Ichthyosis hystrix is a localized variant of Bullous Congenital Ichthyosiform Erythroderma by Broq, and also, localized form of Epidermolytic hyperkeratosis according to Frost and Van Scott's classification of ichthyosi-form dermatoses. Clinically it is generally considered to be widespread systematized epidermal nevus, and demonstrating an autosomal dominant pattem of inheritance, and characterized by verrucous scale at or shortly after birth with particular involve- ment of flexural areas. A case of 21 year old girl with ichthyosis hystrix was reported, The skin lesion showed multiple rice to pea sized linear verrucous papules on left forearm, neck, axilla and upper chest. The skin biopsy was done, the finding shows remarked laminated hyperkeratosis, acanthosis, papillomatosis, and vacuoliization on malpighian layer. Diagnosis was confirmed by clinicaI apperance and histopathologicaI finding. The method of treatment is 0. 05% Vitamin A acid ointment application and show marked improvement.
Axilla ; Biopsy ; Classification ; Diagnosis ; Female ; Forearm ; Humans ; Hyperkeratosis, Epidermolytic ; Ichthyosis* ; Neck ; Nevus ; Papilloma ; Parturition ; Peas ; Porcupines* ; Skin ; Skin Diseases ; Thorax ; Tretinoin* ; Vitamin A* ; Vitamins* ; Wills ; Young Adult

The present study was carried out to find a simple and safe in. vitro test for phtotoxic drugs. Authors selected two strains of Salmonella typhimurium(TA98 and TA102) which have been used in Ames test for the detsction of mutagenecity af various chemical substances. Both strains are genuine products of genetic enzineering. The etrain TA98 should be highly vulnerable to ultraviolet radition because it lacks normal I)NA excision repair gene. The strain TA102 was chosen as control since it maintained the DNA repair gene. These strains were subjected to increasing dosea of UVA with or without pretreatment of 8 methoxypsoralsn(8-MOP) which is a prototype of photatoxic druge. The authors made use of a perforated stain-less steel template which provided a simple and eosy monitoring of ultraviolet irradiation effects i.e. clear zones due to inhibition of the atrains could be determined. By using this methad, the authors acquired the following results .' I. 8-MOP alone exerted no inhibition on both strains at concentration upto 100mg /ml. 2. UVA irradiation alone showed no growth inhibition at dose upto 5J/cm. 3. UVA irradiation after pretreatment with 8 MOP resulted varying growth inhibition in proportion to irradiation doses. 4. Authors found a suitable concentration of 8-MOP for this test is 10pg/ml. With this ccncentrstion, minimal phatatoxic dose of UVA were O.l J/cm for the strain TA98 and 1.0J/cm for the strain TA102 respectively.
Dermatitis, Phototoxic ; DNA Repair ; Methoxsalen ; Salmonella ; Steel

Scleroderma is an incidious and chronic disease of unknown cause. The clinical picute of scleroderma is characterized by the apperance of circumscribed of diffuse, hard, smooth, ivory colored areas that are immobile upon upon the underlying tissues. It may be classified to as a localized form (morphea) and a systemic scleroderma or progressive systemic sclerosis (PSS) which is characterized by diffuse involvment of the connective tissue of the skin and certain intemal organs. The authors observed 3 cases of PSS and 6 cases of morphea. They have complained of marked sclerosis or thickening of skin or slowly developing dyspneadue to interstitial pulmonary fibrosis which were confirmed by chest X-ray. Recently, penicillamine-D has been advocated as a beneficial agent in scleroderma by Asboe-Hansen. therefore, penicillamine-D was administered to the above patients. Two of PSS were markedly improved with normal skin softness. Two of morphea were also moderately improved. Other patients remained stationary or no effect.
Chronic Disease ; Connective Tissue ; Humans ; Pulmonary Fibrosis ; Scleroderma, Diffuse ; Scleroderma, Localized ; Scleroderma, Systemic ; Sclerosis ; Skin ; Thorax

A 21-year-old male suffered from generalized anhidrosis and heat intolerance for about 15 years. Other clinical features included multiple angiokeratoma on the back, scrotum and extremities, together with ocular involvement. The electronmicroscopic studies of the skin demonstrated laminated intracytoplamic inclusions in the endothelial cells, pericytes of dermal vessels, fibroblasts and eccrine glandular cells. a Galactosidase assay of leukocytes from the patient and his uncle showed nearly absence of the activity,
Angiokeratoma ; Endothelial Cells ; Extremities ; Fibroblasts ; Galactosidases ; Hot Temperature ; Humans ; Hypohidrosis* ; Leukocytes ; Male ; Pericytes ; Scrotum ; Skin ; Young Adult

