Sweet syndrome

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Sweet syndrome

Karolyn A. Wanat, Joel M. Gelfand and William D. James

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports

(Courtsey of Misha Rosenbach MD.)

Sweet syndrome is a neutrophilic dermatosis, characterized clinically by multiple painful, well-demarcated, non-scarring erythematous plaques or pustules on the face, neck, upper trunk, and extremities. There can be a pseudovesicular appearance. Fever, leukocytosis, arthralgias, myalgias, headaches, and general malaise may occur. Oral, ocular, and internal organ involvement is rare. On histopathology, there is a diffuse neutrophilic infiltrate in the upper dermis without evidence of primary leukocytoclastic vasculitis. Neutrophilic dermatosis of the dorsal hands (NDDH) is classified as an anatomically limited subset of Sweet syndrome.

Management strategy

Sweet syndrome is often idiopathic, but may be associated with malignancy (most commonly acute myelogenous leukemia, but also lymphomas, dysproteinemias, and carcinomas), inflammatory bowel disease, infection (commonly Streptococcus or Yersinia), medication, radiation, and pregnancy. Work-up includes skin biopsy, complete physical examination, and laboratory studies. Treatment of the underlying associated condition or discontinuation of the causative medication may lead to resolution of skin lesions. The standard treatment is corticosteroids. For patients with contraindications to corticosteroids, or for therapeutic failures, second-line therapies may be used.

Specific investigations

History and physical examination

Complete blood count with differential

Erythrocyte sedimentation rate or C-reactive protein

Cultures as indicated by history and physical examination

Pregnancy test (in women of childbearing potential)

Medication history

Sweet’s syndrome revisited: a review of disease concepts.

Cohen PR, Kurzrock R. Int J Dermatol 2003; 42: 761–78.

An excellent review of the literature.

The relationship between neutrophilic dermatosis of the dorsal hands and Sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum.

Walling HW, Snipes CJ, Gerami P, Piette WW. Arch Dermatol 2006; 142: 57–63.

A report of nine cases of NDDH and review of all 43 cases reported.

Histiocytoid Sweet syndrome.

Heymann WR. J Am Acad Dermatol 2009; 61: 693–4.

The term ‘histiocytoid’ refers to the histiologic presence of immature neutrophils; the clinical implications are identical to those of the classical Sweet syndrome.

Chronic recurrent lymphocytic Sweet syndrome as a predictive marker of myelodysplasia.

Vignon-Pennamen MD, Julliard C, Rybojad M, Wallach D, Danie MT, et al. Arch Dermatol 2006; 142: 1170–6.

Nine cases of chronic Sweet syndrome in which the initial biopsies demonstrated a lymphocytic infiltrate with atypical mononuclear cells positive for CD68 and myeloperoxidase, suggesting a myeloid origin; this presentation appeared to be a predictive marker of underlying myelodysplasia.

Necrotizing Sweet syndrome.

Kroshinsky D, Alloo A, Rothschild B, Cummins J, Tan J, Montecino R, et al. J Am Acad Dermatol 2012; 67: 945–54.

Three cases of acute necrotizing neutrophilic dermatosis characterized by deep tissue neutrophilic infiltration and soft tissue necrosis. The authors state that ‘the diagnosis of NSS should be entertained in all cases of suspected necrotizing fasciitis failing to respond to therapy and lacking a causative organism.’

Neonatal Sweet syndrome.

Gray PE, Bock V, Ziegler DS, Wargon O. Pediatrics 2012; 129: e1353–9.

Sweet syndrome in the neonatal period requires thorough investigation as it can be associated with a serious underlying disorder.

First-line therapies

Oral corticosteroids	C
Topical and intralesional corticosteroids	C

Sweet’s syndrome: a review of current treatment options.

Cohen PR, Kurzrock R. Am J Clin Dermatol 2002; 3: 117–31.

A review of the literature regarding treatment for Sweet syndrome.

Corticosteroid therapy (oral prednisone 0.5–1.5 mg/kg daily tapered over 4 to 6 weeks) results in rapid relief of systemic symptoms (within one to two days) and skin lesions within 3 to 9 days. Up to one-third of patients will relapse.

High-potency topical corticosteroids and intralesional corticosteroids may be useful as monotherapy for patients with limited or mild disease, or as adjuvant treatment.

Second-line therapies

Potassium iodide	C
Colchicine	C
Indomethacin	C
Pulse intravenous corticosteroids	D
Dapsone	D
Clofazimine	D
Doxycycline	E
Metronidazole	E

Potassium iodide in dermatology: a 19th century drug for the 21st century – uses, pharmacology, adverse effects, and contraindications.

