Abstract

Langerhans cell histiocytosis (LCH) is an infrequent clonal proliferative disorder of myeloid dendritic cells. It has a wide variety of cutaneous manifestations and retains the possibility of systemic implications. Because LCH is predominantly a disease of childhood, there are well-established clinical definitions, as well as guidelines regarding workup and treatment, in the context of pediatric disease. Here we present a case of isolated cutaneous LCH in an adult male, followed by a discussion of our diagnostic plan and treatment course. The patient exhibited a small, excoriated, yellow papule on his inferior forehead during a skin examination. The specimen underwent tangential shave biopsy; histopathologic evaluation with appropriate immunohistochemical staining confirmed a diagnosis of cutaneous LCH. After thorough investigation via serologic and imaging diagnostics, we confirmed isolated cutaneous disease. The patient underwent wide local excision (WLE) with no evidence of recurrence. It is crucial to appropriately screen all patients diagnosed with cutaneous LCH for internal organ involvement. The authors aim to highlight the need for further investigations to ultimately dictate standardized management and treatment for isolated cutaneous LCH in the adult population.

Highlights

  • Langerhans cell histiocytosis (LCH), previously termed histiocytosis X, is a rare inflammatory, neoplastic condition of myeloid dendritic cells that express an immunophenotype positive for CD1a, langerin (CD207), S100, and cytoplasmic Birbeck granules [1, 2]

  • The authors aim to highlight the need for further investigations to dictate standardized management and treatment for isolated cutaneous LCH in the adult population

  • LCH is divided into four distinct syndromes: Letterer-Siwe disease, Hand-Schüller-Christian disease, eosinophilic granuloma, and congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker disease)

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Summary

Introduction

Langerhans cell histiocytosis (LCH), previously termed histiocytosis X, is a rare inflammatory, neoplastic condition of myeloid dendritic cells that express an immunophenotype positive for CD1a, langerin (CD207), S100, and cytoplasmic Birbeck granules [1, 2]. Based on similarities in immunophenotypic markers, LCH was thought to arise from skin Langerhans cells, the primary antigen-presenting cells in the epidermis. Newer gene expression analyses demonstrated that the neoplastic cells in LCH derive from immature myeloid precursor cells from the bone marrow [1,2,3]. LCH is further categorized based on the extent of disease (single or multisystem) and involvement of at-risk organs

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