Necrobiotic Xanthogranuloma - Case Report and Literature Review.

Abstract

Necrobiotic xanthogranuloma is a rare type of non-Langerhans histiocytosis, whose main clinical features are the development of red-brown, purplish or yellowish skin papules and nodules, which evolve by forming infiltrated plaques. The periorbital region is the most commonly affected site. Some cases have lesions on the torso or extremities, with no facial involvement. Extracutaneous involvement of the ocular, respiratory, and cardiac tissues have also been described. Most patients have an associated monoclonal gammopathy (IgG k and λ). The treatment is difficult, with progression and recurrence. We present the clinical case of a 65-year-old woman, who was hospitalized for multiple erythematous plaques and placards, with fine squames and telangiectasis on the surface, disseminated within the scalp, ears, trunk, lower limbs; some plaques have a circinate border with reddish-purple, slightly protruding edges and a whitish and erosive atrophic center. The lesions within the scalp are alopecic. The disease began 15 years ago, the patient being diagnosed with Psoriasis vulgaris and treated with dermatocorticoids and Cignolin, with no remarkable results. Paraclinical investigations did not reveal any associated pathologies. Histopathological and immunohistochemical examination confirmed the diagnosis of necrobiotic Xanthogranuloma. The patient was treated with antihistamines, Neuromultivit, Vit E 100mg/day, Oximed spray, Atoderm emollient cream, Neopreol ointment, with slow favorable evolution. The physical examination and laboratory investigations for the diagnosis and surveillance of malignant diseases should be performed on a regular basis in patients with NXG. Our patient had lesions with a course of 15 years, with no development of multiple myeloma or other systemic involvement.