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BMC Veterinary Research Feb 2023Autoimmune subepidermal blistering diseases (AISBDs) are rare skin disorders of animals that were first identified in dogs but several AISBDs are now recognised in other... (Review)
Review
Autoimmune subepidermal blistering diseases (AISBDs) are rare skin disorders of animals that were first identified in dogs but several AISBDs are now recognised in other companion animal species. Most AISBDs in animals are homologues of the human diseases and are thought to share similar pathomechanisms of epidermal and/or mucosal blister formation caused by autoantibodies targeting structural proteins of the basement membrane zone (BMZ). Disruption of their structural function by the autoantibodies and/or recruited inflammation leads to BMZ fragility, which presents clinically as vesicles, bullae and, later, deep erosions and ulcers. Canine AISBDs are the best characterised, particularly the more common variants such as mucous membrane pemphigoid (48%), epidermolysis bullosa acquisita (EBA) (26%), and bullous pemphigoid (10%). Exceedingly rare AISBDs in the dog are junctional EBA, mixed AISBD, type-1 bullous systemic lupus erythematosus, linear IgA dermatosis, and pemphigus gestationis. The diagnosis of a specific AISBD is made by combining the clinical features (breed, age, lesion distribution) with histological evidence of subepithelial clefting, but not all AISBDs can be differentiated in this manner and specialised immunological testing is required. This latter, unfortunately, is not readily available and, therefore, the specific AISBD diagnosis often remains unconfirmed. While this limits further understanding of these diseases, it does not prevent clinicians from treating their patients, as the treatment approaches are similar for the different AISBDs in dogs. This review primarily focuses on canine AISBDs, the species for which these diseases have been best characterised, and shorter descriptions of variants in other species are also provided.
Topics: Humans; Animals; Dogs; Skin; Pemphigus; Epidermis; Autoantibodies; Breeding; Dog Diseases
PubMed: 36849885
DOI: 10.1186/s12917-023-03597-1 -
Frontiers in Immunology 2019Lichen planus pemphigoides (LPP) is a very rare autoimmune sub-epidermal blistering disease associated with lichenoid skin changes. Initially thought to be a mere... (Review)
Review
Lichen planus pemphigoides (LPP) is a very rare autoimmune sub-epidermal blistering disease associated with lichenoid skin changes. Initially thought to be a mere variant of more common inflammatory dermatoses, particularly Bullous Pemphigoid (BP) or Lichen Planus (LP), a growing body of evidence suggests that it is a disease entity in its own right. In common with a range of autoimmune blistering diseases, including BP, pemphigoid gestationis (PG), mucous membrane pemphigoid (MMP) and linear IgA dermatosis (LAD), a key feature of the disease is the development of autoantibodies against type XVII collagen (COL17). However, accurately establishing the diagnosis is dependent on a careful correlation between the clinical, histological and immunological features of the disease. Therefore, we present an up to date summary of the epidemiology and etiopathogenesis of LPP, before illustrating the predisposing and precipitating factors implicated in the development of the disease. In addition to a selective literature search, we compare reports of potential drug-induced cases of LPP with pharmacovigilance data available via OpenVigil. We subsequently outline the cardinal clinical features, important differential diagnoses and current treatment options. We conclude by demonstrating that an improved understanding of LPP may not only lead to the development of novel treatment strategies for the disease itself, but may also shed new light on the pathophysiology of more common and treatment-refractory autoimmune blistering diseases.
Topics: Adolescent; Adrenal Cortex Hormones; Adult; Aged; Aged, 80 and over; Autoantibodies; Autoantigens; Blister; Child; Child, Preschool; Diagnosis, Differential; Female; Humans; Lichen Planus; Male; Middle Aged; Non-Fibrillar Collagens; Pemphigoid, Bullous; Young Adult; Collagen Type XVII
PubMed: 31312198
DOI: 10.3389/fimmu.2019.01389 -
Frontiers in Medicine 2020Treatment regimens for pemphigoid gestationis (PG) are non-standardized, with most evidence derived from individual case reports or small series. To systematically...
Treatment regimens for pemphigoid gestationis (PG) are non-standardized, with most evidence derived from individual case reports or small series. To systematically review current literature on treatments and clinical outcomes of PG and to establish recommendations on its therapeutic management. An a priori protocol was designed based on PRISMA guidelines. PubMed, Scopus, and Web of Science databases were searched for English-language articles detailing PG treatments and clinical outcomes, published between 1970 and March 2020. In total, 109 articles including 140 PG patients were analyzed. No randomized controlled trials or robust observational studies detailing PG treatment were found. Systemic corticosteroids ± topical corticosteroids and/or antihistamines were the most frequently prescribed treatment modality ( = 74/137; 54%). Complete remission was achieved by 114/136 (83.8%) patients. Sixty-four patients (45.7%) were given more than one treatment modality due to side effects or ineffectiveness. Leaving aside topical corticosteroids as monotherapy ± antihistamines in patients with mild disease, systemic corticosteroids ± topical corticosteroids and/or antihistamines led to complete remission in the highest proportion of patients (83%), while steroid-sparing treatments ± topical corticosteroids and/or antihistamines were associated with the lowest proportion of flares (55.5%). The review has been drafted based on a limited number of single case reports and small case series. Underreporting/underdiagnosis of patients with mild-to-moderate PG, partial/absent follow-up, absence of precise description of neonatal outcomes and lack of validated objective scores for measuring disease severity are other limitations of our study. Our systematic review was affected by publication bias. Systemic corticosteroids are the most frequently used treatment for PG. Whilst most patients achieve complete remission, many of them have refractory/persistent disease requiring multiple lines of therapy. Therefore, we provided an algorithm for PG treatment integrating the results of this systematic review with current knowledge available for bullous pemphigoid. High-quality studies will further help assess the effectiveness of different treatment options for PG.
