We report two cases of extramammary Paget's disease associated with ad enocarcinoma with metascases. Histologically, Iaget cells in the epidermis and the hair follicles and adenocarcinoma in the der mis was observed in both cases. Of special interest was the demonstration of Eaget cells extending from the involved epidermis to the underlying dermis in case 2, There were metastatic lesioris of 3rd lumbar vertebra in case 1 and of the liver and lymph nodes in rase 2.
Adenocarcinoma* ; Dermis ; Epidermis ; Hair Follicle ; Liver ; Lymph Nodes ; Neoplasm Metastasis* ; Paget Disease, Extramammary* ; Spine

Targetoid hemosiderotic hemangioma is a benign vascular tumor with a peculiar clinical presentation and histologic features sometimes difficult to differentiate from the patch stage of Kaposi's sarcoma, progressive lymphangioma, eosinophilic hemangioma, retiform hemangioendothelioma. We report a case of targetoid hemosiderotic hemangioma on upper back and right upper arm of a 28-year-old male. He has a 5mm × 5mm sized slightly raised violaceous papule surrounded by an ecchymotic halo on upper back and right upper arm. We took a biopsy of the lesion and identified it as targetoid hemosiderotic hemangioma. This is a case of targetoid hemosiderotic hemangioma with typical clinical and histopathological findings.
Adult ; Arm ; Biopsy ; Eosinophils ; Hemangioendothelioma ; Hemangioma* ; Humans ; Lymphangioma ; Male ; Sarcoma, Kaposi

Bullous pemphigoid may, very rarely, occur in infants, although it is usually a bullous disease of the elderly which is characterized by subepidermal bullae on erythematous and normal skin. We describe an infant with severe subepidermal blistering disorder with typical features for bullous pemphigoid.
Aged ; Blister ; Humans ; Infant* ; Pemphigoid, Bullous* ; Skin

The optimal and alternative antimicrobial regimens to treat neurosyphilis in human immunodeficiency virus (HIV) infected patients remain controversial. Little is known concerning the efficacy of ceftriaxone. A 75-year-old heterosexual man visited an outpatient clinic due to incidentally detected positive serum Venereal disease research laboratory (VDRL) and Fluorescent treponemal antibody absorbed tests in routine preoperative checkup. Because of benzathine penicillin was unavailable to use, 4-week regimen with doxycycline was started. After 2 weeks doxycycline treatment, a severe headache suddenly occurred. Cerebrospinal fluid (CSF) analysis showed mild lymphocyte-dominant pleocytosis, increased protein, and weakly reactive VDRL test. ELISA for HIV antibody and Western blot assay revealed he is infected with HIV. Initial CD4+ T cell count was 220/mm3 and the load of HIV RNA was 5,200 copies/mL. The most probable diagnosis was deemed to be neurosyphilis, considering the possibilities of Jarisch-Herxheimer reaction or doxycycline-induced pseudotumor cerebri. In the absence of procaine penicillin G in our hospital, intravenous ceftriaxone was administered and highly active antiretroviral therapy was started. After 14days of treatment, the patient no longer had a headache and the CSF profile was improved. Additional follow-up CSF analysis was done 3months after treatment. CSF pleocytosis and protein level were decreased and the CSF VDRL was converted to negative. Ceftriaxone may be a good alternative in the treatment of neurosyphilis in HIV infected patient.
Aged ; Ambulatory Care Facilities ; Antiretroviral Therapy, Highly Active ; Blotting, Western ; Ceftriaxone ; Cell Count ; Doxycycline ; Enzyme-Linked Immunosorbent Assay ; Ethylenediamines ; Follow-Up Studies ; Headache ; Heterosexuality ; HIV ; Humans ; Leukocytosis ; Neurosyphilis ; Penicillin G Benzathine ; Penicillin G Procaine ; Pseudotumor Cerebri ; RNA ; Sexually Transmitted Diseases

