Bart's syndrome was initially described as a genodermatosis characterized by congenital localized absence of the skin with blistering and nail deformities 1-3. However, it is considered as any type of epidermolysis bullosa(EB) with localized congenital absence of the skin on the extremities. A 33-day-old fbmale baby was presented with congenital absence of the skin over the left shin and dorsa of both feet which were covered with the thin, translucent, and brown-red glistening membranes. Blistering of the right calf and left great toe nail deformity were also noted. She was diagnosed as a recessive dystrophic EB by the histopathological, ultra- structural and immunomapping studies.
Blister ; Congenital Abnormalities ; Extremities ; Foot ; Membranes ; Skin ; Toes

No abstract available.
Gram-Positive Bacterial Infections* ; Teicoplanin*

Pseudocyst of the auricle is a disease of degenerative cartilage associated with asymptomatic swelling of the external ear caused by an intracartilaginous accumulation of serous fluid. Histological examination shows an intracartilaginous cavity without an epithelial lining. This disease is characterized by frequent relapses despite various therapeutic approaches. In this report we describe one patient with pseudocyst of the auricle who was successfully treated with intralesional injection of 1 mg/mL of minocycline hydrochloride.
Cartilage ; Ear, External ; Humans ; Injections, Intralesional* ; Minocycline* ; Recurrence

Adult T-cell leukemia/lymphoma (ATLL) is an often aggressive and fatal malignancy of mature CD4+ T lymphocytes, characterized by leukemia, lymphadenopathy, hypercalcemia and lytic bone lesions. Diagnosis of ATLL is based cm abnormal T lymphocytes in the peripheral blood and histologically and cytologically proven lymphoid malignancy with T cell surface antigens. Anti-HTLV-I serum antibodies and demonstration of clonality to HTLV-I proviral DNA are included. We report a case of ATLL in a 64-year-old man who had a painful nodule on the right cheek and multiple scattered papules and nodules on the trunk for 2 months. He had multiple cervical and axillary lymph node enlargements and hepatosplenomegaly. On laboratory finding peripheral leukocytes count was 33,400/mm3 with 63% atypical lymphocytes. Clover-shaped lyrnphocytes with multilobulated nuclei were found in peripheral blood and bone marrow. Histologic findings showed dense bandlike infiltrations of atypical lymphocytes in the dermis with angiocentricity. The surface phenotype of these abnormal cells were CD2+, CD3+, CD4+, CD30+ and HLA-DR+ and HTLV-I specific antibodies were found.
Adult* ; Antibodies ; Antigens, Surface ; Bone Marrow ; Cheek ; Dermis ; Diagnosis ; DNA ; Human T-lymphotropic virus 1 ; Humans ; Hypercalcemia ; Leukemia ; Leukemia-Lymphoma, Adult T-Cell* ; Leukocytes ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphoma* ; Middle Aged ; Phenotype ; T-Lymphocytes

The histology of herpes virus infection of the skin is characterized as an intraepidermal vesicle formed as the result of acantholysis and ballooning degeneration of kerationocytes. Dermal changes is limited to polymorphous inflammatory cells infiltrate, leukocytoclastic vasculitis, and herpetic folliculitis. Involvement of eccrine epithelium is very rare finding. We report two cases of herpes zoster with prominent viral infection of the eccrine sweat glands.
Acantholysis ; Eccrine Glands ; Epithelium ; Folliculitis ; Herpes Zoster ; Skin ; Sweat Glands ; Vasculitis

MRI is useful for demonstrating the anatomy of various congenital cardiac lesions and plays an important role in the diagnosis of congenital cardiac lesions. Its large field of view and unlimited imaging planes enable the depiction of complex lesions, the complicated three-dimensional relations of the cardiac chambers and anomalies of the central pulmonary arteries, the systemic and pulmonary veins, and aorta. We describe the normal MR anatomy and MR imaging findings of a variety of congenital pathologies of the heart, in order to provide a better understanding and facilitate the interpretation of the MR features of various congenital heart diseases.
Aorta ; Diagnosis ; Heart ; Heart Defects, Congenital* ; Heart Diseases ; Magnetic Resonance Imaging* ; Pathology ; Pulmonary Artery ; Pulmonary Veins

Pneumopericardium, as a form of barotrauma, refers to the presence of air within the pericardial sac. The causes of pneumopericardium are various. Clinically, pneumopericardium is typically present with dyspnea and precordial chest pain. On physical examination, heart sounds are usually distant, and precordial tympany may be elicited. The diagnosis can be made by clinical and radiographic findings. Management of pneumopericardium depends on many factors, such as the age of the patient, the suspected causes, and the extent of clinically observed respiratory compromise. The case shown here, a patient who developed pneumopericardium, involved an associated underlying pulmonary parenchymal process as the cause of pneumopericardium.
Barotrauma ; Chest Pain ; Diagnosis ; Dyspnea ; Heart Sounds ; Humans ; Physical Examination ; Pneumopericardium*

The purpose of this study was to develop a muffin containing dried leek powder. The complete analysis was conducted using the Design Expert 7 program (Stat - Easy Co., Minneapolis, MN, USA). The leek muffins were produced with varying amounts of leek powder (A), sugar (B), and butter (C). According to response surface methodology (RSM), there were 16 experimental points, including two replicates. The leek muffin formulation was optimized using rheology and sensory analyses. For the results, a quadratic model was applied in determining lightness, volume, hardness, and sensory characteristics, including flavor, texture, and overall quality; redness and height were represented by a linear model. Lightness decreased and redness increased with increasing amounts of leek powder (p<0.001). In addition, hardness displayed significant differences (p<0.001) with increasing amounts of leek powder. The sensory evaluation results showed significant differences for color, flavor, texture, and overall quality (p<0.05). Based on the numerical and graphical methods, the optimal formulation was determined as 8.30 g of leek power, 88.37 g of sugar, and 81.70 g of butter.
Butter ; Hardness ; Light ; Linear Models ; Rheology

Rowell's syndrome is described as erythema multiforme(EM)-like lesions in the presence of lupus erythematosus(LE) associated with immunoserological characteristics of speckled antinuclear antibodies and positive tests for anti-Ro/La antibodies and rheumatoid factor. We report a case of Rowell's syndrome in a 28-year-old woman with systemic lupus erythematosus for 13 years, who presented with targetoid eruptions on the right arm and with positive immunoserologic findings as above.
Adult ; Antibodies ; Antibodies, Antinuclear ; Arm ; Erythema ; Female ; Humans ; Lupus Erythematosus, Systemic ; Rheumatoid Factor