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Sci Rep 2022 Jun 22;12(1):10519. Epub 2022 Jun 22.

Department of Pathology, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Korea.

The gold standard for diagnosing bullous pemphigoid (BP) is the detection of linear deposition of IgG and/or C3 at the dermoepidermal junction using direct immunofluorescence (DIF). Because DIF has several disadvantages, primarily the requirement for frozen specimens, we assessed the diagnostic value of immunohistochemical (IHC) staining for BP detection. Eighty-eight patients with bullous lesions were included in this study. Read More

Emerg Med J 2022 Jul;39(7):520-539

Pediatrics, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.

MMW Fortschr Med 2022 Jun;164(12):25

Springer Medizin Verlag GmbH, Aschauer Str. 30, 81549, München, Deutschland.

Actas Dermosifiliogr 2022 Jun 6;113(6):636-639. Epub 2021 Oct 6.

Servicio de Dermatología, Hospital Clínico Universitario Lozano Blesa, Zaragoza, España.

Front Immunol 2022 1;13:903174. Epub 2022 Jun 1.

Dermatology Hospital of Jiangxi Province, Jiangxi Provincial Clinical Research Center for Skin Diseases, Candidate Branch of National Clinical Research Center for Skin Diseases, Dermatology Institute of Jiangxi Province, The Affiliated Dermatology Hospital of Nanchang University, Nanchang, China.

Mucous membrane pemphigoid (MMP) and anti-laminin (LM) γ1 pemphigoid, two subtypes of subepidermal autoimmune bullous diseases characterized by autoantibodies against epidermal basement membrane zone proteins, mainly show mucosal and skin lesions, respectively. The known autoantigens of MMP includes BP180, BP230, LM332, integrin α6β4 and type VII collagen, and anti-LMγ1 pemphigoid targets LMγ1. In this study, we present an unique MMP case with oral mucosal lesions, which showed positive IgA signals on basement membrane zone in indirect immunofluorescence using normal human skin and on dermal side in indirect immunofluorescence using salt-split skin, positive IgA autoantibodies against LMγ1 by immunoblotting of epidermal extracts, positive IgA autoantibodies against LMα5 by immunoblotting of LM521 recombinant protein (rLM521) and positive IgG autoantibodies against LMγ1 by immunoblotting of rLM111 and rLM521 at first visit (Day 0). Read More

Front Immunol 2022 20;13:884067. Epub 2022 May 20.

Chair of Vegetative Anatomy, Institute of Anatomy, Faculty of Medicine, Ludwig-Maximilians-Universität Munich, Munich, Germany.

Pemphigus vulgaris (PV) is an autoimmune bullous skin disease caused primarily by autoantibodies (PV-IgG) against the desmosomal adhesion proteins desmoglein (Dsg)1 and Dsg3. PV patient lesions are characterized by flaccid blisters and ultrastructurally by defined hallmarks including a reduction in desmosome number and size, formation of split desmosomes, as well as uncoupling of keratin filaments from desmosomes. The pathophysiology underlying the disease is known to involve several intracellular signaling pathways downstream of PV-IgG binding. Read More

BMC Med Genomics 2022 Jun 18;15(1):136. Epub 2022 Jun 18.

Center for Reproductive Medicine, Changhai Hospital, Naval Medical University, Shanghai, China.

Background: Uniparental disomy (UPD) is a condition in which both chromosomes are inherited from the same parent, except for imprinting disorders. Uniparental isodisomy (UPiD) may result in a homozygous variant contributing to an autosomal recessive disorder in the offspring of a heterozygous carrier. Junctional epidermolysis bullosa intermediate (JEB intermediate) is an autosomal recessive inherited disease that is associated with a series of gene variants, including those of COL17A1. Read More

Front Immunol 2022 25;13:884241. Epub 2022 May 25.

Chair of Vegetative Anatomy, Instiute of Anatomy, Faculty of Medicine, Ludwig-Maximilian-Universität München (LMU) Munich, München, Germany.

Desmosomes are important epidermal adhesion units and signalling hubs, which play an important role in pemphigus pathogenesis. Different expression patterns of the pemphigus autoantigens desmoglein (Dsg)1 and Dsg3 across different epidermal layers have been demonstrated. However, little is known about changes in desmosome composition in different epidermal layers or in patient skin. Read More

J Dtsch Dermatol Ges 2022 06;20(6):798-806

Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Würzburg.

J Dtsch Dermatol Ges 2022 06;20(6):777-786

German Center for the Documentation of Severe Skin Reactions (dZh), Department of Dermatologie, Medical Center and Medical Faculty - University of Freiburg, Germany.

Background: Stevens-Johnson syndrome and toxic epidermal necrolysis are severe, primarily drug-induced reactions of skin and mucosa. Since they differ in the extent of skin detachment but not in etiology, they are grouped together as epidermal necrolysis (EN). Due to nationwide registration, representative data are available at the German Center for the Documentation of Severe Skin Reactions (dZh). Read More

Rev Paul Pediatr 2022 10;40:e2021134. Epub 2022 Jun 10.

