CITATION: Jordaan, H.F., De Goede, F.H. & Sandler, M. 1989. Acute febrile neutrophilic dermatosis (Sweet's syndrome). A report of 2 cases .  S Afr Med J, 75(4):336-338.The original publication is available at http://www.samj.org.zaENGLISH ABSTRACT: Two cases of Sweet's syndrome are described. The diagnostic criteria, clinical spectrum, complications, pertinent differential diagnoses and treatment modalities of this relatively rare clinical condition are described. The association of Sweet's syndrome with underlying haematological malignant disease is stressed.Publisher’s versio

Jordaan, H. F.

De Goede, F. H.

Sandler, M.

Раздел II. «Культурология. Журналистика. Лингвострановедение и современные иностранные языки

Филимонова, О. В.

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Word coinage and ways of forming neologisms

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336 SAMT VOL 75 1 APR 1989Acute febrile neutrophilic dermatosis(Sweet's syndrome)A report of 2 c~sesH. F. JORDAAN, F. H. DE GOEDE, M. SANDLERSummary·Two cases of Sweet's syndrome are described. The diagnosticcriteria, clinical spectrum, complications, pertinent differentialdiagnoses and treatment modalities of this relatively rareclinical condition are described. The association of Sweet'ssyndrome with underlying haematological malignant diseaseis stressed.S Air Med J 1989; 75: 336-338.In 1964 Sweet l reported 8 cases of a condition he called acutefebrile neutrophilic dermatosis; this later became known asSweet's syndrome. A diagnosis of Sweet's syndrome involvessatisfying the 2 major criteria and at least 2 of the minorcriteria.2 The major criteria include: (I) the abrupt onset of acutaneous eruption consisting of tender erythematous or viola-ceous plaques or nodules confined predominantly to sun-exposed areas; and (it) neutrophilic infiltration in the dermiswithout evidence of a leucocytoclastic vasculitis. Minor criteriainclude: (I) a fever or infection which antedates the onset ofthe skin eruption; (il) fever, polyarthralgia, conjunctivitis,haematuria and/or proteinuria accompanying the skin lesions;(iii) leucocytosis or underlying haematological malignantdisease; and (iv) a good clinical response to systemic steroidsin a lesion previously unresponsive to antibiotic therapy.Two cases of Sweet's syndrome, 1 accompanied by fatalhaematological malignant disease, are described.CaSe reportsCase 1A 40-year-old woman presented to hospital with suddenonset of fever, diffuse myalgia, polyarthralgia and the develop-ment of painful skin lesions over the anterior chest wall andshoulders which later spread to involve the dorsal surfaces ofthe arms and legs. One month before, the patient had beentreated for an upper respiratory tract infection.On examination the patient was acutely ill, pyrexial and hada marked skin eruption involving the anterior and posteriorthorax. This consisted of red papules and nodules approxi-mately 0,5 - 2· cm in diameter, some of which had centralvesicle formation (Fig. 1). Evidence of polyartbralgia wasmanifested by a reduced range of movement and tenderness toDepartments of Dermatology and Internal Medicine,University of Stellenbosch and Tygerberg Hospital, Parow-vallei, CPH. F. JORDAAN, M.B. CH.B., M.MED. (DERM.)F. H. DE GOEDE, M.B. CH.B.M. SANDLER, M.B. CH.B., PHD., M.R.C.P.Reprint requests to: Dr M. Sandler, Dept of Internal Medicine, TygerbergHospital, PO Box 03, Tygerberg, 7505 RSA.Accepted 10 Nov 1988.Fig. 1. Case 1 - red papules and nodules on the upper chestwall.palpation of the following joints: shoulders, elbows, wrists,proximal interphalangeal, knees and ankles. Cardiovascular,abdominal and neurological examinations revealed no abnorma-lities.Special investigations: A full blood count showed haemo-globin 11,9 gldl, white cell count 13 x 109/1 with 85% neutro-phils, and erythrocyte