scholarly journals Histomorphologic and Direct Immunofluorescence Findings of Autoimmune Bullous Disease

2018 ◽  
Author(s):  
Deniz Ates
Keyword(s):  
Direct Immunofluorescence ◽  
Bullous Disease ◽  
Autoimmune Bullous Disease

scholarly journals IgA Pemphigus in a Child – a Case Report

2017 ◽  
Vol 9 (1) ◽  
pp. 14-21
Author(s):  
Branislav Lekić ◽  
Mirjana Gajić-Veljić ◽  
Svetlana Popadić ◽  
Miloš Nikolić
Keyword(s):  
Case Report ◽  
Complete Remission ◽  
Direct Immunofluorescence ◽  
Cell Surfaces ◽  
Bullous Disease ◽  
Autoimmune Bullous Disease ◽  
Iga Pemphigus ◽  
Subcorneal Pustular Dermatosis ◽  
Keratinocyte Cell ◽  
The Face

Abstract IgA pemphigus (IGAP) is a rare autoimmune bullous disease characterized by IgA deposits on keratinocyte cell surfaces. The IGAP is classified into: 1) subcorneal pustular dermatosis (SPD) type, and 2) intraepidermal neutrophilic (IEN) IgA dermatosis type. So far, only 9 children with IGAP have been described in the literature, of whom only 3 with SPD type. We report a 3-year-old boy with SPD type of IGAP. Clinically, he presented with pruritic vesicles, pustules and erosions on the face, trunk, groin area, and extremities. Histopathology showed subcorneal pustules containing a few acantholytic cells. Direct immunofluorescence (DIF) test of Tzanck smear showed intercellular IgA deposits on the surface of the groups of epidermal cells. Oral dapsone and prednisone induced remission after two weeks; the treatment was discontinued 11 months later, and complete remission was achieved during 19 months without any treatment. Direct immunofluorescence of Tzanck smear is a simple, sensitive, rapid and non-aggressive test, very suitable for the diagnosis of IGAP in children.

scholarly journals Severe Multi-Resistant Pemphigus vulgaris: prolonged remission with a single cycle of Rituximab

2013 ◽  
Vol 88 (4) ◽  
pp. 639-642 ◽  
Author(s):  
Isabela Soubhia Corral ◽  
Thais Helena Proença de Freitas ◽  
Renata Telles Rudge de Aquino ◽  
Daniella Abbruzzini S. Koller ◽  
Maria Elisa Ruffolo Magliari ◽  
...  
Keyword(s):  
Pemphigus Vulgaris ◽  
Immunosuppressive Drugs ◽  
Direct Immunofluorescence ◽  
Clinical Relapse ◽  
Single Cycle ◽  
Bullous Disease ◽  
Autoimmune Bullous Disease ◽  
Systemic Corticosteroids ◽  
Daily Dose

Pemphigus vulgaris is an autoimmune bullous disease whose therapy is based on systemic corticosteroids, with or without immunosuppressants. Rituximab is a chimeric monoclonal antibody of the IgG class, directed at a specific CD20 B cell surface antigen, used in pemphigus vulgaris empirically since 2002, with success in 90% of the cases and long periods of remission. Male patient, 33 years old, diagnosed with pemphigus vulgaris, confirmed by histopathology and direct immunofluorescence. He was treated for seven months with numerous treatments, including immunosuppressive drugs, with an unsatisfactory response, until he had complete remission with the use of rituximab. During a 34-month follow-up period, the patient presented a slight clinical relapse, which was successfully controlled with prednisone in a daily dose of 120mg, soon reduced to 20mg.

Bullous Scabies Simulating Pemphigoid

2011 ◽  
Vol 15 (1) ◽  
pp. 55-57 ◽  
Author(s):  
T. Roxana Stan ◽  
Stefano Piaserico ◽  
Matteo Bordignon ◽  
Roberto Salmaso ◽  
Edoardo Zattra ◽  
...  
Keyword(s):  
Skin Lesion ◽  
Bullous Pemphigoid ◽  
Steroid Treatment ◽  
Social Conditions ◽  
Direct Immunofluorescence ◽  
Benzyl Benzoate ◽  
Bullous Disease ◽  
Autoimmune Bullous Disease ◽  
Skin Specimen ◽  
Desmoglein 3

Background: Scabies is a contagious infestation affecting subjects of all ages, races, and social conditions. Objective: We report a case of a 79-year-old man who developed a bullous pemphigoid-like eruption. He presented to our unit 4 months after the onset of symptoms. An autoimmune bullous disease was suspected. Direct immunofluorescence on a skin specimen and anti-desmoglein 1, anti-desmoglein 3, and anti-bullous pemphigoid antigen 180 were negative. Surprisingly, the histology of a skin lesion demonstrated the presence of scabies, which was successfully treated with benzyl benzoate 20%. Conclusion: The diagnosis of bullous scabies should be considered for any bullous eruptions accompanied by papules and itching resistant to steroid treatment and with negative immunopathologic findings.

