-
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 -
Frontiers in Immunology 2022Pemphigoid diseases (PD) are autoimmune skin blistering diseases characterized by autoantibodies directed against proteins of the cutaneous basement membrane zone (BMZ).... (Review)
Review
Pemphigoid diseases (PD) are autoimmune skin blistering diseases characterized by autoantibodies directed against proteins of the cutaneous basement membrane zone (BMZ). One of the major antigens is type XVII collagen (BP180), a transmembrane glycoprotein, which is targeted in four PDs: bullous pemphigoid, mucous membrane pemphigoid, linear IgA dermatosis, and pemphigoid gestationis. To date, different epitopes on BP180 have been described to be recognized by PD disease patients' autoantibodies. Different BP180 epitopes were associated with distinct clinical phenotypes while the underlying mechanisms are not yet fully understood. So far, the main effects of anti-BP180 reactivity are mediated by Fcγ-receptors on immune cells. More precisely, the autoantibody-antigen interaction leads to activation of complement at the BMZ and infiltration of immune cells into the upper dermis and, by the release of specific enzymes and reactive oxygen species, to the degradation of BP180 and other BMZ components, finally manifesting as blisters and erosions. On the other hand, inflammatory responses independent of Fcγ-receptors have also been reported, including the release of proinflammatory cytokines and internalization and depletion of BP180. Autoantibodies against BP180 can also be found in patients with neurological diseases. The assumption that the clinical expression of PD depends on epitope specificity in addition to target antigens, autoantibody isotypes, and antibody glycosylation is supported by the observation that epitopes of PD patients differ from those of PD patients. The aim of the present review is to describe the fine specificities of anti-BP180 autoantibodies in different PDs and highlight the associated clinical differences. Furthermore, the direct effects after binding of the autoantibodies to their target are summarized.
Topics: Autoantibodies; Autoantigens; Autoimmune Diseases; Carrier Proteins; Complement System Proteins; Epitopes; Humans; Immunoblotting; Nervous System Diseases; Non-Fibrillar Collagens; Pemphigoid, Bullous; Receptors, IgG; Collagen Type XVII
PubMed: 36032160
DOI: 10.3389/fimmu.2022.948108 -
Human Pathology Oct 2023Pathologists provide valuable input in the dermatological care of pregnant patients in various contexts. This article provides dermatopathology updates on cutaneous...
Pathologists provide valuable input in the dermatological care of pregnant patients in various contexts. This article provides dermatopathology updates on cutaneous changes associated with pregnancy, organized based on the following classification system: physiological skin changes in pregnancy, specific dermatoses of pregnancy, dermatoses modified in pregnancy, and skin neoplasms in pregnancy. Awareness of the impact of pregnancy on the skin by pathologists is important, as this is an opportunity to contribute to diagnostic precision in this patient population.
PubMed: 37209919
DOI: 10.1016/j.humpath.2023.05.005 -
Journal of the European Academy of... Oct 2021Apart from bullous pemphigoid (BP), the association of other autoimmune bullous diseases (AIBDs) with neurological conditions is poorly understood.
BACKGROUND
Apart from bullous pemphigoid (BP), the association of other autoimmune bullous diseases (AIBDs) with neurological conditions is poorly understood.
OBJECTIVE
To estimate the association between a wide array of AIBDs and neurological conditions.
METHODS
A retrospective cross-sectional study recruited patients with BP, mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), pemphigoid gestationis (PG), pemphigus vulgaris (PV) and pemphigus foliaceus (PF). These patients were compared with their age- and sex-matched control subjects with regard to the lifetime prevalence of Parkinson's disease (PD), Alzheimer's disease (AD), stroke, epilepsy and multiple sclerosis (MS). Logistic regression was used to calculate OR for specified neurological disorders.
RESULTS
The current study included 1743, 251, 106, 126, 860 and 103 patients diagnosed with BP, MMP, EBA, PG, PV and PF, respectively. These patients were compared with 10 141, 1386, 606, 933, 5142 and 588 matched controls, respectively. Out of the investigated neurological conditions, PD associated with BP (OR, 2.71; 95% CI, 2.19-3.35); AD with BP (OR, 2.11; 95% CI, 1.73-2.57), MMP (OR, 2.37; 95% CI, 1.03-5.47), EBA (OR, 6.00; 95% CI, 1.90-18.97) and PV (OR, 2.24; 95% CI, 1.40-3.60); stroke with BP (OR, 1.84; 95% CI, 1.55-2.19) and EBA (OR, 2.79; 95% CI, 1.11-7.01); and epilepsy with BP (OR, 2.18; 95% CI, 1.72-2.77) and PV (OR, 1.80; 95% CI, 1.19-2.73). MS did not significantly cluster with any of the six AIBDs.
CONCLUSION
In addition to BP, EBA and PV were found to cluster with neurological comorbidities. Patients with these AIBDs with compatible symptoms may be carefully assessed for comorbid neurological disorders.
