Childhood Bullous Pemphigoid

Pablo, Maria Isabel Martinez-De; González-Enseñat, Maria Antònia; Vicente, A.; Gilaberte, Montserrat; Mascaró, José M.

doi:10.1001/archderm.143.2.215

Cited by 28 publications

(3 citation statements)

References 25 publications

(20 reference statements)

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“…Besides medical history, physical examination and histopathological analysis, we use tests such as immunofluorescence, indirect immunofluorescence (with salt-split skin technique), immunoblotting and ELISA for diagnosis. 1,11,13,17,18,19,20 …”

Section: Discussionmentioning

confidence: 99%

Bullous pemphigoid in a 3-month-old infant: case report and literature review of this dermatosis in childhood

Reis-Filho

Silva

Aguirre

et al. 2013

An. Bras. Dermatol.

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Bullous pemphigoid is an autoimmune subepidermal blistering dermatosis that is uncommon in childhood. We report a case of a female infant, 3 months old, which presented clinical and laboratory data for the confirmatory diagnosis of bullous pemphigoid. The authors used immunohistochemical staining for collagen type IV that allowed the differentiation of bullous pemphigoid from other subepidermal bullous diseases. Opportunely we review the clinical, immunological, therapeutic and prognostic features of this pathology in children.

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Section: Discussionmentioning

confidence: 99%

Bullous pemphigoid in a 3-month-old infant: case report and literature review of this dermatosis in childhood

Reis-Filho

Silva

Aguirre

et al. 2013

An. Bras. Dermatol.

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“…The antigens subset indicates a relationship between Bullous Pemphigoid (BP) and LABD that could be explained by "the epitope spreading phenomenon" in which the primary disease, such as ordinary BP, exposes the BMZ to the immune system, which induces autoantibodies against BMZ proteins without pathogenicity. For that reason, BP and LABD may overlap in infantile patients with mucosal involvement (47). LABD histologic and therapeutic findings are similar to those associated with BP and dermatitis herpetiformis (DH), even if LABD and DH are two different diseases in terms of pathomechanisms and clinical features.…”

Section: Pathologymentioning

confidence: 99%

Linear Immunoglobulin a Bullous Dermatosis in Children

et al. 2022

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Linear Immunoglobulin A Bullous Disease (LABD) is a rare dermatosis whose pathomechanisms are not yet completely understood. LABD has different features characterizing adults and children in terms of potential triggers, clinical manifestations, and prognosis. The aim of the present study is to review all neonatal and pediatric cases of LABD and summarize the major characteristics. Childhood LABD is mainly idiopathic with a benign prognosis. Neonatal cases are difficult to differentiate from infectious diseases and usually have a poor prognosis. Drugs are one of the possible triggers that can activate autoimmune responses through antigen mimicry and epitope spreading as well as different stimuli (e.g., infections, inflammatory diseases, trauma). The gold standard for the diagnosis is based on direct immunofluorescence. Prognosis is generally favorable but often depends on the prompt dermatological diagnosis, treatment and follow-up guaranteed by a multidisciplinary team, including pediatricians for this group of age.

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“…Here, the antigen is also BP180/NC16A but direct IF of the skin biopsy shows linear IgA fluorescence. Also IgA antibodies against the NC16A domain of BP180 have been found ( 60 ). IgA does, however, not activate the complement system.…”

Section: Bullous Skin Diseasesmentioning

confidence: 99%

Complement System in Dermatological Diseases â€“ Fire Under the Skin

Panelius¹,

Meri

2015

Front. Med.

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The complement system plays a key role in several dermatological diseases. Overactivation, deficiency, or abnormality of the control proteins are often related to a skin disease. Autoimmune mechanisms with autoantibodies and a cytotoxic effect of the complement membrane attack complex on epidermal or vascular cells can cause direct tissue damage and inflammation, e.g., in systemic lupus erythematosus (SLE), phospholipid antibody syndrome, and bullous skin diseases like pemphigoid. By evading complement attack, some microbes like Borrelia spirochetes and staphylococci can persist in the skin and cause prolonged symptoms. In this review, we present the most important skin diseases connected to abnormalities in the function of the complement system. Drugs having an effect on the complement system are also briefly described. On one hand, drugs with free hydroxyl on amino groups (e.g., hydralazine, procainamide) could interact with C4A, C4B, or C3 and cause an SLE-like disease. On the other hand, progress in studies on complement has led to novel anti-complement drugs (recombinant C1-inhibitor and anti-C5 antibody, eculizumab) that could alleviate symptoms in diseases associated with excessive complement activation. The main theme of the manuscript is to show how relevant the complement system is as an immune effector system in contributing to tissue injury and inflammation in a broad range of skin disorders.

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Childhood Bullous Pemphigoid

Cited by 28 publications

References 25 publications

Bullous pemphigoid in a 3-month-old infant: case report and literature review of this dermatosis in childhood

Bullous pemphigoid in a 3-month-old infant: case report and literature review of this dermatosis in childhood

Linear Immunoglobulin a Bullous Dermatosis in Children

Complement System in Dermatological Diseases â€“ Fire Under the Skin

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