Generalized papular rash as a rare sentinel sign of pediatric lupus: Hyper-IgE in a 13-year-old girl

• Author Details:   
• Natalia Erica Jahja
• Zahrah Hikmah ^*
• Willy Sandhika
• Anang Endaryanto

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Systemic lupus erythematosus (SLE) is an autoimmune disorder with diverse dermatologic manifestations. This case report describes a 13-year-old Indonesian girl presenting with generalized erythematous papules, a rare cutaneous sentinel of pediatric SLE. Despite markedly elevated immunoglobulin E (IgE) levels (>22,000 IU/mL) mimicking hyper-IgE syndrome, histopathological confirmation of subacute cutaneous lupus erythematosus (SCLE) and renal involvement established the diagnosis of SLE. Elevated IgE initially obscured the diagnosis, its association with severe immune dysregulation underscored the need for awareness in pediatric SLE. Treatment with intravenous methylprednisolone, mycophenolate sodium, and hydroxychloroquine led to rapid resolution of skin lesions and renal improvement. This case highlights atypical skin lesions as early signs of systemic autoimmunity, requiring dermatopathological and renal function evaluation.

Keywords: Pediatric lupus, Hyper-IgE, Subacute cutaneous lupus erythematosus, Generalized papular rash.