1/10. Keratotsis punctata of the palmar creases: report of two cases associated with ichthyosis vulgaris.Two patients with keratosis punctata of the palmar creases are described. The association with ichthyosis vulgaris and other disorders of keratinization is discussed. In both cases, histopathology revealed a close relation between the keratotic plug and the sweat glands. The role of genetic factors and manual activity in the pathogenesis is discussed. Treatment with oral etretinate resulted in a good improvement in the first patient, but prolonged low-dose maintenance therapy was required to prevent recurrence.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
2/10. An unusual central retinal dystrophy associated with ichthyosis vulgaris.A number of ichthyosis syndromes may have retinal abnormalities such as the retinitis pigmentosa-like diffuse rod-cone dystrophy in Refsum's syndrome and the maculopathy in sjogren-larsson syndrome. We present two sisters who have an unusual, almost identical, bilaterally symmetric central retinal dystrophy associated with ichthyosis vulgaris in the absence of other systemic disorders. We believe that this dystrophy has not been previously described in patients with any of the known varieties of ichthyosis.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
3/10. New-onset ichthyosis and diabetes in a 14-year-old.Diabetes has not been linked to acquired ichthyosis or ichthyosis vulgaris. We report a newly diagnosed diabetic 14-year-old girl with bilateral tibial and sacral ichthyosiform plaques and a hemoglobin A1c of 20.1%. The patient had no personal or family history of atopy or ichthyosis and lacked keratosis pilaris or hyperlinear palms. A biopsy specimen of an ichthyosiform plaque showed compact lamellar orthohyperkeratosis and hypogranulosis, histopathology consistent with either ichthyosis vulgaris or acquired ichthyosis. We speculate that our patient's new-onset diabetes induced acquired ichthyosis.- - - - - - - - - - ranking = 0.4keywords = vulgaris (Clic here for more details about this article) |
4/10. Dermatophyte infection in ichthyosis vulgaris.A 31-year-old male who had been suffering from ichthyosis vulgaris developed hyperkeratosic lesions on the palms and soles and ungual alterations of both hands and feet. These lesions were resistant to topical corticosteroid and emollient cream treatments and were found to be caused by trichophyton rubrum. association of ichthyosis vulgaris and dermatophytosis--both very common disorders--is to our knowledge a very rare event.- - - - - - - - - - ranking = 1.2keywords = vulgaris (Clic here for more details about this article) |
5/10. Hypoplastic thyroid, growth hormone deficiency, corneal opacities, cataract and hyperkeratotic skin disease: a possible new ichthyosis syndrome associated with endocrinopathies.A 56 year old man presented with ichthyosis vulgaris since early childhood, clinically characterised by fine scaling of the trunk and hyperkeratotic scales on the exterior surfaces of the upper and lower extremities. The patient also showed hypothyroidism due to hypoplastic thyroid, cataract, hypercholesterinemia with concommitant arcus cornealis and biliary concrements. Renal lithiasis caused by calcio-oxalate was additionally present. Endocrinological screening revealed growth hormone deficiency in the 1.55 m tall man-(secondary) osteoporosis was observed. The clinical symptomatology indicates that this case cannot be considered as a subtype of the inherited ichthyosis group, but suggests a new syndrome as a separate nosologic entity.- - - - - - - - - - ranking = 0.2keywords = vulgaris (Clic here for more details about this article) |
6/10. Mixed sclerosing dysplasia of the bone associated with ovarian and skin problems.The clinical, radiologic, histopathologic, and genetic features of a sclerosing bone dysplasia found in three members of a family are described. The bone disorder affected all metaphyseal-diaphyseal regions of all long bones, the skull, and three metacarpals. All affected members also had ichthyosis vulgaris and premature ovarian failure. These features have not been reported before, indicating a new syndrome.- - - - - - - - - - ranking = 0.2keywords = vulgaris (Clic here for more details about this article) |
7/10. Keratohyalin granules are heterogeneous in ridged and non-ridged human skin: evidence from anti-filaggrin immunogold labelling of normal skin and skin of autosomal dominant ichthyosis vulgaris patients.Recent biochemical and morphological investigations have provided evidence for a heterogeneous composition of keratohyalin in human skin. A major component is filaggrin. In interfollicular epidermis the heterogeneity of keratohyalin is not directly visible, whereas in normal ridged skin bicomponent keratohyalin is revealed by electron microscopy. skin biopsies of ridged and non-ridged skin of normal individuals and patients with autosomal dominant ichthyosis vulgaris (ADI)--characterized by defective keratohyalin synthesis and lack of filaggrin--were investigated by routine transmission electron microscopy and immunogold postembedding techniques using a commercial monoclonal anti-filaggrin antibody. In normal interfollicular epidermis filaggrin labelling was demonstrated on keratohyalin granules and in the lowermost cornified cells, whereas in ADI patients crumbly keratohyalin granules were present that did not show specific labelling for filaggrin. In normal ridged skin only the major (more electron-dense) component reacted with anti-filaggrin, whereas the attached (less electron-dense) component did not react. Ridged skin of ADI patients contained globular keratohyalin that did not react with anti-filaggrin, thus corresponding to the attached keratohyalin component in normal ridged skin. Our results provide a visible counterpart to the recent biochemical investigations of keratohyalin protein heterogeneity and contribute to the understanding of terminal differentiation in human skin and of the defective keratohyalin synthesis in ADI.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
8/10. Twenty-nail dystrophy (trachyonychia) caused by lichen planus in a patient with alopecia universalis and ichthyosis vulgaris.A 7-year-old girl with alopecia universalis had dystrophy of all 20 nails. A nail biopsy specimen disclosed features of lichen planus. The patient also had ichthyosis vulgaris and hypogammaglobulinemia. We are not aware of any previous reports of these associations, which we believe to be noncoincidental.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
9/10. keratosis punctata of the palmar creases. A report on three generations, demonstrating an association with ichthyosis vulgaris and evidence of involvement of the acrosyringium.Five patients in the same family showed small keratotic plugs of the palmar creases. Transmission in lineage was consistent with an autosomal dominant pattern of inheritance. The syndrome was highly associated with ichthyosis vulgaris in several members of the family. The histopathology in two patients revealed a close relation between the plugs and the acrosyringium.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
10/10. A study of the steroid sulfatase gene in families with X-linked ichthyosis using polymerase chain reaction.We have studied the steroid sulfatase (STS) gene in three Japanese families with X-linked ichthyosis (XLI), using polymerase chain reaction (PCR). PCR was performed using three sets of intraexonic primers covering exons 1, 5 and 10. In affected individuals from two of the families, dna was not amplified in any of the three exons, suggesting that XLI in these families was due to the complete deletion of the STS gene. In affected individuals in the remaining family, dna was amplified in predicted sizes in exons 1 and 5, but not in exon 10, suggesting that XLI in this family was due to partial deletion of the STS gene including exon 10. These results suggested that STS gene deficiency is heterogeneous in Japanese families with XLI. PCR is useful for the rapid diagnosis of XLI, the differentiation of XLI from ichthyosis vulgaris, and genetic counseling of XLI families. The PCR method was not applicable for carrier detection.- - - - - - - - - - ranking = 0.2keywords = vulgaris (Clic here for more details about this article) |
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