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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 -
JAAD Case Reports Nov 2023
PubMed: 37842158
DOI: 10.1016/j.jdcr.2023.08.013 -
JAAD International Dec 2023
PubMed: 37786789
DOI: 10.1016/j.jdin.2023.08.015 -
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 -
Cureus Sep 2023Neonatal alloimmune thrombocytopenia (NAIT) is a complex condition, stemming from the transplacental passage of alloantibodies from a pregnant mother directed against...
Neonatal alloimmune thrombocytopenia (NAIT) is a complex condition, stemming from the transplacental passage of alloantibodies from a pregnant mother directed against fetal platelet antigens. This case report discusses a rare instance of severe NAIT initially presenting as inadequate weight gain. After a clinical workup yielded negative findings for an infection and the resolution of the patient's thrombocytopenia following the administration of platelet products and intravenous immunoglobulin (IVIG), hematology deduced that this patient's NAIT was secondary to maternal history of gestational pemphigus. We describe the pathophysiology and current understanding of NAIT, pemphigoid gestationis (PG), as well as an analysis of their association. This intersection of NAIT and maternal PG underscores the importance of considering potential interactions between maternal autoimmune conditions overall and their impact on fetal health.
PubMed: 37692187
DOI: 10.7759/cureus.44946 -
Cureus Mar 2024Background Autoimmune vesiculobullous diseases (AIBDs) are a group of diseases characterized by blisters of the skin/mucosa due to the presence of circulating...
Background Autoimmune vesiculobullous diseases (AIBDs) are a group of diseases characterized by blisters of the skin/mucosa due to the presence of circulating autoantibodies against antigens in the epidermis or the dermo-epidermal junction. Direct immunofluorescence (DIF) for immunoglobulin (Ig)G, IgC3, and IgA on fresh-frozen tissue is the gold standard diagnostic test for AIBDs. However, DIF in the absence of frozen tissue is challenging for the diagnosis of AIBDs. This study aimed to analyze the practical utility of DIF using paraffin-embedded skin biopsy rather than fresh frozen tissue for the diagnosis of AIBDs. Methodology This cross-sectional comparative study included 30 cases of AIBDs. DIF for IgG and IgA was performed on paraffin-embedded tissue (PE-DIF) after proteinase digestion on histopathologically confirmed 15 pemphigus vulgaris (PV), three pemphigus foliaceous (PF), four bullous pemphigoid (BP), three dermatitis herpetiformis (DH), three subcorneal pustular dermatosis (SCPD), and one case each of linear IgA disease and pemphigoid gestationis (PG). PE-DIF staining pattern was compared with the DIF on fresh frozen tissue (FF-DIF). Results All cases of PV and PF showed an intercellular IgG chicken wire staining pattern similar to FF-DIF. However, background staining was more intense in PV cases while less intense in PF cases. Three BP cases showed linear IgG staining in PE-DIF. DH, SCPD, linear IgA disease, and PG cases did not show IgG positivity. Out of three DH cases, two cases showed granular IgA positivity while linear IgA positivity along the basement membrane was seen in a single case of linear IgA disease. Negative IgG staining was observed in SCPD. Immunofluorescence in PE-DIF was rapidly deteriorating than in FF-DIF. Conclusions DIF done on paraffin-embedded tissue can be used as a supplement and salvage technique with histopathology for the diagnosis of AIBDs, particularly when a cryostat facility for frozen tissue is not available and the patient is unable to undergo a second biopsy procedure.
PubMed: 38665766
DOI: 10.7759/cureus.56916 -
JAAD Case Reports Apr 2024
PubMed: 38463656
DOI: 10.1016/j.jdcr.2024.01.026