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Farida Benhadou, Guillaume Hellgren, Fabienne Willaert, Véronique Del Marmol
Tumor necrosis factor-α (TNF-α) normally binds to TNF-α receptors, leading to the inflammatory response of autoimmune diseases. Adalimumab is a TNF-inhibiting, anti-inflammatory, biological medication which binds to TNF-α, thus reducing this inflammatory response. The use of TNF-α-inhibiting medication, such as adalimumab, being the first FDA-approved treatment for hidradenitis suppurativa, has drastically changed the management of dermatological diseases. One rarely reported manifestation that occurs as a side effect associated with the use of TNF-α-blocking agents is erythroderma...
January 2018: Case Reports in Dermatology
Alexander K C Leung, Benjamin Barankin, Kin Fon Leong
BACKGROUND: Staphylococcal-scalded skin syndrome (SSSS), also known as Ritter disease, is a potentially life-threatening disorder and a pediatric emergency. Early diagnosis and treatment is imperative to reduce the morbidity and mortality of this condition. The purpose of this article is to familiarize physicians with the evaluation, diagnosis, and treatment of SSSS. DATA SOURCES: A PubMed search was completed in Clinical Queries using the key terms "Staphylococcal scalded skin syndrome" and "Ritter disease"...
March 5, 2018: World Journal of Pediatrics: WJP
D Miyamoto, C W Maruta, C G Santi, P Zoroquiain, A B T Dias, J J Mansure, M N Burnier, V Aoki
BACKGROUND: Erythroderma is a severe manifestation of pemphigus foliaceus (PF), a blistering disease mediated by IgG autoantibodies against desmoglein-1. Increasing evidence supports the contribution of angiogenic mediators in the pathogenesis of erythroderma. OBJECTIVE: To evaluate the in situ expression of vascular endothelial growth factor (VEGF) and endoglin in PF patients with erythroderma. METHODS: Formalin-fixed paraffin-embedded skin samples obtained from patients with erythrodermic PF (n=19; 12 patients with endemic PF), non-erythrodermic PF (n=17), pemphigus vulgaris (PV; n=10), psoriasis (n=10), and healthy individuals (HI; n=10) were processed in an automated immunohistochemistry platform utilizing anti-VEGF and anti-endoglin as primary antibodies...
February 28, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Javier Arellano, Mathias Yagnam, Martín Vidal, Yamile Corredoira
HIV infection can be manifested with different skin symptoms, which are sometimes considered infection markers. Erythrodermic psoriasis is a possible manifestation, which is a widespread form of psoriasis. We report a clinical case of a young man suspected of HIV infection due to a psoriatic erythroderma confirmed by biopsies, associated with Kaposi sarcoma. Afterwards, HIV infection was confirmed by serological tests. Antiretroviral therapy was started, with positive response at one month of treatment. Erythrodermic psoriasis can be considered a skin marker of HIV infection when occurs in previously healthy patients or in recalcitrant psoriasis...
December 2017: Revista Chilena de Infectología: órgano Oficial de la Sociedad Chilena de Infectología
Suvarna Samudrala, Sukumar Dandakeri, Ramesh M Bhat
BACKGROUND: Although dermatology is largely considered as an outpatient specialty, dermatological conditions comprise 5-8% of cases presenting to the emergency department. The need for a dermatological intensive care unit is widely acknowledged due to the increasing incidence of acute skin failure. Very few studies have been done to characterize the common conditions seen in the emergency department and intensive care units. We undertook this study to analyze the spectrum of dermatological conditions presenting to the emergency department and the clinical profile of patients admitted to the intensive care unit...
February 26, 2018: International Journal of Dermatology
Julia J Scarisbrick, Emmilia Hodak, Martine Bagot, Rene Stranzenbach, Rudolf Stadler, Pablo L Ortiz-Romero, Evangelia Papadavid, Felicity Evison, Robert Knobler, Pietro Quaglino, Maarten H Vermeer
Our current mycosis fungoides (MF) and Sézary Syndrome (SS) staging system includes blood-classification from B0-B2 for patch/plaque/tumour or erythroderma based on manual Sézary counts but results from our EORTC survey confirm these are rarely performed in patch/plaque/tumour MF, and there is a trend towards using flow cytometry to measure blood-class. Accurately assigning blood-class effects overall stage and the 'global response' used to measure treatment responses in MF/SS and hence impacts management...
