Hailey-Hailey disease(benign familial chronic pemphigus) is a rare autosomal dominant disorder characterized by blisters at sites of friction such as the neck, axillae and groin which are caused by suprabasal epidermal acantholysis. We report two cases of Hailey-Hailey disease in the one family. One of the two cases has asymptomatic multiple longitudinal white bands in the fingernails associated with typical skin lesions. The nail lesions have not been described until reported by Burge in 1992 and it may be a characteristic finding in Hailey-Hailey disease.
Acantholysis ; Axilla ; Blister ; Friction ; Groin ; Humans ; Nails ; Neck ; Pemphigus, Benign Familial* ; Skin

The monoclonal antibody Ki-1(CD30) was first described in 1982 and was reported to react selectively with Reed-Sternberg cells in Hodgkins disease, highly activated B and T cells and large cell lymphomas of both T-and B-cell origin. Lymphomatoid papulosis(LyP) is characterized by recurrent erythematous papules or nodules that undergo spontaneous healing with hyperpigmentation and scarring. Histologically, two major types of LyP can be distinguished: type A is characterized by the presence of variable numbers of Ki-1+ large, atypical lymphocytes with some Reed-Sternberg like cells and type B is characterized by Ki-1- atypical cerebriform mononuclear cells similar to those in mycosis fungoides. We report a case of Ki-1+ lymphomatoid papulosis which was histopathologically recognized as a type B lesion but represented a Ki-1 positive reaction.
B-Lymphocytes ; Cicatrix ; Hodgkin Disease ; Hyperpigmentation ; Lymphocytes ; Lymphoma ; Lymphomatoid Papulosis* ; Mycosis Fungoides ; Reed-Sternberg Cells ; T-Lymphocytes

Kaposis sarcoma is a multicentric neoplastic vascular tumor involving the skin or internal organs. We report a case of classic Kaposis sarcoma in a 69-year-old male who had positive serum cytomegalovirus antibodies and disseminated multiple erythematous to purplish colored confluent papules and edema on both lower legs. The histopathological finding showed abnormally proliferated and dilated vessels, vascular spaces, spindle cells, and extravasated erythrocytes. He was treated with radiotherapy, but died due to ventricular tachycardia.
Aged ; Antibodies ; Cytomegalovirus ; Edema ; Erythrocytes ; Humans ; Leg* ; Male ; Radiotherapy ; Sarcoma, Kaposi* ; Skin ; Tachycardia, Ventricular

We report two cases of recurrent extramarnmary Pagets disease after wide local excision. Both cases involved the scrotum and the penoserotal junction, respectively. On histopathological examination, we could observed many Paget cells confined to the epidermis and the hair follicle, but not invading the underlying dermis in both cases. There was no evidence of internal malignancy. Both cases were treated with wide local reexcision.
Dermis ; Epidermis ; Hair Follicle ; Paget Disease, Extramammary* ; Scrotum

No abstract available.
Eosinophilia*

BACKGROUND: Although therapeutic plasmapheresis (TP) is a useful procedure in removing pathogenic antibodies and toxic substances from the patient, adverse reactions could arise from the use of replacement fluids and anticoagulants. Comprehensive analysis on those adverse effects had been rarely reported in Korea. METHODS: We retrospectively investigated the clinical records and the TP records from 3,962 TP sessions for 581 patients between January 1995 and October 2008 at Asan Medical Center, and we analyzed the adverse reactions related to TP. RESULTS: Adverse reactions were seen in 142 patients (24.4%) in 348 TP procedures (8.8%). Citrate toxicity was most frequently seen in 83 procedures (23.9%) followed by chills in 72 procedures (20.7%), allergic reactions in 69 procedures (19.8%) and hypotension in 60 procedures (17.2%). Citrate toxicity, chills and allergic reactions were seen more frequently in the TP procedures using FFP than in the TP procedures using albumin (P=0.001). The prevalence of citrate toxicity was significantly lower in the cases where calcium gluconate was administered (P<0.001), while it was significantly higher in the patients whose hematocrit was below 28.5% (P<0.001). In terms of severity, the mild, moderate and severe adverse reactions were 36.8%, 56.3% and 6.9%, respectively. CONCLUSION: TP is a relatively safe method of treatment, but it is important to predict and prevent adverse reactions and to respond appropriately to these adverse reactions.
Antibodies ; Anticoagulants ; Calcium Gluconate ; Chills ; Citric Acid ; Gluconates ; Hematocrit ; Humans ; Hypersensitivity ; Hypotension ; Plasmapheresis ; Prevalence ; Retrospective Studies

