1/130. Phakomatosis pigmentovascularis: A new case with renal angiomas and some considerations about the classification.We report phakomatosis pigmentovascularis detected in a Caucasian child characterized by the presence of a nevus flammeus and nevus anemicus on the face, a telangiectatic linear nevus of the right leg, and a very extensive blue spot covering 60% of the body surface, with ocular melanosis. Multiple angiomatous lesions of the kidney are associated without alterations of the central nervous system (CNS). This association has not been reported before; it could be a further expression of the complex of developmental defects. Our case corresponds exactly to type IIb in the classification of phakomatosis pigmentovascularis proposed by Hasegawa. As this classification seems very extensive, the higher incidence of cases corresponding to the second subtype suggests that we should identify it by the term phakomatosis pigmentovascularis, while the others could be considered as only very uncommon variants.- - - - - - - - - - ranking = 1keywords = ocular (Clic here for more details about this article) |
2/130. Pigmented purpuric dermatosis in a young male.The pigmented purpuric dermatoses (PPD) are a group of disorders that most often occur on the lower extremities of middle-aged adults as asymptomatic, yellow-orange patches with petechiae. The PPD represent a benign, often chronic, capillaritis of unknown cause. We present a case of PPD on the abdomen of a young male and emphasize careful observation in such a patient to reveal possible progression to purpuric mycosis fungoides.- - - - - - - - - - ranking = 1.8382366299326keywords = mycosis (Clic here for more details about this article) |
3/130. Brachycephalosyndactyly with ptosis, cataract, colobomas, and linear areas of skin depigmentation.A male patient with brachycephalosyndactyly syndrome associated with ocular and skin anomalies is reported and it is suggested that this patient has a previously undescribed disorder.- - - - - - - - - - ranking = 1keywords = ocular (Clic here for more details about this article) |
4/130. Koraxitrachitic syndrome: a syndromic form of self-healing collodion baby with residual dappled atrophy of the derma.We report on a child with a generalized skin disorder associated with other minor anomalies. At birth, the child presented as a collodion baby, with patchy erythema, generalized irregular dermal atrophy, alopecia, absent eyelashes and eyebrows, and conjunctival pannus. He also had hypertelorism, prominent nasal root, large mouth, micrognathia, brachydactyly, syndactyly involving all interdigital spaces, and camptodactyly of fingers III-V. The hyperkeratotic membrane thinned progressively, leaving a mottled reticulated skin atrophy, with patchy areas of yellowish hyperpigmentation and papyraceous areas. hair and nails were dystrophic. Mental development was borderline normal. The histological hallmarks of the skin manifestations combined orthokeratotic hyperkeratosis and marked atrophy of the dermis. The dermal extracellular matrix was immature, and factor xiii-a positive dendrocytes were rare and globular rather than dendritic. We frame as a hypothesis that the disease is due to or associated with a defect in maturation of a subset of dermal dendrocytes during fetal life. This entity may be designed as the koraxitrachitic syndrome (kappaomicronrhoalphaxi:grapnel- taurhoalphachiiotatauepsilonsigma: roughness) copyright 1999 Wiley-Liss, Inc.- - - - - - - - - - ranking = 0.036070741091674keywords = eye (Clic here for more details about this article) |
5/130. Latanoprost-induced iris color darkening: a case report with long-term follow-up.PURPOSE: To determine the effect on iris color of discontinuing latanoprost (LP) treatment in a patient with pronounced iris color darkening, and to assess the possible role of sympathetic innervation. methods: In a patient demonstrating pronounced iris color darkening in both eyes after treatment with LP for 6 months, magnified iris color photographs were taken at 3- to 6-month intervals for 5 years after discontinuation of LP treatment. Pupillary testing for sympathetic insufficiency was performed with cocaine 10% or hydroxyamphetamine 1%. RESULTS: The iris color did not appreciably change after discontinuing LP. The cocaine-induced increase in pupillary diameter was considerably greater for the control subject than for the patient who demonstrated the LP-induced color change. CONCLUSIONS: Latanoprost-induced iris color darkening does not appreciably change for several years after discontinuing treatment. Some eyes that show LP-induced darkening may have relative ocular sympathetic insufficiency.- - - - - - - - - - ranking = 1.0360707410917keywords = ocular, eye (Clic here for more details about this article) |