Kinky hair disease is X-linked recessive neurodegenerative disorder produced by defects in a gene(ATP7A) that encodes an intracellular copper-transporting ATPase. About 90-95% of the patients have a severe clinical course leading to death in early childhood. ATP7A mutations associated with Menkes disease show great variety from cytogenetic abnormalities to partial gene deletions to single base-pair changes. We experienced a 15 month-old boy with loss of developmental milestones, hypotonia, seizures and failure to thrive. On laboratory findings, the levels of serum copper and ceruloplasmin were low. Electron microscopy of hair illustrated pathognomic pili torti and other abnormalities such as trichorrhexis nodosa and trichoptilosis(longitudinal splitting of the shaft). Brain magnetic resonance image showed diffuse cerebral and cerebellar atrophy with tortousity of cerebral blood vessels. Genetic defect was evaluated. Our sequencing data on the amplified exon 19 of ATP7ase genomic DNA confirmed point mutation, G1255A, resulting in a glycine-to-arginine conversion. So, we report a brief view with the related literatures.
Adenosine Triphosphatases ; Atrophy ; Blood Vessels ; Brain ; Ceruloplasmin ; Chromosome Aberrations ; Copper ; DNA ; Exons ; Failure to Thrive ; Gene Deletion ; Hair ; Humans ; Infant ; Male ; Menkes Kinky Hair Syndrome* ; Microscopy, Electron ; Muscle Hypotonia ; Neurodegenerative Diseases ; Point Mutation ; Seizures

Brunner's gland hyperplasia is a rare tumor of the duodenum and might also be an unusual cause of gastrointestinal bleeding. In symptomatic patients, treatment requires either surgical resection or endoscopic polypectomy. We report a case of upper gastrointestinal bleeding from a pedunculated Brunner's gland hyperplasia in the duodenal bulb. Endoscopic resection using the detachable snare and hemoclipping was instituted to remove a large pedunculated polyp. The pathologic diagnosis was Brunner's gland hyperplasia with adenomyomatous hyperplasia.
Adult ; Brunner Glands/*pathology/surgery ; Duodenum/*pathology ; Gastrointestinal Hemorrhage/etiology ; Humans ; Hyperplasia/complications/diagnosis/pathology ; Laparoscopy ; Male ; Precancerous Conditions/pathology ; Stents

Acute generalized exanthematous pustulosis(AGEP) presents with the abrupt onset of a wide spread pustules on an erythematous base and rapid spontaneous healing. The most possible causes of AGEP seem to be antibiotics, viral infection and exposure to mercury. We report five cases of acute generalized exanthematous pustulosis, all of which presented wide spreaded tiny pustules on an erythematous base. Histopathologic examination showed that there are subcorneal pustules, rete ridge elongations and perivascular infiltration. We suspected that the possible causes of five cases were pharyngitis and various drugs each.
Acute Generalized Exanthematous Pustulosis* ; Anti-Bacterial Agents ; Pharyngitis

A 61-year-old man presented with cognitive dysfunction and an 8-month history of progressive gait disturbance. Brain MRI revealed communicating hydrocephalus. Under the impression of normal pressure hydrocephalus, cerebrospinal fluid (CSF) study was done and showed unusually increased opening pressure (40 cm H2O) and protein concentration (650 mg/dl). Spine MRI was performed to rule out the possibility of intraspinal tumor. It revealed cauda equina mass, which was confirmed to be schwannoma pathologically. Intraspinal tumor should be suspected in patients with abnormally increased CSF opening pressure and/or CSF protein who presented as normal pressure hydrocephalus.
Brain ; Cauda Equina* ; Cerebrospinal Fluid ; Gait ; Humans ; Hydrocephalus ; Hydrocephalus, Normal Pressure* ; Magnetic Resonance Imaging ; Middle Aged ; Neurilemmoma* ; Spine

