1/183. Hereditary perioral pigmented follicular atrophoderma associated with milia and epidermoid cysts.Eight members of a single family all presented the characteristic changes of facial, especially perioral, pigmented follicular atrophoderma, with numerous milia and epidermoid cysts. For this condition. diagnosis at a glance may be possible because of the perioral cutaneous manifestations. Histopathological examination of follicular atrophoderma revealed proliferation of basaloid cells continuous with the epidermis and coarse collagen fibres, with a decreased density of elastic fibres around the basaloid cells. Two of the eight individuals also showed generalized hypohidrosis. The eight affected persons were the proband, her son, mother, uncle, two younger sisters, cousin and nephew: an autosomal dominant mode of transmission was suggested from this family tree. The patients' symptoms resembled those of Bazex-Dupre-Christol syndrome, except for the different distribution of the follicular atrophoderma and the absence of basal cell carcinoma and hypotrichosis. This disease may be an entirely new syndrome characterized by perioral pigmented follicular atrophoderma associated with milia and epidermoid cysts.- - - - - - - - - - ranking = 1keywords = pigment (Clic here for more details about this article) |
2/183. Upward extension of an atrophic tract of the retinal pigment epithelium associated with congenital macular toxoplasmosis.PURPOSE: To report an unusual case of gravitational atrophic tract of the retinal pigment epithelium in a 20-year old woman. methods: Case Report. RESULTS: The patient had macular cicatricial congenital toxoplasmic chorioretinitis in both eyes. In the right eye, an atrophic tract of the retinal pigment epithelium originating from the upper margin of the macular scar extended upwards toward the retinal periphery. CONCLUSION: The unusual upward direction of the atrophic tract of retinal pigment epithelium may be explained by the in utero head position during the active phase of the chorioretinal disease.- - - - - - - - - - ranking = 18.23260217017keywords = retinal pigment, pigment (Clic here for more details about this article) |
3/183. Plantar fat pad atrophy after corticosteroid injection for an interdigital neuroma: a case report.A case of bilateral interdigital (Morton's) neuroma treated with steroid injection therapy developed plantar fat pad atrophy, shown on magnetic resonance imaging. Some pathologic changes at the site of injection (such as subcutaneous fat atrophy, depigmentation of the skin, and telangiectasias) are well known disadvantages of local steroid injection for the treatment of the Morton's neuroma. Scientific literature reports these problems (mainly as an aesthetic problem) in the dorsal aspect of the foot. In this work, the authors describe a case in which the steroid injection therapy has caused some changes in the plantar aspect of the feet, with serious functional problems. Fat pad atrophy is a serious problem in the foot and can cause a painful metatarsal syndrome with some important effects on the gait.- - - - - - - - - - ranking = 0.16666666666667keywords = pigment (Clic here for more details about this article) |
4/183. Extensive chorioretinal atrophy in Vogt-Koyanagi-Harada disease.PURPOSE: To report extensive chorioretinal atrophy during the long-term course of Vogt-Koyanagi-Harada (VKH) disease not treated properly in the initial phase. CASES: Four patients with VKH disease were examined more than 10 years after onset of the disease. OBSERVATIONS: They presented initially with classic features of VKH disease, except 1 patient who had developed bilateral, acute angle-closure glaucoma as the initial sign. Two patients received systemic corticosteroid therapy at the acute phase of the disease. During the follow-up of 13-34 years subsequent to onset, these patients had chronic recurrent anterior uveitis with apparently stable depigmented fundus. Eventually, they developed diffuse, extensive chorioretinal atrophy that resulted in severe visual loss. One patient had an unusual familial occurrence of the disease. CONCLUSIONS: Failure to prescribe proper corticosteroid therapy in the initial phase of VKH disease may lead to chronic recurrent uveitis. Long-standing uveitic reactions may eventually result in severe visual loss due to extensive chorioretinal degeneration.- - - - - - - - - - ranking = 0.16666666666667keywords = pigment (Clic here for more details about this article) |
5/183. Anterior segment ischemia and sector iris atrophy: after strabismus surgery in a patient with chronic lymphocytic leukemia.A 69-year-old woman with chronic lymphocytic leukemia developed segmental iris atrophy and iridocyclitis after routine surgery for exotropia. Both the clinical picture and fluorescein angiogram indicated anterior segment ischemia. It is postulated that this was related to hyperviscosity of the blood caused by a high white blood cell count (114,000/cu mm). The possibility of anterior segment ischemia should be kept in mind when contemplating strabismus or retinal detachment surgery in the presence of hematologic disorders likely to increase blood viscosity. In these cases a minimal amount of surgery should be done with proper supportive therapy. strabismus surgery should be done in stages allowing for hemodynamic compensation between procedures.- - - - - - - - - - ranking = 0.14374783715024keywords = detachment, retinal detachment (Clic here for more details about this article) |
