Journal of Plastic, Reconstructive & Aesthetic Surgery (2013) 66, 867e869

CASE REPORT

Sweets syndrome mimicking alkali burn: A clinical conundrum

E. OHalloran a,*, N. Stewart b, T.P. Vetrichevvel a, S. Rea a,b, F. Wood a,b
a b

Burn Injury Research Unit, School of Surgery, University of Western Australia, Perth, Australia Burn Service of Western Australia, Royal Perth Hospital, Perth, Australia

Received 25 October 2012; accepted 7 November 2012

KEYWORDS
Sweets syndrome; Acute febrile neutrophilic dermatosis; Alkali burn; Skin infection; Upper limb

Summary Introduction: Sweets syndrome or acute febrile neutrophilic dermatosis presents most commonly on the hands, upper extremities and face. The disease is of clinical relevance to surgeons as it could mimic an infective aetiology but debridement negates such wounds. Case: A 34-year-old man was referred from a peripheral hospital with suspected infected alkali burn to the hands. A builder by profession, he had been working outdoors with possible exposure to cement-concrete mix, 5 days previously. At presentation, the dorsal aspect of the thenar eminence appeared erythematous and oedematous, with pustules and blisters with central ulcerations. Haematological investigation revealed a neutrophilic leucocytosis and raised CRP. On the second day of admission the patient became febrile. He was treated with analgesia, IV Tazocin (Pipperacillin and Tazobactam) and the wounds were surgically debrided and covered using autologous cell therapy via the Recell kit. Two days following surgery, microbiology of wound swabs, tissue samples and blood cultures yielded no growth. The wound was noted to be extending beyond the zone of injury and a new area of erythema was evident on the neck. A diagnosis of idiopathic acral Sweets syndrome was conrmed when histopathological investigation showed a moderate inammatory cell inltrate in the dermis. A rapid response to oral corticosteroids was clinically evident after 48 h and the lesions were completely healed at 4 weeks follow-up. Conclusion: We recommend thorough clinical history and examination, systematic wound review, tissue biopsy and culture in conjunction with dermatology opinion in cases of suspected Sweets syndrome. Surgical debridement should be avoided as it has the potential to negate such wounds secondary to pathergy phenomenon. 2012 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

* Corresponding author. Tel.: 61 8 64 888587; fax: 61 8 64 888580. E-mail address: ohalloranemily@gmail.com (E. OHalloran). 1748-6815/$ - see front matter 2012 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.bjps.2012.11.003

868 Sweets syndrome or acute febrile neutrophilic dermatosis presents most commonly on the hands, upper extremities and face. The disease is of clinical relevance to surgeons as it could mimic an infective aetiology but debridement negates such wounds. A 34-year-old man was referred from a peripheral hospital with suspected infected alkali burn to the hands. A builder by profession, he had been working outdoors with possible exposure to cement-concrete mix, 5 days previously. He had been treated empirically with intravenous and oral Flucloxacillin for two days, but the lesions on his hands persisted with associated throbbing pain. At presentation, the dorsal aspect of the thenar eminence appeared erythematous and oedematous, with pustules and blisters with central ulcerations (Figure 1). There was no history of diabetes mellitus, inammatory bowel disease, recent illness or drug intake before the onset of skin lesions. Differential diagnosis included infected alkali burn, allergic contact dermatitis or photosensitivity injury. Haematological investigation revealed a neutrophilic leucocytosis (white cell count 11.6 109/L, neutrophils 7.96 109/L) and raised CRP (12 mg/L). Serum immunoelectrophoresis and rheumatoid factor were unremarkable. Baseline chest radiograph was normal. On the second day of admission the patient became febrile (38.1  C). Considering the timely exposure to cement-concrete mix, the classical distribution of the lesions and the presence of pain rather than itch, a diagnosis of infected alkali burns was made. He was treated with analgesia, IV Tazocin (Pipperacillin and Tazobactam) and the wounds were surgically debrided and covered using autologous cell therapy via the Recell kit.

