... treatment, and early hematopoietic stem cell transplantation will increase the patients’ chances of survival, especially for late-onset forms. Graphical Abstract Graphical Abstract autoimmunity erythroderma Omenn syndrome RAG1/2 severe combined immunodeficiency vasculitis The recombination...

... of epidermolytic hyperkeratosis (bullous congenital ichthyosiform erythroderma) . J Invest Dermatol   1992 ; 99 : 19 – 26 . 3 Virtanen M , Smith SK , Gedde‐Dahl T Jr  et al.   Splice site and deletion mutations in keratin (KRT1 and KRT10) genes: unusual...

... (54%) had nasal colonization. Staphylococcus aureus was isolated from 44 patients, 33 (31%) each from skin and nares. Colonization was highest in erythrodermic SS (48%), similar to atopic dermatitis (64%), and lowest in MF without erythroderma (26%), psoriasis (21%), and the general...

... not revealed any further metastatic disease. He is under regular review by dermatologists, plastic surgeons and oncologists. His prognosis remains poor. 1 Patient 1 at 21 years old. The characteristic clinical features of NBCIE are evident, namely generalized erythroderma and diffuse scaling. The larger...

...), tamsulosin HCl (June–November 2006) and terazosin HCl (November–December 2006). Under the suspicion of drug‐induced skin eruption, tamsulosin HCl and terazosin HCl were discontinued sequentially. However, the eruption persisted and progressed from the abdomen and upper chest to erythroderma within 3 months...

.... ⁵ In a series of 41 Sudanese children with rickets due to vitamin D deficiency, three had ichthyosis, but the 25‐hydroxyvitamin D levels were not described. ⁶ Rickets has also been described in ichthyosis vulgaris ⁷ and nonbullous ichthyosiform erythroderma, ³...

.../standard_publication_model ) erythroderma IgE pemphigoid nodularis psoriasis suplatast tosilate Sir, Bullous pemphigoid (BP) is a chronic autoimmune blistering skin disease characterized by the presence of immunoglobulin (Ig) G autoantibodies specific for the hemidesmosomal BP antigens. ¹ Skin...

... ichthyosiform erythroderma Inherited ichthyoses are disorders of cornification that broadly refer to a group of heterogeneous skin disorders characterized clinically by generalized scaling. ¹ Among these, autosomal recessive congenital ichthyosis is a subgroup comprising three major types...

... by mild epidermal hyperkeratosis over flexural areas, blister formation and the development of superficially denuded areas of hyperkeratotic skin. It is clinically difficult to distinguish severe IBS from mild bullous congenital ichthyosiform erythroderma (BCIE, MIM 113800). In the current literature, 19...

... with blood flow cytometric findings to diagnose primary SS. Many cases will lack the epidermotropism usually seen in mycosis fungoides. Leukemia cutis Cutaneous T-cell lymphoma Exfoliative erythroderma Mycosis fungoides Anatomic Pathology / PRIMARY SÉZARY SYNDROME Primary Sézary Syndrome Commonly Shows...

.../journals/pages/open_access/funder_policies/chorus/standard_publication_model ) erythroderma mycobacteria pneumonia tuberculosis We postulate that the erythroderma in this patient with pulmonary tuberculosis may have been induced by inflammatory mediators. It has been shown that the alveolar macrophages...

...F.B. Müller; I. Haußer; D. Berg; C. Casper; R. Maiwald; A. Jung; H. Jung; B.P. Korge Abstract Summary Netherton syndrome (NS) is a rare autosomal recessive disease with variable expression. It is defined by a triad of symptoms: congenital ichthyosiform erythroderma, trichorrhexis invaginata...

... of keratinization, especially in the case of BIE. References 1 Langtry JA , Carr MM , Ive FA et al. Ichthyosiform erythroderma associated with generalized pustulosis . Br J Dermatol 1998 ; 138 : 502 – 5 . 2 Bale SJ , Compton JG , DiGiovanna JJ . Epidermolytic...

...‐like membrane and diffuse erythema, and had been diagnosed as a collodion baby. PPK and generalized ichthyosis with erythroderma developed subsequently. The constricting bands appeared at the age of 3 years, and affected the proximal interphalangeal (PIP) joints of all fingers. There was diffuse well...

...E. Virolainen; K‐M. Niemi; A. Gånemo; J. Kere; A. Vahlquist; U. Saarialho‐Kere Abstract Congenital ichthyoses are a group of heterogeneous disorders of cornification. Autosomal recessive congenital ichthyosis (ARCI) can be clinically subdivided into congenital ichthyosiform erythroderma...

... bullous congenital ichthyosiform erythroderma (BCIE) and ichthyosis bullosa of Siemens (IBS). Previous studies have shown that these genodermatoses are due to mutations in the KRT1 and KRT2E genes, respectively. We report a new amino acid substitution mutation in codon 155...

...B. Mevorah; M. Landau; A. Gat; P. De Viragh; S. Brenner 1 Erythroderma is evident, with foci of greyish scaling. Although a severe ichthyosiform erythroderma may clinically resemble our case, the lichenoid tissue reaction practically rules out the possibility that our patient was affected by any...

.../chorus/standard_publication_model ) Abstract We report novel mutations in the transglutaminase (TGase) 1 gene (TGM1) in a Japanese boy with non‐bullous congenital ichthyosiform erythroderma (NBCIE). The patient showed fine, grey or light‐brown scales on an erythematous skin. An in situ...

... ichthyosiform erythroderma, and hypernatraemic dehydration which she had for several days. Subsequently, she failed to thrive, with recurrent bacterial infections until 5 months of age, and very high serum IgE levels (1200 U/mL). Trichorrhexis invaginata and pili torti were identified at 18 months. The second...

...Oxford University Press This chapter reviews the various types of congenital ichthyoses that can present in the newborn with erythroderma, hyperkeratosis, scales, or fissures. The presenting symptoms frequently overlap in these conditions. Erythroderma is a finding frequently seen in the ichthyoses...