DISSEMINATED BCG INFECTION RESEMBLING LANGERHANS CELL HISTIOCYTOSIS IN AN INFANT WITH SEVERE COMBINED IMMUNODEFICIENCY: A Case Report

Abstract

We present a very rare congenital immunologic disease, severe combined immunodeficiency syndrome (SCID) in 6-months-old-boy with prolonged mucocutaneous candidiasis, severe anaemia, skin rash similar to the infiltrative eczema of Langerhans cell histiocytosis (LCH) and subcutaneous nodules with histiocytic infiltration. Laboratory findings show profound absence of humoral and cell-mediated immunity. Pathology specimens analysis of subcutaneous nodule revealed numerous S-100 protein and Cd1a negative histiocytes, occupied by BCG intracellular growth. Histopathology and immunohistochemistry confirmed the diagnosis of BCG dissemination. BCG vaccination in infants with SCID can lead to life threatening dissemination, resembling to the infiltrative eczema of LCH and may mislead the clinician.

Successful unrelated mismatched cord blood transplantation in an infant with severe combined immunodeficiency and Mycobacterium bovis bacillus Calmette‐Guèrin disease

“…The vast majority of people with strong immune systems however, never develop tuberculosis disease. BCG vaccination in infants with SCID can lead to life-threatening dissemination, resembling infiltrative eczema and may mislead the clinician (11). Moreover, BCG disease could not possibly be cured with courses of the currently available antituberculous agents unless immune reconstitution occurs (1,12).…”

Vaccine-Acquired Rotavirus in Infants with Severe Combined Immunodeficiency

“…Infections associated with the administration of live viral vaccines in infants with SCID have been described previously. 4,5 Live viral vaccines are typically contraindicated in patients with known severe immunodeficiencies 6 ; however, the rotavirus-vaccine series is recommended to be started at 2 months of age, which is before SCID is typically diagnosed in infants for whom there is no family history of immunodeficiency. We describe three infants with SCID in whom vaccine-associated disease developed after receipt of rotavirus vaccine.…”

Disseminated BCG Infection in Severe Combined Immunodeficiency

“…Histopathological features are characterized by diffuse histiocytic infiltrates without caseation necrosis or apparent epithelioid granuloma formation (1,5,6). Thus, there could be a risk that these patients are misdiagnosed as having histiocytic neoplasms (1,5,6), such as Langerhans' histiocytosis and non-Langerhans' cell histiocytosis. The presence of neutrophils may be a useful feature for histopathological differential diagnosis, and in contrast to ordinary tuberculoid lesions, numerous acid-fast bacilli can be detected (1, 3-7).…”

“…These include multiple papulo-nodular or subcutaneous nodular lesions (1,(3)(4)(5)(6)(7)(8). Histopathological features are characterized by diffuse histiocytic infiltrates without caseation necrosis or apparent epithelioid granuloma formation (1,5,6). Thus, there could be a risk that these patients are misdiagnosed as having histiocytic neoplasms (1,5,6), such as Langerhans' histiocytosis and non-Langerhans' cell histiocytosis.…”