PubMed: 33330568
DOI: 10.3389/fmed.2020.604945 -
Clinics (Sao Paulo, Brazil) 2009Pemphigoid gestationis, also known as herpes gestationis, is a rare autoimmune blistering disease associated with pregnancy. It usually occurs during the second or third...
INTRODUCTION
Pemphigoid gestationis, also known as herpes gestationis, is a rare autoimmune blistering disease associated with pregnancy. It usually occurs during the second or third trimester, but it may be present at any stage of pregnancy or the puerperium. The clinical, histologic, and immunopathological features of pemphigoid gestationis are similar to those of the pemphigoid group of disorders.
METHODS
We hereby report seven patients who were diagnosed with pemphigoid gestationis and followed at the Autoimmune Blistering Disease Clinic in the Department of Dermatology of the University of Sao Paulo Medical School between 1996 and 2008.
DISCUSSION
Demographic and clinical findings, such as median age, sites of involvement, and gestational age of onset or C3 of our patients, coincide with those described in previous reports. The majority of patients (85%) exhibited complement C(3) and immunoglobulin G (IgG) deposition along the basement membrane zone (BMZ) on immunofluorescence. Herpes gestationis factor (HG) factor was postitive in four out of six patients (67%), and three out of five patients recognized the bullous pemphigoid recombinant antigen (BP180) by ELISA.
CONCLUSION
This study revealed a good outcome of the newborns from pemphigoid gestationis affected mothers, based on the absence of pemphigoid gestationis cutaneous lesions, mean birth weight, and normal Apgar scores and gestational age at birth.
Topics: Adult; Complement C3; Female; Humans; Immunoglobulin G; Infant, Newborn; Pemphigoid Gestationis; Pemphigoid, Bullous; Pregnancy; Pregnancy Outcome; Pruritus; Urticaria; Young Adult
PubMed: 19936176
DOI: 10.1590/S1807-59322009001100002 -
American Family Physician Jan 2007Common skin conditions during pregnancy generally can be separated into three categories: hormone-related, preexisting, and pregnancy-specific. Normal hormone changes... (Review)
Review
Common skin conditions during pregnancy generally can be separated into three categories: hormone-related, preexisting, and pregnancy-specific. Normal hormone changes during pregnancy may cause benign skin conditions including striae gravidarum (stretch marks); hyperpigmentation (e.g., melasma); and hair, nail, and vascular changes. Preexisting skin conditions (e.g., atopic dermatitis, psoriasis, fungal infections, cutaneous tumors) may change during pregnancy. Pregnancy-specific skin conditions include pruritic urticarial papules and plaques of pregnancy, prurigo of pregnancy, intrahepatic cholestasis of pregnancy, pemphigoid gestationis, impetigo herpetiformis, and pruritic folliculitis of pregnancy. Pruritic urticarial papules and plaques of pregnancy are the most common of these disorders. Most skin conditions resolve postpartum and only require symptomatic treatment. However, there are specific treatments for some conditions (e.g., melasma, intrahepatic cholestasis of pregnancy, impetigo herpetiformis, pruritic folliculitis of pregnancy). Antepartum surveillance is recommended for patients with intrahepatic cholestasis of pregnancy, impetigo herpetiformis, and pemphigoid gestationis.
Topics: Female; Humans; Pregnancy; Pregnancy Complications; Skin; Skin Diseases
PubMed: 17263216
DOI: No ID Found -
Journal of Obstetrics and Gynaecology... Dec 2014
PubMed: 25404804
DOI: 10.1007/s13224-013-0432-0 -
Frontiers in Pharmacology 2020Pregnancy may induce the onset or exacerbation of autoimmune bullous diseases such as pemphigus or pemphigoid gestationis. A shift toward T helper (Th) 2 immune response... (Review)
Review
Pregnancy may induce the onset or exacerbation of autoimmune bullous diseases such as pemphigus or pemphigoid gestationis. A shift toward T helper (Th) 2 immune response and the influence of hormonal changes have been evoked as possible triggering factors. Therapeutic management of this setting of patients may represent a challenge, mainly due to safety concerns of some immunosuppressive drugs during pregnancy and lactation. In this narrative review, we provided a comprehensive overview of the therapeutic management of autoimmune bullous diseases in pregnant and breastfeeding women, focusing on pemphigus and pemphigoid gestationis.
PubMed: 33117178
DOI: 10.3389/fphar.2020.583354 -
JAAD Case Reports Nov 2023
PubMed: 37842158
DOI: 10.1016/j.jdcr.2023.08.013 -
Journal of Obstetrics and Gynaecology... Dec 2020Pemphigoid gestationis is a rare subepidermal bullous dermatosis of pregnancy, caused by the interaction of IgG1 auto-antibodies with 180 kD BP Antigen 2. This disease...
Pemphigoid gestationis is a rare subepidermal bullous dermatosis of pregnancy, caused by the interaction of IgG1 auto-antibodies with 180 kD BP Antigen 2. This disease can lead to preterm delivery, but the neonate is affected in only 10% cases. The treatment of choice in pemphigoid gestationis is systemic corticosteroids.
PubMed: 33417630
DOI: 10.1007/s13224-019-01302-7 -
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