sedimentation rate (ESR) 120 mm/1st h(Westergen). Urinalysis revealed haematuria, proteinuria,hyaline and granular casts. The following tests were eithernormal or negative: ECG, chest radiography, blood culture,protein electrophoresis, serology for viral and rickettsial infec-tions, and collagen vascular disorder investigations which werenegative for antinuclear and rheumatoid factors. Examinationof biopsy specimens of the skin lesions revealed typical neutro-philic infiltrates without evidence of vasculitis; these wereindicative of Sweet's syndrome (Fig. 2).Fig. 2. Case 1 - histological section of a skin lesion showingspongiosis, sub-epidermal oedema and a nodular infiltrate ofIymphocytes, mature neutrophils and nuclear dust.Although the polyanhralgia improved slightly with the initialtreatment of intramuscular injections of diclophenac sodium,once corticosteroids were started (prednisone 60 mg daily) thepatient responded within 72 hours and the fever, skin lesions,polyarthralgia and myalgia abated. The steroids were graduallytapered off over the next month by which time the patient wasasymptomatic and the skin lesions had completely cleared.The patient was followed up for a further month, found to beasymptomatic and then .returned home to the Netherlands.She was subsequently lost to further follow-up.ease 2A 73-year-old man presented to hospital with a 4-weekhistory of intermittent fever, malaise, progressive loss of weightand the development of painful skin lesions on the side of theneck. He had no significant past medical history. Treatmentby his general practitioner with tetracycline 250 mg 4 timesdaily had not altered the clinical course or affected the skinlesions and he was therefore referred to Tygerberg Hospitalfor further evaluation.On examination the patient was apyrexial, clinically anaemicwith the presence of generalised lymphadenopathy, especiallyprominent in both axillae. Examination of the skin revealedthe presence of two reddish-purple firm non-tender lesionsapproximately 3 x 3 cm in diameter on the right side of theneck. Both lesions showed evidence of early blister formation(Fig. 3). Examination showed that the cardiovascular, respira-tory, abdominal and neurological ·systems were normal. A fullblood count revealed the presence of a pancytopenia; the ESRwas 140 mm/1st h (Westergren). The following investigationswere normal or negative: urinalysis, blood chemistry, repeatedblood cultures, liver function and chest radiography. Bonemarrow aspirate and trephine biopsy showed the presence ofacute myeloid leukaemia, which had developed against a back-ground of myeloid dysplasia. Skin biopsy showed the presenceof diffuse neutrophilic infiltration in the dermis withoutevidence of leucocytoclastic vasculitis. No immature cells werenoted. A diagnosis of Sweet's syndrome was made in associa-tion with an underlying haematological malignant disease.Despite extensive therapy with fresh whole blood, granulocyteand platelet support in addition to numerous intravenousantibiotic regimens, the patient's course was that of rapidlyprogressive deterioration and subsequent death.DiscussionThese 2 cases were chosen for discussion because: (I) bothcases adequately fulfil the criteria for the diagnosis of Sweet'ssyndrome as set out by Su and Liu;2 (il) they emphasise therange of skin lesions found in Sweet's syndrome (case 1 -multiple, small, tender red nodules on sun exposed areas; case2 - 2 large violaceous non-tender nodules); and (iil) theassociation of Sweet's syndrome with underlying haemato-logical malignant disease is highlighted.The majority of cases of Sweet's syndrome described thusfar have involved women aged between 30 years and 60 years.However, cases involving children3 and· men4 have beenreported. Although in most cases the aetiology was idiopathic,the associations with Sweet's