6/130. Increased eyelid pigmentation associated with use of latanoprost.PURPOSE: To report increased eyelid pigmentation as an adverse side effect associated with topical latanoprost. METHOD: Case report. A 62-year-old Korean woman with normal-tension glaucoma developed bilateral increased eyelid skin pigmentation 4 months after beginning treatment with latanoprost in both eyes. Clinical examinations were performed, and external photographs were taken. RESULT: Latanoprost was discontinued. Periodic examinations revealed that the eyelid skin pigmentation gradually diminished 1 month after the cessation of the drug, and decrease in pigmentation continued over 4 months of follow-up. CONCLUSION: An increase in eyelid skin pigmentation is a possible complication of topical latanoprost therapy, and the cessation of the drug can result in loss of induced pigmentation in humans.- - - - - - - - - - ranking = 0.16231833491253keywords = eye (Clic here for more details about this article) |
7/130. Alkaptonuric ochronosis presenting as palmoplantar pigmentation.We describe a 37-year-old woman who presented with palmoplantar pigmentation, thickening and pitting of 4 years duration. Bluish pigmented patches were seen over the sclera of her eyes. Her lumbar spine showed typical calcification of the intervertebral discs. Addition of Benedict's reagent to a urine sample of the patient gave rise to greenish brown precipitate and brownish black supernatant. Alkalinization of urine turned it black. A biopsy of the palmar lesion demonstrated irregular breaking up, swelling and homogenization of collagen bundles in the reticular dermis. Yellow-brown (ochre coloured) pigment was seen lying within the collagen bundles and also freely in the deeper dermis confirming our clinical diagnosis of alkaptonuric ochronosis. To the best of our knowledge this is probably the second report of alkaptonuria presenting with palmoplantar pigmentation.- - - - - - - - - - ranking = 0.018035370545837keywords = eye (Clic here for more details about this article) |
8/130. Recurrent digital fibroma, focal dermal hypoplasia, and limb malformations.Recurrent digital fibroma of infancy generally is considered a sporadic tumor of childhood. We describe the case of a mother with recurrent digital fibroma at a young age who gave birth to a daughter with focal dermal hypoplasia, coloboma of the iris and eyelids, anal atresia, and extensive limb malformations. When the infant was 3 months old, fibromas started to appear at the fingertips. The cases of three additional patients are described, with a similar combination of multiple digital fibromas, pigmented marks on the temporal region, and limb malformations. One of these patients has consanguineous parents. The clinical findings overlap partially with Gorlin-Goltz syndrome, which has been renamed by some authors "microphthalmia with linear skin defects" (MLS). Since the skin signs are clearly different, however-more like those of Setleis syndrome ("forceps mark" temporal dysplasia)-the patients described here seem to have a new combination of congenital malformations. Deletion of distal Xp, known to occur in some MLS patients, was not detected using cosmids in fluorescence in situ hybridization. This pattern of digital fibroma with congenital malformations seems to represent a new syndrome.- - - - - - - - - - ranking = 0.018035370545837keywords = eye (Clic here for more details about this article) |
9/130. Pigmented paravenous retinochoroidal atrophy: a literature review supported by a unique case and insight.Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare disorder of unknown origin characterised by bone corpuscle pigmentation accumulation along the distribution of the retinal veins. In addition there are peripapillary pigmentary changes as well as areas of chorioretinal atrophy adjacent to the perivenular pigmentary changes. The finding of PPRCA is usually incidental and does not affect vision. The literature regarding this condition is reviewed. Its natural course has been considered controversial. observation of the initial insult leading to PPRCA has not previously been reported. A case that provides a photographic record over 20 years of the development of this condition from the initial insult, including the gradual development of the characteristic fundus appearance, is described. The initial presentation was with a sudden reduction of vision and gross diffuse macular oedema in one eye, which was rapidly followed by similar involvement of the fellow eye despite treatment. Thus it was possible to examine the patient at the stage of the initial insult, 5 years before the development of the typical and pathognomonic retinal changes of PPRCA. The clinical and electrophysiological findings are discussed, as also is the relevance of this case to the literature reviewed.- - - - - - - - - - ranking = 0.036070741091674keywords = eye (Clic here for more details about this article) |
10/130. Reticulate acropigmentation of Kitamura: first case reports from nepal.Two young Nepalese females, aged 34 and 17 years, presented with multiple small brown macules in a reticulate pattern on the distal extensor aspects of their limbs. The first patient's daughter also had similar lesions. The second patient had similar lesions on her eyelids and palmar pits as well. The clinical diagnosis of reticulate acropigmentation of Kitamura (RAK) was made in both the patients and was confirmed histopathologically. These are the first case reports of this condition from nepal.- - - - - - - - - - ranking = 0.018035370545837keywords = eye (Clic here for more details about this article) |
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