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Australasian Journal of Dermatology (2015) 56, e74–e76
doi: 10.1111/ajd.12142
BRIEF REPORTS
Sweet’s syndrome following surgery: Cutaneous trauma as a possible aetiological co-factor in neutrophilic dermatoses Rashi Minocha, Deshan F Sebaratnam and James YJ Choi Department of Dermatology, Westmead Hospital, Westmead, New South Wales, Australia
CASE HISTORY ABSTRACT A 47-year-old man presented with an acute, cutaneous eruption of exquisitely painful papules at the operative site 4 weeks after a right tibial osteotomy. Initially this was managed as a postoperative wound infection; however the exacerbation and spread of the cutaneous eruption prompted further investigation. Histopathology and clinical findings were consistent with the development of Sweet’s syndrome and resolution was obtained after the initiation of dapsone. We propose that surgery may produce an acute inflammatory response in a similar manner to pathergy reactions, which play an aetiological role in other neutrophilic dermatoses, such as pyoderma gangrenosum. We conclude that there may be greater overlap between these neutrophilic dermatoses than previously appreciated. Key words: acute febrile neutrophilic dermatosis, aetiology, neutrophilic dermatosis, pathergy, surgery, Sweet’s syndrome.
INTRODUCTION Sweet’s syndrome (SS) is an uncommon, neutrophilic dermatosis characterised by the acute development of intensely painful cutaneous lesions, pyrexia and neutrophilia.1 We present the first reported case of SS initiated by surgery.
Correspondence: Dr James YJ Choi, Department of Dermatology, Westmead Hospital, Hawkesbury Road, Westmead, NSW 2145, Australia. Email: [email protected] Rashi Minocha, MBBS. Deshan F Sebaratnam, MBBS (Hons) Mmed. James YJ Choi, FRACP FACD. Conflict of interest: none. Submitted 18 October 2013; accepted 25 December 2013. © 2014 The Australasian College of Dermatologists
A 47-year-old man presented to his local medical officer 4 weeks after a right tibial osteotomy with a 1-week history of tender erythematous papules at the operative site, which had evolved to violaceous nodules (Fig. 1). The lesions were associated with malaise, night sweats and febrile episodes to > 38°C. His past medical history was otherwise unremarkable. Serology demonstrated a leucocytosis to 10.1 × 109/L with neutrophils encompassing > 70% of the leucocyte count and a C-reactive protein count of 23.4 mg/L. His blood tests were otherwise unremarkable, apart from the detection of perinuclear anti-neutrophil cytoplasmic antibody; however proteinase 3 and myeloperoxidase antibodies were negative. He received a dose of vancomycin, gentamicin and cephalothin perioperatively. He was referred to his orthopaedic surgeon who initially managed his case as a postoperative wound infection, and he was treated with i.v. flucloxacillin. Blood cultures and wound swabs completed during his admission were negative. He was discharged on cephalexin and flucloxacillin and the lesions resolved over the next fortnight but recurred 4 weeks later on his left elbow and right ankle. Our patient was referred to the infectious diseases department and despite a range of antibiotic regimens he continued to have ongoing flares of intensely painful violaceous nodules in association with fever and significant malaise. After 4 months of treatment and a series of unremarkable imaging findings, infection was deemed unlikely to be the driving force underlying his condition and he was referred for a dermatology review accordingly. A provisional diagnosis of SS was considered and biopsies of the lesions were completed for histological review (Fig. 2). A dense neutrophilic infiltrate was observed extending to the middermis in association with marked dermal oedema. There was extravasation of erythrocytes in association with activated endothelial cells and abundant leucocytoclasis; however, no frank vasculitis was appreciable. His clinical and histological findings were consistent with SS. Further
Abbreviation: SS
Sweet’s syndrome
Sweet’s syndrome following surgery investigations, including a vasculitic screen, blood film, bone scan and colonoscopy found no evidence of haematological or other malignancy, gastrointestinal pathology, infection, connective tissue disease or vasculitis.
e75
Our patient was treated with colchicine (0.5 mg thrice daily); however, he failed to respond after 4 weeks of therapy and was accordingly changed to dapsone (50 mg daily for the first week and 100 mg daily thereafter), which led to the prompt resolution of his disease within 6 weeks of treatment.
DISCUSSION
Figure 1 Sweet’s syndrome. A tender violaceous papule over right knee developed 3-weeks postoperatively. The papule would evolve to a painful nodule with malaise, fever and arthralgias, with similar eruptions occurring distal to the postoperative site in the succeeding weeks.
