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Blistering disease

Antigona Begolli Gerqari, Mybera Ferizi, Merita Kotori, Aferdita Daka, Syzana Hapciu, Ilir Begolli, Mirije Begolli, Idriz Gerqari
Tattooing is a procedure where ink is applied to an area of the skin, mostly intraepidermally (1). This procedure is carried out mainly for aesthetic purposes. Lately, it has been used as a corrective medical procedure following amputation of mammilla. The procedure is aggressive (2), and the fact that skin is punctured many times with the same needle which cannot be fully sterilized may cause infection of the treated area with bacterial, fungal, or viral agents that may lead to health consequences manifesting in the form of verrucae vulgaris, molluscum contagiosum, and herpes simplex...
April 2018: Acta Dermatovenerologica Croatica: ADC
Sergio Santos-Alarcón, Cesar Benavente-Villegas, Isabel García-Briz, Magdalena Moneva-Léniz, Celia Sanchis-Sánchez, Almudena Mateu-Puchades
Dear Editor, Gestational pemphigoid (GP) is a rare autoimmune bullous dermatosis in pregnancy. GP usually occurs during the second or third month of pregnancy. It clinically manifests as the development of either early-onset urticarial lesions or late-onset subepidermal blisters that may linger for weeks or even months. Herein we report the case of a 45-year-old woman with the distinctive clinical onset of GP. A forty-five-year-old woman, gravida I, para 0, at 27 weeks gestation, was referred for evaluation to our Department with an extensive pruritic eruption that had developed over the previous 7 days...
April 2018: Acta Dermatovenerologica Croatica: ADC
Thomas Sajda, Animesh A Sinha
Pemphigus vulgaris (PV) is an autoimmune skin blistering disease effecting both cutaneous and mucosal epithelia. Blister formation in PV is known to result from the binding of autoantibodies (autoAbs) to keratinocyte antigens. The primary antigenic targets of pathogenic autoAbs are known to be desmoglein 3, and to a lesser extent, desmoglein 1, cadherin family proteins that partially comprise the desmosome, a protein structure responsible for maintaining cell adhesion, although additional autoAbs, whose role in blister formation is still unclear, are also known to be present in PV patients...
2018: Frontiers in Immunology
Iakov Shimanovich, Julia Marie Nitz, Mareike Witte, Detlef Zillikens, Christian Rose
BACKGROUND: Mucous membrane pemphigoid (MMP) is an autoimmune subepithelial blistering disease with predominant involvement of mucosal surfaces. It is usually diagnosed by direct immunofluorescence microscopy of frozen biopsies, demonstrating linear deposits of complement, IgG or IgA along the basement membrane. The aim of the present study was to investigate the utility of immunohistochemistry on formalin-fixed, paraffin-embedded tissue biopsies for the diagnosis of MMP and to compare its sensitivity to that of direct immunofluorescence microscopy...
May 12, 2018: Journal of Oral Pathology & Medicine
C H Tan, L Y Kuan, Y K Heng, J Y Pan, S I Tee, Y L Lim, L S Tan
No abstract text is available yet for this article.
May 10, 2018: Clinical and Experimental Dermatology
Yanling Liao, Larisa Ivanova, Rajarajeswari Sivalenka, Trevor Plumer, Hongwen Zhu, Xiaokui Zhang, Angela M Christiano, John A McGrath, Jodi P Gurney, Mitchell S Cairo
Recessive dystrophic epidermolysis bullosa (RDEB) is a devastating inherited skin blistering disease caused by mutations in the COL7A1 gene that encodes type VII collagen (C7), a major structural component of anchoring fibrils at the dermal-epidermal junction (DEJ). We recently demonstrated that human cord blood-derived unrestricted somatic stem cells promote wound healing and ameliorate the blistering phenotype in a RDEB (col7a1-/- ) mouse model. Here, we demonstrate significant therapeutic effect of a further novel stem cell product in RDEB, that is, human placental-derived stem cells (HPDSCs), currently being used as human leukocyte antigen-independent donor cells with allogeneic umbilical cord blood stem cell transplantation in patients with malignant and nonmalignant diseases...
May 10, 2018: Stem Cells Translational Medicine
Aparna K Sanath, A Santha Devy, Sanath Aithal, G Sathish Kumar, B Ganesh Prasad, P S Pradeep
Background: Pemphigus is a group of autoimmune blistering diseases characterized by loss of keratinocyte cell adhesion that leads to blister formation clinically. Induction of apoptosis or of proapoptotic proteins by pemphigus immunoglobulin G (IgG) may be part of the mechanism by which IgG induces acantholysis. Some of the current data suggest that activation of proapoptotic proteins such as bax and caspase cysteine proteinases may sensitize cells to the acantholytic effects of pemphigus IgG...
