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Suspected case of chronic bullous disease of childhood in a rural area of Southern Mozambique
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  1. Rosauro Varo1,2,
  2. Sheila Fernández-Luis3,
  3. Antonio Sitoe2,
  4. Quique Bassat1,2,4
  1. 1Instituto de Salud Global Barcelona, Barcelona, Spain
  2. 2Centro de Investigacao em Saude de Manhica, Manhica, Maputo, Mozambique
  3. 3Hospital Universitario de Salamanca, Salamanca, Castilla y León, Spain
  4. 4ICREA, Pg. Lluís Companys 23, 08010 Barcelona, Spain
  1. Correspondence to Dr Quique Bassat, quique.bassat{at}isglobal.org

https://doi.org/10.1136/bcr-2016-218315

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    Description

    Chronic bullous disease of childhood (CBDC) is the most common, non-hereditary, autoimmune blistering disorder of childhood. This rare condition, characterised by linear IgA staining on direct immunofluorescence of the basement membrane of the squamous epithelium, has been considered the paediatric variant of adult linear IgA disease,1 although CBDC tends to occur in children less frequently, usually as a response to neoplasms or drug hypersensitivity.

    The clinical hallmark of this condition is the abrupt appearance on normal or erythematous skin of many large, tense bullae, filled with clear or sometimes haemorrhagic fluid and acquiring the typical rosette-like pattern of ‘string of pearls’ (figure 1), with or without associated pruritus. Bullae predominantly appear in the genital and buttocks area, although they can also appear in the trunk, extremities and face (figure 2). Mucous membranes may also be affected. Secondary impetiginisation may be frequent, but scarring sequelae are not common. The disease is often self-limiting, but may persist for months or even years, with occasional recurrences, before complete resolution, usually by puberty.

    Figure 1
    Figure 1

    Classic ‘string of pearls’ or ‘cluster of jewels’ pattern of lesions with tense vesicles arising at the periphery of old lesions, located in the extremities and genital area of an HIV-negative male Mozambican child aged 8 years, with no other significant diseases.

    Figure 2
    Figure 2

    Generalised distribution in the same child of a heterogeneous set of cutaneous lesions, including vesicles, tense bullae and impetiginised erosions. Lesions resolved completely about 4 months after the initiation of oral corticosteroids, as dapsone was unavailable in Mozambique.

    Diagnostic confirmation requires direct immunofluorescence studies, where IgA linear deposition at the dermal–epidermal junction of peri-lesional skin areas can be observed. In resource-constrained settings, however, where such diagnostic methods are seldom available, diagnosis remains clinical, but requires the exclusion of other diseases such as bullous pemphigoid, dermatitis herpetiformis or erythema multiforme.2 ,3

    The majority of cases respond well to long-term oral dapsone, or sulfapyridine. Alternatively, oral or systemic corticosteroids or tacrolimus can be effective, and sometimes may need to be complemented with topical or oral antibiotics.

    Learning points

    • Albeit rare, chronic bullous disease of childhood is the most frequent non-hereditary autoimmune blistering disease in childhood.

    • In African settings, where diagnostic methods remain scarce, it is important to recognise clinically this disease, and initiate a specific long-term treatment with dapsone or corticosteroids.

    • In spite of its impressive appearance, the disease is often self-limited and disappears by puberty.

    References

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      1. Mintz EM,
      2. Morel KD
      . Clinical features, diagnosis, and pathogenesis of chronic bullous disease of childhood. Dermatol Clin 2011;29:459–62, ix. doi:10.1016/j.det.2011.03.022
      OpenUrlPubMed
    2. ↵
      1. Patsatsi A
      . Chronic bullous disease or linear IgA dermatosis of childhood—revisited. J Genet Syndr Gene Ther 2013;4:151.
      OpenUrl
    3. ↵
      1. Sansaricq F,
      2. Stein SL,
      3. Petronic-Rosic V
      . Autoimmune bullous diseases in childhood. Clin Dermatol 2012;30:114–27. doi:10.1016/j.clindermatol.2011.03.018
      OpenUrlCrossRefPubMed
    View Abstract

    Footnotes

    • Contributors RV managed the case, reviewed the literature, wrote and prepared the final manuscript. SF-L managed the case and reviewed the manuscript. AS managed the case and reviewed the manuscript. QB managed the case and reviewed the manuscript.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.