Surgery has only been implicated previously in two patients with a known history of SS at the surgical site after vertebral fusion2 and the excision of actinic keratosis;3 however it has not yet been reported as an initiating feature of the disease. SS has, however, been reported in association with trauma in the setting of burns4 as well as pathergy reactions following venipuncture, peripheral cannulation5 and skin biopsy.6 The pathophysiology of SS has yet to be elucidated and is thought to be multifactorial. Increased T helper 1 cytokine expression has been implicated as a causative factor and is known to elevate interleukin (IL)-1, IL-2, IL-4 and interferon gamma; which are linked to neutrophilic leukocytosis and macrophage stimulation.7 IL-1 also notably enhances the production of granulocyte colony stimulating factor and granulocyte macrophage colony stimulating factor; cytokines widely recognised as causative in many cases of SS in the oncology literature.8 The prominent inflammatory response observed in pathergy is also thought to be caused by an aberrant T-cell-mediated response, possibly initiated by an autoimmune reaction to antigenically modified tissue caused by cutaneous trauma.9 Pathergy is a well-recognised phenomenon in other neutrophilic dermatoses, such as pyoderma gangrenosum and there may be greater overlap among the neutrophilic dermatoses than previously appreciated.10 In the postoperative setting, alterations to normal tissue anatomy and physiology could liberate self-antigens,
Figure 2 Biopsy of one the lesions demonstrated (a) a dense dermal infiltrate (b) occupying the papillary dermis extending down the mid dermis. (c) Significant dermal oedema was noted with the infiltrate composed of (d) abundant neutrophils with leucocytoclasia and erythrocyte extravasation. © 2014 The Australasian College of Dermatologists
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R Minocha et al.
which are ordinarily sequestered, initiating SS in a manner similar to the acute inflammatory responses observed in pathergy reactions. This process could also occur in other settings, such as cutaneous infection,11 with disturbances associated with the inflammatory processes causing trauma and initiating a similar response. Given the close temporal relationship with his surgery, the initial location of his lesions overlying his surgical site and the absence of any other causative factors, we propose that the SS is likely to have been initiated by his orthopaedic surgery. As our understanding of the pathogenesis of SS and the other neutrophilic dermatoses advances, the role of cutaneous trauma as an aetiological co-factor may be more clearly determined.
3. 4. 5.
6.
7.
8.
9.
REFERENCES 1.
2.
Anavekar NS, Williams R, Chong AH. Sweet’s syndrome in an Australian hospital: a retrospective analysis. Australas. J. Dermatol. 2007; 48: 161–4. Propst DA, Bossons CR, Sutterlin CE. Sweet’s syndrome associated with spinal surgical intervention: a case report. Spine 1998; 23: 1708–10.
© 2014 The Australasian College of Dermatologists
10.
11.
Ramos IS, Wiering CT, Tebcherani AJ et al. Sweet’s syndrome on surgical scar. An. Bras. Dermatol. 2006; 81 (Suppl. 3): 324–6. Phua YS. Sweet’s syndrome triggered by scalding: a case study and review of the literature. Burns 2010; 36: 49–52. Awan F, Hamadani M, Devine S. Paraneoplastic Sweet’s syndrome and the pathergy phenomenon. Ann. Haematol. 2007; 86: 613–4. Bi XL, Gu J, Yan M et al. A case of Sweet’s syndrome with slack skin and pathergy phenomenon. Int. J. Dermatol. 2008; 47: 842–4. Giasuddin AS, El-Orfi AH, Ziu MM et al. Sweet syndrome: is the pathogenesis mediated by helper T cell type cytokines? J. Am. Acad. Dermatol. 1998; 39: 940–3. Bidyasar S, Montoya M, Suleman K et al. Sweet syndrome associated with granulocyte colony stimulating factor. J. Clin. Oncol. 2008; 26: 4355–6. Richard R. Pyoderma gangrenosum after coronary artery bypass grafting. Ann. Thorac. Surg. 1993; 55: 1016–8. Salmon P, Rademaker M, Edwards L. A continuum of neutrophilic disease occurring in a patient with ulcerative colitis. Australas. J. Dermatol. 1998; 39: 116–8. Dinh H, Murugasu A, Gin D. Sweet’s syndrome associated with cellulitis. Australas. J. Dermatol. 2007; 48: 105–9.
