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Postepy Dermatologii I Alergologii Apr 2023
PubMed: 37312912
DOI: 10.5114/ada.2023.127648 -
JAMA Dermatology Feb 2022This case series characterizes immune profiles of pregnant people with pemphigoid gestationis.
This case series characterizes immune profiles of pregnant people with pemphigoid gestationis.
Topics: Autoantibodies; Autoantigens; Female; Humans; Non-Fibrillar Collagens; Pemphigoid Gestationis; Pemphigoid, Bullous; Pregnancy
PubMed: 34985500
DOI: 10.1001/jamadermatol.2021.5294 -
American Journal of Obstetrics &... Sep 2023
Use of a pregnancy dermatology clinical scoring system to differentiate between pemphigoid gestationis and polymorphic eruption of pregnancy: practical considerations for the obstetrician.
Topics: Pregnancy; Female; Humans; Pemphigoid Gestationis; Obstetricians; Dermatology; Pregnancy Complications
PubMed: 37399893
DOI: 10.1016/j.ajogmf.2023.101078 -
JAAD International Dec 2023
PubMed: 37786789
DOI: 10.1016/j.jdin.2023.08.015 -
PloS One 2021Autoimmune bullous diseases vary in their clinico-epidemiological features and burden across populations. Data about these diseases was lacking in Sudan. We aimed to...
OBJECTIVES
Autoimmune bullous diseases vary in their clinico-epidemiological features and burden across populations. Data about these diseases was lacking in Sudan. We aimed to describe the epidemiological profile and to estimate the burden of autoimmune bullous diseases in Sudan.
METHODS
This was a retrospective cross-sectional study conducted at Khartoum Dermatological and Venereal Diseases Teaching Hospital. We used routinely collected health care data, and included all patients with an autoimmune bullous disease who presented to the hospital between 2001 and 2016.
RESULTS
Out of the 4736 patients who were admitted to the hospital during the study period, 923 (19.5%) had an autoimmune bullous disease. The average rate of patients at the hospital was 57.7 per year representing 1.3 per 100,000 population per year. After exclusion of patients where the final diagnosis was missing, 585 were included in the further analysis. Pemphigus vulgaris was the most common disease (50.9%), followed by bullous pemphigoid (28.2%), linear IgA disease/chronic bullous disease of childhood (8.4%), and pemphigus foliaceous (8.2%). Pemphigoid gestationis and IgA pemphigus constituted 1.4% and 1.2% of the cohort, respectively. Paraneoplastic pemphigus, mucous membrane pemphigoid, lichen planus pemphigoidis, bullous systemic lupus erythematosus, and dermatitis herpetiformis were rare. None of the patients had epidermolysis bullosa acquisita.
CONCLUSIONS
The clinico-epidemiological characteristics vary among the types of autoimmune bullous diseases. Females were more predominant in most of them. Sudanese patients tended in general to present at a younger age than other populations. The pool of Sudanese patients with autoimmune bullous diseases is large which requires investigation for the local risk factors and presents a field for future trials.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Autoimmune Diseases; Child; Child, Preschool; Cross-Sectional Studies; Epidermolysis Bullosa Acquisita; Female; Humans; Male; Middle Aged; Pemphigoid, Benign Mucous Membrane; Pemphigoid, Bullous; Retrospective Studies; Sudan; Young Adult
PubMed: 34255799
DOI: 10.1371/journal.pone.0254634 -
Journal of the European Academy of... Jun 2023Adverse pregnancy outcomes (APO) occur in 35% of patients with pemphigoid gestationis (PG). No biological predictor of APO has been established yet.
BACKGROUND
Adverse pregnancy outcomes (APO) occur in 35% of patients with pemphigoid gestationis (PG). No biological predictor of APO has been established yet.
OBJECTIVES
To assess a potential relationship between the occurrence of APO and the serum value of anti-BP180 antibodies at the time of PG diagnosis.
METHODS
Multicentre retrospective study conducted from January 2009 to December 2019 in 35 secondary and tertiary care centres.
INCLUSION CRITERIA
(i) diagnosis of PG according to clinical, histological and immunological criteria, (ii) ELISA measurement of anti-BP180 IgG antibodies determined at the time of PG diagnosis with the same commercial kit and (iii) obstetrical data available.
