Prurigo pigmentosa is a chror ic pruritic inflammatory dermatosis characterized by erythematous papules in a reticulated pattern that resolve leaving a reticulated, mottled hyperpigmentation and rapidly response to dapsone therapy. Most cases that have been reported are from Japan. We report two cases of prurigo pigmentosa in a 21 year-old female and a 23-year-old female. Histopathologic findings of erythematous papules showed exocytosis, spongiosis, liquefaction degeneration of basal cell layer and perivascular lymphohistiocytic infiltration on the upper dermis. We treated them with dapsone, 100mg daily, which resulted in a remarkable regression of the ery thematous papules and improvenien of pruritus.
Dapsone ; Dermis ; Exocytosis ; Female ; Humans ; Hyperpigmentation ; Japan ; Prurigo* ; Pruritus ; Skin Diseases ; Young Adult

Cirsoid aneurysm of the calp is a congenital arteriovenous malformation which is the result of abnormal connections betwe:.n the external carotid artery and scalp veins with a subsequent dilatation of vascular channels nd formation of aneurysm. We report a case of cirsoid aneurysm of the scalp on the left parieto-occipital area of 14-year-old female who had had 8 x 5 x 0.8 cm sized pulsating soft mass for 2 years. 4 vessel angiog raphy revealed a arteriovenous malformation that emerge mainly from a superficial temporal artery and occipital artery of the left external carotid artery and partially from the right superficial temporal artery and drined into the left scalp vein. The mass was removed by excision with ligation of the feeding arter es and followed up for 2 years without recurrence.
Adolescent ; Aneurysm* ; Arteries ; Arteriovenous Malformations ; Carotid Artery, External ; Dilatation ; Female ; Humans ; Ligation ; Recurrence ; Scalp* ; Temporal Arteries ; Veins

Kaposi's sarcoma is a neoplasm of multifocal origin which manifest primarily as vascular nodules in the skin and other organs. We report a case of Kaposis sarcoma of classic type in a 17-year-old boy, who had a 4 x 5cm sized, dusky erythematous plaque with multiple spots of acupuncture on the left foot for 1 year. Histopathologic examination showed dilated, bizarre-shaped vessels, ndothelial cell proliferation, and spindle cell formation with vascular slits throughout the dermis. He was treated with 3000 rads of radiation(300 rad per day for l0 successive days). Six months after radiotherapy, gross findings were much improved and histopathologic findings showed only fibrosismc hemosiderin deposits in the dermis.
Acupuncture ; Adolescent ; Cell Proliferation ; Dermis ; Foot ; Hemosiderin ; Humans ; Male ; Radiotherapy* ; Sarcoma, Kaposi* ; Skin

Cutaneous manifestations of sarcoidosis are highly variable and can be devided into the specific lesions which reveal noncaseating granuloma and the nonspecific lesio is which are referred to as cutaneous changes. Among speciific lesions, annular lesions may be foimied from coalescing papular lesions that may clear centrally and elevate peripherally. We report a case of sarcoidosis with multiple annular lesions on the face in a 33-year-old female who had systemic involvements in the intrathoracic organs and eyes. The main histopathologir. findings were well-demarcated islands of epitheloid cells with a few giant cells in the dermis. In addition, a slight admixfure of lymphoid cells was present at the margins of the epitheloid cell granulomas. Complete regression of t!he skin lesions was obtained with systemic cortiocosteroid therapy and no recurrence has been found for 2 1/2years.
Adult ; Dermis ; Female ; Giant Cells ; Granuloma ; Humans ; Islands ; Lymphocytes ; Recurrence ; Sarcoidosis* ; Skin

Dermatophytes have been demonstrated infrequently in viable tissues such as dermis, subcutis, lymph nodes, bones and other organs. Mycetoma-like lesions caused by dermatophytes, known as pseudomycetoma, have been very rarely reported. A 56-year-old woman presented with several protruding bean to chestnut-sized subcutaneous nodules on the occipital region of the scalp. She had a long term history of epidermolysis bullosa acquisita and intermittent administration of systemic corticosteroids for 5 years. Histopathology of the subcutaneous nodules showed a well encapsulated granuloma containing characteristic lobulated granules consisted of mycelial aggregates. Trichophyton mentagrophytes grew out on the cultures of Sabouraud's media with excised tissues. Surgical excision and itraconazole 200mg per day for 10 weeks was tried and skin lesions are improved with focal recurrence.
Adrenal Cortex Hormones ; Arthrodermataceae ; Dermis ; Epidermolysis Bullosa Acquisita ; Female ; Granuloma ; Humans ; Itraconazole ; Lymph Nodes ; Middle Aged ; Recurrence ; Scalp ; Skin ; Trichophyton*

