Biologics in autoimmune bullous diseases: Current scenario

Autoimmune bullous diseases can be intraepidermal (pemphigus group of disorders) or subepidermal (pemphigoid group of disorders). The treatment of these disorders chiefly comprises corticosteroids and immunosuppressant adjuvants like azathioprine and mycophenolate mofetil. Autoantibodies are the main mediators of these diseases. Rituximab, a chimeric anti-CD20 monoclonal antibody targeting B-cells, has emerged as an excellent treatment option for refractory pemphigus vulgaris in the last decade. Since then, many new biologics have been proposed/explored for managing autoimmune bullous diseases. These hold potential for greater efficacy and lesser adverse effects than conventional immunosuppressants. In this review, we discuss the role of various biologics in the treatment of autoimmune bullous diseases, followed by a brief discussion on the drawbacks to their use and new developments in this area.

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Rituximab in autoimmune bullous diseases: mixed responses and adverse effects

British Journal of Dermatology ◽

10.1111/j.1365-2133.2006.07646.x ◽

2007 ◽

Vol 156 (2) ◽

pp. 352-356 ◽

Cited By ~ 112

Author(s):

E. Schmidt ◽

C.S. Seitz ◽

S. Benoit ◽

E.B. Bröcker ◽

M. Goebeler

Keyword(s):

Adverse Effects ◽

Autoimmune Bullous Diseases ◽

Bullous Diseases ◽

Mixed Responses

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Treatment of resistant pemphigus vulgaris with an anti-CD20 monoclonal antibody (Rituximab)

Clinical and Experimental Dermatology ◽

10.1046/j.1365-2230.2003.01283.x ◽

2003 ◽

Vol 28 (4) ◽

pp. 366-368 ◽

Cited By ~ 58

Author(s):

H. L. Cooper ◽

E. Healy ◽

J. M. Theaker ◽

P. S. Friedmann

Keyword(s):

Monoclonal Antibody ◽

Pemphigus Vulgaris ◽

Anti Cd20

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Development of a Disease Registry for Autoimmune Bullous Diseases: Initial Analysis of the Pemphigus Vulgaris Subset

Acta Dermato Venereologica ◽

10.2340/00015555-1854 ◽

2015 ◽

Vol 95 (1) ◽

pp. 86-90 ◽

Cited By ~ 16

Author(s):

A Shah ◽

K Seiffert-Sinha ◽

D Sirois ◽

V Werth ◽

B Rengarajan ◽

...

Keyword(s):

Pemphigus Vulgaris ◽

Disease Registry ◽

Initial Analysis ◽

Autoimmune Bullous Diseases ◽

Bullous Diseases

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Vitamin D in autoimmune bullous disease

Acta Biochimica Polonica ◽

10.18388/abp.2020_2905 ◽

2020 ◽

Author(s):

Stefan Tukaj

Keyword(s):

Vitamin D ◽

Lupus Erythematosus ◽

Skin Diseases ◽

Experimental Studies ◽

Epidemiological Studies ◽

Diabetes Mellitus Type 1 ◽

Inflammatory Skin Diseases ◽

Autoimmune Bullous Diseases ◽

Bullous Diseases

Numerous epidemiological studies have suggested a link between vitamin D deficiency and the development of various autoimmune diseases, including diabetes mellitus type 1, rheumatoid arthritis, inflammatory bowel disease, multiple sclerosis or systemic lupus erythematosus. More recently, such a link has been also proposed for autoimmune bullous diseases (AIBD). This is a relatively rare and potentially life-threatening, organ-specific group of inflammatory skin diseases characterized by the presence of tissue-bound and circulating autoantibodies against various molecules present in desmosomes (in pemphigus diseases) or hemidesmosomes (in pemphigoid diseases). In addition to the well-known role of vitamin D in calcium and phosphate homeostasis, the hormonally active vitamin D metabolite, 1,25-dihydroxyvitamin D3 (calcitriol), exerts potent effects on cellular differentiation and regulation of immune responses via binding to the vitamin D receptor present in most cells of the immune system. Since cells of both, the innate and adaptive immune systems, are known to be relevant in AIBD, the role of vitamin D analogues in the treatment of patients with these disorders deserves much attention. This mini-review summarizes recent epidemiological and experimental studies on vitamin D involvement in the autoimmune bullous diseases.

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An Unusual Case of Pemphigus Vulgaris

Skin Appendage Disorders ◽

10.1159/000521333 ◽

2022 ◽

pp. 1-3

Author(s):

Laura Marano ◽

Gabriella Fabbrocini ◽

Giuseppe Monfrecola ◽

Fabrizio Martora

Keyword(s):

Skin Disorder ◽

Unusual Case ◽

Pemphigus Vulgaris ◽

Pemphigus Foliaceus ◽

Case Presentation ◽

Autoimmune Bullous Diseases ◽

Atypical Manifestations ◽

Life Threatening ◽

Bullous Diseases ◽

Careful History

Introduction: Pemphigus is a potential life-threatening skin disorder belonging to the group of the autoimmune bullous diseases affecting the skin and mucosa. The most common subtypes are pemphigus foliaceus (PF) and pemphigus vulgaris. Case Presentation: We present the case of a young woman with scalp manifestations diagnosed as seborrhiasis who came to our office where a more careful history and clinical examination directed us toward another diagnostic suspicion. The histological examination confirmed our suspicion of pemphigus and therefore we believe it is important to report our experience to avoid misdiagnosis. Discussion/Conclusion: Our case may be useful in the literature to identify cases of PF with atypical manifestations that may mimic other diseases.

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