scholarly journals Assessing the Correlation Between Autoimmune Bullous Disease Quality of Life Questionnaires (ABQOL, TABQOL, DLQI) and Disease Severity Indices in Pemphigus and Bullous Pemphigoid

2020 ◽  
Author(s):  
Shan Cao ◽  
Lulu Sun ◽  
Zhongxiang Shi ◽  
Baoqi Yang ◽  
Furen Zhang
Keyword(s):  
Quality Of Life ◽  
Disease Severity ◽  
Bullous Pemphigoid ◽  
Strong Correlations ◽  
Disease Area ◽  
Area Index ◽  
Severity Of Disease ◽  
Bullous Disease ◽  
Autoimmune Bullous Disease

Abstract Background: Pemphigus and bullous pemphigoid (BP) are autoimmune blistering diseases (AIBDs) that affect the skin and mucous membranes, and adversely impact quality of life (QOL). Few studies have assessed the correlation between the severity of disease and QOL in patients with pemphigus and BP. Objectives: To identify the correlation between the severity of AIBDs and QOL using the Autoimmune Bullous Disease Quality of Life (ABQOL), Treatment Autoimmune Bullous Disease Quality of Life (TABQOL), and Dermatology Life Quality Index (DLQI) questionnaires in Chinese patients with pemphigus and BP at baseline, and after 1, 3 and 6 months of treatment. Methods: Pemphigus and BP patients were invited to complete the ABQOL, TABQOL, and DLQI questionnaires. We measured the pemphigus disease area index (PDAI), autoimmune bullous skin disorder intensity score (ABSIS), and antibodies of desmoglein1,desmoglein3 (DSG1/DSG3) for pemphigus; and the bullous pemphigoid disease area index (BPDAI), ABSIS, and antibodies of BP180/ BP230 for BP as disease severity indices. The correlations between the severity of disease and QOL were analyzed by Spearman’s correlation coefficient (r). Results: Eighty-five patients were included: 55 with pemphigus and 30 with BP. The pemphigus subtypes included pemphigus vulgaris (PV, n = 32), pemphigus foliaceus (PF, n = 22), and paraneoplastic pemphigus (PNP, n = 1). We found significantly strong correlations between QOL (ABQOL, TABQOL, DLQI) and severity of disease (PDAI/BPDAI, ABSIS) with (r = 0.87, 0.77, 0.83; r = 0.86, 0.73, 0.80) for pemphigus and BP, respectively. Mild or strong correlations were also observed between QOL and antibody titers in pemphigus with DSG1/DSG3(r=0.32/0.36) and BP with BP180/BP230( r = 0.73/0.17) respectively. Conclusion: The QOL of patients with pemphigus and BP decreased with increased severity of the AIBDs. As the disease severity descended, so the QOL improved. The QOL indices should be used in clinical trials and to manage patients’ treatment, especially during the active disease stage, despite the mild correlation observed after 1 month of treatment.The PDAI to be better at assessing disease severity than the ABSIS in patients with pemphigus, and ABSIS are better than BPDAI in BP patients for correlation with the QOL indices.

Bacteremia in autoimmune bullous disease patients undergoing double-filtration plasmapheresis

2018 ◽  
Vol 30 (4) ◽  
pp. 402-404
Author(s):  
Chika Ohata ◽  
Hiroshi Koga ◽  
Hiroshi Saruta ◽  
Norito Ishii ◽  
Takekuni Nakama
Keyword(s):  
Double Filtration Plasmapheresis ◽  
Bullous Disease ◽  
Autoimmune Bullous Disease ◽  
Double Filtration

Dermatological emergencies

2019 ◽  
pp. 691-710
Author(s):  
Punit S. Ramrakha ◽  
Kevin P. Moore ◽  
Amir H. Sam
Keyword(s):  
Herpes Zoster ◽  
Toxic Epidermal Necrolysis ◽  
Stevens Johnson Syndrome ◽  
Drug Reactions ◽  
Eczema Herpeticum ◽  
Bullous Disease ◽  
Autoimmune Bullous Disease ◽  
Johnson Syndrome ◽  
Generalized Pustular Psoriasis ◽  
Cutaneous Drug Reactions

This chapter discusses dermatological emergencies, including cutaneous drug reactions, erythroderma, urticaria and angio-oedema, autoimmune bullous disease, eczema herpeticum, herpes zoster, generalized pustular psoriasis, and Stevens–Johnson syndrome and toxic epidermal necrolysis.

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