syndrome have included, amongothers, bronchiectasis,5 lupus erythematosus,6 rheumatoidarthritis,? sub-acute thyroiditis,S DressIer's syndrome,9 andBehc;:et's disease. lo However, the most important associationwith Sweet's syndrome is undoubtedly the presence of under-lying malignant disease. In this context it must be noted thatof all the cases of Sweet's syndrome described to date, 10-12%were associated with malignant disease. II These authorsSAMJ VOL 75 1 APR 1989 337Fig. 3. Case 2 - two reddish purple tumours on the side of theneck.reported 40 cases of Sweet's syndrome in aSSOCiation withmalignant disease; 35 of these patients had evidence of a blooddyscrasia and the remaining 5 had solid neoplasms. In almost50% of the patients with haematological malignant disease thediagnosis was acute myeloid leukemia. 11 Over the past decadeSweet's syndrome, atypical Sweet's syndrome, pyodermagangrenosum and atypical pyoderma gangrenosum have beenreported in association with various forms of leukaemia. Theseconditions most probably represent points on a continuum ofnon-infectious non-metastatic inflammatory neutrophilicdermatoses, which occur in patients with derangements inmyeloid cell proliferation. 12 Since the clinical picture of Sweet'ssyndrome includes the presence of a fever, polyarthralgia,diffuse myalgia, conjunctivitis, painful skin rashes and a leuco-cytosis, the most relevant conditions to be considered in thedifferential diagnosis must include acute systemic infections(bacterial, rickettsial), collagen vascular disorders and acuteblood dyscrasias. Successful treatment regimens for Sweet'ssyndrome have included the use of potassium iodide,13 colchi-cine,14 dapsone l5 and indomethacin. 16 However, short coursesof high-dose corticosteroids are considered to be the treatmentof choice.4 The suggested dosage regimen involves a startingdose of 1 mg/kg/d which is gradually reduced in step-wisefashion over a period of 10-14 days. Although recurrenceshave been reported to occur in approximately 30% of casesowing to inadequate therapy,4 in our experience (40 cases over7 years) the relapse rate after completion of steroid therapy onthe above dosage regimen occurred in less than 10% of cases.338 SAMT VOL 75 1 APR 1989We have described 2 cases which fulfil the criteria of Sweet'ssyndrome and, to our knowledge, are the first in South Africa todocument a case of acute febrile neutrophilic dermatosis in asso-ciation with acute myeloid leukaemia.REFERENCESI. Sweet RD. An acute febrile neutrophilic dermatosis. Br] Dermtlloll964; 76:349-356.2. Su WPD, Liu HH. Diagnostic criteria for Sweet's syndrome. GuIis 1986; 37:167-174.3. Kibbi AG, Zaynoum ST, Kurban AK, Najjar SS. Acute febrile neutrophilicdermatosis (Sweet's syndrome): case report and review of the literature.Pediarr Dermatol 1985; 3: 40-44.4. Storer JS, Nesbin LT, Galen WK, DeLeo VA. Sweet's syndrome. Int]Dermaroll983; 22: 8-12.5. Sweet RD. Further observations on acute neutrophilic dermatosis. Br ]Dermarol 1968; 80: 800-805.6. Goene DK. Sweet's syndrome in subacute lupus erythematosus. ArchDermarol 1985; 121: 789-791.7. Harary AM. Sweet's syndrome associated with rheumatoid arthritis. ArchIntern Med 1983; 143: 1993-1995.8. AIcalay J, Filhaber A, David M, Sandbank M. Sweet's syndrome andsubacute thyroiditis. Dermarologica 1987; 174: 28-29.9. Rodriquez-cte la Sema A, Domingo-Pedrol P, Biancb-Torra L, Perez-PerezA, Obrador-Mayol D. Acute febrile neutrophilic dermatosis (Sweet'ssyndrome) associated with post-myocardial infarction syndrome (Dressier'ssyndrome). Arch Intern Med 1985; 145: 1522-1524. •10. Mizoguchi M, Chikakane K, Goh K, Asahina Y, Masuda K. Acute febrileneutrophilic dermatosis (Sweet's syndrome) in Behc;er's disease. Br ]Dermaroll987; 116: 727-734.11. Cohen PR, Kurzrock R. Sweet's syndrome and malignancy. Am] Med 1987;82: 