Pênfigo Neonatal Em Filho De Mãe Com Pênfigo Vulgar: Relato De Caso

Carvalho, Adriana Amaral; Neto, Dinamar Amador dos Santos; Carvalho, Mirelle Augusta dos Reis; Eleutério, Sabrina Jeane Prates; Xavier, Alessandra Rejane Ericsson de Oliveira

doi:10.1590/1984-0462/;2019;37;1;00004

Cited by 8 publications

(7 citation statements)

References 14 publications

(18 reference statements)

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“…Neonatal pemphigus occurs in 30% to 45% of children of PV carriers, by the passage of maternal antibodies to the fetus through the placenta. 39 It is manifested by vesicles, blisters, and erosions from the moment of birth, and the involvement of mucous membranes is rare. 40 Neonatal pemphigus is transient and tends to disappear spontaneously within three weeks, as it results from the transfer of antibodies that are progressively eliminated.…”

Section: Clinical Picturementioning

confidence: 99%

Pemphigus vulgaris

Porro

Seque

Ferreira

et al. 2019

An. Bras. Dermatol.

119

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Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin. Clinical suspicion can be confirmed by cytological examination, histopathological examination, and direct and indirect immunofluorescence tests. The treatment is performed with systemic corticosteroids, and immunosuppressive drugs may be associated, among them azathioprine and mycophenolate mofetil. More severe cases may benefit from corticosteroids in the form of intravenous pulse therapy, and recent studies have shown a beneficial effect of rituximab, an anti-CD20 immunobiological drug. It is a chronic disease with mortality around 10%, and septicemia is the main cause of death. Patients need long-term and multidisciplinary follow-up.

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Section: Clinical Picturementioning

confidence: 99%

Pemphigus vulgaris

Porro

Seque

Ferreira

et al. 2019

An. Bras. Dermatol.

119

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“…Another sign that may be present is Asboe-Hansen sign also referred to as the ‘indirect Nikolsky’ or ‘Nikolsky II’ which occurs when a gentle pressure on intact bulla forces the fluid to spread under the skin away from the site of pressure -‘bulla-spread phenomenon’ ( 1 , 3 , 4-8 ). In the case of pregnant women with active pemphigus, there is a chance that the newborn could develop neonatal pemphigus as a result of the transmission of maternal IgG (consisting of autoantibodies against Dsg3) through the placenta ( 8 , 19 ). The clinical picture in neonatal pemphigus is not as severe compared to the disease that caused it since it is not a systemic disease.…”

Section: Pemphigus Vulgaris (Pv)mentioning

confidence: 99%

“…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 ( 19-21 ). Neonatal pemphigus has a good prognosis ( 19 ).…”

Section: Pemphigus Vulgaris (Pv)mentioning

confidence: 99%

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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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“…Neonatal pemphigus vulgaris (NPV) is a transient immunobullous disease induced by transplacental transfer of maternal autoantibodies from a mother with PV to her newborn (Fenniche et al, 2006;Gushi et al, 2008;Turrentine et al, 2014;Carvalho et al, 2019;Fenner et al, 2020;Foster et al, 2021). First reported in 1975(Salihbegović-Opalić et al, 1975Zhao et al, 2016), few cases exist in the literature, as female patients with pemphigus can experience infertility, premature delivery and stillbirth (Ouahes et al, 1997).…”

Section: Neonatal Pemphigus Vulgarismentioning

confidence: 99%

Autoimmune Pemphigus: Latest Advances and Emerging Therapies

Lim

Bohelay

Hanakawa

et al. 2022

Front. Mol. Biosci.

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Pemphigus represents a group of rare and severe autoimmune intra-epidermal blistering diseases affecting the skin and mucous membranes. These painful and debilitating diseases are driven by the production of autoantibodies that are mainly directed against the desmosomal adhesion proteins, desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1). The search to define underlying triggers for anti-Dsg-antibody production has revealed genetic, environmental, and possible vaccine-driven factors, but our knowledge of the processes underlying disease initiation and pathology remains incomplete. Recent studies point to an important role of T cells in supporting auto-antibody production; yet the involvement of the myeloid compartment remains unexplored. Clinical management of pemphigus is beginning to move away from broad-spectrum immunosuppression and towards B-cell-targeted therapies, which reduce many patients’ symptoms but can have significant side effects. Here, we review the latest developments in our understanding of the predisposing factors/conditions of pemphigus, the underlying pathogenic mechanisms, and new and emerging therapies to treat these devastating diseases.

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Pênfigo Neonatal Em Filho De Mãe Com Pênfigo Vulgar: Relato De Caso

Cited by 8 publications

References 14 publications

Pemphigus vulgaris

Pemphigus vulgaris

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

Autoimmune Pemphigus: Latest Advances and Emerging Therapies

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