Bullous Pemphigoid of Childhood

Chimanovitch, Iakov; Hamm, Henning; Georgi, M.; Kroiss, Maximilian; Stolz, Wilhelm; Apitz, Christian; Bröcker, Eva‐Bettina; Zillikens, Detlef

doi:10.1001/archderm.136.4.527

Cited by 43 publications

(5 citation statements)

References 33 publications

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“…In the literature, all mothers of BP infants were healthy without a medical history of bullous disease. In six cases, mothers' sera were tested to circulating BP180 antibodies, but only by IIF, a low sensitivity method, and they were found to be negative 9,11 , 12,14 , 17,18 …”

Section: Discussionmentioning

confidence: 99%

“…BP is very rare in infants. Only 20 cases have been reported in the literature 8–19 . The clinical presentation is characteristic, with multiple tense bullae of the palms and soles, spreading quickly to the trunk, face, scalp and limbs with erythematous and oedematous plaques covered by tense and clear blisters and crusts, frequently distributed in annular and polycyclic patterns, and with severe pruritus.…”

Section: Discussionmentioning

confidence: 99%

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Absence of anti-BP180 antibodies in mothers of infants with bullous pemphigoid

Chiavérini¹,

Hamel‐Teillac²,

Gilbert³

et al. 2005

British Journal of Dermatology

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Absence of anti-BP180 antibodies in mothers of infants with bullous pemphigoid

Chiavérini¹,

Hamel‐Teillac²,

Gilbert³

et al. 2005

British Journal of Dermatology

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“…Though the autoantibodies in younger patients with BP recognize the same epitopes within the non-collagenous 16A domain of BP180 as in classic BP, differences in the clinical course and response to therapy have been described. 35…”

Section: Clinical Aspects and Classificationmentioning

confidence: 99%

Bullous pemphigoid

Miyamoto

Santi

Aoki

et al. 2019

An. Bras. Dermatol.

166

181

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Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. Diagnosis relies on (1) the histopathological evaluation demonstrating eosinophilic spongiosis or a subepidermal detachment with eosinophils; (2) the detection of IgG and/or C3 deposition at the basement membrane zone using direct or indirect immunofluorescence assays; and (3) quantification of circulating autoantibodies against BP180 and/or BP230 using ELISA. Bullous pemphigoid is often associated with multiple comorbidities in elderly individuals, especially neurological disorders and increased thrombotic risk, reaching a 1-year mortality rate of 23%. Treatment has to be tailored according to the patient's clinical conditions and disease severity. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.

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“…'s results. They consider childhood BP a true variant of BP because the fine specificity of autoantibodies to BP180 is the same in BP of childhood and adulthood 10 . Thus, we diagnosed the present case as BP.…”

Section: Discussionmentioning

confidence: 71%

“…They consider childhood BP a true variant of BP because the fine specificity of autoantibodies to BP180 is the same in BP of childhood and adulthood. 10 Thus, we diagnosed the present case as BP. A differential diagnosis with congenital syphilis has been made by serological tests.…”

Section: Discussionmentioning

confidence: 82%

Childhood bullous pemphigoid treated by i.v. immunoglobulin

Xiao

Wang

et al. 2007

The Journal of Dermatology

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Bullous pemphigoid is an acquired autoimmune subepidermal blistering disorder mostly seen in the elderly. Childhood bullous pemphigoid is very rare. For the first time we report a case of childhood bullous pemphigoid associated with infantile eczema. Two weeks after a routine vaccination, a 3.5-month-old boy with infantile eczema developed a generalized blistering disorder. Histopathology revealed a subepidermal blister. Direct immunofluorescence showed linear depositions of C3 along the basement membrane zone. Indirect immunofluorescence studies demonstrated the presence of circulating immunoglobulin G antibodies directed against the epidermal side of salt-split skin. Enzyme-linked immunosorbent assay demonstrated serum level of anti-BP180 antibody elevated. The patient was successfully treated by high-dose i.v. immunoglobulin.

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Bullous Pemphigoid of Childhood

Cited by 43 publications

References 33 publications

Absence of anti-BP180 antibodies in mothers of infants with bullous pemphigoid

Absence of anti-BP180 antibodies in mothers of infants with bullous pemphigoid

Bullous pemphigoid

Childhood bullous pemphigoid treated by i.v. immunoglobulin

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