Oral 7, Cutaneous manifestations of disseminated BCG in a child with severe combined immunodeficiency
Authors: Brodie, T.; Goodyear, H.; Hackett, S.
Source: British Journal of Dermatology, Volume 156, Number 6, June 2007 , pp. 1405-1405(1)
Publisher: Blackwell Publishing
An 8-month-old girl of consanguineous parents presented with multiple subcutaneous nodules on her limbs and trunk. Over the preceding 2 weeks, her bacillus Calmette-Guérin (BCG) wound, which had been weeping since it was administered at birth, had become significantly worse. Both BCG and skin nodules had progressed to deep ulcers surrounded by areas of erythema with scaling. Previous contacts with health services included concerns regarding delayed development and a prolonged admission for a chest infection at 3 months of age (positive for respiratory syncytial virus with Campylobacter in her stool), associated with significant growth faltering requiring nasogastric feeding. This clinical picture raised the suspicion of severe combined immunodeficiency (SCID). Skin biopsy and Ziehl-Neelsen staining demonstrated numerous acid alcohol-fast bacilli within granulomatous lesions. Analysis of lymphocyte subsets, absent lymphocyte proliferations and molecular genetics confirmed the diagnosis of SCID with maternofetal engraftment complicated by disseminated BCG. The patient has recently received a matched unrelated donor human stem cell transplantation.Severe complications following BCG vaccination are rare and almost exclusively confined to patients with immunological impairment, in particular, SCID, chronic granulomatous disease, AIDS and complete DiGeorge syndrome.1 Individuals may present firstly to a dermatologist with subcutaneous nodules or a rash.2 The case described is used to highlight the importance of recognizing the cutaneous manifestations of disseminated BCG and to discuss the pathogenesis of the disease. References 1 Casanova JL, Jouanguy E, Lamhamedi S et al. Immunological conditions of children with BCG disseminated infection. Lancet 1995; 346:581. 2 Al-Bhal LA. Pathological findings for bacilli Calmette-Guérin infections in immunocompetent and immunocompromised patients. Am J Clin Pathol 2000; 113:703-8.
Document Type: Abstract
article url: http://www.ingentaconnect.com/content/bsc/bjd/2007