Section:
Diagnostic Challenge
Published:
2021-04-02
A 10-year-old female patient, with a history of severe combined immunodeficiency (SCID), hospitalized a month ago with daily fever, with peaks up to 40.0ºC and generalized lymphadenopathy. Treated with broad-spectrum antibiotics without improvement and managed with empirical first-line antituberculosis treatment on previous occasions due to similar symptoms.
Physical examination:
Patient is stable, listless and feverish . Cardiopulmonary is well, the abdomen is slightly globose, with hepatosplenomegaly. Generalized lymphadenopathy. On the skin, a necrotic-looking crusted lesion is observed at the edge of the left nostril and in the central submandibular area, and in the facial area adjacent to the eye, there is a small umbilicated lesion with adjacent erythema.
The blood count showed pancytopenia.
Thoracic-abdominal CT: mediastinal, axillary, retroperitoneal and mesenteric lymphadenopathy. Diffuse hepatosplenomegaly. Bibasal atelectasis.
What is your diagnosis?
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