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International Journal of Women's... Oct 2022
PubMed: 35919284
DOI: 10.1097/JW9.0000000000000026 -
Clinics in Dermatology 2020Rituximab is a monoclonal antibody targeting CD20 on B cells with proven efficacy for pemphigus vulgaris, now an FDA-approved indication. Other autoimmune bullous... (Review)
Review
Rituximab is a monoclonal antibody targeting CD20 on B cells with proven efficacy for pemphigus vulgaris, now an FDA-approved indication. Other autoimmune bullous diseases can be challenging to treat and have significant associated morbidity and mortality, but data supporting the use of rituximab in pemphigoid group diseases remain limited. Although rituximab demonstrates efficacy for clinical improvement and remission in pemphigoid, concern for adverse events may also limit the use of this medication. We review the current evidence fo rthe use of rituximab in pemphigoid diseases, pertinent dosing schedules and laboratory monitoring, and the associated common and rare adverse events. Review of the literature to date not only supports consideration of rituximab for treatment of refractory pemphigoid group diseases but also reflects tolerability and an acceptable safety profile.
Topics: Autoimmunity; Drug Administration Routes; Drug Monitoring; Female; Humans; Leukoencephalopathy, Progressive Multifocal; Male; Pemphigoid Gestationis; Pemphigoid, Benign Mucous Membrane; Pemphigoid, Bullous; Pemphigus; Pregnancy; Risk; Rituximab
PubMed: 32563354
DOI: 10.1016/j.clindermatol.2019.07.023 -
The Journal of Pediatrics Aug 2021
Topics: Conservative Treatment; Exanthema; Female; Humans; Infant, Newborn; Infectious Disease Transmission, Vertical; Male; Pemphigoid Gestationis; Pregnancy
PubMed: 33887331
DOI: 10.1016/j.jpeds.2021.04.017 -
Journal of the European Academy of... Sep 2019Autoimmune Blistering Diseases of the Pemphigoid type is characterised by sub-epidermal blisters (SEB) with circulating autoantibodies against components of the basement... (Review)
Review
Autoimmune Blistering Diseases of the Pemphigoid type is characterised by sub-epidermal blisters (SEB) with circulating autoantibodies against components of the basement membrane zone (BMZ). The main disorders to date include bullous pemphigoid (BP), pemphigoid gestationis, mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), linear IgA disease (LABD), dermatitis herpetiformis (DH), lichen planus pemphigoides and bullous lupus. This is in contrast to pemphigus and related disorders, which demonstrate intraepidermal acantholysis and a positive Nikolsky sign. The classification and management is based on clinical, histological and direct and indirect immunofluorescence findings. There are, however, overlapping clinical and histological features between the conditions and clinical heterogeneity within each disease.
Topics: Autoimmune Diseases; Humans; Skin Diseases, Vesiculobullous
PubMed: 31087464
DOI: 10.1111/jdv.15679 -
Clinical and Experimental Dermatology Dec 2021
Topics: Adult; Antibodies, Monoclonal, Humanized; Female; Humans; Interleukin-4 Receptor alpha Subunit; Pemphigoid Gestationis; Postpartum Period; Pregnancy
PubMed: 34048080
DOI: 10.1111/ced.14765 -
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 -
Clinical, Cosmetic and Investigational... 2021Pemphigoid gestationis (PG) is a rare autoimmune bullous skin disorder which usually presents with intense pruritus and urticarial lesions that may evolve into vesicles...
Pemphigoid gestationis (PG) is a rare autoimmune bullous skin disorder which usually presents with intense pruritus and urticarial lesions that may evolve into vesicles and tense blisters. In majority of patients, it starts in the second or third trimester of pregnancy and resolves spontaneously after delivery. Lesions appear in the periumbilical area in 90% of patients and rapidly spread centrifugally to other parts of the body. The diagnosis needs to be confirmed by direct immunofluorescence test (DIF) with indirect immunofluorescence test (IIF), ELISA and immunoblot techniques playing role in diagnosis and/or monitoring antibodies level. Mild symptoms of PG can be treated with topical therapy only, but in severe course of the disease the treatment may be escalated to oral corticosteroids. We present an unusual case of PG started 2 weeks after delivery with an updated overview on the epidemiology, pathology, clinical picture, treatment, and complications of the disease.