Sterling JB, Heymann WR. J Am Acad Dermatol 2000; 43: 691–7.

The usual dosage is 40–60 mg orally three times a day up to 300 mg three times a day of potassium iodide. A supersaturated solution of potassium iodide (SSKI) may also be used. Start at three drops orally three times daily and increase by one drop per dose up to 10 drops (500 mg) three times daily until clear, then taper. SSKI is contraindicated in pregnancy, hyperkalemia, and in patients with renal disease. Baseline thyroid function tests and periodic monitoring are required for patients on SSKI.

Long-term suppression of chronic Sweet’s syndrome with colchicine.

Ritter S, George R, Serwatka LM, Elston DM. J Am Acad Dermatol 2002; 47: 323–4.

A case report of successful long-term treatment using colchicine.

Indomethacin treatment of eighteen patients with Sweet’s syndrome.

Jeanfils S, Joly P, Young P, Le Corvaisier-Pieto C, Thomine E, Lauret P. J Am Acad Dermatol 1997; 36: 436–9.

A prospective open-label uncontrolled study of 18 patients treated with indomethacin 150 mg daily for 7 days, followed by 100 mg daily from days 7 to 21. Seventeen of 18 patients responded within 48 hours, and no relapses occurred.

Association of acute neutrophilic dermatosis and myelodysplastic syndrome with (6;9) chromosome translocation: a case report and review of the literature.

Megarbane B, Bodemer C, Valensi F, Radford-Weiss I, Fraitag S, MacIntyre E, et al. Br J Dermatol 2000; 143: 1322–4.

Pulse corticosteroid therapy was successful for refractory and/or recurrent Sweet syndrome. Intravenous methylprednisolone up to 1000 mg daily for 3 to 5 days was used, followed by a low-dose taper of an oral corticosteroid with or without another immunosuppressant.

Sweet’s syndrome and malignancy in the UK.

Bourke JF, Keohane S, Long CC, Kemmett D, Davies M, Zaki I, et al. Br J Dermatol 1997; 137: 609–13.

A review of 87 cases with Sweet syndrome. Dapsone was used successfully as first-line therapy in five of six cases.

Dapsone is used as monotherapy or combination therapy. Initial oral doses range from 100–200 mg daily.

Sweet’s syndrome in association with generalized granuloma annulare in a patient with previous breast cancer.

Anthony F, Holden CA. Clin Exp Dermatol 2001; 26: 668–70.

A report of Sweet syndrome successfully treated with clofazimine.

Clofazimine 200 mg daily for 4 weeks followed by 100 mg daily for 4 weeks has been reported to be an effective dosage regimen in six patients.

Sweet’s syndrome.

Amichai B, Lazarov A, Halevy S. J Am Acad Dermatol 1995; 33: 144–5.

A patient with Sweet syndrome associated with a chlamydial infection responded to tetracycline.

Tetracycline, doxycycline, and minocycline have been effective in Sweet syndrome with and without associated Chlamydia or Yersinia infections. Interestingly, there are several reports of minocycline as a cause of Sweet syndrome.

Sweet’s syndrome in association with Crohn’s disease: report of a case and review of the literature.

Rappaport A, Shaked M, Landau M, Dolev E. Dis Colon Rectum 2001; 44: 1526–9.

A patient successfully treated with metronidazole and prednisone.

Third-line therapies

Cyclosporine	E
Interferon	E
Cyclophosphamide	E
Chlorambucil	E
Etretinate	E
Etanercept	E
Thalidomide	E
Anakinra	E
IVIG	E

Peripheral ulcerative keratitis: an extracutaneous neutrophilic disorder. Report of a patient with rheumatoid arthritis, pustular vasculitis, pyoderma gangrenosum, and Sweet’s syndrome with an excellent response to cyclosporine therapy.

Wilson DM, John GR, Callen JP. J Am Acad Dermatol 1999; 40: 331–4.

One patient with Sweet syndrome responded to cyclosporine.

Initial doses of 2–10 mg/kg daily have been used successfully and should generally be limited to short-term use given its potential renal toxicity when used chronically.

Systemic interferon-alpha treatment for idiopathic Sweet’s syndrome.

Bianchi L, Masi M, Hagman JH, Piemonte P, Orlandi A. Clin Exp Dermatol 1999; 24: 443–5.

One patient responded to interferon-α 3 million units intramuscularly three times weekly plus hydroxyurea 500 mg twice daily, tapered to 500 mg daily for 30 days. The patient maintained remission for 2 years with intramuscular interferon-α as monotherapy.

