A Rare Case of Juvenile Systemic Lupus Erythematosus with Disseminated Histoplasmosis
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Published on: November 1, 2015
Piyabi Sarkar1, Keya Basu1, Mamata Guha Mallick Sinha1
1Department of Pathology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India.
Abstract:
Histoplasmosis is a systemic fungal infection caused by dimorphic fungus, Histoplasma capsulatum. Immunocompetent individuals usually have self-limiting or localized disease whereas immunocompromised individuals develop disseminated disease. The occurrence of progressive disseminated histoplasmosis in juvenile systemic lupus erythematosus is extremely rare with only one reported case in literature showing such association. Therefore, we report a case of severe opportunistic fungal infection caused by Histoplasma in a 13-year-old girl who was diagnosed with juvenile lupus erythematosus, subsequently developed septic shock and died of the disease despite of aggressive antifungal therapy.
Insights
Progressive disseminated histoplasmosis is a rare, severe fungal infection in children with juvenile systemic lupus erythematosus. This case highlights the critical risk of opportunistic infections in immunocompromised youth, emphasizing the need for vigilance.
Area of Science:
- Medical Mycology
- Immunology
- Pediatric Rheumatology
Background:
- Histoplasmosis is a systemic fungal infection caused by *Histoplasma capsulatum*.
- Immunocompromised individuals are at high risk for disseminated disease.
- Juvenile systemic lupus erythematosus (JSLE) significantly increases susceptibility to opportunistic infections.
Purpose of the Study:
- To report an extremely rare case of progressive disseminated histoplasmosis in a patient with JSLE.
- To highlight the severe consequences of opportunistic fungal infections in pediatric autoimmune diseases.
- To underscore the importance of early diagnosis and aggressive management.
Main Methods:
- Case report of a 13-year-old female diagnosed with JSLE.
- Clinical presentation and diagnostic workup for disseminated fungal infection.
- Treatment course involving antifungal therapy and management of septic shock.
Main Results:
- The patient developed severe opportunistic histoplasmosis despite aggressive antifungal treatment.
- The infection rapidly progressed to septic shock, leading to a fatal outcome.
- This case represents only the second documented instance of this association in medical literature.
Conclusions:
- Progressive disseminated histoplasmosis is a life-threatening complication in pediatric patients with JSLE.
- Aggressive antifungal therapy may not always prevent fatal outcomes in severe opportunistic infections.
- Vigilance for fungal infections is crucial in immunocompromised pediatric patients with autoimmune conditions.
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