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Rash in a child with systemic secrets
*Corresponding author: Subhra Kamal Saha, Department of Haematology, Nil Ratan Sircar Medical College and Hospital, Kolkata, West Bengal, India. subhrakamal.saha12@gmail.com
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Received: ,
Accepted: ,
How to cite this article: Saha S. Rash in a child with systemic secrets. J Hematol Allied Sci. 2026;6:100-1. doi: 10.25259/JHAS_10_2026
Langerhans cell histiocytosis (LCH) is a rare clonal proliferation of dendritic cells with variable clinical presentations. Some children present with isolated skin or bone lesions, while others develop multisystem disease with potential involvement of vital organs such as the hepatobiliary system. Early recognition of systemic involvement is essential to prevent long-term complications.
A 2-year-old child who presented with failure to thrive, delayed developmental milestones with persistent Papules and papulovesicles, scaly skin lesions resembling seborrheic dermatitis, and hepatomegaly. The rash was unresponsive to conventional topical therapies, prompting further evaluation. Physical examination revealed significant hepatomegaly, raising suspicion for a systemic histiocytic disorder.
Complete hemogram showing normocytic normochromic anemia, eosinophilia, and normal platelet count. Bone marrow aspiration examination showed Infiltration by Langerhans cells with grooved (“coffee-bean”) nuclei.
- (a) Scaly lesion over the palm; (b) papules and papulovesicles, scaly skin lesions resembling seborrheic dermatitis; (c) papulovesicular lesion, scaly lesion over the anterior abdomen; (d) skin biopsy IHC showing CD1a positivity.
A skin biopsy was done from the lesion over the anterior abdomen. Histopathological examination showed infiltration by cells with characteristic nuclear grooves and eosinophilic cytoplasm. Immunohistochemistry demonstrated positivity for CD1a and Langerin (CD207), confirming the diagnosis of LCH. Subsequent systemic evaluation established multisystem involvement.
The child was started on a low-dose cytarabine-based treatment protocol in accordance with current pediatric LCH management guidelines. Following initiation of therapy, the patient exhibited marked clinical improvement, with resolution of cutaneous lesions and reduction in hepatosplenomegaly.
This case highlights the need for heightened clinical suspicion for LCH in young children with persistent rashes accompanied by organomegaly. Skin biopsy with appropriate immunohistochemical markers remains the cornerstone of diagnosis, and timely initiation of systemic therapy can lead to favorable clinical outcomes.
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Declaration of patient consent:
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Conflicts of interest:
There are no conflicts of interest.
Use of artificial intelligence (AI)-assisted technology for manuscript preparation:
The authors confirm that there was no use of artificial intelligence (AI)-assisted Technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.
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