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Blistering skin disorders
Published in Rashmi Sarkar, Anupam Das, Sumit Sethi, Concise Dermatology, 2021
It is not a single disease, but a group of heterogeneous, chronic blistering diseases primarily affecting mucosal surfaces. It is the second most common autoimmune blistering disorder in central Europe. Historically, because of the scarring complications that may develop clinically, it was described as cicatricial pemphigoid but is now categorized as mucous membrane pemphigoid. Cicatricial pemphigoid is now used for blistering disorders where skin lesions heal with scarring, but mucosal lesions are absent. The mucous membranes that are most frequently affected are the oral cavity, the eyes, and nasopharynx.
Linear IgA disease
Published in Lionel Fry, Atlas of Bullous Diseases, 2020
LAD has to be distinguished from other bullous diseases. As the bullae are subepidermal, they are tense and do not rupture as easily as pemphigus blisters. If LAD presents late in life then it has to be distinguished from bullous pemphigoid (BP). If there is severe involvement of the mucous membranes then a distinction has to be made from mucous membrane pemphigoid (MMP). The distinction of LAD from other autoimmune bullous diseases may be made by direct immunofluorescent studies. The distinction between LAD and IgA mucous membrane pemphigoid may be the most difficult. In this instance, it has been proposed that that the disease should be classified as mucous membrane pemphigoid. Conversely, there are so-called ‘mixed’ immunobullous disorders (see Chapter 10), with both linear IgG and IgA deposits along the basement membrane region. In these cases the course of the disease is usually more like LAD, and has a good response to dapsone and sulfonamides. The exact nosology of this group of patients is unclear, but the natural history is that of LAD.
Female-specific pruritus
Published in Miranda A. Farage, Howard I. Maibach, The Vulva, 2017
Michael Joseph Lavery, Carolyn Stull, Shoshana Korman Grossman, Gil Yosipovitch
LP (Figure 20.4) is an autoimmune mucocutaneous disorder that mainly affects postmenopausal women. LP may affect the genitalia and may occur alongside extragenital involvement affecting the scalp, nails, and mucous membranes (oral, esophageal, urinary tract, and anus). The constellation of erosive LP involving the vulva, vagina, and gingiva is termed Hewitt–Pelisse syndrome (vulvovaginal–gingival syndrome) (32). This can clinically mimic mucous membrane pemphigoid. In addition, an association with hepatitis C infection has been reported (33).
Autoimmune blistering diseases: promising agents in clinical trials
Published in Expert Opinion on Investigational Drugs, 2023
Henning Olbrich, Christian D. Sadik, Enno Schmidt
Mucous membrane pemphigoid (MMP) is distinguished clinically from BP and other pemphigoid diseases by predominant affection of mucosae, while skin lesions are absent or mild [14]. Mono- or multisite disease can be found and typical localizations include the oral, ocular, genital or urological, tracheal, nasopharyngeal, and laryngeal mucosa besides skin [15]. Manifestations include erythema, erosions, ulcerations, and subsequent scarring; in particular, ocular MMP can induce symblephara, entropion, and trichiasis and often cause blindness. MMP is a rare disease affecting mainly the elderly with an incidence of 1–2 per million per year [5,6]. Pathomechanisms are heterogeneous. DIF can show linear deposits of IgG, IgA, IgM, and/or complement C3 at the basement membrane with an n-, u-, or undetermined serration pattern; however, initial DIF findings may be negative due to a low concentration of tissue-bound antibodies. The main target antigens are BP180 (mainly C-terminal epitopes) and laminin 332; BP230 and type VII collagen can also be recognized and individual patients with antibodies against α6β4 integrin have been described [16–21]. Laminin 332 autoantibodies are associated with neoplasms in about 25% of MMP patients [22–24].
Dipeptidyl peptidase IV inhibitor-associated bullous pemphigoid: a recently recognized autoimmune blistering disease with unique clinical, immunological and genetic characteristics
Published in Immunological Medicine, 2019
BP usually develops in individuals around 70–80 years old, with no sex prevalence [1]. The prevalence in Europe has been reported to be 21–66/million [1], and the number of BP cases is increasing, probably due to demographic aging. BP is known to be likely to develop in patients with neurological diseases such as Alzheimer’s disease, multiple sclerosis and cerebral infarction [10], and a prodromal phase of chronic eczema or prurigo nodularis may proceed the onset of BP [11]. Clinically, typical BP patients show itchy urticarial erythema with tense blisters on the entire body (Figure 1(A)). The mucous membranes, including those in the ocular, oral and nasopharyngeal regions, are involved in around 20 percent of patients. Patients with extensive mucous membrane lesions are classified as mucous membrane pemphigoid, which is a pemphigoid variant [1]. Histopathologically, BP blisters form in the subepidermis with numerous eosinophilic infiltrates (Figure 1(B)). Immunologically, BP patients have circulating autoantibodies directing the dermal-epidermal junction (DEJ) in skin, and direct immunofluorescence (DIF) studies on peri-blistering lesions have revealed in vivo deposits of IgG and/or C3 along the DEJ (Figure 1(C)) [2]. Circulating IgG-class autoantibodies directing the DEJ are detectable by indirect immunofluorescence (IIF) using human skin substrate. When IIF is performed on human skin with artificial blisters induced by 1 M NaCl incubation, the BP-IgG autoantibodies react to the epidermal side of the blisters (Figure 1(D)).
Direct Immunofluorescence Findings in 145 Consecutive Patients Receiving a Conjunctival Biopsy for Cicatrizing Conjunctivitis
Published in Ocular Immunology and Inflammation, 2021
D. Kneiber, E.H. Kowalski, M. Kroloff, P.M. Patel, V.A. Jones, E.Y. Tu, J. Sugar, I. Aronson, K.T. Amber
We read Anesi et al.’s findings with great interest.1 The authors detail the results of diagnostic testing on one of the largest cohorts of patients referred for evaluation of ocular mucous membrane pemphigoid (oMMP). Given the controversy surrounding the utilization of immunosuppressive treatment in cases with a negative direct immunofluorescence (DIF) and high clinical suspicion for oMMP2,3 which they discuss, we recently surveyed our own database to determine the number of positive DIF results. We queried the University of Illinois at Chicago’s Skin Immunology database, assessing specimens sent to rule out ocular mucous membrane pemphigoid. After exclusion of eight cases with inadequate records, 145 records were available for review.