Universidade Federal do Paraná, Curitiba, PR, Brazil.

Objective: The aim of this study was to evaluate the coronavirus disease 2019 (COVID-19) cutaneous manifestations described in pediatric patients and discuss their relevance for early diagnosis.

Data Source: The study consisted of a systematic review of original articles indexed in PubMed and Embase databases, as well as gray literature articles found through Google Scholar. A search strategy, based on PICO (Patient, Intervention, Comparison, Outcome) Tool, with the terms "child," "infant," "childhood," "adolescents," "teenagers," "COVID-19," "SARS-CoV-2," and "skin manifestations," was performed to optimize the findings. Read More

An Bras Dermatol 2022 Jun 11. Epub 2022 Jun 11.

Department of Dermatology, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.

Epidermolysis bullosa acquisita is a rare autoimmune disease, characterized by the synthesis of anti-collagen VII autoantibodies, the main component of hemidesmosome anchoring fibrils. The antigen-antibody binding elicits a complex inflammatory response, which culminates in the loss of dermo-epidermal adhesion of the skin and/or mucous membranes. Skin fragility with bullae, erosions, and milia in areas of trauma characterizes the mechanobullous form of the disease. Read More

Actas Dermosifiliogr 2022 05 19;113(5):510-511. Epub 2021 Nov 19.

Servicio de Dermatología, Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas de Gran Canaria, Las Palmas, España.

J Eur Acad Dermatol Venereol 2022 Jun 13. Epub 2022 Jun 13.

Dermatology Unit and Skin Bank, Department of Medical, Surgical and Neurosciences, Siena University Hospital, Siena, Italy.

Background: The spectrum of pustular skin disorders (PSD) is large and particularly challenging, including inflammatory, infectious and amicrobial diseases. Moreover, although pustules represent the unifying clinical feature, they can be absent or not fully developed in the early stage of the disease. The line-field confocal optical coherence tomography (LC-OCT) is a recently developed imaging technique able to perform a non-invasive, in vivo, examination of the epidermis and upper dermis, reaching very high image resolution and virtual histology. Read More

Front Immunol 2022 27;13:907424. Epub 2022 May 27.

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC, United States.

In the endemic variants of pemphigus foliaceus (PF), in Brazil and Tunisia, patients generate pathogenic IgG4 anti-desmoglein 1 autoantibodies. Additionally, these patients possess antibodies against salivary proteins from sand flies that react with Dsg1, which may lead to skin disease in susceptible individuals living in endemic areas. This minireview focuses on recent studies highlighting the possible role of salivary proteins from () in EPF from Brazil and () in EPF from Tunisia. Read More

Arerugi 2022 ;71(4):328-333

Department of Pediatrics, Kasugai Municipal Hospital.

We describe here the case of a 7-year-old male patient with Stevens-Johnson syndrome (SJS), which was suspected to be caused by treatment with tipepidine hibenzate (Asverin). The day after taking tipepidine hibenzate and L-carbocysteine (Carbocysteine DS) for relief of a cold, he began presenting with the following symptoms: fever above 38°C, wheezing, and decreased oxygen saturation. Two days later, mucous membrane rashes, such as erosions on the lips, eye mucosa, vulva, and blisters on the trunk appeared, and SJS was thus diagnosed. Read More

Biomed Res Int 2022 31;2022:6493156. Epub 2022 May 31.

Department of General Surgery and Surgical-Medical Specialties, School of Dentistry, University of Catania, AOU "Policlinico-San Marco", Via S. Sofia 78, 95124 Catania, Italy.

Epidermolysis bullosa (EB) is a group of skin disorders with skin fragility characterized by blistering from minimal mechanical trauma with rupture at the dermoepidermal junction. There are four major classical heritable EB types, due to mutations in as many as 20 distinct genes: EB simplex (EBS), junctional EB (JEB), dystrophic EB (DEB), and Kindler EB (KEB). This study is aimed at reporting case series on patients ( = 8; males, = 5 and females, = 3, age range 12-68 years) affected by EB and performs a review of the literature on this topic. Read More

Paediatr Drugs 2022 Jul 9;24(4):307-319. Epub 2022 Jun 9.

Division of Dermatology, Department of Medicine, Sunnybrook Health Sciences Centre, Toronto, ON, Canada.

Pediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threating blistering diseases triggered by medications that affect the skin and mucosae. Drug-induced epidermal necrolysis is a better term for medication-triggered cases because there is a spectrum of disease severity that otherwise is divided into the separate entities of SJS, overlap SJS/TEN, and TEN. This manuscript reviews the management of drug-induced epidermal necrolysis (DEN), including diagnosis, investigations to exclude differential diagnoses, and treatment. Read More

Rev Soc Bras Med Trop 2022 6;55:e0601. Epub 2022 Jun 6.

Universidade Estadual Paulista, Instituto de Biociências, Botucatu, SP, Brasil.

