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A 36-year-old male ex-smoker was referred for the management of pulmonary Langerhans cell histiocytosis (LCH) confirmed by surgical biopsy (figure 1A). He had been treated with desmopressin for 5 years for diabetes insipidus. One year before admission, dyspnoea on exertion had appeared. At the same time, the patient developed painful nail abnormalities on all 10 fingers with subungual hyperkeratosis, purpuric striae, paronychial erythema, longitudinal grooving, splinter haemorrhages, onycholysis, lunula deformation (figure 1B), with no underlying bone involvement on hand X-ray examination. Nail samples were negative for bacteria and fungi. Maculopapular and crusted lesions in the axillary fold, the scalp and the external auditory canal were also present. A nail biopsy showed hyperplastic epidermis, surmounted by thick parakeratosis. The upper dermis was infiltrated by pale histiocytes with eosinophilic cytoplasm and reniform nuclei that positively stained for CD1a antigen, …
Contributors LD, J-DB, GL and AT managed the patient. MB analysed and interpreted histological findings. All authors contributed to writing the manuscript. All authors have approved the version of the manuscript submitted for publication.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.