Erythema elevatum diutinum (EED) is emerging as a specific HIV-associated dermatosis which can be easily misdiagnosed as Kaposi's sarcoma or bacillary angiomatosis. Until now, no case of HIV-associated EED had been reported in Korea. We report a case of EED in a 49-year-old man with HIV infection. The patient was diagnosed with HIV-infection and treated with a combination of anti-retroviral agents and dapsone. Two years after the start of treatment the lesion had regressed.
Angiomatosis, Bacillary ; Anti-Retroviral Agents ; Dapsone ; Erythema ; HIV ; HIV Infections ; Humans ; Korea ; Middle Aged ; Sarcoma, Kaposi ; Skin Diseases ; Vasculitis, Leukocytoclastic, Cutaneous

The port-wine stain has always been a difficult lesion to treat cosmetically. Skin biopsoes prior to treatment port-wine stains and at 11- 42 months after CO2 laser treatment of port-wine stain were taken in 6 patients of both sexes. Histology after CO2 laser surgery in successfully treated lesions is remarkable for a few small-caliber dilated vessels with relativelly free of erythrocytes. The histology after CO2 laser surgery also revealed a large amount of collagen deposited and a few appendageal structures in the dermis.
Angiomatosis ; Therapeutics ; Diagnosis ; lasers

Meyer-Schwickerath(1959) has successfully treated the angiomatosis tumor by means of Xenon-light coagulation with minimal damage to the affected eye ball. Though cryothenny was first tried by Lincoff et al. (1967) it resulted only in the shrinkage of the tumor. Amoils and Smith(1969) succeeded through repeated application of cryothermy to completely destory the tumor itself which was followed by disapperance of the surrounding choroidal and retinal vessels. In our case, an angiomatosis tumor in the right eye, one and a half times the size of the optic disc, was shrunk to two-thirds of the disc size by an application of cryothermy, repeated three times at minus 60 degrees C for about two minutes at varying intervals. Then the tumor was completely destroyed by means of Xenon-arc photocoagulation repeated two times each at about three weeks' intervals. In the left eye, even though the angiomatosis retinae was complicated by secondary retinal detachment, the tumors were partially destroyed by diathermy application in the same manner that ignipuncture is ordinarily done. Subsequently after reattachment of the retine by performing an encircling buckle procedure with autogenic fascia lata, the tumors were completely destroyed by means of Xenon-arc photocoagulation, with prevention of the complications of angiomatosis retinae such as secondary glaucoma or phthisis bulbi. Angiomatosis tumor can be destroyed by cryothermy but in the case of a large tumor, cryothermy will be required in repeated applications. This may cause atrophy of choroid and retina so severe that a distinct line of demarcartion between the diseased and normal retina will be formed. Therefore repeated applications of cryothermy increase the danger of rupture of the retina. According cryothermy will be effective only in the treatment of small angiomatosis tumor. In the case of a large angiomatosis tumor or other complicated condition, Xenon-arc photocoagulation is an easy and suitable method of treatment to destroy the tumor.
Angiomatosis* ; Atrophy ; Choroid ; Diathermy ; Fascia Lata ; Glaucoma ; Light Coagulation ; Retina ; Retinal Detachment ; Retinal Vessels ; Rupture ; von Hippel-Lindau Disease*

PURPOSE: We reported the CT and MR findings of 2 cases with Sturge-Weber syndrome which were not accompanied by facial nevi. MATERIALS AND METHODS: They were examined with both CT and MR in one case and with MR only in the other case. RESULTS: CT was better than MR in the demonstration of the characteristic cortical calcification. MR was superior to CT in the depiction of the abnormalities of the surrounding parenchyma and the intense enhancement of pial angiomatosis with Gd-DTPA enhancement. CONCLUSION: Gd-DTPA enhanced MR imaging could be useful in the demonstration of the presence and extent of pial angiomatosis in patients with suspected Sturge-Weber syndrome.
Angiomatosis ; Gadolinium DTPA ; Humans ; Magnetic Resonance Imaging ; Nevus* ; Sturge-Weber Syndrome*

