Simultaneous occurrence of two rare cutaneous markers of poor prognosis in myelodysplastic syndrome: erythema elevatum diutinum and specific lesions

Abstract

We report the concomitant occurrence of erythema elevatum diutinum and specific skin lesions in a patient with a myelodysplastic syndrome (MDS). This patient's course, and review of other reported cases, support the opinion that neutrophilic dermatoses are associated with a poor prognosis of MDS. The simultaneous appearance of these manifestations could be the consequence of a particular chemotactism of myeloid cells, expressed after acute transformation.

From acute febrile neutrophilic dermatosis to neutrophilic disease: Forty years of clinical research

“…The occurrence of a ND is usually of poor prognostic significance. 31 Monoclonal gammopathies are often of the IgA type. Most of them are benign.…”

Chronic Recurrent Lymphocytic Sweet Syndrome as a Predictive Marker of Myelodysplasia

“…However, 7 of the 9 patients died in a follow-up period of 3 to 9 years, confirming the poor prognosis of Sweet syndrome associated with myelodysplasia. 17 Interestingly, cutaneous lesions clinically characteristic of Sweet syndrome, but with lymphocytic infiltrate and atypical mononuclear myeloperoxidase-positive cells, preceded myelodysplasia in all 9 cases.…”

Abscess-Forming Neutrophilic Dermatosis: Report of Three Cases Associated with Hemopathies

“…Myelodysplastic syndrome (MDS) and other hematological disorders, such as leukemia or more rarely myeloma, have recently been recognized to be associated with skin diseases characterized by a neutrophilic infiltration in the dermis, which clinically resembles acute febrile neutrophilic dermatosis (Sweet’s syndrome) or pyoderma gangrenosum [1, 2, 3, 4, 5, 6], erythema elevatum et diutinum [7, 8]and neutrophilic eccrine hidradenitis [9, 10]. Although the detailed mechanisms of these diseases are unknown, a role of hematopoietic cytokines, such as granulocyte-macrophage colony-stimulating factor (GM-CSF) and granulocyte colony-stimulating factor (G-CSF), has been suggested.…”