Centipedes are venomous arthropods that have an elongated body, divided into many segments, presenting a pair of legs in each segment, adapted pairs of paws that simulate fangs and inject venom causing intense pain, with local erythema and edema, and rarely, blisters and skin necrosis. We present the case of a young woman pricked on her upper lip with intense swelling and local pain and discuss the real danger of envenomation and the therapeutic measures that should be taken. Read More

J Dtsch Dermatol Ges 2022 06 8;20(6):753-771. Epub 2022 Jun 8.

Department of Dermatology and Allergy, University Hospital Bonn, Bonn, Germany.

Generalized pustular psoriasis (GPP) is a rare, severe, potentially life-threatening, autoinflammatory, neutrophilic skin disease that may be accompanied by fever and leukocytosis. This paper describes the current state of knowledge on GPP in terms of classification, (differential) diagnosis and prevalence. We present a comparison of the genetics and pathoimmunology of GPP and psoriasis vulgaris with the central mechanisms of autoimmunology and autoinflammation. Read More

Dermatol Online J 2022 03 15;28(2). Epub 2022 Mar 15.

Department of Dermatology, Hospital Universitari Son Espases, Palma, Spain.

Dermatol Online J 2022 Mar 15;28(2). Epub 2022 Mar 15.

Section of Dermatology and Venereology, Department of Medicine, University of Verona, Verona, Italy.

A 76-year-old man came to our attention for the presence of itchy skin lesions localized on the trunk. The patient had a nodular melanoma removed two years earlier. Because of metastatic pulmonary melanoma, he underwent a lung lobectomy and began adjuvant therapy with nivolumab. Read More

Front Immunol 2022 18;13:863095. Epub 2022 May 18.

INSERM U1234, Normandie University, Rouen, France.

Background: Immunoglobulin G (IgG) levels are maintained by the IgG-recycling neonatal Fc-receptor (FcRn). Pemphigus vulgaris and pemphigus foliaceus are debilitating autoimmune disorders triggered by IgG autoantibodies against mucosal and epidermal desmogleins. Recently, a phase 2 clinical trial (NCT03334058; https://clinicaltrials. Read More

J UOEH 2022 ;44(2):215-219

Department of Dermatology, University of Occupational and Environmental Health, Japan.

A 73-year-old male noticed a localized nose erosion that we thought was possibly an exacerbation of skin erosion due to the direct influence of friction from wearing a mask. Blood examination revealed a remarkable increase in serum anti-desmoglein-1 and anti-desmoglein-3 antibodies. A skin biopsy showed acantholysis in the epidermal granular layer. Read More

Cutis 2022 Apr;109(4):221-223

Dr. Kou is from Des Moines University College of Osteopathic Medicine, Iowa. Dr. Kolb is from Olmsted Medical Center, Rochester, Minnesota. Dr. Kelada is from Roseville Family Healthcare, California. Dr. Schmieder is from the Department of Dermatology, Orange Park Medical Center, Florida.

Blisters and subsequent desquamation of the skin in the presence of acute edema is a well-known clinical phenomenon. In this case report, we describe a new variant that we have termed anasarca-induced desquamation in a 50-year-old man with molting of the entire cutaneous surface after acute edema, in a setting of 40-lb weight gain over 5 days. Laboratory workup for infectious causes and punch biopsies of skin lesions ruled out Stevens-Johnson syndrome and staphylococcal scalded skin syndrome, which have a similar clinical presentation to anasarca-induced desquamation. Read More

Cutis 2022 04;109(4):E11-E13

School of Medicine, Duke University, Durham, North Carolina. Drs. Shah and Bellet are from the Department of Pediatrics. Dr. Bellet also is from the Department of Dermatology.

Cutis 2022 02;109(2):E22-E24

Drs. Ibraheim and Martin are from the University of Texas Health Science Center at Houston. Dr. Ibraheim is from the McGovern Medical School; Dr. Martin is from the Department of Dermatology. Dr. Nguyen is from Village Dermatology, Houston.

Cutis 2022 02;109(2):E9-E12

Department of Dermatology and Cutaneous Surgery, University of South Florida College of Medicine, Tampa.

J Dtsch Dermatol Ges 2022 06 3;20(6):798-805. Epub 2022 Jun 3.

Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Würzburg, Germany.

Background And Objectives: Bullous pemphigoid (BP) is associated with neuropsychiatric disorders. Other comorbid diseases are discussed controversially. We evaluated the prevalence of comorbidity in BP patients in a representative area of Germany. Read More

Sci Rep 2022 Jun 2;12(1):9229. Epub 2022 Jun 2.

Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria.

Autoimmune diseases caused by pathogenic IgG4 subclass autoantibodies (IgG4-AID) include diseases like MuSK myasthenia gravis, pemphigus vulgaris or thrombotic thrombocytopenic purpura. Their etiology is still unknown. Polymorphisms in the human leukocyte antigen (HLA) gene locus, particularly in HLA-DRB1, are known genetic susceptibility factors for autoimmune diseases. Read More