Pemphigus vegetans is a rare variant of pemphigus vulgaris and is characterized by vegetating lesions in the inguinal folds and mouth and by the presence of autoantibodies against desmoglein 3. Two clinical subtypes of pemphigus vegetans exist, which are initially characterized by flaccid bullae and erosions (the Neumann subtype) or pustules (the Hallopeau subtype). Both subtypes subsequently develop into hyperpigmented vegetative plaques with pustules and hypertrophic granulation tissue at the periphery of the lesions. Oral administration of corticosteroids alone does not always induce disease remission in patients with pemphigus vegetans. We report here on a 63-year-old woman with pemphigs vegetans. She had a 2-year history of vegetating, papillomatous plaques on the inguinal folds and erosions of the oral mucosa. The enzyme-linked immunosorbent assay was positive for anti-desmoglein 3, but it was negative for anti-desmoglein 1. She was initially treated with systemic steroid, but no improvement was observed. The patient was then successfully treated with a combination of systemic steroid and dapsone with a good clinical response.
Administration, Oral ; Adrenal Cortex Hormones ; Autoantibodies ; Blister ; Dapsone ; Desmoglein 3 ; Enzyme-Linked Immunosorbent Assay ; Female ; Granulation Tissue ; Humans ; Middle Aged ; Mouth ; Mouth Mucosa ; Pemphigus

Bowen's disease is a squamous cell carcinoma in situ and has the potential to progress to a squamous cell carcinoma. The authors treated two female patients (a 39-year-old and a 41-year-old) with Bowen's disease in the vulva area using topical photodynamic therapy (PDT), involving the use of 5-aminolaevulinic acid and a light-emitting diode device. The light was administered at an intensity of 80 mW/cm2 for a dose of 120 J/cm2 biweekly for 6 cycles. The 39-year-old patient showed excellent clinical improvement, but the other patient achieved only a partial response. Even though one patient underwent a total excision 1 year later due to recurrence, both patients were satisfied with the cosmetic outcomes of this therapy and the partial improvement over time. The common side effect of PDT was a stinging sensation. PDT provides a relatively effective and useful alternative treatment for Bowen's disease in the vulva area.
Adult ; Bites and Stings ; Bowen's Disease* ; Carcinoma, Squamous Cell ; Female ; Humans ; Photochemotherapy* ; Recurrence ; Sensation ; Vulva*

Eccrine angiomatous hamartoma (EAH) is a rare benign disease that is characterized by an abnormal proliferation of eccrine glands and vascular elements. It is generally congenital, but it can appear before puberty. It usually presents as a single plaque or nodule, but multiple patch-like lesions are also possible. EAH is mostly asymptomatic, but it is sometimes associated with pain or hyperhidrosis. It generally does not require aggressive treatment, but the lesion can be excised due to pain, enlargement and cosmetic reasons. A 3-week-old Korean female presented with a hemorrhagic skin lesion on the right foot since birth. There was no specific birth history. The lesion first appeared on the third toe of the right foot and quickly spread to almost half of the right foot. Histopathology examination revealed acanthosis in the epidermis and a proliferation of eccrine ducts, glands and capillaries. The eccrine glands were immunohistochemically-positive for carcinoembryonic antigen.
Capillaries ; Carcinoembryonic Antigen ; Cosmetics ; Eccrine Glands ; Epidermis ; Female ; Foot ; Hamartoma ; Hemorrhage ; Humans ; Hyperhidrosis ; Parturition ; Puberty ; Reproductive History ; Skin ; Toes