January 2018: Journal of Oral and Maxillofacial Pathology: JOMFP
E Q Wang, M Radjenovic, M A Castrillón, G Feng, D F Murrell
BACKGROUND: Autoimmune blistering diseases (AIBD) is known to negatively impact upon quality of life (QoL), however there is a paucity of research on the effect of AIBD on work productivity. AIBD can be quite disfiguring in terms of a patient's appearance due to its blistering nature. OBJECTIVE: To determine the impact of AIBD on work productivity and to determine whether patients are stigmatized at work due to their appearance. METHODS: Sixty one patients with AIBD completed the Work Productivity and Activity Impairment Questionnaire-Specific Health Problem (WPAIQ-SHP), the Dermatology Life Quality Index (DLQI), the Autoimmune Bullous Disease Quality of Life (ABQOL), and the Treatment of Autoimmune Bullous Disease Quality of Life questionnaires (TABQOL)...
May 6, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Ayman Abdelmaksoud, Michelangelo Vestita
We read with interest a study by Miyamoto et al. published in the Journal of European Academy of Dermatology and Venereology. In their study, Miyamoto et al. found higher expression of vascular endothelial growth factor (VEGF) and endoglin in the tissue of erythrodermic pemphigus foliaceous (PF) compared to non-erythrodermic PF. According to the authors, both VEGF and endoglin may pose a prognostic predicting factors for adequate tissue repair in pemphigus patients; PF& pemphigus vulgaris (PV). The authors stated "therapy such as topical propranolol may enable adequate healing and disease remission"...
May 6, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
M Mockenhaupt
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acutely occurring, unpredictable, often life-threatening reactions that are a huge challenge in clinical practice. They are characterized by extensive blistering of skin and mucosa and are considered as one disease entity of different severity. Thus, they are summarized as SJS/TEN or EN (for epidermal or epithelial necrolysis). The diagnosis can be confirmed through synopsis of clinical pattern and histopathological findings. To identify the inducing factors, it is crucial to obtain a detailed and thorough medication and infection history...
May 2, 2018: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
Dario Didona, Giovanni Di Zenzo
Autoimmune blistering diseases are characterized by autoantibodies against structural adhesion proteins of the skin and mucous membranes. Extensive characterization of their autoantibody targets has improved understanding of pathogenesis and laid the basis for the study of antigens/epitopes diversification, a process termed epitope spreading (ES). In this review, we have reported and discussed ES phenomena in autoimmune bullous diseases and underlined their functional role in disease pathogenesis. A functional ES has been proposed: (1) in bullous pemphigoid patients and correlates with the initial phase of the disease, (2) in pemphigus vulgaris patients with mucosal involvement during the clinical transition to a mucocutaneous form, (3) in endemic pemphigus foliaceus, underlining its role in disease pathogenesis, and (4) in numerous cases of disease transition associated with an intermolecular diversification of immune response...
2018: Frontiers in Immunology
Fang Wang, Yanting Ye, Ze-Yu Luo, Qian Gao, Di-Qing Luo, Xingqi Zhang
Background: The pathogenesis of Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) is not fully understood. Our previous study reported that chemokine CCL27 was overexpressed in serum of SJS/TEN patients. The objective of this study was to investigate the levels of CCL27 and TNF-α in serum and blister fluid from patients with SJS/TEN during the acute stage or resolution phase. Methods: A total of 27 patients with SJS/TEN and 39 healthy donors were recruited to the study...
2018: Clinical and Translational Allergy
Yosuke Mai, Wataru Nishie, Kazumasa Sato, Moeko Hotta, Kentaro Izumi, Kei Ito, Kazuyoshi Hosokawa, Hiroshi Shimizu
Bullous pemphigoid (BP) is a common autoimmune blistering disease in which autoantibodies mainly target the hemidesmosomal component BP180 (also known as type XVII collagen) in basal keratinocytes. Various triggering factors are known to induce BP onset, including radiotherapy, burns, ultraviolet exposure, surgery, and the use of dipeptidyl peptidase-IV inhibitors (DPP4i), which are widely used antihyperglycemic drugs. Here, we present a case of BP triggered by a thermal burn under medication with DPP4i. A 60-year-old man with type II diabetes had been treated with the DPP4i linagliptin for 1 year...