Lipoid pneumonia is chronic, interstitial, proliferative inflammation resulting from aspiration of lipoid material. Mineral oil is a hydrocarbon that physicians often use to treat chronic constipation in children and adults. Mineral oil may not elicit a normal protective cough reflex and may impair mucociliary transport. We experienced a case of exogenous lipoid pneumonia caused by aspiration of mineral oil given as a laxatives confirmed by fiberoptic bronchoscopy with bronchoalveolar lavage and bronchial biopsy in a 9-month-old boy with chronic cough and radiologic evidence of parenchymal lung disease.We reported this case with a brief review of related literatures.
Adult ; Biopsy ; Bronchoalveolar Lavage ; Bronchoscopy ; Child ; Constipation ; Cough ; Humans ; Infant ; Inflammation ; Laxatives* ; Lung ; Male ; Mineral Oil ; Mucociliary Clearance ; Pneumonia* ; Reflex

No abstract available.
HLA-DR Antigens* ; Thyroid Gland* ; Thyroid Neoplasms*

To exanime in vivo tissue reactions of glass fibers, we injected glass fibers to rats subcutaneously. We made fibers of average dimensions of approximately 2 nm in diameter and 60 nm in length. After instilation of glass fiber we sacrificed rats sequentially at 1, 3 and 6 months. At 1 month after injection of glass fibers, the exposure area turned to yellow color and formed well-demarcated round mass. The average size of the mass was 1X0.3 cm. Grossly detectable mass was decreased in size at 6 months compared to 1 or 3 months. Microscopically, strong foreign body reaction to glass fibers, inflammation and fibrosis were observed until 6 months. Foreign body reaction was increased up to 3 months, but it was decreased after 6 months. In scanning electron microscope, there was many bundles of glass fibers around the inflammation area, but the size of glass fibers were gradually reduced from 1 month to months. These results suggest that subcutaneous exposure of glass fiber can provoke strong tissue reaction including foreign body granulomas, inflammation and fibrosis. But glass fiber itself did not produce any neoplastic changes.
Animals ; Fibrosis ; Foreign-Body Reaction ; Glass* ; Granuloma, Foreign-Body ; Inflammation ; Rats*

BACKGROUND: Angioleiomyoma of the skin is a rare smooth muscle tumor and may develop anywhere in the body. Comprehensive clinicopathological analysis of angioleiomyomas developed in Korean has not been reported in domestic dermatological journals. OBJECTIVE: The purpose of this study was to evaluate the clinical and pathological characteristics of angioleiomyoma and the relationship between the tumor and the nerve with immunohistochemical stains. METHODS: Fifty nine cases of angioleiomyoma were reappraised from the files of the pathology of Seoul National University, Green Cross Reference Laboratory Center and Korea Veterans Hospital. Brief clinical histories of all cases were examined and microscopic findings in 35 cases were classified according to subtypes. Paraffin blocks were recut and stained in 16 cases for desmin, smooth muscle actin and S-100 protein. RESULTS AND CONCLUSION: 1. Male to female ratio was 1:1.27. 2. The age of the patients was mostly over 20 years and the mean age was 51 years in men and 40 years in women. 3. Tumors occurred most frequently in the lower extremity(38 cases), and followed by upper extremity(18 cases), head(2 cases), and trunk(1 case). Women had predominance of the legs in location site and men showed equal distribution in the lower and upper extremities.
Actins ; Angiomyoma* ; Coloring Agents ; Desmin ; Female ; Hospitals, Veterans ; Humans ; Korea ; Leg ; Male ; Muscle, Smooth ; Paraffin ; Pathology ; S100 Proteins ; Seoul ; Skin ; Smooth Muscle Tumor ; Upper Extremity