Franziska Hübner, Ewan A Langan, Andreas Recke
Lichen planus pemphigoides (LPP) is a very rare autoimmune sub-epidermal blistering disease associated with lichenoid skin changes. Initially thought to be a mere variant of more common inflammatory dermatoses, particularly Bullous Pemphigoid (BP) or Lichen Planus (LP), a growing body of evidence suggests that it is a disease entity in its own right. In common with a range of autoimmune blistering diseases, including BP, pemphigoid gestationis (PG), mucous membrane pemphigoid (MMP) and linear IgA dermatosis (LAD), a key feature of the disease is the development of autoantibodies against type XVII collagen (COL17)...
2019: Frontiers in Immunology
Mirjana Ziemer, Manfred Kunz, Monica Schüürmann, Daniel Wagenknecht, Konstantin Dumann, Robin Reschke
We present four clinicopathological correlated cases of young patients with cryothermic dermatitis artefacta. They were initially misdiagnosed as primary bullous dermatoses or fixed drug eruptions. Cryothermic dermatitis artefacta can imitate authentic dermatoses such as linear IgA bullous dermatosis, herpes virus infection, bullous pemphigoid or fixed drug eruption. It should be considered as differential diagnosis in uncommon cases of recurrent bullae in adolescent and young adult patients. We summarize helpful clinical and histopathological criteria for correct diagnosis and therewith causative treatment...
June 7, 2019: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
Everton Carlos Siviero do Vale, Oscar Cardoso Dimatos, Adriana Maria Porro, Claudia Giuli Santi
Dermatitis herpetiformis and linear IgA bullous dermatosis are autoimmune diseases that present with pruritic urticarial papules and plaques, with formation of vesicles and blisters of subepidermal location, mediated by IgA antibodies. Mucosal lesions are present only in linear IgA bullous dermatosis. The elaboration of this consensus consisted of a brief presentation of the different aspects of these dermatoses and, above all, of an updated literature review on the various therapeutic options that were discussed and compared with the authors' experience, aiming at the treatment orientation of these diseases in Brazil...
April 2019: Anais Brasileiros de Dermatologia
Giovanni Genovese, Luigia Venegoni, Daniele Fanoni, Simona Muratori, Emilio Berti, Angelo Valerio Marzano
BACKGROUND: Linear IgA bullous dermatosis (LABD) is a rare autoimmune subepithelial vesiculobullous disease due to IgA autoantibodies directed against different antigens of the basement membrane zone (BMZ) of the skin and/or mucosae. It affects mainly preschool-aged children and adults, with only few studies on large series. The aim of this study was to assess possible differences between adults and children regarding clinical presentation, immunopathologic features, management and course of the disease...
May 24, 2019: Orphanet Journal of Rare Diseases
Lorena Visentainer, Juliana Yumi Massuda, Maria Letícia Cintra, Renata Ferreira Magalhães
We report an uncommon presentation of bullous dermatosis by linear IgA. There are few cases reported in the literature with this form of presentation starting with mucosal lesions and then evolving into a similar bullous pemphigoid pattern. In addition, we emphasize the importance of direct immunofluorescence for the definitive diagnosis.
May 2019: Clinical Case Reports
M S Díaz, L Morita, B Ferrari, S Sartori, M F Greco, L Sobrevias Bonells, M A González-Enseñat, M A Vicente Villa, M Larralde
Linear IgA bullous dermatosis is an acquired subepidermal immunoglobulin-mediated vesiculobullous disease. In this retrospective, observational, descriptive study, we describe the clinical characteristics, treatments, and outcomes of 17 patients with linear IgA bullous dermatosis. Two children had been vaccinated 2 weeks before the onset of symptoms, 2 had had bronco-obstructive respiratory symptoms, and 1 had received intravenous antibiotic therapy. We also observed an association with autoimmune hepatitis in one patient and alopecia areata in another...
