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Disseminated cutaneous granulomatosis as a manifestation of myelodysplastic syndrome

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Disseminated cutaneous granulomatosis as a manifestation of myelodysplastic syndrome Dear Editor, Disseminated cutaneous granulomatosis (DCG) consists in a heterogeneous clinicopathological spectrum. In addition to well-defined entities, such as sarcoidosis, granuloma… Click to show full abstract

Disseminated cutaneous granulomatosis as a manifestation of myelodysplastic syndrome Dear Editor, Disseminated cutaneous granulomatosis (DCG) consists in a heterogeneous clinicopathological spectrum. In addition to well-defined entities, such as sarcoidosis, granuloma annulare, etc., some cases do not fit into any of these categories and may be associated with myelodysplastic syndromes (MDS), as in our two illustrative cases. A 65-year-old man presented in 2019 with a 2-month history of eruption associated with asthenia and loss of 2 kg in body weight. Lesions began as bright pink-orange pruritic papules on the forearms and neckline and secondarily extended to the whole body in confluent plaques (Fig. 1a). Skin biopsy showed abundant dermal, nodular, and interstitial histiocytic infiltrates with multinucleated giant cells, minimal neutrophilic component, and focal necrotic changes in collagen fibers (Fig. 1b,c). No pathogens were presented in culture. Blood analysis revealed anemia (hemoglobin level: 12.1 g/dl), monocyte count: 2.8 G/l, and myelemia: 0.54 G/l. Blood cells phenotyping showed 96% CD16 monocytes, which led to a diagnosis of chronic myelomonocytic leukemia (CMML). Myelogram showed granular line hyperplasia, without maturation hiatus, 0.5% blasts, and no karyotype abnormalities. Results of computed tomography were normal. In the absence of unfavorable prognostic criteria, we started systemic corticosteroids (20 mg/ day). The pruritus disappeared after 7 days, and the rash resolved completely within 6 weeks, along with a decrease in monocytosis and myelemia. Owing to the recurrence of skin lesions and increase in monocytes when tapering corticosteroids, we added azacitidine 2 months later. After 7 months, the partial improvement of DCG allowed further reduction in corticosteroids to 10 mg/day and stabilization of monocytosis. A 70-year-old woman presented in 2014 with a 3-month history of confluent micropapules in pink sheets on extensor areas of the upper limbs, trunk, and neck (Fig. 2a). Skin biopsy showed a dense dermal nodular and interstitial histiocytic infiltrate associated with some neutrophils and focal alteration of collagen fibers (Fig. 2b,c). Cultures were negative. Systemic steroids (10 mg/day) allowed rapid resolution of the lesions within a few days. As a result of steroid dependence, we introduced methotrexate (7.5 mg/week). Upon the occurrence of poorly tolerated normocytic anemia (hemoglobin level: 8 g/dl), a myelogram showed non-specific dyserythropoiesis. Despite

Keywords: disseminated cutaneous; cutaneous granulomatosis; myelodysplastic syndrome; granulomatosis manifestation; manifestation myelodysplastic

Journal Title: International Journal of Dermatology
Year Published: 2020

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