The lacrimal fossa can be invovled by a wide specturm of orbital pathology. The correct diagnosis is importantto avoid unnecessary procedure and to do appropriate management. 14 patients with mass lesions in the lacrimalfossa were evaluated with CT and clinical findings. The results were as follows: 1. Final diagnosis of 14 caseswith lacrimal fossa tumors was pleomorphic adenoma in 3 cases, adenoid cystic carcinoma in 1 case, pseudotumor incases, lymphoma in 2 cases, neurofibroma in 1 case, chloroma in 1 case and metastatic adenocarcinoma in 1 case. 2.The duration of symptoms of pleumorphic adenoma was more than 1 year and characteristic CT findings were globularmasses with pressure erosion of the adjacent bone. Patient wih adenoid cystic carcinoma had a short history ofsymptoms. CT showed a fusiform mass but intracranial extension with frank destruction of sphenoid bone. 3.Patients wtih pseudotumor and lymphoma had symptoms for less than 1 year. The CT findings were ill-definedinfiltrative patterns with scleral thickening and the differential diagnosis of them was difficult. 4. The marginsof neurofibroma and chloroma were well defined while that of the metastatic adenocarcinoma was ill-defined. 5. Thedegree and the extent of the contrast enhancement gave no benefit in the differential diagnosis of each diseaseentities and even of the benign and malignant lesions.
Adenocarcinoma ; Adenoma ; Adenoma, Pleomorphic ; Carcinoma, Adenoid Cystic ; Diagnosis ; Diagnosis, Differential ; Humans ; Lymphoma ; Neurofibroma ; Orbit ; Pathology ; Sarcoma, Myeloid ; Sphenoid Bone ; Unnecessary Procedures

The Kniest syndrome is characterized by disproportionate dwarfism and Kyphoscoliosis which may be associated with flat facies with prominent eyes, cleft palate, hearing loss, myopia and limited joint motion. The skeletal abnormalities are recognizable at birth with shortening and deformity of the extremities and stiff joints. Marked lumbar lordosis and kyphoscoliosis develop in childhood, resulting in disproportionate shortening of the trunk. We experienced a case of kniest syndrome, confirmed by clinical features, radiological features, and histological examination of cartilage. A brief review of the related literature is presented.
Animals ; Cartilage ; Cleft Palate ; Congenital Abnormalities ; Dwarfism ; Extremities ; Facies ; Hearing Loss ; Joints ; Lordosis ; Myopia ; Parturition

No abstract available.
Acrospiroma*

We described a case of diffuse biphasic cutaneous amyloidosis, a unique form of localized cutaneous amyloidosis. A 41-year-old man has gradually developed a lichenoid papular and a grouped spotted pigmented macular eruption on the trunk and upper extremities over the past 15 years. Histopathologic examination revealed that amyloid deposits were present in the papillary dermis. It was confirmed by Congo red staining, immunohistochemistry and electron microscopy. There was no evidence of systemic amyloidosis.
Adult ; Amyloidosis* ; Congo Red ; Dermis ; Humans ; Immunohistochemistry ; Microscopy, Electron ; Plaque, Amyloid ; Upper Extremity

No abstract available.
De Lange Syndrome*

PURPOSE: To compare the variability of standard automated perimetry (SAP) between patients with preperimetric glaucoma (PPG) and normal controls. METHODS: This study included 67 eyes, classified into the following groups: 30 eyes, normal controls; and 37 eyes, PPG. All subjects were examined with 24-2 Humphrey static perimetry. The visual field was divided into superonasal, superotemporal, inferonasal, and inferotemporal sectors. The variability of SAP was obtained using the standard deviation of sensitivity points at each location. We compared the variability of SAP between the normal controls and PPG patients. RESULTS: The variability of SAP was higher in the PPG group compared with the normal control group (p < 0.001). There was also a significant correlation between the variability of SAP and intraocular pressure fluctuations and retinal nerve fiber layer thickness (both p < 0.05). The variability of SAP in the PPG group was higher (p < 0.05) in all areas except the inferotemporal sector compared with the normal control group. CONCLUSIONS: The variability of the SAP increased in PPG patients compared with normal controls, even when the visual field test results were normal.
Glaucoma ; Humans ; Intraocular Pressure ; Nerve Fibers ; Retinaldehyde ; Visual Field Tests ; Visual Fields