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Beyond Hepatitis: A Rare Case of Multisystem Langerhans Cell Histiocytosis in a Child

Year: 2025

Type: article

Abstract: Langerhans cell histiocytosis (LCH) is a rare neoplastic disorder involving abnormal proliferation of dendritic cells, with clinical presentations ranging from isolated lesions to life-threatening multisystem involvement. This case report describes a 26-month-old girl who presented with progressive jaundice, fever, hepatomegaly, pruritic scalp rashes with greasy scales, and maculopapular to papulocrusted skin lesions. Laboratory findings revealed severe anemia, elevated inflammatory markers, high bilirubin levels, elevated transaminases, markedly increased GGT (Gamma-Glutamyl Transferase) and ALP (Alkaline Phosphatase) levels, suggestive of cholestatic injury, along with low albumin and a reversed albumin-to-globulin ratio. A provisional diagnosis of high-risk multisystem LCH was made, and a skin biopsy was performed, demonstrating characteristic histiocytic infiltration with immunopositivity for CD1a and S-100, thereby confirming the diagnosis. She was initiated on chemotherapy with vinblastine in combination with prednisolone at a referred center and demonstrated a favorable clinical response. This case underscores the diagnostic challenges of Langerhans cell histiocytosis, particularly in resource-limited settings, and highlights the importance of looking beyond common diagnoses, such as acute viral hepatitis, when the clinical presentation does not fully align. Careful recognition of systemic features, especially involvement of the liver and skin, combined with early skin biopsy and a multidisciplinary approach, is essential for timely diagnosis and effective management of rare conditions like high-risk multisystem LCH.

Source: Clinical Case Reports

DOI: 10.1002/ccr3.71336

Authors: Dilip Neupane, Rishi Ram Banjade, Dhiraj Joshi, Pusp Raj Awasthi, Santosh Mishra

Institution: Kathmandu Medical College Teaching Hospital

Language: English

Topic: Histiocytic Disorders and Treatments

Subfield: Physiology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $1,820