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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 -
Journal of Obstetrics and Gynaecology... Dec 2014
PubMed: 25404804
DOI: 10.1007/s13224-013-0432-0 -
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 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 Mar 2023
PubMed: 36915856
DOI: 10.1016/j.jdcr.2023.01.026 -
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 -
Cureus Jan 2020Bullous pemphigoid is an autoimmune blistering disorder that typically presents in elderly patients as pruritic tense subepidermal blisters on the lower trunk, axilla,...
Bullous pemphigoid is an autoimmune blistering disorder that typically presents in elderly patients as pruritic tense subepidermal blisters on the lower trunk, axilla, and groin. It is caused by circulating and tissue-bound autoantibodies directed against bullous pemphigoid antigen 1 or bullous pemphigoid antigen 2 or both. Dyshidrosiform bullous pemphigoid is a rare variant of bullous pemphigoid, and it usually presents as itchy, potentially hemorrhagic, or purpuric blisters on the palms and/or soles of elderly individuals; subsequently, typical bullous lesions of bullous pemphigoid appear on other body sites. In our study, we report the features of two men with dyshidrosiform bullous pemphigoid and review the characteristics of individuals with this rare subtype of bullous pemphigoid. Including the men whose condition is described in this paper, at least 72 patients with dyshidrosiform bullous pemphigoid have been reported so far. However, complete features of the condition have not been described for all of the individuals. Based on the cases reported so far, the condition was slightly more common in women and the onset of the disease, for most of the patients, occurred between the ages of 61 and 94 years. The patients usually presented with blisters on both their palms and soles (66%) or just their soles (31%); 77% of the patients had progression of bullous pemphigoid to other areas of their body. Whether hemorrhagic blisters or purpuric lesions are associated with dyshidrosiform bullous pemphigoid remains to be determined; these features were present in 91% of the 22 patients who were described in the case reports yet were only observed in 5% of the individuals from a single larger series of 20 patients. The mainstay of therapy for dyshidrosiform bullous pemphigoid is systemic corticosteroids, with or without topical corticosteroids, and/or systemic dapsone or immunosuppressants; nearly all of the patients showed improvement after the treatment was initiated. Similar to individuals with bullous pemphigoid, at least nine of the dyshidrosiform bullous pemphigoid patients, including both patients in this report, had either a neurologic condition (seven patients) or both a neurologic condition and a psychiatric disorder (two patients). Usually, an autoimmune bullous disease, particularly dyshidrosiform bullous pemphigoid, is not initially considered in patients who present with blisters restricted to the palms and/or soles. Indeed, the lesion morphology of dyshidrosiform bullous pemphigoid mimics several other conditions that are characterized by blisters on the hands and feet, such as allergic and irritant contact dermatitis, chronic bullous disease of childhood, cutaneous T-cell lymphoma, dermatophyte infection, dyshidrosis or pompholyx, epidermolysis bullosa acquisita, erythema multiforme, herpes gestationis, lichen planus, linear IgA disease, scabies, and systemic contact dermatitis. In conclusion, the possibility of dyshidrosiform bullous pemphigoid should be considered in elderly individuals who present with the new onset of palmar and/or plantar blisters that are either recurrent or recalcitrant to therapy or would subsequently also appear on other areas of the body.
PubMed: 32064205
DOI: 10.7759/cureus.6630 -
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 -
International Journal of Women's... Jun 2017
PubMed: 28560301
DOI: 10.1016/j.ijwd.2016.11.004 -
World Journal of Clinical Cases Dec 2021Pemphigoid gestationis (PG) is a rare autoimmune blistering disease that usually presents in the second or third trimester, with an incidence of 1 per 50000 pregnancies....
BACKGROUND
Pemphigoid gestationis (PG) is a rare autoimmune blistering disease that usually presents in the second or third trimester, with an incidence of 1 per 50000 pregnancies. PG tends to recur with an earlier onset and a more severe course in subsequent pregnancies. Skin biopsy markers can be confirmed by direct immunofluorescence staining.
CASE SUMMARY
Our patient was diagnosed with PG at 8 mo of gestation with fresh bullous lesion marks on the abdomen and limbs. Termination of the pregnancy was performed by cesarean section at 37 + 4 wk of gestation. The patient delivered an infant weighing 3620 gm. The infant had urticaria-like and vesicular skin lesions and was diagnosed with PG. The patient was discharged on prednisolone and in a satisfactory condition. The infant was discharged after anti-inflammatory therapy for one week.
CONCLUSION
PG is a rarely reported disease, and 10% of newborns develop mild clinical symptoms consisting of urticaria-like or vesicular skin lesions. We intend to remind clinicians to consider this condition when a patient presents with such lesions so that treatment can be started early and neonatal morbidity can be taken into account.
PubMed: 35004996
DOI: 10.12998/wjcc.v9.i34.10645