1220-1226.12. Caughman W, Stem R, Haynes H. Neutrophilic dermatosis of myeloproli-ferative disorders: atypical forms of pyoderma gangrenosum and Sweet'ssyndrome associated with myeloproliferative disorders.] Am Acad Dermarol1983; 9: 751-758.13. Horio T, Inamura S, Dunro K, Furukawa F, Ofuji S. Treatment of acutefebrile ne!'trophilic dermatosis (Sweet's syndrome) with potassium iodide.Dermatologica 1980; 160: 341-347.14. Suehiza S, Tagami H, Inoue F, Matsumoto K, Yoshikuni K. Colchicine inthe treatment of acute febrile neutrophilic dermatosis (Sweet's syndrome).Br] Dermaroll983; 108: 99-101.15. Aram H. Acute febrile neutrophilic dermatosis (Sweet's syndrome): responseto dapsone. Arch Dermarol1I984; 120: 245-247.16. Hoffman GS. Treatment of Sweet's syndrome (acute febrile neutrophilicdermatosis) with indomethacin.] RheumarolI977; 4: 201-206.Pulmonary oedema from a widow spider biteA case reportL. H. VISSER, S. N. KHUSISummary Case reportThe clinical course of a patient biUen by a widow spider(Latrodectus indistinctus Pickard-Cambridge (Araneae:Theridiidae» is described. Although this spider is common insouthern Africa, c.ase reports are rare. To date details of thedevelopment of pulmonary oedema after a bite have not beenpublished in South Africa.S Atr Med J 1989; 75: 338-339.Spiders are ubiquitous in southern Africa; however, mostspider bites are either unrecognised or require little medicalattention. A recent review! described the four spiders knownto be medically important in southern Africa. They are dividedinto two categories according to the action of their venom -neurotoxic and cytotoxic.The widow spider (Latrodectus indistincIUs Pickard-Cambridge (Araneae: Theridiidae)) belongs to the neurotoxicgroup.!The signs and symptoms that appeared in a healthy youngwoman after she had been bitten by L. indistinctus are described.The development of pulmonary oedema was an unusual com-plication; it has oilly been described once before.2Tshilidzini Hospital, PO Shayandima, VendaL. H. VISSER, ARTS EXAMEN NEDERLANDS. N. KHUSI, M.B.CH.B.Accepted 8 Aug !988On 18 January 1988 a 24-year-old black woman was bittenon the left thigh by a spider. Severe pain was immediatelyexperienced at the bite site. On the way to hospital she feltnauseous and vomited once.On arrival at Tshilidzini Hospital the patient appeared veryanxious and complained of generalised body pain and weak-ness. The blood pressure was 200/140 mmHg, pulse 88/minand temperature 36,6°C. No cellular reaction was seen at thebite site. The remainder of the physical examination wasunremarkable. She was admitted for observation. Mepyrarninemaleate 50 mg and pethidine 50 mg were administered intra-muscularly.Over the next 24 hours the blood pressure normalised(120/80 mmHg), but the general malaise persisted and thepatient developed a continuous headache, periods of shiveringand profuse sweating. Intravenous fluids were then given.Laboratory investigations showed a negative malaria smearand a white cell count of 15,3 x 109/1.On the afternoon of 19 January the patient complained ofdifficulty in breathing with coughing of frothy sputum. Onexamination she appeared to be tachypnoeic and had bilateralbasal crepitations on auscultation.A chest radiograph showed obvious lung oedema. By thattime a total amount of 450 ml of Ringer's lactate had beengiven intravenously. Urine output was good and there were nosigns of cardiac failure. Immediate treatment with morphine10 mg intramuscularly and aminophylline 250 mg slowly wasstarted. Oxygen was administered and furosemide 40 mg wasgiven intravenously 8 hourly.

Acute febrile neutrophilic dermatosis (Sweet's syndrome). A report of 2 cases

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Acute febrile neutrophilic dermatosis (Sweet's syndrome). A report of 2 cases

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