Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty eight patients with various dermatological conditions were treated orally with the new aromatic derivate of retinoic acid, Ro 10-9359. The initial average dose was 48,3 mg/day and the maintenance dose was 26,6 mg/day. Duration of treatment ranged between 3 to 6 months. Evolution of erythema, infiltration and hyperkeratosis showed changes statistically significant (p < 0,05) and excellent to good results were obtained in 23 out of the 28 treated patients. On the basis of this study it is concluded that Ro 10-9359 is a promising drug for the treatment of several skin diseases, specially ichthyosis, Darier's disease, oral lichen planus, erythrokeratoderma variabilis and psoriasis. No serious side effects were reported; dryness of the lips, scaling of palms and soles, pruritus and thinning of the skin were the most common. In no case treatment was discontinued due to side effects. Laboratory controls did not show deviations from the normal values.
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PMID:[Oral treatment of various dermatosis with the aromatic derivative of retinoic acid Ro 10-9359]. 39 25

Interferon-gamma (IFN-gamma) suppresses the synthesis of collagen by fibroblasts in vitro and the synthesis of collagen in vivo in animal models. Therefore, recombinant human IFN-gamma was examined for its ability to clinically modify keloids. Subjects were treated by injection of either 0.01 or 0.1 mg of recombinant human IFN-gamma into one lesional site and diluent alone into another lesional site three times per week for 3 weeks. Keloids were measured and photographed before beginning therapy and weekly thereafter. Three days after the final injection, biopsies were performed on treated and control sites. Six of eight subjects who finished the course of treatment demonstrated reduction in size at the treated site with an average reduction in height of 30.4% vs 1.1% for control sites. Lesions treated with recombinant human IFN-gamma demonstrated alterations in both the epidermis and dermis. The epidermis showed thinning of the suprapapillary plates, compact hyperkeratosis, focal or diffuse parakeratosis, exocytosis of lymphocytes, and an increased quantity of mucin. The dermis contained a diminished quantity of thickened collagen bundles and active fibroblasts and an increased number of inflammatory cells and quantity of mucin. These results suggest the feasibility of using IFN-gamma in the treatment of abnormal fibrosis. Dose-ranging studies are required to establish whether IFN-gamma can fulfill a true clinical need in the treatment of keloidal scarring.
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PMID:A controlled trial of intralesional recombinant interferon-gamma in the treatment of keloidal scarring. Clinical and histologic findings. 212 Nov 4

Twelve cases of pachyonychia congenita were reviewed. The mode of inheritance was autosomal dominant. The clinical features of these patients included thickened nails, hyperkeratosis of the palms and soles, thinning of hair or alopecia, painful bullae or ulcerations of the palms and soles, leukokeratosis oris, verrucous lesions of the extremities, hyperhidrosis, premature eruption of teeth, paronychial infections, epidermal cysts with milia, and corneal dyskeratosis at times associated with cataracts. Biopsy from the plantar lesions usually revealed marked hyperkeratosis, acanthosis, moderate hypergranulosis, and minimal dermal inflammatory infiltration. Treatment with keratolytic agents and lubricants is indicated to areas of palmar and plantar hyperkeratosis but usually produces only transient benefit. Squamous cell carcinoma developed in one of the patients over the site of chronic plantar ulcerations. Areas of chronic bullous formation or ulceration should be observed for possible skin malignancy.
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PMID:Pachyonychia congenita: a clinical study of 12 cases and review of the literature. 214 Apr 47

We report a patient with hyperkeratosis lenticularis perstans (HLP) manifesting as multiple reddish-brown hyperkeratotic papules on the lower extremities. Typical histologic features of HLP include hyperkeratosis, thinning or absence of the granular layer and a band-like infiltrate in the upper dermis underlying an atrophic epidermis. In order to determine the cellular composition of the infiltrate, skin biopsy specimens were studied immunohistochemically using a series of commercially available monoclonal antibodies. The dermal infiltrate consists predominantly of helper/inducer T cells (Leu-4+, Leu-3a+). Suppressor/cytotoxic T cells (Leu-2a+) were fewer at the periphery of the infiltrate. The majority of T cells were activated as they expressed HLA-DR-antigen. Large numbers of Leu-6+ Langerhans' cells were observed at the dermo-epidermal interface. Few natural killer cells (Leu-11b+) were noted within the dermal infiltrate. These findings support the hypothesis than an active cellular immune reaction involving the epidermis is of pathogenic importance for HLP.
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PMID:Hyperkeratosis lenticularis perstans (Flegel's disease). In situ characterization of T cell subsets and Langerhans' cells. 245 81

The experiments on rats kept for 1, 2, 3 and 4 months in phosphorous-producing workshops revealed the influence of elementary phosphorous and its nonorganic derivatives on the mouth mucosa tissues. At the early stages of the experiments the above agents caused the increase in the number of the epithelial layer cell elements, and strengthening of the keratosis process, leading to the development of hyperkeratosis. By the end of the experiment the development of dystrophic and atrophic processes in the epithelium were observed, which sometimes caused thinning of the epithelial layer. The connective tissue of submucous basis was characterized by microcirculation disorders and dystrophic disturbances of blood vessel walls becoming more pronounced by the end of the experiment.
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PMID:[Morphology of the oral mucosa in rats exposed to increased phosphorus levels]. 394 6