Aberrant expression of BORIS/CTCFL (Brother of the Regulator of Imprinted Sites/CTCF-like protein) is reported in different malignancies. In this study, we characterized the entire promoter region of BORIS/CTCFL, including the CpG islands, to assess the relationship between BORIS expression and lung cancer. To simplify the construction of luciferase reporter cassettes with various-sized portions of the upstream region, genomic copies of BORIS were isolated using TAR cloning technology. We analyzed three promoter blocks: the GATA/CCAAT box, the CpG islands and the minisatellite region BORIS-MS2. Polymorphic minisatellite sequences were isolated from genomic DNA prepared from the blood of controls and cases. Of the three promoter blocks, the GATA/CCAAT box was determined to be a critical element of the core promoter, while the CpG islands and the BORIS-MS2 minisatellite region were found to act as regulators. Interestingly, the polymorphic minisatellite region BORIS-MS2 was identified as a negative regulator that repressed the expression levels of luciferase reporter cassettes less effectively in cancer cells compared with normal cells. We also examined the association between the size of BORIS-MS2 and lung cancer in a case–control study with 590 controls and 206 lung cancer cases. Rare alleles of BORIS-MS2 were associated with a statistically significantly increased risk of lung cancer (odds ratio, 2.04; 95% confidence interval, 1.02–4.08; and P=0.039). To conclude, our data provide information on the organization of the BORIS promoter region and gene regulation in normal and cancer cells. In addition, we propose that specific alleles of the BORIS-MS2 region could be used to identify the risk for lung cancer.
Alleles ; Clone Cells ; Cloning, Organism ; CpG Islands ; DNA ; Gene Expression* ; Luciferases ; Lung Neoplasms* ; Lung* ; Minisatellite Repeats* ; Promoter Regions, Genetic

Pulmonary mucormycosis is an opportunistic infection in patients with severe underlying illness such as immunocompromised diseases or uncontrolled diabetes mellitus. While patients with leukemia and lymphoma usually resent with diffuse parenchymal disease, diabetic patients usually have a localized endobronchial disease involving central airways. We report upon a case of pulmonary mucormycosis in diabetes mellitus patient presenting as an endobronchial mass, which was cured with antifungal therapy, rigid bronchoscopic mass removal and right pneumonectomy.
Diabetes Mellitus ; Humans ; Leukemia ; Lymphoma ; Mucormycosis* ; Opportunistic Infections ; Pneumonectomy

Pseudomembranous colitis, although uncommon, is an important complication of antibiotics that is related to a variety of deleterious effects on the gastrointestinal tract. Rifampicin is one of the 1st line agents in the treatment of tuberculosis and a large number of patients are exposed to its potential adverse effects. We report upon a patient that had diarrhea due to pseudomembranous colitis after receiving antitubeculous medication, and which was probably caused by rifampicin. A 77-year-old man was admitted with diarrhea of three weeks duration. One month previously, he suffered from left pleuritic chest pain and left pleural effusion was noticed at chest X-ray. One week prior to the onset of diarrhea, he was started on empirically isoniazid, rifampicin, ethambutol and pyrazynamide as antituberculous medication. On admission, he complained of diarrhea, left pleuritic chest pain, dyspnea and sputum. On physical examination, breathing sound was decreased in the left lower lung field and bowel sound increased. Pleural biopsy revealed chronic granulomatous infalmmation, which was compatible with tuberculosis. Sigmoidoscopy showed whitish to yellowish pseudomembrane with intervening normal mucosa, and his stool was positive for C.difficle toxin. He was diagnosed as pseudomembranous colitis and treated with oral metronidazole and vancomycin. The diarrhea did not recur after reinstitution of the anti-tuberculous medication without rifampicin. In patients with severe diarrhea receining anti-tuberculous medication, rifampicin induced pseudomembranous colitis should be excluded.
Aged ; Anti-Bacterial Agents ; Biopsy ; Chest Pain ; Clostridium difficile ; Diarrhea ; Dyspnea ; Enterocolitis, Pseudomembranous* ; Ethambutol ; Gastrointestinal Tract ; Humans ; Isoniazid ; Lung ; Metronidazole ; Mucous Membrane ; Physical Examination ; Pleural Effusion ; Respiratory Sounds ; Rifampin* ; Sigmoidoscopy ; Sputum ; Thorax ; Tuberculosis ; Vancomycin

