Q J Med 2014; 107:859 doi:10.1093/qjmed/hcu066

Advance Access Publication 22 March 2014

Clinical picture Necrobiotic xanthogranuloma extremities and the face (periorbital lesions).1 Histopathology of NX shows typical necrobiotic areas surrounded by granulomas composed of lymphocytes, Touton giant cells, foamy histiocytes and foreign body-type multinucleated giant cells affecting all layers of the dermis.1 The lesions tends to be progressive and treatment is often unsuccessful. Alkylating agents (cyclophosphamide, chlorambucil, melphalan), alone or in combination with systemic cortocsteroids, is the treatment most commonly used. Furthermore, good response to pulsed high-dose oral dexamethasone plus maintenance therapy with oral prednisolone have previously been reported in a recalcitrant case of NX.2 Haematological disorders are present in up to 80% of cases (IgG monoclonal gammopathy of unknown significance or smoldering myeloma mainly), so care of patients with NX should include lifelong follow-up to detect the development of multiple myeloma.3 Photographs and text from: F. Jae´n-A´guila, J.A. Vargas-Hitos and J. Jime´nez-Alonso, Internal Medicine Department, ‘‘Virgen de las Nieves’’ University Hospital, 9th Floor, Avda, Fuerzas Armadas No 2, 18014 Granada, Spain. email: [email protected] Conflict of interest: None declared.

References 1. Spicknall KE, Mehregan DA. Necrobiotic xanthogranuloma. Int J Dermatol 2009; 48:1–10. 2. Chave TA, Chowdhury MM, Holt PJ. Recalcitrant necrobiotic xanthogranuloma responding to pulsed high-dose oral dexamethasone plus maintenance therapy with oral prednisolone. Br J Dermatol 2001; 144:158–61.

Figure 1. Confluent nodular lesions and plaques with elevated borders on the upper chest highly suggestive of necrobiotic xanthogranuloma.

3. Wood AJ, Wagner MV, Abbott JJ, Gibson LE. Necrobiotic xanthogranuloma: a review of 17 cases with emphasis on clinical and pathologic correlation. Arch Dermatol 2009; 145:279–84.

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A 66-year-old women presented with a 1-year history of yellowish nodular lesions and plaques with erythematous borders on the neck, upper chest (Figure 1) and the periocular skin of the left eye. Skin biopsy showed dermis granulomatous inflammation alternating with areas of necrobiosis. Main laboratory finding was an IgG-k monoclonal spike on serum protein electrophoresis (3090 mg/dl). Bone marrow biopsy showed 7.5% plasmatic cells and she was diagnosed with necrobiotic xanthogranuloma (NX) with smoldering myeloma. Treatment with oral dexamethasone 8 mg/daily was initiated and progressively reduced, observing a gradual improvement of the lesions and a decrease of IgG-k values 2 months later. NX is a chronic dermatological disease characterized by yellow or red–orange plaques or nodules that predominantly affects to the trunk, the