Helicobacter pylori (H. pylori) is currently considered the most important exogenous factor in the genesis of gastritis and peptic ulcer disease. However, the optimum regimen for the eradication of H. pylori remains unclear. The purpose of this study was to evaluate the eradication rate of H. pylori, the side effects, and the patients' compliance with regard to various drug regimens. We also analyzed factors influencing the eradication of H. pylori. One hundred and eighty patients were included and divided into four groups: 42 patients (Group I) received tripotassium dicitrato bismuthate (240 mg b.i.d.), metronidazole (250 mg t.i.d.) and amoxicillin (500 mg t.i.d.) for 14 days; 55 patients (Group 2) received omeprazole (20 mg b.i.d.) and amoxicillin (1000 mg b.i.d.) for 14 days; 36 patients (Group 3) were treated with omeprazole (20 mg b.i.d.), metronidazole (250 mg t.i.d.) and amoxicillin (500 mg t.i.d.) for 14 days; and 47 patients (Group 4) received omeprazole (20 mg q.d.) and amoxicillin (500 mg t.i.d.) for 14 days and then tripotassium dicitrato bismuthate(240 mg b.i.d.) and nizatidine (150 mg q.d.) for 14 days. The diagnosis of H. pylori was made by histology. The eradication of H. pylori was defined both by histology (H&E and Giemsa stain) and by rapid urease test (CLOR) showing negative for H. pylori 4 weeks after the completion of therapy. Of the 180 patients, 95 patients had non-ulcer dyspepsia, 40 patients had gastric ulcer and 45 patients had duodenal ulcer. The eradication rate of H. pylori was highest (89.3%) in Group 3, as compared with Group 1 (68.9%), Group 2 (65.4%), and Group 4 (48.9%). The eradication rate was significantly higher in Group 3 than in Groups 2 and 4 (p< 0.05). There was no significant difference in the eradication rate among clinical diagnosis, sex and age. But, in the conventional triple therapy (Group 1), the eradication rate was higher in male (78.6%) than in female (46.2%). The side effects in order, were nausea (22.1%), dizziness (19.5%), abdominal pain (11.6%) and diarrhea (97%), and there was no difference among the drug regimens. The compliance of the patients was good (more than 80% irrespective of drug regimen). On the basis of these findings, the side effects of the drugs seemed minimal, and the compliance of patients was good irrespective of the drug regimen. In conclusion, the triple therapy with omeprazole, metronidazole and amoxicillin was the most effective regimen and could be recommended for H. pylori eradication.
Adult ; Anti-Ulcer Agents/*therapeutic use ; Antibiotics/*therapeutic use ; Duodenal Ulcer/microbiology ; Dyspepsia/microbiology ; Female ; Helicobacter Infections/*drug therapy ; *Helicobacter pylori ; Human ; Male ; Middle Age ; Stomach Ulcer/microbiology

Retroperitoneal fibrosis is a disease characterized by proliferation of fibro-inflammatory tissue, which mainly encloses the abdominal aorta, iliac arteries and (enlarges to the retroperitoneum to surrounds) ureters. Two leading pathogenesis are an exacerbated local inflammatory reaction to aortic atherosclerosis and a manifestation of systemic autoimmune disease. Because of insidious disease course, most cases are diagnosed late when bilateral urinary obstruction caused oliguria and symptoms related to uremia. In this case, the patient complained left flank pain which might have been caused by left ureter stone, but through abdominal CT scan, the patient was diagnosed as left unilateral retroperitoneal fibrosis. Abdominal CT scan showed encircling mass around abdominal aorta and left ureter with hydronephrosis. The patient had no underlying disease and didn't take any medication; she was diagnosed as idiopathic unilateral retroperitoneal fibrosis and started prednisolone, 40 mg daily. Three months have passed since the therapy was started; most fibrous tissue was disappeared, hydronephrosis was resolved and flank pain was relieved.
Aorta, Abdominal ; Atherosclerosis ; Autoimmune Diseases ; Flank Pain ; Humans ; Hydronephrosis ; Iliac Artery ; Oliguria ; Prednisolone ; Retroperitoneal Fibrosis ; Uremia ; Ureter