Acute or chronic aortic dissection may lead to the rupture, which is the major cause of death. A dissecting aneurysm of ascending aorta(Stanford type A dissection) can rupture into the superior vena cava producing a aortocaval fistula, which is rare, but has been reported mostly in the cases of abdominal aortic aneurysm. We report a case of 67-year-old man with type A chronic dissection and aortocaval fistula, presenting symptoms of superior vena syndrome. The preoperative diagnosis was composed of radiologic examinations, including computed tomography, magnetic resonance imaging angiography and aortography. The dissecting aneurysm was resected and replaced, and the aortocaval fistula was repaired under deep hypothermic circulatory arrest. The details are described here.
Aged ; Aneurysm, Dissecting ; Angiography ; Aortic Aneurysm, Abdominal ; Aortography ; Cause of Death ; Circulatory Arrest, Deep Hypothermia Induced ; Diagnosis ; Fistula* ; Humans ; Magnetic Resonance Imaging ; Rupture ; Vena Cava, Superior

Tracheal bronchus is a aberrant, accessory or ectopic bronchus arising almost invariably from the right lateral wall of the trachea and may be related to inflammatory conditions affecting the lung, including recurrent pneumonia and bronchiectasis. Recently we experienced a case of tracheal bronchus associated with pulmonary actinomycosis. The 37-year-old male patient had suffered recurrent hemoptysis and had been medicated as a presumptive diagnosis of tuberculosis, but neither clinical nor radiologic improvement was not seen. Right upper lobectomy was performed and pulmonary actinomycosis was confirmed by the histologic examination. Postoperatively, the patient was medicated with penicillin and ampicillin for 3 months and completely recovered without any evidence of recurrence during the 6-month followup period.
Actinomycosis* ; Adult ; Ampicillin ; Bronchi* ; Bronchiectasis ; Diagnosis ; Follow-Up Studies ; Hemoptysis ; Humans ; Lung ; Male ; Penicillins ; Pneumonia ; Recurrence ; Trachea ; Tuberculosis

Though leiomyoma is the most common tumor of esophagus, it accounts for only 1% of all esophageal tumors. Most of the leiomyomas are intramural type originating from the muscularis propria and only 1% of them is intraluminal pedunculated type originating from muscularis mucosae. Recently, a 30-year-old male was admitted to our hospital because of dysphagia. Radiologic examination showed that intraluminal tumor 5cm in diameter was found at the cervical esophagus. Endoscopic examination showed that the tumor was covered with normal mucosa. The patient underwent surgical excision through the left cervical approach. After full, longitudinal esophagotomy, the intraluminal pedunculated tumor was successfully enucleated. Esophageal leiomyoma was confirmed histopathologically. Postoperative course was uneventful and the patient was relieved from dysphagia.
Adult ; Deglutition Disorders ; Esophageal Neoplasms ; Esophagus* ; Humans ; Leiomyoma* ; Male ; Mucous Membrane

Objectives: . Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by recurrent epistaxis, telangiectasia, and visceral arteriovenous malformations (AVMs). Activin A receptor-like type 1 (ACVRL1/ALK1) and endoglin (ENG) are the principal genes whose mutations cause HHT. No multicenter study has yet investigated correlations between genetic variations and clinical outcomes in Korean HHT patients. Methods: . Seventy-two members from 40 families suspected to have HHT based on symptoms were genetically screened for pathogenic variants of ACVRL1 and ENG. Patients with genetically diagnosed HHT were also evaluated. Results: . In the HHT genetic screening, 42 patients from 24 of the 40 families had genetic variants that met the pathogenic criteria (pathogenic very strong, pathogenic strong, pathogenic moderate, or pathogenic supporting) based on the American College of Medical Genetics and Genomics Standards and Guidelines for either ENG or ACVRL1: 26 from 12 families (50%) for ENG, and 16 from 12 families (50%) for ACVRL1. Diagnostic screening of 42 genetically positive HHT patients based on the Curaçao criteria revealed that 24 patients (57%) were classified as having definite HHT, 17 (41%) as having probable HHT, and 1 (2%) as unlikely to have HHT. Epistaxis was the most common clinical presentation (38/42, 90%), followed by visceral AVMs (24/42, 57%) and telangiectasia (21/42, 50%). Five patients (12%) did not have a family history of HHT clinical symptoms. Conclusion . Only approximately half of patients with ACVRL1 or ENG genetic variants could be clinically diagnosed as having definite HHT, suggesting that genetic screening is important to confirm the diagnosis.