2018: Frontiers in Immunology
K T Amber, S A Grando
Managing corticosteroid related comorbidities, including bone mineral density loss (BMDL), is an essential part of caring for the autoimmune blistering disease (AIBD) patient. The American College of Rheumatology (ACR) issued an evidence-based BMDL prophylaxis guideline for patients receiving systemic corticosteroids. 2010 guidelines divided patients as low, medium, and high-risk. Accordingly, for patients greater than 50 and on corticosteroids for ≥3 months, low-risk patients should receive bisphosphonate prophylaxis if receiving ≥7...
April 28, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Stefanie Haeberle, Xiaoying Wei, Katja Bieber, Stephanie Goletz, Ralf J Ludwig, Enno Schmidt, Alexander H Enk, Eva N Hadaschik
BACKGROUND: Autoimmune bullous dermatoses (AIBD) are severe autoantibody-mediated skin diseases. The pathogenic relevance of autoreactive CD4+ T cells for the induction of autoantibody production remains to be fully evaluated. Scurfy mice lack functional regulatory T cells (Treg), develop spontaneous activation of autoreactive CD4+ T cells and display severe erosive skin lesions suggestive of AIBD. OBJECTIVE: To determine whether AIBD develop in Treg-deficient scurfy mice...
April 25, 2018: Journal of Allergy and Clinical Immunology
J D Ho, Al-Haseni, S Smith, J Bhawan, D Sahni
Complex regional pain syndrome (CRPS, formerly reflex sympathetic dystrophy) is a poorly understood syndrome occurring most commonly after peripheral trauma.(1) Diagnostic features include pain, autonomic dysregulation, sensory/motor abnormalities and trophic changes involving the affected limb.(1,2) Dermatologic findings include erythema, atrophy, xerosis, erosive disease, and reticulated erythematous patches.(3,4) Exceptionally, blistering has been reported.(5-7) Given its rarity, the clinical and histopathologic findings of bullous CRPS are not well described...
April 27, 2018: Journal of Cutaneous Pathology
Urda Rüdrich, Manuela Gehring, Eleni Papakonstantinou, Anja Rabenhorst, Judith Engmann, Alexander Kapp, Karin Hartmann, N Helge Meyer, Bernhard F Gibbs, Ulrike Raap
Bullous pemphigoid (BP) is characterized by substantial skin and blood eosinophilia as well as intensive pruritus. Since the pruritogenic cytokine interleukin (IL)-31 is increased in inflammatory skin diseases the aim of this study was to determine whether IL-31 plays a role in BP. Using immunofluorescence, IL-31 expression was analysed in eosinophils derived from blister fluids and skin from patients with BP and IL-31 levels in blister fluids, serum and culture supernatants were determined by enzyme-linked immunoassay (ELISA)...
April 24, 2018: Acta Dermato-venereologica
Jörg Tittelbach, Melanie Peckruhn, Peter Elsner
BACKGROUND: Dermatitis artefacta is a relevant and frequently unrecognized clinical condition associated with self-harming behavior, in which unconscious manipulation causes skin lesions. While atypical lesions and an unusual disease course may give rise to clinical suspicion of a self-induced disorder, questioning and examining these patients usually fails to confirm or clarify this suspicion. In this setting, the dermatopathologist may be faced with the question whether there are any histological signs corroborating the diagnosis of dermatitis artefacta...
April 24, 2018: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Valéria Bumiller-Bini, Gabriel Adelman Cipolla, Rodrigo Coutinho de Almeida, Maria Luiza Petzl-Erler, Danillo Gardenal Augusto, Angelica Beate Winter Boldt
Skin blisters of pemphigus foliaceus (PF) present concomitant deposition of autoantibodies and components of the complement system (CS), whose gene polymorphisms are associated with susceptibility to different autoimmune diseases. To investigate these in PF, we evaluated 992 single-nucleotide polymorphisms (SNPs) of 44 CS genes, genotyped through microarray hybridization in 229 PF patients and 194 controls. After excluding SNPs with minor allele frequency <1%, out of Hardy-Weinberg equilibrium in controls or in strong linkage disequilibrium ( r 2  ≥ 0...
2018: Frontiers in Immunology
Trinidad Hasbún, Ximena Chaparro, Viera Kaplan, Felipe Cavagnaro, Alex Castro
Henoch-Schönlein purpura (HSP) or IgA Vasculitis is the most common childhood vasculitis. The classic tetrad of signs and symptoms include palpable purpura, arthralgia, abdominal pain and renal disease. The occurrence of hemorrhagic bullae in children with HSP is rarely encountered. Objec tive: To report an unusual cutaneous manifestation of HSP in children. CASE REPORT: A 14-year-old girl complained about a 2-week painful bullous rash in both lower extremities and multiple arthral gias...
February 2018: Revista Chilena de Pediatría
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