Chronic bullous disease of childhood and pneumonia in a neonate with VATERL association and hypoplastic paranasal sinuses

Salud, Charissa Mia D.; Nicolas, Marie Eleanore O.

doi:10.1016/j.jaad.2009.02.009

Cited by 14 publications

(16 citation statements)

References 5 publications

(2 reference statements)

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“…Linear IgA bullous dermatosis is rare in neonates, and there have been only six other reported neonatal cases (1–6), one of which was associated with bullous pemphigoid (1) and another in a patient with vertebral, anal, tracheoesophageal, renal, and limb defects (VATERL) association (6). Almost all of the patients, including ours, were male and developed bullae within the first week of life (Table 1).…”

Section: Discussionmentioning

confidence: 99%

“…The only exception was the neonate with VATERL who was a girl and developed bullae at 10 days of life. Because of concern regarding sepsis shortly after birth, this patient received multiple antibiotics, fresh frozen plasma, and furosemide before the development of her bullae (6). In contrast, most of the other patients underwent an examination for infection, including administration of empiric antiviral and antibacterial therapy pending results, after they presented with bullae.…”

Section: Discussionmentioning

confidence: 99%

“…In the case associated with bullous pemphigoid, DIF revealed weak linear IgG and C3 deposition at the dermal–epidermal junction, and IgA was noted on the epidermal side only after performing immunofluorescence with salt‐split skin (1). The coexistence of IgA with other immunoreactants is less frequently associated with drug‐induced LABD (7), so although this was a possibility in the patient with VATERL, it was considered less likely because of the presence of IgG and C3 (6).…”

Section: Discussionmentioning

confidence: 99%

“…Although tracheoesophageal anomalies may be seen with VATERL association, the patient reported by Salud et al did not have this manifestation. However, during the course of the presentation and treatment of her LABD, she developed respiratory complications that led to her eventual death (6). In addition, all previously reported patients required treatment with systemic medications including corticosteroids, diaminophenylsulfone, dapsone, and intravenous immunoglobulin.…”

Section: Discussionmentioning

confidence: 99%

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Neonatal Linear Immunoglobulin A Bullous Dermatosis: A Rare Presentation

Julapalli

Brandon²,

Rosales

et al. 2011

Pediatric Dermatology

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Linear immunoglobulin A bullous dermatosis (LABD) is an autoimmune blistering disease that most commonly presents in preschool-aged children. There have been few neonatal reports, all of which had life-threatening aerodigestive complications requiring mechanical intervention and systemic therapy. We present a case of LABD in a neonate who had an uncomplicated course and was treated conservatively with only low-potency topical corticosteroids and wound care before resolution of his skin lesions.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Neonatal Linear Immunoglobulin A Bullous Dermatosis: A Rare Presentation

Julapalli

Brandon²,

Rosales

et al. 2011

Pediatric Dermatology

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“…The disease has a chronic relapsing course and in the majority of cases resolves before puberty (1,5). A few neonatal cases of LABD have been described (7)(8)(9)(10)(11)(12)(13). Mucosal involvement was predominant in these patients.…”

mentioning

confidence: 99%

A Case of Neonatal Linear IgA Bullous Dermatosis with Severe Eye Involvement

Romani

Diociaiuti²,

D’Argenio³

et al. 2015

Acta Derm Venerol

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Neonatal Linear IgA Bullous Dermatosis Mediated by Breast Milk–Borne Maternal IgA

et al. 2021

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IMPORTANCE Neonatal linear immunoglobulin A (IgA) bullous dermatosis (LABD) is a rare disease that can be fatal when associated with respiratory failure. All previously reported cases of neonatal LABD have been in newborns with healthy asymptomatic mothers, and the pathogenic IgA was of unknown origin.OBJECTIVE To clarify the origin of IgA associated with LABD in neonates born of healthy asymptomatic mothers. DESIGN, SETTING, AND PARTICIPANTSThis case study analyzed the laboratory findings of a single breast-fed newborn male with neonatal LABD admitted to the Keio University Hospital in Tokyo and his healthy asymptomatic mother. The healthy newborn developed life-threatening blisters and erosions of the skin and mucous membranes on day 4 after birth. Blood serum, skin, and maternal breast milk were examined for IgA autoantibodies.MAIN OUTCOMES AND MEASURES Histopathologic and immunofluorescence analyses of specimens (serum, skin, and breast milk) from the patient and his mother.RESULTS Histopathologic evaluation of the newborn's skin revealed subepidermal blisters with neutrophil infiltrates, and immunofluorescence testing showed linear IgA deposition along the basement membrane zone (BMZ), which lead to the diagnosis of neonatal LABD. Indirect immunofluorescence using normal human skin after treatment with 1-mol/L sodium chloride showed the patient to have circulating IgA binding to the dermal side of BMZ. Immunohistochemical staining proved the deposition of secretory IgA in the neonatal skin by demonstrating the presence of J chain-not been seen in other LABD cases-indicating that the autoantibodies producing the blisters were derived from the maternal breast milk. Although no circulating IgA against the skin was detected in mother's sera, the breast milk contained IgA that reacted with the dermal side of the BMZ. No new blister formation was observed after cessation of breastfeeding. CONCLUSIONS AND RELEVANCEThe results of this case study suggest a passive transfer of pathogenic IgA to a newborn from an asymptomatic mother via breast milk. In prior reports, no serum from asymptomatic mothers of newborns with LABD had IgA autoantibodies binding to skin components; however, in this case, we found that the maternal breast milk contained IgA autoantibodies associated with neonatal LABD. In neonatal LABD, maternal breast milk should be examined for IgA autoantibodies and breast milk feeding should be discontinued as soon as neonatal LABD is suspected.

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Chronic bullous disease of childhood and pneumonia in a neonate with VATERL association and hypoplastic paranasal sinuses

Cited by 14 publications

References 5 publications

Neonatal Linear Immunoglobulin A Bullous Dermatosis: A Rare Presentation

Neonatal Linear Immunoglobulin A Bullous Dermatosis: A Rare Presentation

A Case of Neonatal Linear IgA Bullous Dermatosis with Severe Eye Involvement

Neonatal Linear IgA Bullous Dermatosis Mediated by Breast Milk–Borne Maternal IgA

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