E. OHalloran et al. Two days following surgery, microbiology of wound swabs, tissue samples and blood cultures yielded no growth, in particular Methicillin resistant Staphylococcus aureus (MRSA) and Vancomycin resistant enterococcus (VRE) were negative. The wound was noted to be extending beyond the zone of injury and a new area of erythema was evident on the neck. Hence, a dermatology opinion was requested. A diagnosis of idiopathic acral Sweets syndrome was conrmed when histopathological investigation showed a moderate inammatory cell inltrate in the dermis. A rapid response to oral corticosteroids was clinically evident after 48 h and the lesions were completely healed at 4 weeks follow-up (Figure 2). Classical Sweets syndrome lesions typically affect women aged 30e50 years after a mild respiratory illness. Lesions start as tender, purple-red papules or nodules commonly on the hands, upper extremities or face, associated with fever. However, the lesions might enlarge and coalesce, with increasing dermal oedema resulting in a pseudo-vesicular appearance,1 mimicking the appearance of wound infection. Other morphological variants of Sweets syndrome like bullous and pustular could also appear like infected burns. Sweets syndrome could be associated with many conditions including malignancy, medications (granulocyte-colony stimulating factor and antibiotic co-trimoxazole), gastro-intestinal infections and inammatory bowel disease.2 Skin lesions may be the rst evidence of a serious underlying disorder. Cutaneous pathergy, a form of skin hypersensitivity in which new skin lesions occur at sites of trauma is common in Sweets syndrome. Pathergy in Sweets syndrome has been reported at sites of venipunture, biopsy, insect bite, thermal burns,3 radiation therapy and previous sunburn.4 Systemic steroids are the gold standard treatment of classical Sweets syndrome, while the prognosis of its variants is based on the underlying aetiology.2 Our patient had a classical morphological and temporal correlation with alkali burns and contact dermatitis at the initial stage. Differentiating early Sweets syndrome from infected irritant contact dermatitis is difcult as both can present with fever, limited skin lesions and a predominant neutrophilic inltrate on biopsy. In addition Sweets syndrome could be initiated or worsened as a pathergy response to the alkali burn. Use of wet cement by amateurs, prolonged contact (approximately 2 h), absence of personal protective

Figure 1 Dorsal view of both hands showing oedematous, erythematous lesions with pustules and central erosions.

Figure 2

Lesions healed at 4 weeks follow-up.

Sweets syndrome mimicking alkali burn equipment and lesions limited to site of contact are usual pointers to alkali burns.5 The diagnosis of Sweets syndrome was agged clinically in this case by delayed appearance of lesions unrelated to contact site, sterile septic screen and rapid clinical response to systemic steroids. Idiopathic aetiology was a diagnosis of exclusion after thorough history, examination and relevant investigations including; faecal occult blood testing, chest X-ray and serum protein electrophoresis. We recommend thorough clinical history and examination, systematic wound review, tissue biopsy and culture in conjunction with dermatology opinion in cases of suspected Sweets syndrome. Surgical debridement should be avoided as it has the potential to negate such wounds secondary to pathergy phenomenon.

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Conict of interest
The authors have no conicts of interest in this case. Of note, Fiona Wood is an inventor of the ReCell device and also a founder of Clinical Cell Culture, the company that developed the ReCell device, and is currently a director of Avita Medical (formerly Clinical Cell Culture).

References
1. Honigsmann H, Cohen PR, Wolff K. Acute febrile neutrophilic dermatosis (Sweet Syndrome). In: Wolff K, Goldsmith L, Katz S, Gilchrest B, Paller A, Leffell D, editors. Fitzpatricks dermatology in general medicine. 6th ed. New York: McGraw-Hill; 2003. p. 1056e60. 2. Cohen PR. Sweets syndrome. Orphanet encyclopedia. Available from: http://www.orpha.net/data/patho/GB/uk-Sweet.pdf; October 2003 [last accessed on 01.08.12]. 3. Stransky L, Broshtilova V. Neutrophilic dermatosis of the dorsal hands elicited by thermal injury. Contact Dermatitis 2003;49:42. 4. Phua YS, Al-Ani SA, She RB, de Chalain TM. Sweets syndrome triggered by scalding: a case study and review of the literature. Burns 2010;36:49e52. 5. Peters WJ. Alkali burns from wet cement. Can Med Assoc J 1984 Apr1;130(7):902e4.

Ethical approval
The patient described in this case gave complete and written consent for photographs to be used for external publication (Figure 1, Figure 2).

Funding
No funding was received for this short report.