Abdul Jabbar ( Department of Medicine, The Aga Khan University Hospital, Karachi. )
Acute febrile neutrophilic dermatosis is an uncommon, dramatic skin disease, occurring predominantly in females aged 30 to 60 years1,2. The principal features of Sweet’s syndrome are fever, peripheral blood leucocytosis, multiple raised asymmetric erythematous sharply marginated, painful cutaneous plaques, dense dermal Infiltrate consisting of mature neutrophils and rapid response to steroid treatment. In the majority of patients it is preceded by upper respiratory infection, tonsillitis or flu-like illness by two to three weeks3, Fever and leucocytosis suggest sepsis but the appearance of rash after one to two days should make one think about this differential.
A 45 year old female, known case of chronic myeloid leukemia for four years on hydroxyurea was admitted with fever and erythematous, tender swelling about 12x5 cm on left forearm. She also had mildly erythematosus papular lesions over lateral aspects of both knees and right arm. She was febrile 37.8°C and had hepatosplenomegaly. The initial Investigations revealed a haemoglobm of 12.0 gm/dl, total leucocyte count 12,600/cmm with normal differential and platelet count of 238,000/cmm. Serum electrolytes, PT, APTT, chest x-ray and urine analysis were normal. Peripheral blood smears were negative for malarial parasite on two occasions and four blood cultures did not grow any organism. She was initially diagnosed to have cellulitis and treated with high dose benzyl-penicillin. She continued to spike temperature upto 40°C and complained of nausea, vomiting and severe headache. Her skin lesion on left forearm started to improve but she developed a new erythematosus, markedly indurated tender patch 10x10 cm on right buttock. As the patient was not responding, a punch biopsy was taken from right buttock lesion and she was started on tablet prednisolone 30 mg per day as the lesion clinically looked like erythema nodosum and she did not respond to antibiotics over a week. At the same time her antibiotics were stopped. The lady showed a dramatic response, being afebrile within forty-eight hours and feeling much better. Her lesion resolved in four days time. The skin biopsy revealed features consistent with acute febrile neutrophilic dermatosis (Sweet’s syndrome).
In 1964, Sweet1 first described acute febrile neutrophilic dermatosis and since then more than 130 cases have been reported2. It was initially grouped with erythema nodosum or erythema multiform. The rash appears as populonodular tender lesions which coalesce and evolve over days and weeks3 and later resolve without scarring. They are mostly on face, neck and upper limbs but may also be seen on lower limbs where they resemble erythema nodosum. It is associated with headache, malaise and arthralgia. Inconsistent findings are conjunctivitis or episcleritis2 but these have been noted in over 60 percent of patients in one large series4. An important aspect of Sweet’s syndrome is its association with various systemic conditions In particular acute mycloid leukemia5-9 where It may even precede the hematological malignancy. Our patient was a known case of chronic myeloid leukemia and in retrospect it is important to be aware of this clinical entity to suspect and hence diagnose It. It has also been reported In association with ulcerative colitis1,10 benign monoclonal gammopathy11 and internal malignancies5,12,13 and rheumatoid arthritis14. Histopathologic features are edema of the papillary body and a dense infiltrate of leukocytes (neutrophils) in the lower dermis15. Perivascular foci of leucocytóclasia are quite common and at low magnification may suggest vasculitis15. These patients show prompt response to prednisolone 30-60 mg/d tapered to 10 mg within 2-3 days. They also show dramatic response to potassium iodide 900 mg daily for two weeks. However, recurrence may be seen in upto 50% of patients. Coichicine has also been tried13.
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2. Sweet, RD. An acute febrile neutrophilic dermatosis . 197& Br.J. DermatoL, 1979;100:93-99.
3. Su, W.P. and Liu, H.N. Diagnostic criteria for Sweet\\\'s syndrome. Cutis., 1986;37:167-74.
4. Cohen P.R., Talpaz, M. arid Kurzrock, R. Malignancyassociated Sweet’s syndrome. J. Clin. Oncol., 1988;6:1887-97.
5. Caughman, W., Stem, R. arid Haynes. H. Neutrophilic dermatosis of myeloproliferative disorders. J.Am.Acad. Dermatol., 1983;9:751- 5&
6. Raimer, S.S. and Duncan, C. Pebrile neutrophilic dermatosis in acute myetogenous leukemia. Arch. Dermatol., 1978;114:413-14.
7. Goodfellow, A. and Calvert, H. Sweet’s syndrome and acute myeloid leukemia. Lancet, 1979;2:478-79.
8. Burton, J.L Bullous pyoderma of leukemia. BrJ. DermatoL, 1976;95:209-10.
9. Visani, 0., Patrizi, AL, Ricci, P. et at. Sweet’s syndrome: association with acceterated’ phase of chronic myeloid leukemia. Acts. Haematol. (Basel), 1988;79:207-10.
10. Benton, E.C., Rutherford, D. and Hunter, J.A.A. Sweet’s syndrome and pyoderrna gangrenosum associated with ulcerative colitis. Acta. Derm. Venereol. (Stockh)., 1985;65:77-80.
11. Vestey, J.P., Judge, M. and Savun, J.A. Sweet’s syndrome followed by acute myelornonocytic leukemia. Scott. Mcd.J., 1986;31:184-86.
12. Cooper, P.H., Innes, DJ.and Greer, K.E. Acute Ffebrile neutrophilic dermatosis (Sweet\\\'s syndrome) and myeloproliferative disorders. Cancer, 1983;51:1518-26
13. Suehisa, S., Tagami, H., Inoue, F., Matsumoto, K. and Yoshikuni, K. Coichicine in the treatmentof acute febrile neutrophilicdermatoaia (Sweet’asyndrome). Br.J. Dermatol., 1983;108:99-101.
14. Harary, AM. Sweet’s syndrome associated with rheumatoid arthritis. Arch. Intern. Med., 1983;143:1993-95.
15. Going. 3.3., Going, S.M., Myskow, M.W. and Beveridge, OW. Sweet’s syndrooc histological and imrnuhiatochemical studyof 1$ cases; 3.Clin. Pathot, 1987;40:175-79.