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Frontiers in Immunology 2024Autoimmune blistering disorders (AIBDs) are a heterogeneous group of approximately a dozen entities comprising pemphigus and pemphigoid disorders and dermatitis... (Review)
Review
Autoimmune blistering disorders (AIBDs) are a heterogeneous group of approximately a dozen entities comprising pemphigus and pemphigoid disorders and dermatitis herpetiformis. The exact diagnosis of AIBDs is critical for both prognosis and treatment and is based on the clinical appearance combined with the detection of tissue-bound and circulating autoantibodies. While blisters and erosions on the skin and/or inspectable mucosal surfaces are typical, lesions may be highly variable with erythematous, urticarial, prurigo-like, or eczematous manifestations. While direct immunofluorescence microscopy (IFM) of a perilesional biopsy is still the diagnostic gold standard, the molecular identification of the major target antigens opened novel therapeutic avenues. At present, most AIBDs can be diagnosed by the detection of autoantigen-specific serum antibodies by enzyme-linked immunosorbent assay (ELISA) or indirect IFM when the clinical picture is known. This is achieved by easily available and highly specific and sensitive assays employing recombinant immunodominant fragments of the major target antigens, i.e., desmoglein 1 (for pemphigus foliaceus), desmoglein 3 (for pemphigus vulgaris), envoplakin (for paraneoplastic pemphigus), BP180/type XVII collagen (for bullous pemphigoid, pemphigoid gestationis, and mucous membrane pemphigoid), laminin 332 (for mucous membrane pemphigoid), laminin β4 (for anti-p200 pemphigoid), type VII collagen (for epidermolysis bullosa acquisita and mucous membrane pemphigoid), and transglutaminase 3 (for dermatitis herpetiformis). Indirect IFM on tissue substrates and in-house ELISA and immunoblot tests are required to detect autoantibodies in some AIBD patients including those with linear IgA disease. Here, a straightforward modern approach to diagnosing AIBDs is presented including diagnostic criteria according to national and international guidelines supplemented by long-term in-house expertise.
Topics: Humans; Autoantibodies; Autoimmune Diseases; Autoantigens; Skin Diseases, Vesiculobullous; Enzyme-Linked Immunosorbent Assay
PubMed: 38903493
DOI: 10.3389/fimmu.2024.1363032 -
JAAD Case Reports Nov 2023
PubMed: 37842158
DOI: 10.1016/j.jdcr.2023.08.013 -
Journal of the American Academy of... Jul 2023
Topics: Pregnancy; Female; Humans; Pruritus; Pemphigoid Gestationis; Pregnancy Complications
PubMed: 37187427
DOI: 10.1016/j.jaad.2023.05.016 -
Cureus Nov 2023Pemphigoid gestationis (PG) is a rare autoimmune bullous disease that occurs during pregnancy or the postpartum period. PG has been associated with an increased risk of...
Pemphigoid gestationis (PG) is a rare autoimmune bullous disease that occurs during pregnancy or the postpartum period. PG has been associated with an increased risk of Graves' disease possibly due to shared genetic factors and immune system fluctuations during pregnancy. However, the evidence supporting the association between PG and Graves' disease is mixed. Although dermatologists are cautioned to watch for Graves' disease in patients with a history of PG, this guidance is based on a single cohort where most patients were diagnosed with Graves' disease prior to PG onset. Recent data failed to find an association between Graves' disease and PG but did not capture the lifetime risk of Graves' disease in these patients. Future studies could focus on long-term follow-up of females with PG, shedding light on the lifetime risk profiles of these patients.
PubMed: 38106729
DOI: 10.7759/cureus.48972 -
Frontiers in Immunology 2023The pemphigoid group comprises a number of bullous skin diseases with autoantibodies against different constituents of the basement membrane zone that result in...
The pemphigoid group comprises a number of bullous skin diseases with autoantibodies against different constituents of the basement membrane zone that result in subepidermal detachment and clinically characteristic tense blisters, erosions, urticarial erythema, and itching. Apart from the most frequent type of bullous pemphigoid with antibodies against BP180, which is found predominantly in elderly patients, the disease may present at other ages and different pathogenic conditions. Here, four cases are presented of young age (3 months and 25, 34, and 46 years) and in association with vaccination, pregnancy, or metastatic cancer. Though anti-BP180 was found in all cases, a different pathogenic background may be found in any of them, resulting in characteristic clinical manifestation, yet demanding specifically adapted therapeutic approaches.
Topics: Pregnancy; Female; Humans; Aged; Pemphigoid, Bullous; Blister; Skin Diseases, Vesiculobullous; Autoantibodies; Pruritus
PubMed: 37901243
DOI: 10.3389/fimmu.2023.1272742 -
Journal of the American Academy of... Jul 2023Pemphigoid gestationis (PG) and polymorphic eruption of pregnancy (PEP) may be similar morphologically but confer different maternal and fetal risks. Direct...
BACKGROUND
Pemphigoid gestationis (PG) and polymorphic eruption of pregnancy (PEP) may be similar morphologically but confer different maternal and fetal risks. Direct immunofluorescence is the gold standard test used to differentiate between the 2 diagnoses but is not always available.
OBJECTIVE
To develop and validate a clinical scoring system to differentiate PG from PEP.
METHODS
After developing a scoring system based on differentiating clinical factors reported in existing literature, we tested its diagnostic accuracy in a retrospective international multicenter validation study in collaboration with the European Academy of Dermatology and Venereology's Skin Diseases in Pregnancy Taskforce.
RESULTS
Nineteen pregnancies (16 patients) affected by PG and 39 pregnancies (39 patients) affected by PEP met inclusion criteria. PG had a mean score of 4.6 (SD, 2.5) and PEP had a mean score of -0.3 (SD, 2.0). The area under the curve was 0.93 (95% CI, 0.86-1.00). Univariate analysis revealed that almost all criteria used in the scoring system were significantly different between the groups (P < .05), except for skip pregnancy and multiple gestations, which were then removed from the final scoring system.
LIMITATIONS
Small retrospective study.
CONCLUSION
The Pregnancy Dermatoses Clinical Scoring System may be useful to differentiate PG from PEP in resource-limited settings.
Topics: Female; Pregnancy; Humans; Pemphigoid Gestationis; Retrospective Studies; Pruritus; Pregnancy Complications; Exanthema
PubMed: 36739091
DOI: 10.1016/j.jaad.2023.01.027 -
International Journal of Dermatology Jan 2024
Topics: Pregnancy; Female; Humans; Pemphigoid Gestationis; Retrospective Studies; Pregnancy Complications; Exanthema; Treatment Outcome
PubMed: 37916499
DOI: 10.1111/ijd.16884 -
Journal of the European Academy of... Sep 2023
Topics: Pregnancy; Female; Humans; Pemphigoid Gestationis; Antibodies, Monoclonal, Humanized; Pemphigoid, Bullous
PubMed: 37147906
DOI: 10.1111/jdv.19171