key: cord-346017-wforwxes
authors: Kasperkiewicz, M.; Schmidt, E.; Fairley, J.A.; Joly, P.; Payne, A.S.; Yale, M.L.; Zillikens, D.; Woodley, D.T.
title: Expert recommendations for the management of autoimmune bullous diseases during the COVID‐19 pandemic
date: 2020-04-25
journal: J Eur Acad Dermatol Venereol
DOI: 10.1111/jdv.16525
sha: 
doc_id: 346017
cord_uid: wforwxes

Autoimmune bullous diseases (AIBDs) are potentially life‐threatening disorders comprising intra‐epidermal/epithelial (pemphigus) and sub‐epidermal/epithelial blistering diseases (pemphigoid and dermatitis herpetiformis). Corticosteroids and non‐steroid immunomodulatory agents are the mainstays of treatment. Treatment can be challenging particularly in pemphigus, mucous membrane pemphigoid, and epidermolysis bullosa acquisita which may require more intense immunosuppressive approaches

This article is protected by copyright. All rights reserved dermatitis herpetiformis). Corticosteroids and non-steroid immunomodulatory agents are the mainstays of treatment. Treatment can be challenging particularly in pemphigus, mucous membrane pemphigoid, and epidermolysis bullosa acquisita which may require more intense immunosuppressive approaches. 1, 2 A novel coronavirus named severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is responsible for the recent worldwide coronavirus disease 2019 (COVID-19) pandemic. Since immunosuppressive therapy can generally inhibit antiviral immunity, AIBD patients undergoing immunomodulatory treatment, especially elderly patients with co-morbidities, may be at higher risk of worse outcomes should they develop COVID-19. On the other hand, it has been postulated that immune system over-activation is responsible for the lung injury caused by SARS-CoV-2 and that a subgroup of patients might actually benefit from immunosuppressive drugs. 3 Similar to a recent publication concerning atopic dermatitis, 4 -Maintain immunomodulatory therapy when needed since unjustified withdrawal could lead to uncontrolled AIBD activity associated with high morbidity and mortality. 1,2 -Adhere to the advice from local health authorities in each country.

-Follow standard precautions including social distancing and hygienic procedures.

-Patients with confirmed COVID-19 should initially undergo risk evaluation.

-Azathioprine, mycophenolate mofetil/sodium, cyclophosphamide, methotrexate, and cyclosporine may be stopped for the duration of COVID-19 symptoms, whereas topical corticosteroids, prednis(ol)one ≤10 mg/d, dapsone/sulfapyridine, doxycycline/tetracycline, colchicine, and IVIG can be continued.

age, co-morbidities, and severity of COVID-19 in collaboration between the dermatologist and physician in charge of COVID-19.

-Abrupt termination or considerable dose reduction of systemic corticosteroids should be avoided, particularly in patients with severe forms of AIBDs. Of note, there is some evidence that prednisolone may potentially have beneficial impacts on COVID-19. 3

-Patients with AIBDs on immunosuppressive therapies are generally prone to develop opportunistic infections including viral infections, and microbial pathogens may potentially in turn trigger the bullous disease. Of note, both pemphigus and pemphigoid are associated with increased risk of death due to pneumonia and, in the case of paraneoplastic pemphigus, bronchiolitis obliterans. 1,2 However, there is currently little information specifically pertaining to SARS-CoV-2 and AIBDs.

-Dapsone/sulfapyridine, doxycycline/tetracycline, or IVIG are usually not considered to increase the risk for infections and may even decrease the risk of some infections, thus may be preferred in the COVID-19 pandemic where applicable.

-AIBD patients treated with rituximab were reported to have no additional risks for infections over high-dose corticosteroids without rituximab in general. 5 However, since long-lived SARS-CoV-2-specific plasma cells are not expected to be present in most individuals, AIBD patients treated with rituximab within the last one year may have a more severe/prolonged COVID-19 infection compared to healthy persons.

-Initiation of rituximab or immunoadsorption/plasmapheresis in AIBD patients must be weighed against the risks of conventional immunomodulatory regimens.

Finally, it is advised to remain updated through the WHO/CDC homepage (www.who.int, www.cdc.gov).

Pemphigoid diseases

Associations between immune-suppressive and stimulating drugs and novel COVID-19-a systematic review of current evidence

European Task Force on Atopic Dermatitis (ETFAD) statement on severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2)-infection and atopic dermatitis

First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial

Accepted Article