Letterer-Siwe disease is a non-lipid reticuloendotheliosis of unknown etiology and usually grouped with Hand-Schuller-Christian disease and eosinophilic granuloma as components of histiocytosis-X. It occurs mostly in infancy and, clinically it is the most generalliaed and serious. In recent years, the prognosis of infants with Letterer-Siwe disease has improved, primarily due to more aggressive therapy with corticosteroids and antimetabolic agents. The 14 months old male patient was admitted into the Severance Hospital on 15th, July 1974 for evaluation of a chronic and refractory skin eruptions, abdominal distension with diarrhea and edema on extremities. He had been well until 3 months old age when he developed seborrhea-like skin eruptions which did not improve on treatment for seborrhea. Vigorous treatment was started for the baby from the beginning of admission day. Routine laboratory tests, skin biopsy, bone marrow aspiration and radiologic studies for bony structures wer done. All those studies had showed severe anemia, thrombocytopenia, multiple bony defects in skull and numerous non-lipid histiocytic infiltration in skin and bone marrow all those were consist with Letterer-Siwe disease.
Adrenal Cortex Hormones ; Anemia ; Biopsy ; Bone Marrow ; Dermatitis, Seborrheic ; Diarrhea ; Edema ; Eosinophilic Granuloma ; Extremities ; Histiocytosis, Langerhans-Cell* ; Humans ; Infant ; Male ; Prognosis ; Skin ; Skin Tests ; Skull ; Thrombocytopenia

Dyskeratosis congsnita is a rare congenital disorder characterized by the triad of reticular pigmentation of the skin. dystrophic naila, and leukoplakia of the mucous membrane, and is often associated with severe pancytopenia. A 9-year-old boy had reticular pigmentation of the skin, dystropbic changes of the finger and toe nails, white patches of the buccal mucosa, mild hyperkeratosia of the palms and soles, excesaive lacrimation, dysphagia and severe pancytopenia, Bone marrow showed hypoplastic anemia and decreased cell mediated immunity was noticed.
Anemia, Aplastic ; Bone Marrow ; Child ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Deglutition Disorders ; Dyskeratosis Congenita* ; Fingers ; Humans ; Immunity, Cellular ; Leukoplakia ; Male ; Mouth Mucosa ; Mucous Membrane ; Pancytopenia ; Pigmentation ; Skin ; Toes

The present study evaluates the type, frequency and location of basal cell epithelioma, as well as the age and sex distribution of patients and the recurrence rate of basal cell epithelioma. In this study 116 patients, who visited Severance hospital during the last 15 years from Jan. 1, 1967 to Dec. 31, 1981 and were diagnosed histopathologically as having basal cell epithelioma, were analysed. (countinued..)
Carcinoma, Basal Cell* ; Humans ; Recurrence ; Sex Distribution

Kaposi's sarcorna is a tumor-like condition which characterized by a mixed proliferation of fibroblastic components and vascular structures. The authors report a case of nodular type of Kaposis sarcoma in 54 year-old male who had an eroded hemorrhagic nodule and few papular lesions. These lesions involved only both hands and feet without systemic involvement.
Cytochrome P-450 CYP1A1 ; Fibroblasts ; Foot ; Hand ; Humans ; Male ; Middle Aged ; Sarcoma* ; Sarcoma, Kaposi

The cutaneous metastaaes from carcinoma of breast produce four definite clinical types: inflammatory carcinoma, telangiectatic carcinoma, nodular carcinoma, and carcinoma en cuirasse. We present a case of 51-year-old woman who had purpuric, hard, sclerotic plaque with ulceration, crust and. several pea-sized hemorrhagic papulovesicular eruptions on the right chest wall for one year. Histopathological findings show metastatic ductal carcinoma but, the primary focus was not determined.
Breast ; Carcinoma, Ductal ; Female ; Humans ; Middle Aged ; Thoracic Wall ; Ulcer

We described a 65-year-old woman with a papule on the left temple area of } year duration. The lesion showed typical findings of atypical fibroxanthoma and was located on the lesion of solar keratosis, So sunlight may be the most important one among the various insults to atypical fibroxanthoma of the skin.
Aged ; Female ; Humans ; Keratosis ; Skin ; Sunlight

Keratoacanthoma is a benign neoplasm that develops as a protruding mass especially on the central part of face, We experienced a case of keratoacanthoma that developed on the nasal vestibule after the eradication of verruca vulgaris on the same site by surgical intervention 3 months before. We treated it with total excision and skin graft.
Keratoacanthoma* ; Skin ; Transplants ; Warts*

A case of lymphangioma circumscriptum which was hard to diagnose because of complicated clinical pictures, was described and the pathogenesis of the disease was reviewed, The 23 year-old male patient had palm sized, verrueous, oozing and crusted patch with some grouped vesicles on the right upper thigh since childhood. Histopathologically the verrucous lesion showed marked hyperkeratosis, acanthosis in the epidermis and proliferation and cystic dilatation of lymph vessels lined by a single layer of endothelium in the dermis, subcutis and muscular tissues. He was treated with surgical excision and skin graft and showed no recurrence over 3 months.
Dermis ; Dilatation ; Endothelium ; Epidermis ; Humans ; Lymphangioma* ; Male ; Recurrence ; Skin ; Thigh ; Transplants ; Young Adult

Congenital ichthyosiform erythroderma is most severe type of ichthyosis. This disease is characterized clinically by generallized erythroderma covered with flaccid bullae and verruciform scales which are especially prominent on the flexural areas. This disease demonstrates an autosomal dominant mode of inheritance. The distinctive histologic change is the extreme hyperkeratosis associated with granular and vacuolar degeneration of the mid and upper malphigian layers. Cell kinetic studies indicate an increase in the epidermal mitotic turnover tirne. This 8 months old male patient visited to Dermatologic Department of Severance Hospital on April-28, 1973. Seven days after birth the scales were appeared on his flexural region of limbs and shed to leave a raw surface which forms scales anew. And there were the intermittent appearance of flaccid bullae prominantly involve the flexural area of limb but thereafter appearing the other region of body. Skin biopsy shows that of bullous type of congenital ichthyosiform erythroderma. This baby was expired 4 days after the admission day in spite of vigorous systemic and local trea- tments. The autopsy findings had exhibited fatty necrotic degenerative changes of hepatic cells and septic inflammatory changes of meningeal vessels and meninges itself as the causes of death of this pitiful victim.
Autopsy ; Biopsy ; Cause of Death ; Dermatitis, Exfoliative ; Extremities ; Hepatocytes ; Humans ; Ichthyosiform Erythroderma, Congenital ; Ichthyosis ; Infant ; Male ; Meninges ; Parturition ; Skin ; Weights and Measures ; Wills

Cheilitis glandularis simplex, the clinical term first defined by Puente and Acevedo in 1935, is characterized by the development of hyperplastic mucous glands in the lips, associated with dilated ductal canals; it is manifested clinically by a usually painless swelling of the lips and a variably constant, bothersome, sticky mucoid discharge. Recently we have experienced a case of cheilitis glandularis simplex in a 13-year-old boy and an excellent cosmetic result was obtained after surgical excision and repair.
Adolescent ; Cheilitis* ; Humans ; Lip ; Male