Topics: Autoimmune Diseases; Cross-Sectional Studies; Epidermolysis Bullosa Acquisita; Humans; Retrospective Studies; Skin Diseases, Vesiculobullous
PubMed: 34153122
DOI: 10.1111/jdv.17465 -
Annals of Emergency Medicine Feb 2020
Topics: Adult; Biopsy; Exanthema; Female; Humans; Pemphigoid Gestationis; Pregnancy
PubMed: 31959312
DOI: 10.1016/j.annemergmed.2019.08.421 -
Australian Journal of General Practice Dec 2021Women with rashes or itchy skin during pregnancy will often present initially to the general practitioner. Knowledge of the specific dermatoses of pregnancy...
BACKGROUND
Women with rashes or itchy skin during pregnancy will often present initially to the general practitioner. Knowledge of the specific dermatoses of pregnancy will assist in diagnosis, management and, importantly, facilitation of timely escalation of care of conditions that can potentially affect the fetus.
OBJECTIVE
The aim of this article is to provide a diagnostic framework for approaching a pruritic rash during pregnancy as well as a helpful summary of management of pregnancy-specific dermatoses. It will assist clinicians in the identification of specific dermatoses that pose fetal risks.
DISCUSSION
In addition to considering non-pregnancy specific conditions when approaching pruritus or a pruritic rash in pregnancy, it is important that clinicians also consider pregnancy-specific dermatoses, which have been reclassified into four categories: polymorphic eruption of pregnancy, pemphigoid gestationis, intrahepatic cholestasis of pregnancy (ICP) and atopic eruption of pregnancy. Unlike the other dermatoses, ICP begins with pruritus, and skin changes are secondary. ICP and pemphigoid gestationis are associated with fetal risks such as prematurity and stillbirth.
Topics: Cholestasis, Intrahepatic; Female; Humans; Pemphigoid Gestationis; Pregnancy; Pregnancy Complications; Pruritus; Skin Diseases
PubMed: 34845477
DOI: 10.31128/AJGP-03-21-5900 -
The association of six autoimmune bullous diseases with thyroid disorders: a population-based study.Journal of the European Academy of... Oct 2022The association of autoimmune bullous diseases (AIBDs) with thyroid disorders remains to be profoundly investigated.
BACKGROUND
The association of autoimmune bullous diseases (AIBDs) with thyroid disorders remains to be profoundly investigated.
OBJECTIVE
To evaluate the epidemiological association between six AIBDs and thyroid disorders.
METHODS
A population-based cross-sectional study enrolled patients with bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), pemphigoid gestationis (PG), pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Patients with these six AIBDs were compared with six age- and sex-matched control groups regarding the prevalence of thyroiditis and hyperthyroidism. Logistic regression was used to calculate the odds ratio (OR) and 95% confidence interval (CI) for thyroid disorders.
RESULTS
The study population included 1,743, 251, 106, 126, 860 and 103 patients with BP, MMP, EBA, PG, PV and PF respectively. The corresponding control groups consisted of 10,141, 1,386, 606, 933, 5,142 and 588 matched controls respectively. A significant association was found between thyroiditis and BP (OR, 1.98; 95% CI, 1.18-3.35; P = 0.010), MMP (OR, 7.02; 95% CI, 1.87-26.33; P = 0.004) and PV (OR, 2.73; 95% CI, 1.45-5.15; P = 0.002). With regards to hyperthyroidism, PF was the only AIBD to demonstrate significant comorbidity (OR, 2.42; 95% CI, 1.13-5.21; P = 0.024). EBA and PG were not found to cluster with any of the investigated thyroid conditions.
CONCLUSION
Patients with BP, MMP, PV and PF experience an elevated burden of thyroid disorders. Patients with these AIBDs presenting with suggestive symptoms may be carefully screened for comorbid thyroid disorders.
Topics: Autoimmune Diseases; Cross-Sectional Studies; Epidermolysis Bullosa Acquisita; Humans; Hyperthyroidism; Pemphigoid, Benign Mucous Membrane; Pemphigoid, Bullous; Pemphigus; Skin Diseases, Vesiculobullous; Thyroid Diseases
PubMed: 35611551
DOI: 10.1111/jdv.18266 -
Indian Dermatology Online Journal 2020
PubMed: 33344355
DOI: 10.4103/idoj.IDOJ_93_20 -
BMJ Case Reports Mar 2021Pemphigoid gestationis is a rare autoimmune subepidermal bullous dermatosis occurring during pregnancy and post partum. A 32-year-old woman developed itchy urticarial...
Pemphigoid gestationis is a rare autoimmune subepidermal bullous dermatosis occurring during pregnancy and post partum. A 32-year-old woman developed itchy urticarial wheals over the trunk and extremities at 6 months of gestation. This was not controlled with antihistamines, and 2 months later, the patient developed multiple vesiculobullous lesions. The patient had an exacerbation 3 weeks post-delivery. She did not go into remission for 6 months post partum despite treatment with prednisolone 40 mg/day, doxycycline 100 mg two times per day and dapsone 100 mg/day. The patient went into remission following treatment with three courses of intravenous immunoglobulin 2 mg/kg/course and 2 doses of rituximab 1 g at a 2-week interval.
Topics: Adult; Female; Humans; Immunoglobulins, Intravenous; Pemphigoid Gestationis; Postpartum Period; Pregnancy; Rituximab; Skin Diseases, Vesiculobullous
PubMed: 33727302
DOI: 10.1136/bcr-2020-241496