Disseminated Bacille Calmette-Guérin disease as the initial presentation of X-linked severe combined immunodeficiency--a case report.

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dc.contributor.authorHuang, Li-Hsinen_US
dc.contributor.authorShyur, Shyh-Daren_US
dc.contributor.authorWeng, Jyh-Deren_US
dc.contributor.authorShin-Chi,en_US
dc.contributor.authorTzen, Chin-Yuanen_US
dc.contributor.authorHuang, Fu-Yuanen_US
dc.date.accessioned2009-05-27T17:22:50Z
dc.date.available2009-05-27T17:22:50Z
dc.date.issued2005-12-01en_US
dc.descriptionPublished by the Allergy and Immunology Society of Thailand.en_US
dc.description.abstractBacille Calmette-Guerin (BCG) vaccination is used to prevent severe M. tuberculosis infection. It has been used in many countries for a long time. However, complications do occur, including localized abscesses, regional lymphadenitis and disseminated disease. The latter is often associated with underlying immunodeficiency. We report an 8-month-old male infant presenting with cough and fever who had had a generalized pigmented skin rash for one month. Skin biopsy revealed mycobacterial infection, but his response to treatment was poor and he had a persistent mild fever. Immunological studies revealed an IgG of 49 mg/dl, IgA 4 mg/dl, IgM 28 mg/dl, IgE < 1 mg/dl, CD3 1.1%, CD4 0.6%, CD8 0.6%, CD19 93.9%, CD57 1.1%, activated T cells 0.9%, and CH50 < 6.3%. These findings are compatible with the diagnosis of T(-)B(+)NK- severe combined immunodeficiency. Sequence analysis was performed and showed the presence of missense mutation in IL2Rgamma gene. An X-linked recessive inheritance pattern was proved by sequence analysis of his mother and grandmother. In order to identify the strain of the microorganism, we reviewed pathology of the skin biopsy which consisted of diffuse histiocytic infiltrate with poorly formed granulomas and no necrosis and used polymerase chain reaction (PCR) with the stain-positive clinical specimen and verify the organism found in the child's biopsy as M. bovis BCG strain. The diagnosis of disseminated BCG disease must be considered in any infant with cutaneous mycobacterial lesions, especially with atypical histologic findings. Such a patient's immunologic status should be evaluated and further family study is suggested. A high index of suspicion is needed to make a timely diagnosis, as early intervention with intensive treatment and bone marrow transplantation may be life-saving.en_US
dc.description.affiliationSection of Allergy and Immunology, Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan.en_US
dc.identifier.citationHuang LH, Shyur SD, Weng JD, Shin-Chi , Tzen CY, Huang FY. Disseminated Bacille Calmette-Guérin disease as the initial presentation of X-linked severe combined immunodeficiency--a case report. Asian Pacific Journal of Allergy and Immunology. 2005 Dec; 23(4): 221-6en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/37065
dc.language.isoengen_US
dc.subject.meshBCG Vaccine --adverse effectsen_US
dc.subject.meshDNA, Bacterial --analysisen_US
dc.subject.meshFatal Outcomeen_US
dc.subject.meshHumansen_US
dc.subject.meshInfanten_US
dc.subject.meshInterleukin Receptor Common gamma Subuniten_US
dc.subject.meshMaleen_US
dc.subject.meshMutationen_US
dc.subject.meshMycobacterium Infections --complicationsen_US
dc.subject.meshMycobacterium bovis --geneticsen_US
dc.subject.meshOpportunistic Infections --complicationsen_US
dc.subject.meshReceptors, Interleukin --geneticsen_US
dc.subject.meshSevere Combined Immunodeficiency --complicationsen_US
dc.subject.meshSkin --pathologyen_US
dc.subject.meshSkin Diseases, Bacterial --complicationsen_US
dc.titleDisseminated Bacille Calmette-Guérin disease as the initial presentation of X-linked severe combined immunodeficiency--a case report.en_US
dc.typeCase Reportsen_US
dc.typeJournal Articleen_US
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