Australasian Journal of Dermatology (2015) 56, e74–e76
doi: 10.1111/ajd.12142
BRIEF REPORTS
Sweet’s syndrome following surgery: Cutaneous trauma as a possible aetiological co-factor in neutrophilic dermatoses Rashi Minocha, Deshan F Sebaratnam and James YJ Choi Department of Dermatology, Westmead Hospital, Westmead, New South Wales, Australia
CASE HISTORY ABSTRACT A 47-year-old man presented with an acute, cutaneous eruption of exquisitely painful papules at the operative site 4 weeks after a right tibial osteotomy. Initially this was managed as a postoperative wound infection; however the exacerbation and spread of the cutaneous eruption prompted further investigation. Histopathology and clinical findings were consistent with the development of Sweet’s syndrome and resolution was obtained after the initiation of dapsone. We propose that surgery may produce an acute inflammatory response in a similar manner to pathergy reactions, which play an aetiological role in other neutrophilic dermatoses, such as pyoderma gangrenosum. We conclude that there may be greater overlap between these neutrophilic dermatoses than previously appreciated. Key words: acute febrile neutrophilic dermatosis, aetiology, neutrophilic dermatosis, pathergy, surgery, Sweet’s syndrome.
INTRODUCTION Sweet’s syndrome (SS) is an uncommon, neutrophilic dermatosis characterised by the acute development of intensely painful cutaneous lesions, pyrexia and neutrophilia.1 We present the first reported case of SS initiated by surgery.
Correspondence: Dr James YJ Choi, Department of Dermatology, Westmead Hospital, Hawkesbury Road, Westmead, NSW 2145, Australia. Email: [email protected] Rashi Minocha, MBBS. Deshan F Sebaratnam, MBBS (Hons) Mmed. James YJ Choi, FRACP FACD. Conflict of interest: none. Submitted 18 October 2013; accepted 25 December 2013. © 2014 The Australasian College of Dermatologists
A 47-year-old man presented to his local medical officer 4 weeks after a right tibial osteotomy with a 1-week history of tender erythematous papules at the operative site, which had evolved to violaceous nodules (Fig. 1). The lesions were associated with malaise, night sweats and febrile episodes to > 38°C. His past medical history was otherwise unremarkable. Serology demonstrated a leucocytosis to 10.1 × 109/L with neutrophils encompassing > 70% of the leucocyte count and a C-reactive protein count of 23.4 mg/L. His blood tests were otherwise unremarkable, apart from the detection of perinuclear anti-neutrophil cytoplasmic antibody; however proteinase 3 and myeloperoxidase antibodies were negative. He received a dose of vancomycin, gentamicin and cephalothin perioperatively. He was referred to his orthopaedic surgeon who initially managed his case as a postoperative wound infection, and he was treated with i.v. flucloxacillin. Blood cultures and wound swabs completed during his admission were negative. He was discharged on cephalexin and flucloxacillin and the lesions resolved over the next fortnight but recurred 4 weeks later on his left elbow and right ankle. Our patient was referred to the infectious diseases department and despite a range of antibiotic regimens he continued to have ongoing flares of intensely painful violaceous nodules in association with fever and significant malaise. After 4 months of treatment and a series of unremarkable imaging findings, infection was deemed unlikely to be the driving force underlying his condition and he was referred for a dermatology review accordingly. A provisional diagnosis of SS was considered and biopsies of the lesions were completed for histological review (Fig. 2). A dense neutrophilic infiltrate was observed extending to the middermis in association with marked dermal oedema. There was extravasation of erythrocytes in association with activated endothelial cells and abundant leucocytoclasis; however, no frank vasculitis was appreciable. His clinical and histological findings were consistent with SS. Further
Abbreviation: SS
Sweet’s syndrome
Sweet’s syndrome following surgery investigations, including a vasculitic screen, blood film, bone scan and colonoscopy found no evidence of haematological or other malignancy, gastrointestinal pathology, infection, connective tissue disease or vasculitis.
e75
Our patient was treated with colchicine (0.5 mg thrice daily); however, he failed to respond after 4 weeks of therapy and was accordingly changed to dapsone (50 mg daily for the first week and 100 mg daily thereafter), which led to the prompt resolution of his disease within 6 weeks of treatment.
DISCUSSION
Figure 1 Sweet’s syndrome. A tender violaceous papule over right knee developed 3-weeks postoperatively. The papule would evolve to a painful nodule with malaise, fever and arthralgias, with similar eruptions occurring distal to the postoperative site in the succeeding weeks.