6/183. A case of McLeod syndrome with chronic renal failure.A 50-year-old man with the rare McLeod syndrome, associated with glomerular lesion to the end stage of chronic renal failure and death, is reported. McLeod syndrome is an X-linked recessive disorder on the basis of abnormal expression of the Kell blood group antigens and absence of erythrocyte surface Kx antigen. Most often the clinical and pathological findings are retinitis pigmentosa to blindness, progressive chronic neuropathy, cortical atrophy, dilated cardiomyopathy, and glomerular lesion with chronic renal failure. Among the laboratory parameters the most important are very low level of cholesterol and triglycerides, then various numbers of acanthocytes in peripheral blood smears and sometimes in urine (as in our case).- - - - - - - - - - ranking = 0.16666666666667keywords = pigment (Clic here for more details about this article) |
7/183. Atrophie blanche-like scarring after pulsed dye laser treatment.Pulsed dye laser treatment is well established for the treatment of port-wine stains and other vascular skin lesions. Although hyperpigmentation is quite common, other side effects such as hypopigmentation and atrophic scarring occur infrequently, and hypertrophic scarring is rare.- - - - - - - - - - ranking = 0.33333333333333keywords = pigment (Clic here for more details about this article) |
8/183. Mitochondrial maculopathy: geographic atrophy of the macula in the MELAS associated A to G 3243 mitochondrial dna point mutation.PURPOSE: To report ocular findings in the mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (melas syndrome) in a family with the A to G 3243 mitochondrial (mt) dna point mutation. methods: case reports. Ocular findings are described from four family members with the MELAS associated A to G 3243 mt dna point mutation. RESULTS: Findings included ophthalmoplegia, neurosensory deafness, reduction of photopic and scotopic electroretinogram b-wave amplitudes, and myopathy, as well as macular retinal pigment epithelial atrophy. No family members had nyctalopia, attenuation of retinal blood vessels, or retinal bone spicule pigmentation. CONCLUSION: The finding of slowly progressive macular retinal pigment epithelial atrophy expands the reported phenotypic diversity of patients with A3243G mt dna mutations.- - - - - - - - - - ranking = 5.3759815724294keywords = retinal pigment, pigment (Clic here for more details about this article) |
9/183. Allogenic fetal retinal pigment epithelial cell transplant in a patient with geographic atrophy.PURPOSE: To test the hypothesis that healthy fetal retinal pigment epithelium (RPE) can rescue the remaining viable RPE and choriocapillaries and thereby the photoreceptors in non-neovascular age-related macular degeneration (ARMD) (geographic atrophy [GA]). methods: A 65-year-old legally blind woman with non-neovascular ARMD underwent fetal RPE transplantation. Best-corrected visual acuity testing, detailed fundus examination, fundus photography, fluorescein angiography, scanning laser ophthalmoscope macular perimetry, and humoral and cellular immune response testing were performed. A suspension of RPE was infused into the subretinal space through a retinotomy along the superotemporal arcade at the edge of the area of GA. The patient did not take systemic immunosuppressants. RESULTS: The patient's vision remained unchanged for 5 months after the surgery. fluorescein angiography after transplantation showed leakage and staining at the level of the outer retina. There was progressive subretinal fibrosis in the area of the transplant. Immune response studies showed a weakly positive mixed lymphocyte response against phosducin and rhodopsin. CONCLUSION: Although it is surgically feasible to transplant fetal RPE to the subretinal space of patients with GA, such an allogenic RPE transplant without immunosuppression leads to leakage on fluorescein angiography and eventual fibrosis. A very weak immune response against proteins associated with photoreceptors is also of concern.- - - - - - - - - - ranking = 13.023287264407keywords = retinal pigment, pigment (Clic here for more details about this article) |
10/183. lichen planus with involvement of all twenty nails and the oral mucous membrane.A 57-year-old man had had deformities of all ten fingernails for one and a half years before presentation and deformities of all ten toenails for the previous six months. The surfaces of the nails were rough, with excessive longitudinal striations. The bases of the nails were slightly hypertrophic, and the tips were atrophic and itchy. A longitudinal nail biopsy including the nail matrix revealed the typical histology of lichen planus. Reticulated pigmentation, maceration, and erosion on the buccal mucous membrane were also discovered. Histological analysis of the buccal mucous membrane revealed lichen planus intermingled with eosinophils. Immunological blood analysis revealed elevated CD4 T cells and CD4/CD8 ratio. He worked as a tinsmith and had dental metal. The metal series patch test revealed positive reactions to chromate and tin. Treatment with systemic steroids was quite effective in treating the nail lesions.- - - - - - - - - - ranking = 0.16666666666667keywords = pigment (Clic here for more details about this article) |
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