Light and electron microscopy were used to study specimens from four patients suffering from lamellar ichthyosis. Three of these patients had been treated with etretinate for 10 months. Biopsy specimens taken from the patients before treatment showed hyperkeratosis with focal parakeratosis, a thickened granular layer in which the cellular content of tonofibrils and keratohyalin was moderately diminished, and acanthosis with increased cellular activity. During treatment with etretinate there was moderate clinical improvement. The most conspicuous microscopic change was thinning of the cornified layer. The intercellular spaces of the epidermis showed increased amounts of fine and coarse granular substance. The number of keratinosomes was increased. Stimulation of Langerhans' cells was observed. Two new ultrastructural findings in lamellar ichthyosis were discovered. First, the marginal band of the cornified layer was absent in conventional glutaraldehyde-osmium tetroxide fixation. Second, the corneocytes contained electron-lucent crystals. The treatment with etretinate did not influence these structures. The mechanism of action of retinoids in lamellar ichthyosis is discussed.
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PMID:New observations on the fine structure of lamellar ichthyosis and the effect of treatment with etretinate. 666 37

The aim of the present study was to investigate the response of normal human skin to repeated courses of Sellotape stripping. The skin of healthy volunteers was stripped five times at 24-h intervals. Skin biopsies were taken before stripping (day 0) and on days 2, 4, 7 and 10. The responses were studied using H & E staining and an immunohistochemical analysis of several aspects of epidermal proliferation and keratinization. Although increased proliferation (nuclear binding to Ki-67 binding), acanthosis and parakeratosis were observed, the overall histological picture did not resemble psoriatic histology completely: no micropustules of Kogoj and no thinning of the suprapapillary plate were observed. Involucrin staining followed the recruitment of cycling epidermal cells showing a statistically significant elevation of positive cell layers from day 2 onwards. Filaggrin expression showed an increase from day 2 onwards, which was statistically significant on day 7 and day 10. Using the anti-keratin antibodies KS8.12 (K13 and K16) and RKSE60 (K10) we observed a fast induction of K13/K16 expression, while the staining of keratin 10 showed the same overall intensity at different time intervals. In conclusion, the response to repeated courses of tape stripping provides an adequate model for studies on epidermal proliferation, hypergranulosis and hyperkeratosis. This approach causes a more prolonged induction of these phenomena than a single course of stripping. In contrast to the situation following a single course of stripping, repeated tape stripping induced the expression of filagrin. Therefore the repeated tape stripping model is less compatible with psoriasis than a single course of stripping.
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PMID:Repeated tape stripping of normal skin: a histological assessment and comparison with events seen in psoriasis. 753 89

An 84-year-old male presented with a dermatophyte infection that had spread over nearly the entire body surface. The first signs had developed 48 years before. After treatment with galenic and corticosteroid preparations for diagnoses of "eczema" and "psoriasis", the lesions gradually extended over the body and to the nails. The cutaneous symptoms had worsened in recent year after the patient had started systemic cortisone treatment for bronchial asthma. He also developed diabetes mellitus, papulonodular lesions on the face and limbs, thinning of his hair and eyebrows and hyperkeratosis of the soles and palms.
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PMID:[Universal dermatophytosis (tinea incognito) caused by Trichophyton rubrum]. 775 46

Although nail abnormalities have been reported to occur in 1% to 10% of patients with lichen planus, in children with lichen planus they are rarely mentioned in the literature. An 11-year-old boy had a two-month history of nail dystrophy affecting all the fingernails and the great toenails. The nail plates showed longitudinal ridging and thinning as well as onycholysis and distal splitting. There were no cutaneous or mucous membrane abnormalities. A nail biopsy specimen showed hyperkeratosis, hypergranulosis, and acanthosis in the ventral portion of the proximal nail fold and in the nail matrix. A band-like lymphocytic infiltrate was present in the superficial dermis, and the basal layer showed vacuolar alterations. A diagnosis of lichen planus was made. Treatment was intramuscular triamcinolone 20 mg once a month for six months. Since 1969 only 13 proved pediatric cases of lichen planus limited to the nails have been reported, including two children with 20-nail dystrophy and four with idiopathic atrophy of the nails.
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PMID:Lichen planus limited to the nails in childhood: case report and literature review. 849 65

Monilethrix is characterized by beaded or moniliform hair, which results from the periodic thinning of the hair shaft. The beaded hair thus produced is subject to excess weathering and premature fracturing at the internodes. Clinically, monilethrix presents with short, fragile, broken hair. The follicular abnormalities range from subtle perifollicular abnormalities range from subtle perifollicular erythema and hyperkeratosis to horny follicular papule formation. At the ultrastructural level, cytolysis and keratin tonofilament clumping (epidermolysis) are seen in the cortical cells of the bulb of the hair follicle. Microsatellite markers flanking the keratin gene clusters at 17q12-q21 and 12q11-q13 were used to perform linkage analysis in a monilethrix pedigree. This study demonstrates linkage of monilethrix in a pedigree to microsatellite DNA loci mapping to the region on chromosome 12 containing the type II keratin cluster. A major group of structural hair proteins, the basic type II trichocyte keratins, map within this epithelial cytokeratin gene cluster. This study implicates a mutation in a trichocyte keratin gene in the pathogenesis of a structural hair disorder.
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PMID:Linkage of monilethrix to the trichocyte and epithelial keratin gene cluster on 12q11-q13. 861 25


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