February 21, 2018: European Journal of Cancer
Shahrbanoo Nakhaei, Hamed Heidary, Aliasghar Rahimian, Mahdi Vafadar, Farzaneh Rohani, Gholam Reza Bahoosh, Davoud Amirkashani
Chanarin-Dorfman syndrome (CDS) is a rare autosomal recessive metabolic disorder caused by mutations in gene encoding the domain-5 of α/β-hydrolase enzyme (ABHD5). It is known as a natural lipid storage disorder arising from impaired lipid metabolism often characterized by hepatomegaly, myopathy, ataxia, non-bullous ichthyosiform erythroderma, hearing loss, and mental retardation. In the present study, we report two affected 28-month-old monozygotic twin boys as a new case of CDS. Genetic analysis was performed in patients, and the results showed a homozygote deletion in exon 4 of ABHD5...
February 24, 2018: Iranian Biomedical Journal
Stella X Chen, Brian R Hinds, Aaron M Goodman, Philip R Cohen
Erythroderma is characterized by erythema involving greater than 90% of the body surface area and may be caused by several etiologies, including erythrodermic psoriasis. Psoriasis is an autoimmune skin and systemic condition characterized by erythematous and scaly plaques. Monoclonal B-cell lymphocytosis is an asymptomatic hematological disorder diagnosed by elevated, small, clonal B-cell counts in the peripheral blood. The characteristics of a 71-year-old man with new onset of erythrodermic psoriasis and concurrent monoclonal B-cell lymphocytosis are presented...
December 11, 2017: Curēus
Massimiliano Bergallo, Paolo Fava, Ilaria Galliano, Mauro Novelli, Paola Montanari, Valentina Daprà, Marco Rassu, Pietro Quaglino, Maria Teresa Fierro
Mycosis fungoides (MF) is characterized by longstanding, scaly patch lesions preferentially involving the buttocks and body areas infrequently exposed to sunlight and by a slow evolution over years from patches to plaques and eventually tumors or erythroderma. Lymph node and visceral involvement, as well as large cell transformation, frequently occur in the late stages of the disease [1]. Sézary syndrome (SS) is an erythrodermic cutaneous T-cell lymphoma (CTCL) with leukemic involvement, an aggressive clinical behavior and poor prognosis [2]...
February 6, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Suju Luo, Wenjiao Zhou, Yan Li, Quanzhong Liu
No abstract text is available yet for this article.
February 1, 2018: European Journal of Dermatology: EJD
Jianhua Liang, Hui Qu, Xiaowen Wang, Aiping Wang, Lingling Liu, Ping Tu, Ruoyu Li, Mingyue Wang
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a hypersensitivity reaction characterized by maculopapular rash, exfoliative dermatitis, lymphadenopathy, fever, eosinophilia, and involvement of internal organs. Evidence for reactivation of herpes family viruses has been observed in some DRESS patients, and activated CD8+ T lymphocytes are largely directed against Epstein-Barr virus. Here, we report two cases complicated with this infection. Both patients received antibiotics and non-steroidal anti-inflammatory drugs...
February 2018: Annals of Dermatology
Evangelia Papadavid, Georgios Kokkalis, Georgios Polyderas, Konstantinos Theodoropoulos, Dimitrios Rigopoulos
Background: Apremilast is a new immunomodulatory drug, a small molecule inhibitor of PDE4, which down-regulates the expression of multiple pro-inflammatory cytokines, such as tumor necrosis factor alpha, interleukin 17, interleukin 23. Main observations: We describe a case of a 54-year-old man with erythroderma in the course of psoriasis (PASI=49), with contraindications to other psoriasis therapies, in whom total clearance of skin lesions was achieved by day 20 after therapy with apremilast at a dose of 30 mg bid (ΔPASI = 100)...
