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Disseminated BCG Infection Following Bone Marrow Transplantation for X‐Linked Severe Combined Immunodeficiency
Author(s) -
McKenzie Rowena H. S. B.,
Roux Paul
Publication year - 2000
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1046/j.1525-1470.2000.01754.x
Subject(s) - medicine , ethambutol , rifampicin , bone marrow , isoniazid , tuberculosis , surgery , pathology
Abstract: An 8‐month‐old boy with X‐linked severe combined immunodeficiency (XSCID) developed disseminated bacille Calmette‐Guerin (BCG) infection following BCG vaccination at birth. He initially presented with an abscess at the site of BCG vaccination and was begun on three‐drug antituberculous treatment (rifampicin, isoniazid, and pyrazinimide). Dissemination was subclinical prior to a human leukocyte antigen (HLA)‐identical bone marrow transplant (BMT) from his sister, following which he presented with an acute erythroderma. A skin biopsy specimen revealed granulomas with epithelial histiocytes and giant cells in the reticular dermis, and numerous acid‐fast bacilli (AFB) were present on Ziehl–Nielsen stain. A diagnosis of disseminated BCG disease was made. Despite the addition of a fourth antituberculous agent, ethambutol, he did not recover and developed numerous skin abscesses over the following weeks. Examination of pus from these lesions demonstrated numerous AFB. Clarithromycin was added as a fifth antituberculous agent. Despite five‐drug antituberculous therapy and monthly intravenous immunoglobulin infusions, recurrent abscesses containing AFB developed intermittently until 7 months posttransplant. At follow‐up 1 year post‐BMT he showed good general physical improvement. All abscesses had healed with scarring, and no further skin lesions had occurred.