Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach

Abstract

Autoimmune bullous dermatoses are a heterogeneous group of diseases with autoantibodies against structural skin proteins. Although the occurrence of autoimmune bullous dermatoses during pregnancy is low, this topic deserves attention, since the immunological and hormonal alterations that occur during this period can produce alterations during the expected course of these dermatoses. The authors review the several aspects of autoimmune bullous dermatoses that affect pregnant women, including the therapeutic approach during pregnancy and breastfeeding. Gestational pemphigoid, a pregnancy-specific bullous disease, was not studied in this review.

Case Report: Diagnostic and Therapeutic Challenges in Severe Mechanobullous Epidermolysis Bullosa Acquisita

“…Around 20 EBA cases associated with a hematologic malignancy have been reported so far ( 17 , 18 ), illustrating that repeated paraneoplastic screening may be considered, as is recommended for patients with laminin 332- associated mucous membrane pemphigoid ( 19 ). EBA following pregnancy is an extremely rare event and only reported in few cases ( 20 ). In general, the altered immune pattern with dominance of Th2 lymphocytes during pregnancy favours development of autoimmunity, while Th1 dominant diseases tend to improve ( 20 ).…”

“…EBA following pregnancy is an extremely rare event and only reported in few cases ( 20 ). In general, the altered immune pattern with dominance of Th2 lymphocytes during pregnancy favours development of autoimmunity, while Th1 dominant diseases tend to improve ( 20 ). Whether stretching of the skin, causing damage to the extracellular matrix and connective tissue and thereby exposing maternal antigens plays a role, remains elusive.…”

Burden of pemphigus vulgaris with a particular focus on women: A review

“… 38 The disturbance of the Th1:Th2 balance may trigger or exacerbate Th2-mediated autoimmune diseases, such as pemphigus, during pregnancy. 39 , 40 In addition to this immunological pathway, women’s concern for the potential harm of medications to their pregnancy may affect their adherence to the treatment. 41 Although no estimate of drug cessation during pregnancy in patients with pemphigus exists, it may be an important factor affecting disease exacerbation in pregnancy.…”

Fallbericht: Diagnostische und therapeutische Herausforderungen bei schwerer mechanobullöser Epidermolysis bullosa acquisita

“…Bisher wurden etwa 20 EBA-Fälle im Zusammenhang mit einer hämatologischen Malignität berichtet [17, 18], was veranschaulicht, dass ein wiederholtes paraneoplastisches Screening in Betracht gezogen werden kann, wie es für Patienten mit Laminin-332-assoziiertem Schleimhautpemphigoid empfohlen wird [19]. EBA nach einer Schwangerschaft ist ein extrem seltenes Ereignis und wird nur in wenigen Fällen gemeldet [20]. Im Allgemeinen begünstigt das veränderte Immunmuster mit einer Dominanz von Th2-Lymphozyten während der Schwangerschaft die Entwicklung von Autoimmunität, während Th1-dominante Erkrankungen eher verbessert werden [20].…”