Lymphocytic infiltrates as a presenting feature of Sweet’s syndrome with myelodysplasia and response to cyclophosphamide.

Evans AV, Sabroe RA, Liddell K, Russell-Jones R. Br J Dermatol 2002; 146: 1087–90.

One patient responded to oral cyclophosphamide 50 mg daily, with an occasional oral prednisolone to alleviate exacerbations.

Treatment of recurrent Sweet’s syndrome with coexisting rheumatoid arthritis with the tumor necrosis factor antagonist etanercept.

Yamauchi P, Turner L, Lowe N, Gindi V, Jackson JM. J Am Acad Dermatol 2006; 54: S122–5.

Two patients with rheumatoid arthritis-associated Sweet syndrome had significant clearing with etanercept.

Thalidomide in the treatment of recalcitrant Sweet’s syndrome associated with myelodysplasia.

Browning C, Dixon J, Malone J, Callen J. J Am Acad Dermatol 2005; 53: S135–8.

A patient with refractory Sweet syndrome cleared completely with thalidomide 100 mg daily.

The use of pulse methylprednisolone and chlorambucil in the treatment of Sweet’s syndrome.

Case JD, Smith SZ, Callen JP. Cutis 1989; 44: 125–9.

Chronic and relapsing Sweet syndrome successfully treated with pulse intravenous methylprednisolone. Remission was maintained with oral chlorambucil 4 mg daily.

Efficacy of anti-interleukin-1 receptor antagonist anakinra (Kineret) in a case of refractory Sweet’s syndrome.

Kluger N, Gil-Bistes D, Guillot B, Bessis D. Dermatology 2011; 222: 123–7.

One of three case reports of Sweet syndrome refractory to multiple medications with a rapid and sustained response to anakinra 100 mg/day as monotherapy.

Pediatric Sweet syndrome and immunodeficiency successfully treated with intravenous immunoglobulin.

Haliasos E, Soder B, Rubenstein D, Henderson W, Morell D. Pediatr Dermatol 2005; 22: 530–5.

An immunodeficient child was successfully treated with IVIG and dapsone. The initial IVIG dose was 1 mg/kg and lowered to 0.5 mg/kg, given at 3-week intervals, with dapsone added for small residual papulopustules.

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Sweet syndrome

Sweet syndrome

Management strategy

Specific investigations

Sweet’s syndrome revisited: a review of disease concepts.

The relationship between neutrophilic dermatosis of the dorsal hands and Sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum.

Histiocytoid Sweet syndrome.

Chronic recurrent lymphocytic Sweet syndrome as a predictive marker of myelodysplasia.

Necrotizing Sweet syndrome.

Neonatal Sweet syndrome.

First-line therapies

Sweet’s syndrome: a review of current treatment options.

Second-line therapies

Potassium iodide in dermatology: a 19th century drug for the 21st century – uses, pharmacology, adverse effects, and contraindications.

Long-term suppression of chronic Sweet’s syndrome with colchicine.

Indomethacin treatment of eighteen patients with Sweet’s syndrome.

Association of acute neutrophilic dermatosis and myelodysplastic syndrome with (6;9) chromosome translocation: a case report and review of the literature.

Sweet’s syndrome and malignancy in the UK.

Sweet’s syndrome in association with generalized granuloma annulare in a patient with previous breast cancer.

Sweet’s syndrome.

Sweet’s syndrome in association with Crohn’s disease: report of a case and review of the literature.

Third-line therapies

Peripheral ulcerative keratitis: an extracutaneous neutrophilic disorder. Report of a patient with rheumatoid arthritis, pustular vasculitis, pyoderma gangrenosum, and Sweet’s syndrome with an excellent response to cyclosporine therapy.

Systemic interferon-alpha treatment for idiopathic Sweet’s syndrome.

Lymphocytic infiltrates as a presenting feature of Sweet’s syndrome with myelodysplasia and response to cyclophosphamide.

Treatment of recurrent Sweet’s syndrome with coexisting rheumatoid arthritis with the tumor necrosis factor antagonist etanercept.

Thalidomide in the treatment of recalcitrant Sweet’s syndrome associated with myelodysplasia.

The use of pulse methylprednisolone and chlorambucil in the treatment of Sweet’s syndrome.

Efficacy of anti-interleukin-1 receptor antagonist anakinra (Kineret) in a case of refractory Sweet’s syndrome.

Pediatric Sweet syndrome and immunodeficiency successfully treated with intravenous immunoglobulin.

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