April 20, 2019: Actas Dermo-sifiliográficas
Beatriz Valle Del Barrio, Danila Luraschi, Robert Micheletti, Laurent Hiffler, Ana Paula Arias
An 8-month-old child was admitted to a paediatric intensive care unit in Guinea-Bissau with severe blistering dermatosis. He was treated with broad spectrum antibiotics and dressings, without improvement. After 2 weeks, linear IgA bullous dermatosis was suspected. Owing to lack of dapsone, the child was treated with prednisolone and improved. To avoid corticosteroids side effects, 2 months after starting prednisolone we switched to colchicine, but the boy's condition worsened for reasons of poor adherence, requiring intravenous corticosteroids and antibiotics...
April 2019: Oxford Medical Case Reports
Takanao Matsumoto, Satoshi Nakamura, Norito Ishii, Naoka Umemoto, Masaaki Kawase, Toshio Demitsu, Takashi Hashimoto
No abstract text is available yet for this article.
April 10, 2019: European Journal of Dermatology: EJD
Lorena M C Costa, Mark A Cappel, James H Keeling
Pemphigus herpetiformis (PH), a rare type of pemphigus, is characterized by immunologic findings consistent with pemphigus but with a unique clinical and pathologic presentation. PH was first described as resembling dermatitis herpetiformis clinically, but because of its variable presentation, it can also resemble linear immunoglobulin A bullous dermatosis and bullous pemphigoid. We reviewed reported cases to analyze the most frequent clinical, pathologic, and immunologic characteristics and to propose corresponding diagnostic criteria...
September 2019: International Journal of Dermatology
H A Juratli, M Sárdy
Linear IgA bullous dermatosis is a rare autoimmune blistering disease that occurs in both children and adults. Strings of pearls, crowns of jewels, rosettes and urticarial plaques can occur on the whole integument with emphasis on the face (particularly perioral area) and genitalia. Pruritus is common and may be severe. The presence of IgA deposits along the basement membrane can usually be identified using direct immunofluorescence (DIF) microscopy. The histological and clinical features of this disorder may mimic those of dermatitis herpetiformis...
March 15, 2019: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
Nathan Nartker, Natalie Kudlak, David Crowe
No abstract text is available yet for this article.
March 2019: JAAD Case Reports
Judith Lammer, Rüdiger Hein, Sophie Roenneberg, Tilo Biedermann, Thomas Volz
Linear IgA bullous dermatosis (LABD) is a rare subepidermal autoimmune blistering disease characterized by linear deposition of IgA along the basement membrane zone. Although most reported cases are idiopathic, there is a subset of patients with drug-induced LABD. Various drugs have been associated with the drug-induced form of the disease. This paper reviews the literature on drugs reported to elicit linear IgA dermatosis and its specific clinical presentation. In addition, a case report of a 77-year-old male patient with linear IgA dermatosis induced by vancomycin is described...
February 27, 2019: Acta Dermato-venereologica
A Welfringer-Morin, L Bekel, N Bellon, A Gantzer, O Boccara, S Hadj-Rabia, S Leclerc-Mercier, A Frassati-Biaggi, S Fraitag, C Bodemer
BACKGROUND: Autoimmune bullous dermatoses (AIBDs) in children are uncommon, and their long-term evolution remains unknown. OBJECTIVE: The aim of this retrospective study was to characterize the long-term prognosis of AIBDs that started during childhood. METHODS: We conducted a monocentric retrospective study, in the French dermatology centre, by including all children affected by AIBDs. The long-term outcome was obtained through a phone call questionnaire...
June 2019: Journal of the European Academy of Dermatology and Venereology: JEADV
Camille Pinard, Vivien Hebert, Myriam Lecuyer, Laurine Sacre, Pascal Joly
No abstract text is available yet for this article.
