Neutrophilic Pustulosis Associated with Chronic Myeloid Leukemia: A Special Form of Sweet’s Syndrome

Abstract

Two subjects with Ph-positive chronic myeloid leukemia (CML) in whom pustular Sweet’s syndrome was diagnosed are reported. The first patient was a 47-year-old woman who developed fever, painful ulcers of the oral mucosa and vagina and generalized pustulous skin lesions 2 years after the diagnosis of CML. Histologically, the skin lesions consisted of dense neutrophilic infiltrates with perifollicular disposition. The microbiologic studies were negative. The lesions showed a favorable response to corticosteroids, but fever recurred with every attempt of tapering prednisone; it finally disappeared with the addition of oral cyclophosphamide. The second patient was a 45-year-old man who developed fever and disseminated pustules with histologic features consistent with Sweet’s syndrome and negative microbiologic studies at 2.5 years after diagnosis of CML. The picture showed a dramatic response to prednisone and did not recur after the drug was discontinued. In both patients, CML remained stable after resolution of Sweet’s syndrome.

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

“…In the presented cases, many acneiform papules and pustules were also seen. A similar induction of pustular lesions is observed in neutrophilic dermatoses associated with bowel inflammatory diseases [15]or malignant hemopathies [16]. In our cases, such pustular lesions may have been exacerbated by physical pressure forming dome-shaped abscesses.…”

Clinical features and outcomes of Sweet's syndrome associated with non‐tuberculous mycobacterial infection and other associated diseases

“…11 Cutaneous lesions of Sweet's syndrome typically appear as painful red papules, nodules and plaques, pseudo-vesicles, or variants such as pustules or ulcers. 23 The pustular lesions in Sweet's syndrome may be found in association with chronic myeloid leukemia, 23 inflammatory bowel disease, 24 group D Salmonella cervical lymphadenitis 25 and NTM infection. 9,13 Even though it was not statistically significant, pustular lesions were seen more commonly in Sweet's syndrome with NTM infection than in the other associated diseases group (58.3% vs 40.0%).…”

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.…”