Pilomattiaoma, aften called calcifying epithelioma of Malherbe, is a benign tumor originating from the outer root sheath cell of the hair follicle and extending into the hair matrix, Pilomatricoma usually occurs as a single, asymptomatic dermal or subcutaneous nodule. Multiple lesions are quite unueuel, comprising only 2-3.5% of cases. We report two patients with multiple pilomatricoma.
Hair ; Hair Follicle ; Humans ; Pilomatrixoma*

Bullous pemphigoid(BP) and herpes gestationis(HG) are subepidermal bullous diseases which show clinical and immunological similarities. Both diseases show immune deposits along the basement membrane zone and their autoantibodies bind a common antigenic site within the non-collagenous stretch of the 180 kDa BPAG2 ectodomain. Besides its association with pregnancy, HG has some characteristic features that distinguish it from BP. The serum of patients with HG often contains an IgG that avidly fixes complement, and showes IgG1 subclass predominance. We report here two cases of non-pregnant young women presenting clinical and histological features of bullous pemphigoid or herpes gestationis. The immunopathology, IgG subtyping and immunoblotting studies showed that the autoantibodies in the patients were the characteristic ones of herpes gestationis. The patients might be a subtype of BP that have characteristics of autoantibodies of HG patients.
Autoantibodies* ; Basement Membrane ; Complement System Proteins ; Female ; Humans ; Immunoblotting ; Immunoglobulin G ; Pemphigoid Gestationis* ; Pemphigoid, Bullous* ; Pregnancy

189-192 Six male patients with typical symptoms of Behcet's syndrome were subjected to a clinical examination. One of these was a Chinese patient. The age when the disease started was between 20 to 39 years. All of them became blind but no one was dead.
Asian Continental Ancestry Group ; Behcet Syndrome* ; Humans ; Male

Wilms' tumor is one of the most common primary malignant tumors of the kidney during infancy and childhood and is known to be originated from the primitive cells of metanephric blastema. It presents difficulties when encountered in deciding the presence of anaplasia or in differentiating it from other renal tumors of childhood with different biologic behavior because of its diverse histologic patterns and varying degrees of differentiation. Evaluation of clinical and histopathologic features in terms of prognostication was done of 32 cases of Wilms' tumor which were surgically resected and diagnosed in the period from January 1979 through June 1992. Immunohistochemical reaction for cytokeratin, vimentin, actin and desmin was also analysed on all cases of Wilms' tumor in conjunction with clear cell sarcoma of the kidney(CCSK), malignant rhabdoid tumor of the kidney(MRTK) and congenital mesoblastic nephroma(CMN) to assess the validity of immunohistochemistry in differentiating Wilms' tumor from these renal tumors. Twenty four(75%) cases were diagnosed before the age of 5 and 40.7% were under 2 years old. Mixed type was most common(62.5%), followed by epithelial, blastemal and stromal predominant type in descending order of frequency. Anaplasia was observed in 3 cases(9.4%), two of which were epithelial predominant type and one blastemal predominant type. Treatment modality and presence of anaplasia were significantly correlated with 5 year survival rate of patients. Immunohistochemical stain revealed that all epithelial component of Wilms' tumor were positive for cytokeratin and 56.3% of Wilms' tumor had blastemal component which were positive for both cytokeratin and vimentin. Twenty cases(62.5%) of Wilms' tumor had blastemal component which were positive for cytokeratin with a proportion of more than 5% of reactive cells. Stromal component of Wilms' tumor generally did not show differentiation into the specialized type of tissue and all revealed positive reactions for vimentin among which some revealed positive reactions for actin. Only 3 out of 6 cases with rhabdomyoblastic differentiation were positive for desmin. CCSK, MRTK and CMN which have different biologic behavior and treatment modality compared to Wilm's tumor showed positivity only for vimentin and/or actin. In summary, treatment modality and presence of anaplasia are significantly correlated with patients' survival and the immunohistochemical stain for cytokeratin is very helpful in confirming the presence of blastemal component and useful in the differential diagnosis of Wilms' tumor from other kinds of pediatric renal tumors.
Child ; Male ; Female ; Humans ; Diagnosis, Differential