February 2019: JAAD Case Reports
R Malipatel, V Gnanapriya, A Manocha, Y K Inchara
We report a rare case of systemic lupus erythematosus presenting initially with cutaneous manifestations of linear IgA bullous dermatosis. Later the patient developed renal abnormalities due to thrombotic microangiopathy and lupus nephritis with inflammatory necrotizing vasculitis. Paucity of immune deposits was observed on Immunofluorescence. This association of SLE with these cutaneous and renal lesions is rarely reported in the literature.
November 2018: Indian Journal of Nephrology
Bethsabée Garel, Saskia Ingen-Housz-Oro, Daniele Afriat, Catherine Prost-Squarcioni, Florence Tétart, Benoit Bensaid, Corina Bara Passot, Marie Beylot-Barry, Vincent Descamps, Sophie Duvert-Lehembre, Sabine Grootenboer-Mignot, Géraldine Jeudy, Angèle Soria, Marie Blanche Valnet-Rabier, Annick Barbaud, Frédéric Caux, Bénédicte Lebrun-Vignes
AIMS: Linear immunoglobin A (IgA) bullous dermatosis is a rare autoimmune dermatosis considered spontaneous or drug-induced (DILAD). We assessed all DILAD cases, determined the imputability score of drugs and highlighted suspected drugs. METHODS: Data for patients with DILAD were collected retrospectively from the French Pharmacovigilance network (from 1985 to 2017) and from physicians involved in the Bullous Diseases French Study Group and the French Investigators for Skin Adverse Reactions to Drugs...
March 2019: British Journal of Clinical Pharmacology
Victoria S Humphrey, Jonathan J Lee, Teerawit Supakorndej, Shahid M Malik, Arthur C Huen, Jedrych Jaroslaw
Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder seen in the pediatric and adult populations that is often linked to a medication, infection, or underlying gastrointestinal, hepatobiliary, or autoimmune disease. In this study, we describe the case of a 23-year-old white man whose presentation and diagnosis of LABD ultimately led to the discovery of underlying primary sclerosing cholangitis (PSC) and ulcerative colitis (UC). His dermatitis resolved with topical steroids and dapsone, and he is undergoing systemic treatment for his UC and PSC...
November 15, 2018: American Journal of Dermatopathology
Wolfgang Konschake, Georg Daeschlein, Michael Jünger, Stine Lutze
The rare case of a 61-year-old patient suffering from linear IgA dermatosis is presented. The patient was previously hospitalized with chronic inflammatory bowel disease. The correct diagnosis of the disease was based on clinical and histological findings. Serological methods, such as indirect immunofluorescence, ELISA and immunoblotting are suitable for identification of the autoantibodies. In this case the detection of IgA antibodies along the basal membrane was achieved by direct immunofluorescence. Other bullous dermatoses with similar symptoms, such as an IgG-mediated bullous pemphigoid have to be excluded...
November 20, 2018: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
Tatyana Yetto, Carrick Burns
Linear IgA bullous dermatosis is a rare bullous disease in children and adults that can be associated with autoimmune conditions, malignancies, infections, or medication exposure. The definitive diagnosis relies on the biopsy. A 58-year-old man presented to our clinic with a pruritic vesicular and bullous eruption. Histology showed the classic findings of a subepidermal blister with neutrophilic infiltrate and linear IgA deposition along the dermal-epidermal junction. Upon further evaluation, he was diagnosed with ulcerative proctitis...
July 15, 2018: Dermatology Online Journal
Anthony Rainey, Jena Auerbach, Kairav Shah
Solid organ transplant patients are well established to be at risk of herpes simplex virus and varicella zoster virus infection and reactivation. We present a case of a 41-year-old woman with a history of pancreas and renal transplant who presented with what appeared to be disseminated herpes simplex virus or varicella zoster virus induced rash, but who was ultimately diagnosed and treated as linear IgA bullous dermatosis. This case alerts physicians to other non-infectious dermatoses as a cause of vesiculobullous rash in solid organ transplant patients...
December 2018: Transplant Infectious Disease: An Official Journal of the Transplantation Society
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