Surgery has only been implicated previously in two patients with a known history of SS at the surgical site after vertebral fusion2 and the excision of actinic keratosis;3 however it has not yet been reported as an initiating feature of the disease. SS has, however, been reported in association with trauma in the setting of burns4 as well as pathergy reactions following venipuncture, peripheral cannulation5 and skin biopsy.6 The pathophysiology of SS has yet to be elucidated and is thought to be multifactorial. Increased T helper 1 cytokine expression has been implicated as a causative factor and is known to elevate interleukin (IL)-1, IL-2, IL-4 and interferon gamma; which are linked to neutrophilic leukocytosis and macrophage stimulation.7 IL-1 also notably enhances the production of granulocyte colony stimulating factor and granulocyte macrophage colony stimulating factor; cytokines widely recognised as causative in many cases of SS in the oncology literature.8 The prominent inflammatory response observed in pathergy is also thought to be caused by an aberrant T-cell-mediated response, possibly initiated by an autoimmune reaction to antigenically modified tissue caused by cutaneous trauma.9 Pathergy is a well-recognised phenomenon in other neutrophilic dermatoses, such as pyoderma gangrenosum and there may be greater overlap among the neutrophilic dermatoses than previously appreciated.10 In the postoperative setting, alterations to normal tissue anatomy and physiology could liberate self-antigens,
Figure 2 Biopsy of one the lesions demonstrated (a) a dense dermal infiltrate (b) occupying the papillary dermis extending down the mid dermis. (c) Significant dermal oedema was noted with the infiltrate composed of (d) abundant neutrophils with leucocytoclasia and erythrocyte extravasation. © 2014 The Australasian College of Dermatologists
e76
R Minocha et al.
which are ordinarily sequestered, initiating SS in a manner similar to the acute inflammatory responses observed in pathergy reactions. This process could also occur in other settings, such as cutaneous infection,11 with disturbances associated with the inflammatory processes causing trauma and initiating a similar response. Given the close temporal relationship with his surgery, the initial location of his lesions overlying his surgical site and the absence of any other causative factors, we propose that the SS is likely to have been initiated by his orthopaedic surgery. As our understanding of the pathogenesis of SS and the other neutrophilic dermatoses advances, the role of cutaneous trauma as an aetiological co-factor may be more clearly determined.
3. 4. 5.
6.
7.
8.
9.
REFERENCES 1.
2.
Anavekar NS, Williams R, Chong AH. Sweet’s syndrome in an Australian hospital: a retrospective analysis. Australas. J. Dermatol. 2007; 48: 161–4. Propst DA, Bossons CR, Sutterlin CE. Sweet’s syndrome associated with spinal surgical intervention: a case report. Spine 1998; 23: 1708–10.
© 2014 The Australasian College of Dermatologists
10.
11.
Ramos IS, Wiering CT, Tebcherani AJ et al. Sweet’s syndrome on surgical scar. An. Bras. Dermatol. 2006; 81 (Suppl. 3): 324–6. Phua YS. Sweet’s syndrome triggered by scalding: a case study and review of the literature. Burns 2010; 36: 49–52. Awan F, Hamadani M, Devine S. Paraneoplastic Sweet’s syndrome and the pathergy phenomenon. Ann. Haematol. 2007; 86: 613–4. Bi XL, Gu J, Yan M et al. A case of Sweet’s syndrome with slack skin and pathergy phenomenon. Int. J. Dermatol. 2008; 47: 842–4. Giasuddin AS, El-Orfi AH, Ziu MM et al. Sweet syndrome: is the pathogenesis mediated by helper T cell type cytokines? J. Am. Acad. Dermatol. 1998; 39: 940–3. Bidyasar S, Montoya M, Suleman K et al. Sweet syndrome associated with granulocyte colony stimulating factor. J. Clin. Oncol. 2008; 26: 4355–6. Richard R. Pyoderma gangrenosum after coronary artery bypass grafting. Ann. Thorac. Surg. 1993; 55: 1016–8. Salmon P, Rademaker M, Edwards L. A continuum of neutrophilic disease occurring in a patient with ulcerative colitis. Australas. J. Dermatol. 1998; 39: 116–8. Dinh H, Murugasu A, Gin D. Sweet’s syndrome associated with cellulitis. Australas. J. Dermatol. 2007; 48: 105–9.
