Pemphigus vulgaris in a neonate

Amer, YousefBin; Ajroush, Waleed Al

doi:10.4103/0256-4947.51454

Cited by 8 publications

(5 citation statements)

References 8 publications

(19 reference statements)

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“…The clinical picture in neonatal pemphigus is not as severe compared to the disease that caused it since it is not a systemic disease. The symptoms and signs are reduced to skin lesions, and exanthematous; crusted erosions erupt as a temporary phenomenon over several weeks until the degradation of maternal autoantibodies (19)(20)(21). Neonatal pemphigus has a good prognosis (19).…”

Section: Clinical Featuresmentioning

confidence: 99%

Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review)

et al. 2021

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Pemphigus represents a group of chronic inflammatory disorders characterized by autoantibodies that target components of desmosomes, leading to the loss of intercellular adhesion between keratinocytes and causing intraepithelial blistering. The pemphigus group consists of four main clinical types with several variants: pemphigus vulgaris (with pemphigus vegetans and pemphigus herpetiformis as variants), pemphigus foliaceus, paraneoplastic pemphigus and IgA pemphigus (with two clinical variants: intraepidermal neutrophilic IgA dermatosis and subcorneal pustular dermatosis). Genetic factors are involved in the pathogenesis, with HLA-DR4 (DRB1*0402) and HLA-DRw6 (DQB1*0503) allele more common in patients with pemphigus vulgaris, HLA class II DRB1*0344 and HLA Cw*1445 correlated with paraneoplastic pemphigus, and HLA-DRB1*04:01, HLA-DRB1*04:06, HLA-DRB1*01:01, HLA-DRB1*14, associated with a higher risk of developing pemphigus foliaceus. Autoantibodies are conducted against structural desmosomal proteins in the skin and mucous membranes, mainly desmogleins, desmocollins and plakins. Cell-mediated immunity may also play a role, especially in paraneoplastic pemphigus. Patients may present erythema, blisters, erosions, and ulcers that may affect the skin, as well as mucosal surfaces of the oral cavity, eyes, nose, leading to severe complaints including pain, dysphagia, and fetor. Oral mucosal postbullous erosive lesions are frequently the first sign of disease in pemphigus vulgaris and in paraneoplastic pemphigus, without skin involvement, making the diagnosis difficult. Treatment options classically include immunosuppressive agents, such as corticosteroids and corticosteroid-sparing agents such as azathioprine, mycophenolate mofetil, cyclophosphamide, methotrexate or dapsone. Newer therapies focus on blocking cell signaling events induced by pathogenic autoantibodies and/or targeting specific autoantibodies. The disease evolution is conditioned by the treatment with maximum doses of corticosteroids and the side effects associated with long-term immunosuppressive therapy, which is why patients need a multidisciplinary approach in following the treatment. In this review, we provide a comprehensive overview of the epidemiology, pathophysiology, clinical aspect, diagnosis and management of the main intraepidermal blistering diseases from the pemphigus group.

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Section: Clinical Featuresmentioning

confidence: 99%

Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review)

et al. 2021

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“…The literature review identified 51 original cases of neonatal AIBDs from 48 full‐text papers : 46 from the English‐language literature and 5 in other languages, which were translated. There was an increase in the number of reported cases over the decades.…”

Section: Resultsmentioning

confidence: 99%

Neonatal Autoimmune Blistering Disease: A Systematic Review

Zhao

Chiang

Murrell

2016

Pediatric Dermatology

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We aimed to better understand the pathogenesis, clinical features, prognosis, and treatment of neonatal autoimmune blistering diseases (AIBDs). We searched Medline, Embase, PubMed, Latin American and Caribbean Health Sciences Literature, and reference lists of identified articles. Inclusion criteria were articles published from 1946 to December 2014 in any language. Exclusion criteria were age greater than 4 weeks and no confirmed AIBD diagnosis. We identified 51 cases of neonatal AIBDs: 34 cases of pemphigus (31 pemphigus vulgaris [PV], 3 pemphigus foliaceus [PF]) and 17 cases of pemphigoid diseases (9 bullous pemphigoid [BP], 5 linear immunoglobulin A bullous dermatosis [LABD], 1 BP and LABD, 1 epidermolysis bullosa acquisita, 1 bullous systemic lupus erythematosus). Pemphigoid diseases had a higher male predominance (male:female ratio 4.6:1) than pemphigus (male:female ratio 1:1.06) (p = 0.004). Pemphigus had a higher proportion presenting at birth (79.4%) than pemphigoid diseases (29.4%) (p = 0.008). The most common sites involved were the trunk (63.0%), followed by the head and neck (60.9%). The mucosal membranes were involved in 32.6% of cases (27.6% in pemphigus, 41.6% in pemphigoid diseases). Only 33.3% used systemic therapy, and 75.5% achieved control within 3 weeks. Most PV, PF, and BP cases, but not LABDs, reported maternal disease. In pemphigus cases, 75.0% of mothers had active disease and 25.0% were in control. Pregnant women with PV, PF, and PG of any severity can passively transfer autoantibodies leading to neonatal AIBD. Pemphigoid diseases are more likely to present after birth and may be more male predominant. The presentation of LABDs may be different from that of all other AIBDs.

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“…Predisposing factors include infantile age, infant birth weight, childbearing age, gestational hypertension, Kasabach-Merritt syndrome [50]. Diagnosis is made by history, Fine-Needle Aspiration Cytology (FNAC), MRI, and/color doppler USG, histopathology, and immunohistochemistry ruling out other vascular malformations [51]. Stage specific treatment drugs (α-interferon, propranolol, corticosteroids), surgery and lasers (CO 2 , flash lamp pulsed dye, diode) are the treatment modalities.…”

Section: Hemangiomamentioning

confidence: 99%

Oral Pathology in Paediatric Patients

Shah¹

2018

NCP

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Children and adolescents exhibit a wide spectrum of oral lesions including hard and soft tissue lesions of the oral maxillofacial region. The information regarding prevalence of pediatric oral lesions is scanty but US places the prevalence rate in 4-10% excluding infants [1,2]. These lesions include mucosal conditions, developmental anomalies, neoplastic, reactive or inflammatory lesions. Neonates displaying intraoral lesions decree detailed assessment, diagnosis, management and parental counseling in conjunction with a reassurance. This aids timely diagnosis of both usual and rare oral tissue presentation in neonates [1,2].

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Pemphigus vulgaris in a neonate

Cited by 8 publications

References 8 publications

Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review)

Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review)

Neonatal Autoimmune Blistering Disease: A Systematic Review

Oral Pathology in Paediatric Patients

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