Immune Blistering Disorders

Immune blistering diseases may involve the cutaneous or mucosal surfaces of the female genital structures. Blisters that form on mucosal surfaces erode quickly and appear more commonly as erosions. Diagnosis requires a thorough history and full examinatio

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45

Sarah Shaffer and Colleen K. Stockdale

45.1 Introduction

full examination of all cutaneous and mucosal sites to identify lesions that may be asymptomImmune blistering diseases may involve the cuta- atic, healing, or hidden from view (i.e., vaginal neous or mucosal surfaces of the female genital vault). There is no proven link to malignancy structures. These noninfectious bullous disorders demonstrated for any of the immune blistering are characterized by autoantibodies to antigens diseases [2, 3]. that have a role in adherence of epidermis to derManagement principles and recommended mis or epithelial cells to one another [1]. medications overlap for many of the immune Antibodies affix to a target antigen, trigger com- blistering diseases; where specific differences plement activation, and attract eosinophils and exist, notations are made with the description of neutrophils, which degranulate releasing proteo- the disease. The use of topical steroids is occalytic enzymes that cause blister formation in sionally sufficient, but systemic steroids are often either a subepidermal or intraepidermal location. indicated and should be tapered with clinic Cutaneous and mucosal tissue can be affected; response. Case reports or small case series exist blisters that form on mucosal surfaces erode for most of the immune bullous disorders for the quickly and appear more commonly as erosions. following immune-suppressive medications: azaA biopsy taken from the edge of a blister or ero- thioprine (often noted to be the preferred sion should include a small sample of normal-­ immune-suppressive medication for an acceptappearing epithelium. Histology and direct able side effect profile), cyclophosphamide, immunofluorescence are necessary to diagnose mycophenolate mofetil (nonsteroidal option), and differentiate these diseases. Indirect immu- methotrexate and thalidomide (beware the need nofluorescence of serum for autoantibodies may for contraception if either is used in a reproductive-­ also be considered. aged woman), cyclosporine, dapsone (screen for Unless noted, both subepidermal and intraepi- G6PD deficiency before use), rituximab, nicotindermal blistering diseases occur more commonly amide and tetracycline antibiotics, colchicine, in the elderly, often peaking in or after the sixth antimalarial medications, plasmapheresis, and decade of life. Diagnosis of immune blistering intravenous immunoglobulin (IVIG) [3–12]. disorders requires a thorough history as well as a When treating vulvovaginal manifestations of immune blistering disorders, topical steroid ointments are the predominant form of therapy. S. Shaffer · C. K. Stockdale (*) Care of the vulvar skin should always Department of Obstetrics and Gynecology, University include review of basic guidelines with emphaof Iowa, Iowa City, IA, USA sis on the use of soaks and addition of barriers e-mail: [email protected] © Springer International Publishing AG, part of Springer Nature 2019 J. Bornstein (ed.), Vulvar Disease, https://doi.org/10.1007/978-3-319-61621-6_4

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