Ichthyosiform erythroderma
Progress of a Harlequin Fetus to Nonbullous Ichthyosiform Erythroderma
... babies progress to nonbullous ichthyosifonmeny- throdenrna-lamellar ichthyosis.” The clinical ap- pearance of harlequin fetus with its extremely thick, rigid, angulated scaling, severe e[r] ...
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Elevated n alkanes in congenital ichthyosiform erythroderma Phenotypic differentiation of two types of autosomal recessive ichthyosis
... Because patients with this form of ichthyosis exhibit a wide range of clinical involvement, some authorities have lumped this heterogeneous group into a single disorder, lamellar ichthyo[r] ...
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NONBULLOUS CONGENITAL ICHTHYOSIFORM ERYTHRODERMA: A CASE TREATED WITH METHOTREXATE
... Pediatrics is owned, published, and trademarked by the Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it has.[r] ...
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Epidermolytic hyperkeratosis (bullous congenital ichthyosiform erythroderma) Genetic linkage to chromosome 12q in the region of the type II keratin gene cluster
... Based on these similarities between EHK and epidermolysis bullosa simplex, we used linkage analysis to test the hypothesis that the EHK phenotype is due to mutations in the suprabasilar [r] ...
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Ichthyosis with confetti: clinics, molecular genetics and management
... Ichthyosis with confetti (IWC) is an autosomal dominant congenital ichthyosis also known as ichthyosis variegata or congenital reticular ichthyosiform erythroderma. It manifests at birth with generalized ...
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Ichthyosis and Methotrexate: A Word of Caution
... basis of genetic, clinical, histopathological, and epidermal cellular kinetic considerafions.3 Lamellar ichthyosis (non-bullous congenital ichthyosiform erythroderma) and epidermolytic..[r] ...
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Clinical diversity of epidermolytic hyperkeratosis A study of 5 cases
... congenital ichthyosiform erythroderma to differentiate from non bullous congenital ichthyosiform erythroderma (NBIE) (ShyhDyi Chuang, ...
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Erythroderma: study of clinico etiology and complications from Western India
... Pre-existing dermatoses was present in 54.83 % patients which included chronic plaque psoriasis (25.8%), Non bullous ichthyosiform erythroderma (NBIE) (6.45%), pemphigus foliaceus(6.45%) and ...
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Alitretinoin reduces erythema in inherited ichthyosis
... congenital ichthyosiform erythroderma (two patients who carried ABCA12 mutations, and one who had no identi- fied ...congenital ichthyosiform erythroderma, one with KLICK syndrome, one with ...
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Thirteen year retrospective review of the spectrum of inborn errors of metabolism presenting in a tertiary center in Saudi Arabia
... CHILD: Congenital hemidysplasia with ichthyosiform erythroderma and limb defects; CSF: Cerebrospinal fluid; IEM: Inborn error of metabolism; IEMs: Inborn errors of metabolism; IRB: Insti[r] ...
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A novel mutation of ABHD5 gene in a Chanarin Dorfman patient with unusual dermatological findings
... CDS is an autosomal recessive neutral lipid disease that affects the skin, eyes, central nervous system, skeletal muscle, liver and bone marrow. While the extracuta- neous manifestations are heterogeneous both in charac- ...
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Development of ichthyosiform skin compensates for defective permeability barrier function in mice lacking transglutaminase 1
... generalized ichthyosiform skin ...congenital ichthyosiform erythroderma (CIE) (MIM 242100) (2, 3), although ARCI consists of more heterogeneous disorders with a variety of distinct fea- ...and ...
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Erythroderma and extensive poikiloderma – a rare initial presentation of dermatomyositis: a case report
... on erythroderma in dermatomyositis ...with erythroderma, epidermal necrosis, and symmetrical proximal muscle weakness ...that erythroderma is a predictive factor for a malignancy in patients with ...
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Xeroderma pigmentosum: a rare genodermatosis
... X-linked recessive disorders Dyskeratosis congenita Disorders with malignant potential and keratinization Basal cell nevus syndrome Palmoplantar keratodermas Ichthyosiform dermat[r] ...
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Consequences of beta glucocerebrosidase deficiency in epidermis Ultrastructure and permeability barrier alterations in Gaucher disease
... The presence of ichthyosiform skin changes in both the glucocerebrosidase-deficient transgenic mice and in some type 2 Gaucher patients suggests that blockade of glucosylceramide hydroly[r] ...
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Syndrome of Erythroderma, Failure to Thrive, and Diarrhea in Infancy: A Manifestation of Immunodeficiency
... Our patients share with these cases the triad of generalized erythroderma, failure to thrive, and diarrhea, but the evidence suggests that they have a different condition. None of our pa[r] ...
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Breast Cancer Presenting as Paraneoplastic Erythroderma: An Extremely Rare Case
... Histopathology has been shown to help identify the cause in up to 50% of erythroderma cases. In particular lymphoma; hereditary disorders (ichthyosis, pityriasis rubra pilaris); various types of eczema, pemphigus, ...
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Topical Hypochlorite and Skin Acidification Improves Erythroderma of Omenn Syndrome
... Omenn syndrome is a severe combined immunodeficiency syndrome with low to absent B cells, autoreactive T cells with a restricted T-cell receptor repertoire, and normal natural killer cells. In addition to ...
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Vol 26, No 4 (2016)
... A total of 50 patients were included in the study of which 39 were males and 11 females. The age group ranged from 10 to 75 years. Mean age group was 46.5years. The common skin disorders included in this study were ...
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EFFECTIVE AYURVEDIC TREATMENT ON PSORIATIC ERYTHRODERMA (EKA KUSHTHA) - A CASE STUDY .......
... Psoriatic Erythroderma is a rare type of psoriasis, occurring once or more during the lifetime of 3 percent of people who have psoriasis. It generally appears in the people who have unstable plaque psoriasis. It ...
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