Nail Langerhans Cell Histiocytosis: A Rare Case Series of Two Patients With Single-System and Multisystem Disease Presenting With Nail Lesions.

Am J DermatopatholJun 17, 2026 (epub)

Antonina Kalmykova, Kim Harnisch, Oleksandr Chepets et al.

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Case in brief

Two boys, aged 2 years and 19 months, developed nail abnormalities as the initial feature of Langerhans cell histiocytosis (LCH). The older child had longitudinal nail grooving, onycholysis, subungual hyperkeratosis, and purpuric striae; staging showed mild lymphadenopathy, and BRAF mutation testing was negative. The younger child had diffuse fingernail and toenail dystrophy, followed by skin papules, with LCH confirmed in nail bed, skin, and bone biopsies and disease involving bone and lungs. Both improved with systemic treatment: the first achieved metabolic inactivity at 9 months, and the second had marked improvement across affected sites.

What made this case unusual

Nail dystrophy was the presenting feature in both children despite differing disease extent: one had limited findings on staging, while the other had multisystem involvement of bone and lungs.

Diagnostic / clinical pearl

Unexplained nail changes in a child can precede other signs of LCH; consider the diagnosis and undertake systemic evaluation, since disease extent may vary.

Source

Published in Am J Dermatopathol. This summary was written by xxcode from the publication's abstract and metadata. It is not peer reviewed and is not a substitute for the original article. For clinical decisions, review the original publication.

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AI-generated summaries may contain errors or omissions. Verify clinically important information with the original publication.

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