December 1, 2017: Journal of Dermatological Case Reports
Louise Schøsler, Louise Kronborg Andersen, Maiken Cavling Arendrup, Mette Sommerlund
Dermatophytosis in children caused by Trichophyton rubrum is preferably treated with topical or systemic terbinafine. We report the first case of terbinafine resistance in a child with recurrent T. rubrum dermatophytosis and congenital ichthyosiform erythroderma.
January 16, 2018: Pediatric Dermatology
Xia Yu, Jia Zhang, Yan Gu, Dan Deng, Zhouwei Wu, Lei Bao, Ming Li, Zhirong Yao
BACKGROUND: Congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD) syndrome is a rare X-linked dominant disorder characterized by peculiar cutaneous presentations and ipsilateral skeletal abnormalities. CHILD syndrome is caused by mutations in NSDHL gene, which involves in cholesterol synthesis. OBJECTIVES: To verify the diagnosis of CHILD syndrome and seek effective pathogenesis-based therapy with little side effects. METHOD: We comprehensively evaluated the patient's conditions...
January 17, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Amadeu José Rodrigues Queiróz, Mariana Carvalho Costa, Fabiana Covolo de Souza Santana, Elaine Valim Camarinha Marcos, Gladys Aires Martins, Gustavo Henrique Soares Takano, Izelda Maria Carvalho Costa
Blaschko's lines are trace of the migration of primordial cells to the ectoderm during embryogenesis. We report an intriguing, complex and very rare case of a patient with congenital rash on Blaschko's lines. A 30-year-old female patient presented at birth with erythroderma and generalized desquamation that progressively acquired a linear distribution, under Blaschko's lines, associated with pruritus and fissures in the hands. This article is protected by copyright. All rights reserved.
January 17, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Lisa R Edwards, Barbara B Wilson, Alejandro A Gru
No abstract text is available yet for this article.
January 2018: American Journal of Dermatopathology
Xiujuan Gui, Xinhai Zhu, Liangjun Guo, Guoqiang Tan, Yan Liu, Yi Tan, Qiufang Chen, Yuwei Song, Shaoqiang Lin
RATIONALE: Thymomas are associated with numerous autoimmune disorders, such as myasthenia gravis (MG), pure red cell aplasia (PRCA), and systemic lupus erythematosus (SLE). However, graft-versus-host disease (GVHD)-like erythroderma is a relatively uncommon paraneoplastic disorder associated with thymomas and signifies a poor prognosis. PATIENT CONCERNS: A 35-year-old woman with medical history significant for stage IVa type AB thymoma presented with patchy erythema over face, trunk, and extremities that failed to respond to topical steroids...
December 2017: Medicine (Baltimore)
Gian Paolo Bombeccari, Umberto Garagiola, Francesco Pallotti, Margherita Rossi, Massimo Porrini, Aldo Bruno Giannì, Francesco Spadari
Background: Imatinib mesylate is an inhibitor of the tyrosine kinase Bcr-Abl and a first-line treatment for Philadelphia chromosome-positive chronic myeloid leukaemia (CML). Dermatological side effects include superficial oedema, pustular eruption, lichenoid reactions, erythroderma, and skin rash. Depigmentation of the skin and/or mucosa is uncommon, and hyperpigmentation is rare. Case presentation: We present the case of a 63-year-old Caucasian male with widespread hyperpigmentation of the hard palate associated with a 9-year history of imatinib therapy to treat CML...
December 2017: Maxillofacial Plastic and Reconstructive Surgery
A B Weins, K Scharffetter-Kochanek, T Weiss, K Brockow, T Biedermann, C Psotta-Schachtner, M Mockenhaupt, J M Weiss
No abstract text is available yet for this article.
December 10, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
Dhrubajyoti Sharma, Ankur K Jindal, Amit Rawat, Surjit Singh
Primary immunodeficiency disorders (PIDs) are a group of disorders affecting the capability to fight against infection. These include defects in T cells and B cells affecting cell-mediated and humoral immunity, respectively, combined humoral and cell-mediated immunodeficiency, defects in phagocytosis, complement defects, and defects in cytokine or cytokine signalling pathways which are detrimental for immune function. Depending upon the type and severity, age at onset of symptoms can vary from neonatal period to late childhood...
November 2017: Indian Dermatology Online Journal
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