Kasabach- Merritt syndrame consists of thrombocytopenia, microangiopathic hemolytic anemia, and an acute or chronic censumptive coagulopathy in associati an with a rapidly enlarging hemangioma. Most vascular turrnrs causing Kasabach-Merritt syndon e are benign but the associated coagulopathy may be lifethreatening. Our patient, a 30-day-old female infant, was admitted after an increase in the size of a hemangioma present since birth on the right leg with extension to the lower abdomen and left inguinal area. Her herrnglobin count fell to 7.1g/dl, platelet count was 26,000/ul, prothrombin time was 26 seconds, and hypofibrinogenemia developed. The patient was treated with transfusions of platelets, fresh frozen plasma and red cells and prednisolone. for 2 weeks without improvement. We started interferon alfa-2a therapy. Over the past 6 months, he hemangioma progressively diminished in size withcut any serious complication.
Abdomen ; Anemia, Hemolytic ; Female ; Hemangioma ; Humans ; Infant* ; Interferon-alpha* ; Interferons* ; Kasabach-Merritt Syndrome ; Leg ; Parturition ; Plasma ; Platelet Count ; Prednisolone ; Prothrombin Time ; Thrombocytopenia

Generalized sterile pustular eruptions occur in various dermatoses including pustular psoriasis, erythema multiforme, Sneddon-Wilkinsan disease and others. Recentlr, acute eruptions of disseminated sterile pustules have been reported. The etiology is not related to a gepetic predisposition to psoriasis but to drug ingestion or viral infections, thus termed toxic pustuloderm; (T. P.). In this report, we present a typical case of T. P. observed iu our department. Our patient, an 18-year-old male, with no personal or family history of psoriasis, was given drug medication including amoxicillin for fever and chilling sensation. Pustilar eruptions first, appeared on his face about two days aft.er the medication and gradually spread to the trunk and limbs. The skin examination revealed numerous small pustules on an erythematous base. Laboratory examination revealed neutrophilic leukocytosis and an elevated sedimentation rate skin biopsy showed subeorneal and spongiform neutrophilic pustules Upon interruption of the amoxicillin, the pustules cleared rapidly in 3 days and there has been ri.o recui rence of any rash over a 7-month follow-up period.
Adolescent ; Amoxicillin ; Biopsy ; Eating ; Erythema Multiforme ; Exanthema ; Extremities ; Fever ; Follow-Up Studies ; Giant Cell Tumors ; Humans ; Leukocytosis ; Male ; Neutrophils ; Psoriasis ; Sensation ; Skin ; Skin Diseases

Darier's disease is a well-known genodermatosis characterized by recurrent waxy, hyperkeratotic papules usually occurring over the seborrheic area. The major histopathological changes are characteristic acantholysis and dyskeratosis which are diagnostic of the disease with typical clinical features. There are less common variants including the hypertrophic, vesicobullous and linear type. However, comedonal lesions are very rare. We report an unusual case of Darier's disease, which showed prominent comedonal papules and plaques over the face, scalp and upper trunk with the typical findings of Darier's disease.
Acantholysis ; Darier Disease* ; Scalp

Radiation induced pemphigus, as an example of induced pemphigus, is a rare disease which occurs in patients receiving radiotherapy. A 56-year-old male patient with nasopharyngeal cancer received 3 cycles of chemotherapy and radiotherapy on the neck and anterior chest areas. One month after completion of radio-therapy, eroded and crusted areas developed within the irradiation site. In spite of systemic antibiotic treatment, the skin lesions persisted and spread further to the trunk and upper extremities with new crops of bullae. He was treated with a high dose of prednisolone and the skin lesions cleared. We discuss the suggestive pathogenesis of radiation-induced pemphigus with the comparative results of immunoblotting in various variants of pemphigus.
Cytochrome P-450 CYP1A1 ; Drug Therapy ; Humans ; Immunoblotting ; Male ; Middle Aged ; Nasopharyngeal Neoplasms ; Neck ; Pemphigus* ; Prednisolone ; Radiotherapy ; Rare Diseases ; Skin ; Thorax ; Upper Extremity

BACKGROUND: Extensive study of the p53 gene has established its role as a tumor-suppressor gene, and the mutant p53 gene is expressed in a wide spectrum of human malignancy. The conversion of sulfhydryl groups to disulfide bonds in epidermis is carried out by skin sulfhydryl oxidase(SSO) at the junction of the granular layer and horny layers. So it is of interest to investigate the localization and properties of SSO in epidermal keratinocytic neoplasms. OBJECTIVE: Our purpose is to investigate the expression pattern of the p53 protein and SSO and to gain a better understanding of biologic behavior in epidermal keratinocytic neoplasms. METHODS: We investigated the expression pattern of p53 protein and SSO by immunohistochemical method on formalin-fixed, paraffin-embedded tissue specimens in 8 cases of squamous cell carcinomas, several cases of recancerosis(1 Bowen's disease, 1 actinic keratosis, 1 arsenic keratosis) and benign keratinizing disorders(1 verruca vulgaris, 1 condyloma accuminatum and 2 cases of lichen planus). RESULTS: 1. Seven out of 8 cases of squamous cell carcinama(88%) and 1 case of 1 Bowen's disease showed p53 protein expression 2. SSO was weakly present in squamous cell carcinoma and precancerosis compared to the normal skin. In the well differentiated squamous cell carcinoma and Bowen's disease, the presence of SSO was rather diffuse than poorly differentiated squamous cell carcinoma and concentrated in dyskeratotic cells and horn pearls. 3. p53-stained cells or areas were seldom stained with SSO antibody, especially in invading borders of squamous cell carcinomas. CONCLUSION: Our results suggest that p53 mutation is a common genetic change in epidermal tumorigenesis and it can be useful marker of tumor invasion in the squamous cell carcinoma and precacerosis of skin.
Animals ; Arsenic ; Bowen's Disease ; Carcinogenesis ; Carcinoma, Squamous Cell* ; Epidermis ; Genes, p53 ; Horns ; Humans ; Keratosis, Actinic ; Lichens ; Oxidoreductases* ; Skin* ; Warts

