both lower lobes with internal airspace consolidation. Histologic preparations of the open lung biopsy specimens demonstrated a diffusely scattered palisading micro and macrogranulomas with central focus of neutrophils and necrotic collagen surrounded by histiocytes, histiocytic giant cells. Fibrinoid necrosis involved blood vessels and lung parenchyma. Chronic inflammation, diffuse granulation tissue formation and irregular fibrosis are also found in the lung parenchyma. The histologic findings of middle ear which was previously biopsied showed scattered palisading ill defined microgranulomas mixed with fibrotic tissue.
Adult ; Biopsy ; Blood Vessels ; Collagen ; Cough ; Ear ; Ear, Middle* ; Facial Paralysis ; Fibrosis ; Giant Cells ; Granulation Tissue ; Granuloma ; Histiocytes ; Humans ; Inflammation ; Lung* ; Necrosis ; Neutrophils ; Otitis Media ; Radiography, Thoracic ; Sputum ; Vasculitis ; Wegener Granulomatosis*

Benign pigmented lesions derived from dermal melanocytes include the Mongolian spot, the nevi of Ota and Ito, and the blue nevus. The nevus of Ito differs from the nevus of Ota by its location in the supraclavicular, scapular and deltoid regions. It occurs rarely in association with bilateral nevus of Ota. We present a 70 years old male, showing extensive involvement of dermal melanocytic nevi, so called bilateral nevus of Ota associated with bilateral nevus of Ito.
Aged ; Humans ; Male ; Melanocytes ; Mongolian Spot ; Nevus of Ota* ; Nevus* ; Nevus, Blue ; Nevus, Pigmented

BACKGROUND: Pityriasis rubra pilaris (PRP)is a rare papulosquamous disorder of unknown etiology. Although the clinical and histopathologic features of PRP are relatively well known in western patients, there are few available studies in Korea. OBJECTIVE: The purpose of this study was to evaluate the clinical and histopathologic features of pityriasis rubra pilaris(PRP) in Korea METHODS: The clinical charts, photographies and skin biopsy slides of patients clinically diagnosed as PRP at the initial visit were reviewed. Finally 30 patients were diagnosed as PRP. They were classified into 5 groups according to the Griffiths classification. And then the evaluation of age at onset, sex distribution, clinical feature of the skin lesion, associated symptoms, clinical course and histopathologic features were conducted. RESULTS: 1) The male to female ratio was 1:1.14. The age of onset was varied from 1 to 65 years with two peaks in first and fifth decades. The average ages of onset in adult type and juvenile type were 40.1 and 5.1 years respectively. 2) According to the Griffiths classification, 14(47%) of the patients had type I, 4(13%) had type III, 7(23%) had type IV, and 3(10%) had type V. However, 2(7%)patients could not be classified under any of these five types due to atypical clinical feature or insufficient follow-up periods. 3) The common clinical features during the disease course were follicular hyperkeratosis(83%), knee and/or elbow involvement(83%), palm and/or sole hyperkeratosis(80%). Erythroderma was present in 5 patients(17%) during the disease course. 4) There were no specific symptoms in most cases, but 9 patients had ben suffered from mild itching. 5) 3 patients had a positive family history, and were thought to have inherited autosomal dominant trait. 6) The frequent histopathologic findings were alternating parakeratosis and orthokeratosis (100%), superficial perivascular infiltration(100%), broad rete ridge(97%), acanthosis(93%), hypergranulosis (87%), follicular plugging(73%). CONCLUSION: We investigated the clinical and histopathological features of PRP in Korea. In our study, the clinical and histopathological features of PRP were not different from those observed in western patients.
Adult ; Age of Onset ; Biopsy ; Classification ; Dermatitis, Exfoliative ; Elbow ; Female ; Follow-Up Studies ; Humans ; Knee ; Korea ; Male ; Parakeratosis ; Photography ; Pityriasis Rubra Pilaris* ; Pityriasis* ; Pruritus ; Sex Distribution ; Skin ; Skin Diseases, Papulosquamous

A case of primary melanoma of the spinal cord in 20 year-old male is presented. The symptoms and signs were those of cervical cord compression caused by the melanoma, for which underwent laminectomy and was histologically verified.
Humans ; Laminectomy ; Male ; Melanoma* ; Spinal Cord* ; Young Adult

According to Moertel's classification, synchronous multiple gastric cancer is eonsidered to be a sort of multiple primary cancer, The clinical signifieance of multiplicity in gastric cancer was its location relative to the resection line. Compared with patients with single gastric cancer, multiple gastric cancer were more frequently found among the older men, and they were more commonly found in early gastric cancer. The frequency of multifocality in gastric cancer is 2.2-9% in the world literature reports and is increasing recently, with advance in the diagnostic method of gastric cancer. However, synchronous multiple gastric cancer which has more than four foci is rare. In Korea, there has been only one case reported about quadruple gaatric cancer. Recently, we experienced a case of a 58-year-old male patient with synchronously developed-quadruple gastric cancer on the body of stomach, for whom radical total gastrectomy and esophagojejunostomy was done. All of them were moderately differentiated adenocarcinoma. The mapping about four lesions showed that Borrmanin type III AGC extending to the serosa was placed on the posterior wall of higher body; EGC IIc invading the submucosa, the anterior wall of mid body, EGC III confined to the mucosa, the lesser curvature side of mid to lower body; EGC III localizing to the mucosa, the posterior wall of lower body. So we reported this case with a review of literatures.
Adenocarcinoma ; Classification ; Gastrectomy ; Humans ; Korea ; Male ; Middle Aged ; Mucous Membrane ; Serous Membrane ; Stomach ; Stomach Neoplasms*