RESULTS
Of the 95 patients with PG included, 42 had one or more APO, which mainly corresponded to preterm birth (n = 26), intrauterine growth restriction (IUGR) (n = 18) and small weight for gestational age at birth (n = 16). From a ROC curve, we identified a threshold of 150 IU ELISA value as the most discriminating to differentiate between patients with or without IUGR, with 78% sensitivity, 55% specificity, 30% positive and 91% negative predictive value. The threshold >150 IU was confirmed using a cross-validation based on bootstrap resampling, which showed that the median threshold was 159 IU. Upon adjusting for oral corticosteroid intake and main clinical predictors of APO, an ELISA value of >150 IU was associated with the occurrence of IUGR (OR = 5.11; 95% CI: 1.48-22.30; p = 0.016) but not with any other APO. The combination of blisters and ELISA values higher than 150 IU led to a 2.4-fold higher risk of all-cause APO (OR: 10.90; 95% CI: 2.33-82.3) relative to patients with blisters but lower values of anti-BP180 antibodies (OR of 4.54; 95% CI 0.92-34.2).
CONCLUSION
These findings suggest that anti-BP180 antibody ELISA value in combination with clinical markers is helpful in managing the risk of APO, in particular IUGR, in patients with PG.
Topics: Pregnancy; Female; Humans; Infant, Newborn; Pemphigoid Gestationis; Retrospective Studies; Pemphigoid, Bullous; Blister; Pregnancy Outcome; Non-Fibrillar Collagens; Premature Birth; Enzyme-Linked Immunosorbent Assay; Immunoglobulin G; Autoantigens; Autoantibodies
PubMed: 36802102
DOI: 10.1111/jdv.18973 -
Dermatopathology (Basel, Switzerland) Jun 2022Auto-immune reactions, including auto-immune bullous disease, have been reported following SARS-CoV-2 virus vaccination. Few cases of bullous pemphigoid are described,...
Auto-immune reactions, including auto-immune bullous disease, have been reported following SARS-CoV-2 virus vaccination. Few cases of bullous pemphigoid are described, but there has been no case of pemphigoid gestationis. We report the first case here.
PubMed: 35892478
DOI: 10.3390/dermatopathology9030025 -
Pediatric Dermatology Nov 2021Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by urticarial plaques and/or vesicles and tense bullae. A unique presentation of BP can occur...
Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by urticarial plaques and/or vesicles and tense bullae. A unique presentation of BP can occur during pregnancy, the postpartum period after delivery, or with the initiation of contraception, in which case it is referred to as pemphigoid gestationis (PG). In rare instances, newborns born to mothers with PG may also present with blisters due to transplacental passage of maternal anti-bullous pemphigoid 180 (BP180) or 230 (BP230) immunoglobulin G (IgG). In this report, we present an unusual case of neonatal PG in an infant born to an asymptomatic mother without a previous diagnosis of PG.
Topics: Female; Humans; Infant, Newborn; Mothers; Pemphigoid Gestationis; Pregnancy; Rare Diseases
PubMed: 34713489
DOI: 10.1111/pde.14849 -
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 -
Italian Journal of Dermatology and... Apr 2023Autoimmune bullous diseases (AIBDs) are rare organ-specific diseases characterized by the appearance of blisters and erosions on the skin and mucous membranes. These...
Autoimmune bullous diseases (AIBDs) are rare organ-specific diseases characterized by the appearance of blisters and erosions on the skin and mucous membranes. These dermatoses are marked by the development of autoantibodies targeting the autoantigens located in intercellular junctions, i.e., between keratinocytes or in the basement membrane area. Therefore, the fundamental division of AIBDs into the pemphigus and pemphigoid groups exists. Although AIBDs are uncommon in the general population, their overall incidence is somewhat higher in women of all ages, for which a pregnant women can be likely affected too. While the pemphigoid gestationis is exclusive bullous dermatosis of pregnancy, the other AIBDs can also start or worsen during this period. The appearance of AIBDs in childbearing women is a particularly sensitive situation requiring exceptional clinicians' caution due to the possibility of pregnancy complications with adverse effects and risks to the mother and the child. Also, there are numerous management difficulties in the period of pregnancy and lactation related to the drugs' choice and safety. This paper aimed to outline the pathophysiologic mechanisms, clinical manifestations, diagnostic approach and therapy of the most commonly recognized AIBDs in pregnancy.
Topics: Child; Female; Humans; Pregnancy; Autoimmune Diseases; Skin Diseases, Vesiculobullous; Pemphigus; Pemphigoid, Bullous; Autoantibodies
PubMed: 37153944
DOI: 10.23736/S2784-8671.23.07553-9