LOSW SN TV X THIS FAR AND NO FARTHER

 

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PKZN

Fever and blistering rash in a child

A 3-year-old female presented to the ER with fevers and a worsening blistering skin rash. Two weeks prior to presentation, she was seen by her pediatrician for five days of fever to a Tm of 104, as well as small blisters on her hands and feet. She was diagnosed with coxsackie virus, and her fevers began to improve, becoming more intermittent and lower-grade. However, her rash continued to worsen until she had multiple large, painful, fluid-filled blisters, and her mother brought her to the ER for a second opinion. She had no significant past medical or family history, and no other complaints. On exam, she was ill-appearing, with tender bullous lesions on her hands, feet, and the helices of both ears. She was febrile to 101 and slightly tachycardic, with otherwise normal vital signs. Initial labwork was significant for a hemoglobin of 8.1 and an ESR of 145, with negative COVID PCR and antibody. She was admitted for further workup, where she was found to have elevated immunoglobulins, positive ANA, Ro and Sm antibodies, and low C3 and C4. Infectious workup was negative. A biopsy was performed, and the presumed diagnosis was confirmed. What do you think the diagnosis is?

Thank you everyone for the comments! As some of you suggested, the correct diagnosis is BULLOUS SYSTEMIC LUPUS ERYTHEMATOSUS (BSLE). BSLE is an acute vesiculobullous skin eruption in a patient with SLE. Though most patients have known SLE at the time of the skin eruption, there are reports of bullous rash being the initial presentation of lupus, particularly in pediatric and adolescent patients. Lupus is very rare in a child of this age, and often has a genetic component. This particular patient was found to have a compliment deficiency. She was initially treated with prednisone and dapsone, with resolution of her skin lesions, though she did have some residual scarring. She is currently doing well on belimumab with good control of her underlying SLE.

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