engleski

Isolated cutaneous Langerhans cell histiocytosis in an elderly patient

Introduction & Objectives: Langerhans cell histiocytosis (LCH) is a rare clonal, proliferative disease of Langerhans cells that most commonly develops in children aged 1-3 years, though it can develop at any age. LHC is characterized by a broad spectrum of clinical presentations, ranging from mild, single-organ involvement to severe and progressive multisystem disease. The incidence of LCH is 4-5 per million in children and about 30% of that number in adults. Although dermatological manifestations occur in as many as 50% of cases, disease limited to the skin is uncommon among adult patients. We present a rare case of LCH manifested as a skin- limited disease, occurring in an elderly male patient. Materials & Methods: a single case report Results: A 75-year-old male patient, with a history of hypertension and ishemic heart disease, presented to our Department with symptoms of redness accompanied by burning sensation in the perianal and both inguinal regions. The lesions had begun a year ago and had not responded to prior treatment attempts with topical antibiotics and antimycotics. On physical exam, he had macerated erythematous plaques with multiple ulcerations involving the aforementioned regions. Skin scrapings were negative for fungal elements and direct immunofluorescence of lesional skin showed no immune deposits. However, histopathology showed extensive dermal and focally epidermal infiltrates of large histiocytic- appearing cells, some of which with folded, kidney-shaped nuclei, along with multinucleated giant cells and eosinophils. Immunoperoxidase staining was positive for CD1a+ and CD68+, with high Ki-67 index. The patient denied other constitutional or systemic symptoms including fever or bone pain, and extensive laboratory and imaging investigations failed to detect systemic involvement. The preferred treatment consisting of topical nitrogen mustard was not feasible, due to its unavailability in the country. Furthermore, photochemotherapy could not be performed due to patient's severe ischemic cardiomyopathy. Therefore, the patient's therapy included applications of local corticosteroids, antibacterial creams and anti- irritant creams with a zinc base. This resulted in nearly complete regression but also a swift relapse, at which point the patient refused any further treatment and was lost at follow-up. Conclusions: LCH may present as a skin-limited disease in adult and elderly patients and should be considered in the differential diagnosis of non-specific dermatitis of intertriginous end perigenital regions. This case highlights the importance of immunohistochemistry in the armamentarium of diagnostic methods in dermatology, along with the need for specific treatment guidelines for this rare condition.

Langerhans cell histiocytosis; adult

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