Mixed immunobullous disease of childhood: a good response to antimicrobials

Powell, J.; Kirtschig, Gudula; Allen, J.; Dean, D.; Wojnarowska, Fenella

doi:10.1046/j.1365-2133.2001.04131.x

Cited by 47 publications

(26 citation statements)

References 22 publications

(59 reference statements)

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“…observed that irrespective of whether IgA or IgG is predominantly deposited, the clinical features, response to treatment with dapsone, and prognosis of the childhood disease remain the same. [7] This was true in the case described by us too, as the child had a typical ‘string of pearls’ sign and showed excellent recovery with dapsone, despite IgG predominance. Therefore, the classical cases with predominant IgA deposition (linear IgA disease) as well as mixed immune bullous disease (linear IgG / IgA disease or linear IgA / IgG disease) seem to be practically the same entity.…”

Section: Discussionmentioning

confidence: 54%

Chronic bullous disease of childhood with IgG predominance: What is the locus standi?

et al. 2012

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Linear IgA disease (LAD) is an acquired, autoimmune, subepidermal, blistering disease, characterized by linear deposition of IgA along the dermoepidermal junction on immunofluorescence. Some cases known as ‘mixed immunobullous disease’ show weak staining with other immune reactants like IgG, IgM or C3. We report a rare case of a child having typical manifestations of LAD (chronic bullous disease of childhood), but with IgG predominance rather than IgA. Obviously it is improper to term this as linear IgA disease. Such cases are reported in literature as variants of LAD, with a multitude of terms like mixed immune bullous disease, linear IgG / IgA disease, linear IgA / IgG disease, and so on. In view of the tremendous confusion that these multiple terms cause in the absence of any practical benefit, we propose that the broad term ‘chronic bullous disease of childhood’ be applied to all childhood cases, irrespective of the nature of the immune deposits.

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Section: Discussionmentioning

confidence: 54%

Chronic bullous disease of childhood with IgG predominance: What is the locus standi?

et al. 2012

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“…Most data and experience with macrolide therapy for their anti-inflammatory effects were obtained in patients with inflammatory lung diseases such as cystic fibrosis and asthma bronchiale [11,15] . Furthermore, macrolides have already been successfully used in some non-infectious dermatologic diseases such as bullous pemphigoid, pityriasis rosea and IgA-mediated chronic bullous disease of childhood [7,16] . However, we demonstrate for the first time a beneficial effect of erythromycin in a disorder of keratinization.…”

Section: Discussionmentioning

confidence: 99%

Successful Treatment of Bullous Congenital Ichthyosiform Erythroderma with Erythromycin

Freyhaus¹,

Kaiser²,

Proelss³

et al. 2007

Dermatology

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“…Direct immunofluorescence reveals a smooth linear pattern of IgA deposition along the basement membrane, which is a typical finding for CBDC (Figure 4). Associated deposits of IgG, IgM, and C3 have been reported in cases of CBDC in the literature [31,32]. Direct immunofluorescence on salt-split lesional skin, incubated in 1 m sodium chloride solution, shows typically linear deposition of IgA along the roof of the cavity.…”

Section: Diagnosismentioning

confidence: 92%

Chronic Bullous Disease or Linear IgA Dermatosis of Childhood -Revisited

Patsatsi¹

2013

J Genet Syndr Gene Ther

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Linear IgA dermatosis or chronic bullous disease of childhood (CBDC) is a nonhereditary, autoimmune subepidermal bullous disease, characterized by the presence of continuous linear deposits of IgA autoantibodies along the basement membrane zone. Antigens mainly involved in the pathogenesis of CBDC are a 97-kDa and a 120 -kDa protein, which represent fragments of the extracellular domain of collagen XVII (BP180), a transmembrane protein playing a critical role in maintaining the linkage between the intracellular and the extracellular structural elements involved in epidermal adhesion. Humoral and cellular response contributes to the pathogenetic mechanism leading to blister formation. CBDC is characterized by a clinical polymorphism in each patient, with the "cluster of jewels" pattern being the most typical clinical lesion. For the establishment of CBDC diagnosis, combination of histology and immunofluorescence studies are of utmost importance. The majority of patients respond to dapsone, sulfapiridine or systemic steroids in cases of widespread disease. CBDC tends to resolve spontaneously within several months to 5 years after its onset. In this review article, the key features of this rare autoimmune bullous disease are revisited.

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Mixed immunobullous disease of childhood: a good response to antimicrobials

Cited by 47 publications

References 22 publications

Chronic bullous disease of childhood with IgG predominance: What is the locus standi?

Chronic bullous disease of childhood with IgG predominance: What is the locus standi?

Successful Treatment of Bullous Congenital Ichthyosiform Erythroderma with Erythromycin

Chronic Bullous Disease or Linear IgA Dermatosis of Childhood -Revisited

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