Multiple primary or secondary malignancies after anticancer therapy were recently reported to be increasing in frequency. The authors describe a case of metachronous metastatic pulmonary basaloid carcinoma to the central nervous system that was discovered after chemotherapy and radiation therapy for cervical uterine carcinoma. Two different types of cancer developed within some interval. There's the possibility that a secondary pulmonary neoplasm developed after the chemotherapy and radiotherapy conducted as cervical cancer treatment.
Central Nervous System ; Drug Therapy* ; Lung Neoplasms ; Neoplasm Metastasis* ; Neoplasms, Second Primary ; Radiotherapy ; Uterine Cervical Neoplasms

BACKGROUND: Pilomatricoma is a cystic neoplasm with differentiation toward hair cortex cells. Although it is characterized by several features such as cellular constitiuents, stromal change and inflammation, it is difficult to interpret the histopathogenesis of the lesions, and there is no comprehensive understanding about the evolution of the tumor. OBJECT: The purpose of this study was to characterize the clinical and histopathologic feature of pilomatricoma and to suggest the growth pattern and evolutional stage of this tumor. METHOD: Thirty-eight cases with pilomatricoma in 34 patients who had visited Korea University Hospital during the last 10 years have been collected. We studied the clinical and histopathological characteristics of the cases and tried to classify evolutional stages of them and to elucidate the growth pattern. RESULTS: Most of the tumors were solitary lesions except four patients, who showed two lesions simultaneously. The male to female ratio was 1:1.1. The average age was 16.25 years (9months to 43 years) and 35 cases(92%) were developed in the patients younger than 30 years old. The predilection sites were arm(61%), face(18.2%), neck(13%), thigh(5.2%), shoulder(2.6%) in order. The duration of lesions was from 10 days to 3 years in known cases, with mean duration of 9.8months. Histopathologically tumor size was from 0.3cm to 1.9cm in diameter, with average value of 0.89cm. 29 cases showed inflammatory reaction variably in its severity involving giant cells. Calcification was observed in 22 cases and two of them showed ossification. According to four stage system of previous study, we categorized our cases into the 3 early stage, 8 fully developed stage, 15 early regressive stage, and 12 late regressive stage. Peripheral basaloid epithelium surrounding the tumor was found in 17 cases. 3 cases were surrounded by epithelium perfectly, and 5cases were surrounded by half or more. CONCLUSION: Pilomatricoma is not a cyst but a cystic neoplasm that consists principally of rapidly growing basaloid cells that form well-defined lobular aggregations of diverse sizes and shapes. It shows variable features with differentiation. Since previous four staging system were not clearly differentiated in each stage, we divided pilomatricoma into three phases, developing phase, proliferative phase and regressive phase, according to the constituents of basaloid cells in the periphery.
Adult ; Epithelium ; Female ; Giant Cells ; Hair ; Humans ; Inflammation ; Korea ; Male ; Pilomatrixoma*

Growth hormone (GH) has been available for more than 4 decades for the treatment of GH deficiency. But mass production of recombinant DNA growth hormone has made GH therapy widely available for children with no GH deficiency. The use of GH therapy in children has resulted in adverse effects ranging from minor disturbances such as edema and injection site reactions to more significant, but rare events such as benign intracranial hypertension and slipped capital femoral epiphysis. Yet there has been no report in the dermatological field on skin adverse effects associated with GH therapy. We report here on 2 cases of psoriasis following GH therapy in children.
Child ; DNA, Recombinant ; Edema ; Growth Hormone ; Humans ; Pseudotumor Cerebri ; Psoriasis ; Skin ; Slipped Capital Femoral Epiphyses

Primary small cell neuroendocrine carcinoma in stomach is known to be very rare and only the twelve cases have been reported in the English literature. This tumor appears to be analogous to small cell carcinoma and carcinoid tumors of the lung, and is characterized by a very aggressive clinical course. Recently, we have experienced a 68-year-old man with primary small cell neuroendocrine carcinoma in the stomach, which had liver metastasis and peritoneal seeding. A positive Grimelius stain was present and immunohistochemical studies revealed positivity for neuron-specific enolase in the tumor. For its rarity, we report this case with review of literatures.
Aged ; Carcinoid Tumor ; Carcinoma, Neuroendocrine* ; Carcinoma, Small Cell ; Humans ; Liver ; Lung ; Neoplasm Metastasis ; Phosphopyruvate Hydratase ; Stomach*

McLeod syndrome is a rare X-linked multisystem disorder which forms the core of neuroacanthocytosis syndrome. Neurological symptoms characterized by chorea, seizure, cognitive impairment, and psychosis mostly develop around the 5-6th decades, accompanied by multisystem involvement comprising neuropathy, myopathy, acanthocytosis and hepatosplenomegaly. We hereby present a 60-year-old male who is the first genetically confirmed Korean McLeod syndrome patient. Genetic analysis of his XK gene revealed a previously reported 5 base pair deletion of exon 3 (c.856_860delCTCTA).
Abetalipoproteinemia ; Base Pairing ; Chorea ; Cognition Disorders ; Exons ; Humans ; Korea* ; Male ; Middle Aged ; Muscular Diseases ; Neuroacanthocytosis ; Psychotic Disorders ; Seizures