MR images of seven patients with histologically documented synovial sarcoma were reviewed to find the suggestive or pathognomonic findings of the disease. MR appearance of the tumor was inhomogeneous, multiseptated mass with various degree of internal septation and infiltrative tumor margin. The signal intensity of the mass was slightly hyperintense relative to muscle on T1-weighted image and hyperintense relative to subcutaneous fatty tissue on T2-weighted image.
Adipose Tissue ; Humans ; Magnetic Resonance Imaging* ; Sarcoma, Synovial*

PURPOSE: We evaluated the diagnostic accuracy and clinical usefulness of fluoroscopy-guided bone biopsy. MATERIALS AND METHODS: A total of 31 patients with various skeletal lesions underwent fluoroscopy-guided bone biopsies. The targets were long bones in 16 cases, pelvic bones in 7 cases, spines in 6 cases, and ribs in the rest 2 cases. RESULTS: The overall accuracy was 71%(22/31). The accuracy was 100% in case of primary sarcoma and multiple myeloma. But it was low in malignant lymphoma(1/4), malignant fibrous histiocytoma(0/1), and nonossifying fibroma(0/1). CONCLUSION: Percutaneous fluoroscopy-guided bone biopsy is a simple, safe, not-expensive, and rather accurate diagnostic procedure in evaluation of bone tumors. In addition to that, it is useful in the managment planning and fllow-up of the patients.
Biopsy* ; Humans ; Multiple Myeloma ; Pelvic Bones ; Ribs ; Sarcoma ; Spine

No abstract available.
Carcinoma, Renal Cell* ; Genes, Tumor Suppressor*

Marcus Gunn reported an unusual type of congenital ptosis with a peculiar associated movement of the affected lid in 1883. This movement is thought to be due to a congenital misdirection of some of the 5th cranial nerve fibers into the branch of the 3rd cranial nerve that supplies the levator muscle. Therefore, stimulation of the jaw-winking mechanism sends excess impulses into a poorly innervated levator muscle, enough to momentarily raise the eyelid. We experienced a case of Marcus gunn syndrome in a 14 year-old boy, whose chief complaint was the drooping of the left upper eyelid which was retracted in association with opening of mouth or lateral movement of the jaw to the right side since birth.
Adolescent ; Cranial Nerves ; Equipment and Supplies ; Eyelids ; Humans ; Jaw ; Male ; Mouth ; Parturition

Marcus Gunn reported an unusual type of congenital ptosis with a peculiar associated movement of the affected lid in 1883. This movement is thought to be due to a congenital misdirection of some of the 5th cranial nerve fibers into the branch of the 3rd cranial nerve that supplies the levator muscle. Therefore, stimulation of the jaw-winking mechanism sends excess impulses into a poorly innervated levator muscle, enough to momentarily raise the eyelid. We experienced a case of Marcus gunn syndrome in a 14 year-old boy, whose chief complaint was the drooping of the left upper eyelid which was retracted in association with opening of mouth or lateral movement of the jaw to the right side since birth.
Adolescent ; Cranial Nerves ; Equipment and Supplies ; Eyelids ; Humans ; Jaw ; Male ; Mouth ; Parturition

PURPOSE: To determine the significance of anomalous position of the gallbladder. MATERIALS AND METHODS: Sixteen patients with anomalous position of the gallbladder were evaluated for analysis. The diagnosis was confirmed by ultrasonography(15 patients) and oral cholecystography(1patient). Among those, six patients underwent CT scan and a patient had 99mTc-DISIDA scan. The images were analysed with respect to the location of the GB and configuration and associated abnormality of the liver and hepatobiliary systems. Medical records of each patient were also reviewed. RESULTS: Among 16 patients having an anomalous position of the gallbladder, nine had retrodisplaced gallbladder, four had left-sided gallbaldder, two had suprahepatic gallbladder, and one had floating gallbladder. Except for one patient, fifteen had abnormality in the liver such as focal atrophic or hypoplastic change and liver cirrhosis. Intrahepatic stones were demonstrated in 6 patients. CONCLUSION: Our results showed that anomalous position of the gallbladder was commonly associated with atrophy or hypoplasia of the liver rather than congenital in origin. The possiblity of an anomalous location of gallbladder should be kept in mind when GB is not in its normal location.
Atrophy ; Diagnosis ; Gallbladder* ; Humans ; Liver ; Liver Cirrhosis ; Medical Records ; Technetium Tc 99m Disofenin ; Tomography, X-Ray Computed