A Study of Adult Onset Generalized Eruptive Langerhans Cell Histiocytosis with Rare Association of Nail Pterygium
Journal Title: International Journal of Current Research and Review - Year 2017, Vol 9, Issue 21
Abstract
Background: Langerhans cell histiocytosis(LCH) is a rare proliferative disease which generally affects children and rarely, adults. LCH in adults first presenting in the skin is very rare and often it is misdiagnosed as it mimics number of common skin conditions. Nail changes are another cutaneous feature which is not frequently described in adults. Objectives and Methods: To present two new cases of adult LCH and a review of the literature of generalized cutaneous LCH in adults. Results: Two adults with widespread papulo-nodular cutaneous eruptions in seborrheic distribution. Both the cases had palmoplantar purpuric papules and involvement of nails with pterygium formation, which is a rare finding not frequently reported in LCH. There was pituitary involvement in the form of diabetes insipidus in both the cases though it was clinically symptomatic in only first case. Diagnosis of both the patients was established on the basis of histopathology and immunohistochemistry. Conclusions: Extensive cutaneous and nail involvement (nail dystrophy and pterygium) is possible in adult onset LCH. Thus, high index of suspicion is necessary to make the diagnosis.
Authors and Affiliations
Sunanda Mahajan
Keywords
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- EP ID EP607551
- DOI 10.7324/IJCRR.2017.9218
- Views 127
- Downloads 0
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