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American Journal of Clinical Dermatology Mar 2022Pruritus in pregnancy is a common and burdensome symptom that may be a first sign of a pregnancy-specific pruritic disease (atopic eruption of pregnancy, polymorphic... (Review)
Review
Pruritus in pregnancy is a common and burdensome symptom that may be a first sign of a pregnancy-specific pruritic disease (atopic eruption of pregnancy, polymorphic eruption of pregnancy, pemphigoid gestationis, and intrahepatic cholestasis in pregnancy) or a dermatosis coinciding with pregnancy by chance. Despite its high prevalence, pruritus is often underrated by physicians, and data regarding the safety profiles of drugs for pruritus are very limited. In this review, we illustrate the epidemiology, possible pathophysiology, clinical characteristics, and diagnostic workup of various pregnancy-related diseases and discuss antipruritic treatments. The prevalence of pruritus in pregnancy demonstrates the importance of symptom recognition and the need for an holistic approach, taking into account both the potential benefits for the patient and the potential risks to the fetus.
Topics: Cholestasis, Intrahepatic; Exanthema; Female; Humans; Pemphigoid Gestationis; Pregnancy; Pregnancy Complications; Pruritus
PubMed: 35191007
DOI: 10.1007/s40257-021-00668-7 -
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 -
International Journal of Women's... Oct 2022
PubMed: 35919284
DOI: 10.1097/JW9.0000000000000026 -
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 -
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 -
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 -
Experimental and Therapeutic Medicine Jan 2022Pemphigoid gestationis is considered to be a rare pregnancy exclusive bullous disease, which modifies the course of the pregnancy, with difficulties in the management of...
Pemphigoid gestationis is considered to be a rare pregnancy exclusive bullous disease, which modifies the course of the pregnancy, with difficulties in the management of the pruritus and skin lesions as well as a possible change in the neonatal outcome. Differential diagnosis of skin lesions and pruritus in pregnancy is challenging, and complementary investigations such as skin biopsy or laboratory tests are indispensable. The correct diagnosis and proper treatment could change the natural course of a pregnancy at risk and could improve maternal and fetal morbidity. We present the case of a patient with pemfigoid gestationis with the aim to highlight: i) the management of this pregnancy-associated skin disorder which transfers this pregnancy into a category of high obstetrical risk pregnancy; ii) the particularities of the course of the pregnancy; and iii) the importance in the differential diagnosis of pregnancy dermatoses. The particularity of this case of pemphigoid gestationis was the acute fetal distress in the absence of intrauterine growth restriction that is frequently found in this pathology, and the management of a rare pregnancy skin condition that currently has no standard treatment.
PubMed: 34815775
DOI: 10.3892/etm.2021.10945