Sturge-Weber syndrome is a rare congenital anomaly characterized by angiomatosis of face, eye and leptomeninges and nevus flammeus is distributed along the trigminal nerve in face, often accompanying congenital glaucoma. We experienced a case of Sturge-Weber syndrome in an 11-year-old Korean girl of bilateral facial nevus flammeus with bilateral glaucoma treated by trabeculectomy and laser trabeculoplasty previously, but glaucomatous optic neuropathy progressed due to inadequate intraocular pressure control, and we could obtain successful result after trabeculectomy with mitomycin-C.
Angiomatosis ; Child ; Female ; Glaucoma* ; Humans ; Intraocular Pressure ; Mitomycin ; Optic Nerve Diseases ; Port-Wine Stain ; Sturge-Weber Syndrome* ; Trabeculectomy

No abstract available.
Klippel-Trenaunay-Weber Syndrome*

A 21-year-old Korean male was referred to our department in June, 1999 for the evaluation of extensive reddish patches and gray-bluish pigmentation since birth. Physical examination revealed two kinds of patches over the various parts of the body. Reddish patches suggesting nevus flammeus were located on the left side of face, chest, and both upper and lower extremities. Gray-bluish pigmented patches suggesting nevus of Ota were found on both the periorbital areas. A 0.5×0.6 cm erythematous papule was found on the right anterior chest. The results of its histopathologic examination were compatible with pyogenic granuloma. We made the diagnosis of phakomatosis pigmentovascularis associated with pyogenic granuloma as well as Sturge-Weber syndrome and Klippel-Trenanunay syndrome.
Diagnosis ; Granuloma, Pyogenic* ; Humans ; Klippel-Trenaunay-Weber Syndrome* ; Lower Extremity ; Male ; Neurocutaneous Syndromes* ; Nevus of Ota ; Parturition ; Physical Examination ; Pigmentation ; Port-Wine Stain ; Sturge-Weber Syndrome* ; Thorax ; Young Adult

Sturge-Weber syndrome can be associated with facial port-wine stains and intracranial calcification, and concurrent Klippel-Trenaunay-Weber syndrome has been reported. Klippel-Trenaunay-Weber syndrome is a rare congenital mesodermal phakomatosis characterized by cutaneous hemangiomas, venous varicosities and soft tissue or bone hypertrophy of the affected extremities. This report is presented a rare case of the Sturge-Weber syndrome in combination with the Klippel-Trennaunay syndrome and phakomatosis pigmentovascularis in a 4-month-old infant. He showed nevus flameus on the right leg and both part of the face and back, leptomeningeal angiomatosis on right hemisphere, hypertrophy of the right leg, hemiconvulsion on the left and also evidences of congenital glaucoma and nevus of Ota. Very rare case combined with these three kinds of phakomatosis has been reported.
Humans ; Infant ; Klippel-Trenaunay-Weber Syndrome/*complications/diagnosis ; Male ; Neurocutaneous Syndromes/*complications/diagnosis ; Research Support, Non-U.S. Gov't ; Sturge-Weber Syndrome/*complications/diagnosis

Von Hippel-Lindau disease is a rare disorder which involves multi-organs including cerebellum and retina. Recently we have experienced 2 cases of von Hippel-Lindau disease. The case 1 was a 19-year-old female with chief complaint of severe headache accompanied by vomiting on admission. Computed tomogram of brain showed space occupying lesion in the posterior fossa. She was rapidly deteriorated and diagnosed as brain death on the basis of physical and neurological examination and electroencephalogram. Kidney specimens removed for the transplantation and cerebellum obtained by partial autopsy reveled bilateral renal cell carcinomas and hemangioblastoma, respectively, on the microscopic examination.. The case 2 was a 23-year-old male who visited to our hospital due to headache, gait disturbance, and visual loss in the right eye. The findings of brain CT, vertebral angiography, ophthalmoscope, retinal angiogram, and operation were compatable to cerebello-retinal angiomatosis. With reviewing of some literatures, we present 2 cases of von Hippel-Lindau disease.
Angiography ; Angiomatosis ; Autopsy ; Brain ; Brain Death ; Carcinoma, Renal Cell ; Cerebellum ; Electroencephalography ; Female ; Gait ; Headache ; Hemangioblastoma ; Humans ; Kidney ; Male ; Neurologic Examination ; Ophthalmoscopes ; Retina ; Retinaldehyde ; Vomiting ; von Hippel-Lindau Disease* ; Young Adult

No abstract available.
Humans ; Klippel-Trenaunay-Weber Syndrome