Sorafenib is an oral, multi-targeted tyrosine kinase inhibitor with anti-angiogenic and anti-proliferative activity. It is approved for the treatment of unresectable hepatocellular and advanced renal carcinomas. Cutaneous toxicity is relatively common in patients receiving sorafenib. The most frequent cutaneous side effect is the hand-foot syndrome. Other adverse skin reactions include facial erythema, acral erythema, erythema multiforme, subungual splinter hemorrhage, stomatitis, and alopecia. In Korea, two cases of scrotal and perianal dermatitis after sorafenib therapy were reported. We report a 54-year-old male patient with a 2-week history of scrotal eczema who had been treated for chronic hepatitis type B, liver cirrhosis, and hepatocellular carcinoma. After 2 weeks of oral sorafenib (800 mg/day) administration, thick, scaly patches appeared on his scrotum. A skin biopsy specimen from these lesions revealed superficial dermal perivascular lymphocytic and neutrophilic infiltration, and dilatation of the lymphatics in the superficial dermis. The lesions improved after treatment with a topical and systemic steroid for 2 weeks. Herein, we report a rare case of scrotal erythema associated with sorafenib.
Alopecia ; Biopsy ; Carcinoma, Hepatocellular* ; Dermatitis ; Dermis ; Dilatation ; Eczema ; Erythema Multiforme ; Erythema* ; Hand-Foot Syndrome ; Hemorrhage ; Hepatitis, Chronic ; Humans ; Korea ; Liver Cirrhosis ; Male ; Middle Aged ; Neutrophils ; Protein-Tyrosine Kinases ; Scrotum ; Skin ; Stomatitis

Pityriasis rubra pilaris (PRP) is a chronic papulosquamous disorder of unknown etiology, which may pose therapeutic challenges. There is currently no universally effective treatment for PRP, and some cases are resistant to multiple topical and systemic therapies. Systemic retinoids, methotrexate, several immunosuppressive agents and phototherapy have all been used with varying degrees of success. Recently, a few reports have appeared in the literature, concerning the use of biologics in combination therapies and/or in refractory PRP cases. We report a case of PRP similar to type II with juvenile onset, which was recalcitrant to traditional topical and systemic therapy. He was successfully treated with anti-TNF-alpha monoclonal antibody, infliximab. The patient showed resolution with minimal disease activity, and was maintained on acitretin and emollients. The response to infliximab in our patient and in the previously reported cases confirms a role of anti-TNF-alpha therapy as an effective option in the treatment of PRP.
Acitretin ; Antibodies, Monoclonal ; Biological Agents ; Emollients ; Humans ; Immunosuppressive Agents ; Methotrexate ; Phototherapy ; Pityriasis ; Pityriasis Rubra Pilaris ; Retinoids ; Skin Diseases, Papulosquamous ; Infliximab

Reed syndrome is an autosomal dominantly inherited disease with incomplete penetrance that is characterized by uterine and cutaneous leiomyoma. While the skin tumors are relatively uncommon and benign, women of affected families often develop uterine leiomyoma with associated infertility, pain and bleeding. Here, we report a case of Reed syndrome in a family with multiple cutaneous and uterine leiomyomas. A 30-year-old woman had not only multiple cutaneous leiomyomatosis on cheek and neck, but also uterine multiple leiomyoma. Her mother had lesions that the multiple cutaneous leiomyomatosis on both shoulders and underwent myomectomy for a huge uterine leiomyoma at the age of 45. Her older sister also underwent myomectomy for uterine leiomyoma.
Adult ; Cheek ; Female ; Hemorrhage ; Humans ; Infertility ; Leiomyoma ; Leiomyomatosis ; Mothers ; Neck ; Penetrance ; Shoulder ; Siblings ; Skin

No abstract available.
Foreign-Body Reaction* ; Granuloma* ; Hyaluronic Acid*