Sweet’s Syndrome with Myelofibrosis and Leukemia: Partial Response to Interferon

Abstract

Sweet’s syndrome with malignancy or acute neutrophilic dermatosis (AND) is an unusual cutaneous disorder seen most commonly in association with acute myelogenous leukemia. A large majority of patients with AND and malignancy have neoplasms of hematopoietic, plasma cell or lymphoid nature. The patient reported here had myelofibrosis, chronic myelogenous leukemia and Sweet’s syndrome. The individual lesions responded to intralesional interferon-α₂, which has not to our knowledge been reported previously. This result, however, was not as great as the response of AND to intralesional steroid injections.

Sweet's syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis

“…High potency topical corticosteroids (such as 0.05% clobetasol propionate) in either a cream base, an ointment base, a gel base, or a foam base can be applied to the lesions [13,19-21,30,234,362-365]. Individual lesions have improved following a single injection or multiple intralesional treatments with triamcinolone acetonide when used at a dose ranging from 3 mg/ml to 10 mg/ml [362,366,367]. …”

Pyoderma Vegetans

“…This disease is characterized by an abrupt onset of painful bluishred papules or nodules that coalesce and form irregular plaques. 33,[35][36][37] The lesions tend to enlarge over a period of weeks, with eventual resolution and healing without scarring. Recurrence of lesions is common, with about half of the patients experiencing recurrences, most often in previously involved sites.…”

“…32,37 Peripheral leukocytosis with neutrophilia and an elevated white blood cell count are common laboratory findings, although not all patients with Sweet's syndrome show these findings. 32,33,37 Histological features of this disease include edema of the dermal papillae and a dense infiltrate of neutrophils in the dermis without evidence of leukocytoclastic vasculitis. [32][33][34][35]38 Fragmented neutrophil nuclei are also commonly seen.…”

Hyperfunction of neutrophils in a patient with BCR/ABL negative chronic myeloid leukemia

“…However, hyperresponsiveness of myeloid progenitor cells to G-CSF and enhanced neutrophil functions in the terminal phase, including fMLP-stimulated superoxide production and in vitro production of IL-8 might be strongly related to the pathogenesis of PAP as well as Sweet syndrome and be some clues to elucidate at least a part of BCR/ABL negative CML. 24 1998 CML (1) SS Pertusi et al 25 1996 CML (1) SS Kannan et al 26 1995 CML (1) SS Brodkin and Schwartz 27 1995 CML (1) SS Dieguez et al 28 1993 CML (1) SS Torri et al 29 1993 CML (1) SS Feliu et al 30 1992 CML (2) SS Sanchez-Yus and Palou 31 1992 CML (1) SS Gonzalez-Castro et al 32 1991 CML (1) SS Mijovic et al 33 1991 CML (1) SS Elovaara et al 34 1990 CML (1) SS Bello Lopez et al 35 1990 CML (1) SS Cohen and Kurzrock 36 1989 CML (1) SS Hatch et al 37 1989 CML (1) SS Rauh et al 38 1989 CML (1) SS Visani et al 39 1988 CML (1) SS Marcos Sanchez et al 40 1985 CML (1) SS Odeh 41 1981 CML (1) SS Chmel and Armstrong 42 1978 CML (1) SS Rodriguez-Luaces et al 43 1997 CML (1) PAP Cordonnier et al 3 1994 CML (4) PAP Ito et al 44 1994 CML (1) PAP Miyake et al 45 1992 CML (1) PAP Watanabe et al 46 1990 CML (1) PAP Magy et al 47 1985 CML (1) PAP Aymard et al 48 1984 CML (1) PAP Green et al 49 1980 CML (1) PAP Miyashita et al 50 1977 CML (1) PAP Yamamoto et al 51 1975 CML (1) PAP Nakamura et al 52 1988 Graves (1) SS CML: chronic myeloid leukemia; PAP: pulmonary alveolar proteinosis; SS: Sweet syndrome.…”