No abstract available.
Diagnosis, Differential*

BACKGROUND: Basal cell carcinoma(BCC) is the most common primary cutaneous neoplasm in Korea. Since the majority of BCCs occur on the head and neck and the goal of the BCC treatment is the complete removal of tumor, Mohs micrographic surgery is ideally suited for maximizing cure rate with minimizing tissue loss. OBJECTIVE: The purpose of this study is to evaluate the depth of excision as well as the lateral margins of BCC and to correlate these with the clinicopathologic aspects of the tumors. METHODS: Twenty patients with 21 BCCs (10 primary, 11 recurrent) diagnosed in the Department of Dermatology of Dong-A University Hospital from March, 1992 through December, 1993 were studied prospectively. RESULTS: 1. Sixty percent of tumors in primary BCCs in our series had a 2-mm maximal margin and 81.8% of recurrent BCC were eradicated with 4-mm or more lateral margin. 2. All the recurrent basal cell carcinomas (8 cases) measuring greater than 15-mm required more than 4-mm margin for the total removal. 3. More than 90% of BCC on the nose in our series required the removal of periosteum/perichondrium or the excision of whole layer. CONCLUSION: Mohs micrographic surgery is an appropriate tool to use in proposing guidelines for the treatment of skin cancer because the most accurate method of determining the actual extent of skin cancer can be achieved.
Carcinoma, Basal Cell* ; Dermatology ; Head ; Humans ; Korea ; Methods ; Mohs Surgery* ; Neck ; Nose ; Prospective Studies ; Skin Neoplasms

PURPOSE: To evaluate the feasibility and clinical efficacy of transrectal drainage of a deep pelvic abscess using combined transrectal sonographic and fluoroscopic guidance. MATERIALS AND METHODS: From March 1995 and August 2004, 17 patients (9 men; 8 women; mean age, 39years) suffering from pelvic pain, fever and leukocytosis were enrolled in this retrospective study. Ultrasound (US) or computed tomography (CT), which was obtained prior to the procedure, showed pelvic fluid collections that were deemed unapproachable by the percutaneous transabdominal routes. Transrectal drainage of the pelvic abscess was performed under combined transrectal sonographic and fluoroscopic guidance. The causes of the deep pelvic abscess were postoperative complications (n=7), complications associated with radiation (n=3) and chemotherapy (n=1) as well as unknown causes (n=6). A 7.5-MHz end-firing transrectal US probe with a needle biopsy guide attachment was advanced into the rectum. Once the abscess was identified, a needle was advanced via the biopsy guide and the abscess was punctured. Under US guidance, either a 0.018"or 0.035" guidewire was passed through the needle in the abscess. Under fluoroscopic guidance, the tract was dilated to the appropriate diameter with sequential fascial dilators, and a catheter was placed over the guide wire within the abscess. Clinical success of drainage was determined by a combination closure of the cavity on the follow up images and diminished leukocytosis. The technical and clinical success rate, complications, and patient's discomfort were analyzed. RESULTS: Drainage was technically successful in all patients and there were no serious complications. Surgery was eventually performed in two cases due to fistular formation with the rectum and leakage of the anastomosis site. The procedure was well tolerated in all but one patient who complained of discomfort while the catheter was inserted. The catheter did not interfere with defecation and there was no incidence of catheter expulsion by defecation. CONCLUSION: Transrectal drainage of deep pelvic abscesses using ultrasound and fluoroscopic guidance is a safe, feasible procedure that is well tolerated by patients and is relatively easy to perform.
Abscess* ; Biopsy ; Biopsy, Needle ; Catheters ; Defecation ; Drainage* ; Drug Therapy ; Female ; Fever ; Follow-Up Studies ; Humans ; Incidence ; Leukocytosis ; Male ; Needles ; Pelvic Pain ; Postoperative Complications ; Rectum ; Retrospective Studies ; Ultrasonography*

Sjogren's syndrome is a chronic autoimmune disorder characterized by lymphocytic infiltration of the lacrimal and salivary glands, leading to dryness of eyes (kerato-conjunctivitis sicca) and mouth (xerostomia). The skin lesions in Sjogren's syndrome are usually manifested as xeroderma, but sometimes appear as annular erythema or vasculitis. Central nervous system symptoms may be presented as one of extraglandular manifestations, though rare in incidence, and need differential diagnosis from multiple sclerosis. We report a case of a 45-year-old woman diagnosed as multiple sclerosis at first but later as neurologic manifestation of primary Sjogren's syndrome, showing signs of multiple sclerosis and cutaneous erythematous lesions.
Case Report ; Diagnosis, Differential ; Erythema/pathology ; Erythema/diagnosis* ; Female ; Human ; Middle Age ; Multiple Sclerosis/pathology ; Multiple Sclerosis/diagnosis* ; Sjogren's Syndrome/pathology ; Sjogren's Syndrome/diagnosis*

Median raphe cyst of the penis is an uncommon entity that usually presents as a cystic lesion on the ventral aspect of the penis, and which has a broad spectrum of histopathological appearances. It occurs most commonly near the glans penis, but may occur anywhere from the urethral meatus to the anus. It is solitary and measures only a few millimeters in diameter. Surgical excision is the treatment of choice. Recently we observed an 18-year-old male patient who had had a median raphe cyst since 5 years ago near the urethral orifice of the glans penis.
Adolescent ; Anal Canal ; Humans ; Male ; Penis*