Sweet's Syndrome (SS) in the Course of Acute Myeloid Leukaemia (AML)

Abstract

Firstly described by Robert Douglas Sweet in 1964, febrile neutrophilic dermatosis is a disabling, not only cutaneous disorder, clinically characterised by fever and painful erythematous nodules, with a typical background of neutrophilia. Sweet's syndrome (SS) is a chronic inflammatory reactive disorder of unknown cause and incompletely established pathogenesis, although an interplay between genetic and environmental factors, including infections, is likely to occur. A significant part of cases has been demonstrated to be linked with malignancies, especially in the hematologic setting. Because of the underlying disease and related therapeutic measures, SS may present atypical clinical course, whereas the response to treatment is strictly dependent on the concurrent hematologic disease. Herein we describe a case of a lady who had a refractory form of SS, resulted in a paraneoplastic cutaneous disease, and AML. Surprisingly, clinical remission of SS followed cytotoxic chemotherapy while hematologic disorder obtained a further complete response.

Keywords: Acute leukaemia; Complete response; Epirubicin; Sweet syndrome.

Figure 1

Nodular skin lesions localised at legs (a) and thighs (b). Incisional biopsy revealed normal epidermis and dense inflammatory infiltrate in the dermis (c), mainly composed of lymphocytes and histiocytes (d) [Haematoxylin and eosin stain, x40]. The infiltration extended to the subcutaneous adipose tissue (e), showing a pale neutrophilic background (f) [Haematoxylin and eosin stain, x100]