PubMed: 34168478
DOI: 10.2147/CCID.S297520 -
Journal of Cutaneous Pathology Apr 2023Pemphigoid gestationis (PG) and polymorphic eruption of pregnancy (PEP) are pregnancy-related dermatoses. Definitive diagnosis often relies upon histopathology and...
BACKGROUND
Pemphigoid gestationis (PG) and polymorphic eruption of pregnancy (PEP) are pregnancy-related dermatoses. Definitive diagnosis often relies upon histopathology and direct immunofluorescence (DIF). PG is associated with fetal and neonatal risks, while PEP confers minimal risk.
OBJECTIVE
We aimed to compare histopathologic features to determine key differentiators.
METHODS
A retrospective cohort study of PG and PEP cases, with accompanying DIF, conducted from 1995 to 2020. Skin biopsies were examined independently in a blinded fashion by two dermatopathologists for a list of histopathological features.
RESULTS
Twenty-one cases of PG and 10 cases of PEP were identified. PG had significantly denser eosinophils than PEP (mean 155 vs. 48 cells/5 hpf; p < 0.018). PG was also noted to have eosinophilic spongiosis and eosinophils at the dermal-epidermal junction more frequently compared to PEP (80% PG vs. 10% PEP; p < 0.001). A mean cutoff value of 86 eosinophils and a mean optimal sensitivity and specificity of 81% and 83%, respectively, for eosinophils density's diagnostic power of PEP versus PG were achieved. Subepithelial separation was exclusively seen in PG (40% vs. 0%; p < 0.007).
CONCLUSION
Eosinophilic spongiosis, eosinophilic epitheliotropism, and dense superficial dermal eosinophils were diagnostic of PG. Given overlapping clinicopathologic features, however, DIF results with clinicopathologic correlation, remain the gold standard.
Topics: Pregnancy; Female; Infant, Newborn; Humans; Pemphigoid Gestationis; Retrospective Studies; Pregnancy Complications; Pruritus; Skin Diseases; Exanthema; Autoimmune Diseases
PubMed: 36357351
DOI: 10.1111/cup.14358 -
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 -
American Journal of Clinical Dermatology Aug 2020Autoimmune blistering disease management can be challenging as treatment modalities vary greatly and no single standard of care exists. We consolidated the... (Comparative Study)
Comparative Study Review
A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita.
Autoimmune blistering disease management can be challenging as treatment modalities vary greatly and no single standard of care exists. We consolidated the recommendations of international management guidelines in order to provide optimal management suggestions to physicians. A comprehensive literature search in PubMed/MEDLINE for published blistering disease management guidelines and consensus statements was conducted in November 2019. Search terms included "guideline or guidelines" or "consensus" and "pemphigoid" or "autoimmune blistering disease" or "epidermolysis bullosa acquisita". We included guidelines from established dermatologic societies and expert consensus groups. We excluded literature reviews, guidelines established by an association without dermatologists, or those specific to a single treatment. Guidelines in all languages were considered. Eleven guidelines from dermatologic associations and consensus groups meeting our inclusion criteria were selected. Several differences between recommendations, most notably when to introduce adjuvants for refractory disease, were found in bullous pemphigoid. In mucous membrane pemphigoid, treatment was directed to the sites involved and managed with systemic corticosteroids and immunosuppressants/biologics. There was no universal consensus on the first-line treatment for epidermolysis bullosa acquisita, but a combination of immunosuppressive, anti-inflammatory, and anti-neutrophil therapy was utilized. Comparison of the management guidelines revealed underrepresentation of guidelines from developing nations and key differences between the management styles among dermatologists from Europe and Asia. We attribute these discrepancies to the time elapsed between guidelines, regional differences, and demands of the local healthcare systems.
Topics: Administration, Oral; Administration, Topical; Anti-Inflammatory Agents; Biological Products; Consensus; Dermatologic Agents; Dermatology; Drug Resistance; Epidermolysis Bullosa Acquisita; Female; Humans; Immunosuppressive Agents; Pemphigoid Gestationis; Pemphigoid, Benign Mucous Membrane; Pemphigoid, Bullous; Practice Guidelines as Topic; Pregnancy; Severity of Illness Index; Treatment Outcome
PubMed: 32180161
DOI: 10.1007/s40257-020-00513-3