Sudden-Onset, Discrete, Painful Lesions in a 39-Year-Old Man

July 1, 2002
Stephanie Salas, MD
Stephanie Salas, MD

,
Bernard Karnath, MD
Bernard Karnath, MD

A 39-year-old man sought evaluation ofnewly erupted skin lesions on his arms.Ten days earlier he had fallen on hisforearms, but no bruises appeared atthat time.

Figure A

Figure B

A 39-year-old man sought evaluation ofnewly erupted skin lesions on his arms.Ten days earlier he had fallen on hisforearms, but no bruises appeared atthat time.

The multiple discrete, small, red,painful marks on the forearms weregradually enlarging. The patient deniedfever, chills, nausea, vomiting,cough, or sore throat. He took no medications.During the previous month,he had lost 10 lb and become increasinglyfatigued.

The patient was in no acute distress.No lymphadenopathy was detected.The head, ear, eyes, nose, andthroat; neck; oropharynx; cardiovascular;lung; abdominal; and rectal examinationswere unremarkable. Multipleraised erythematous plaques onthe extensor and flexor surfaces of theforearms bilaterally (Figure) werenoted; some of the lesions were tenderto palpation.

The chest film was unremarkable.A complete blood cell count, electrolytelevels, and liver function test resultswere within normal limits. The erythrocytesedimentation rate (ESR) was103 mm/h. A biopsy of one of the lesionsrevealed a dense, dermal, perivascularpolymorphonuclear infiltrate,which confirmed the suspected diagnosisof Sweet syndrome.

A tapering course of an oral corticosteroidwas prescribed; the diseaseresolved in 6 weeks.

In 1964, Robert Douglas Sweet describedan acute febrile neutrophilicdermatosis in 8 women whowere between the ages of 32 and55 years.1 Four cardinal featureswere noted in these patients:

  • Fever.
  • Peripheral neutrophilia.
  • Raised, painful plaques on thelimbs, face, and neck.
  • Histologically dense dermal infiltrationwith mature polymorphonuclearcells.

In recent years, the eponym"Sweet syndrome" has largely replaced"acute febrile neutrophilic dermatosis"because it is now recognizedthat fever and neutrophilia are variablefeatures of the disease, extracutaneousmanifestations are common,and chronic recurrent forms ofthe disorder exist.2

CLINICAL FEATURES
Approximately 500 cases ofSweet syndrome have been reported3;however, frequent anecdotal reports ofthe disorder suggest that the numberof affected patients may be higher.4Typically, 0.5- to 12-cm, raised,erythematous plaques erupt onthe face, neck, upper chest, back, andextremities.3,4 The plaques are painfuland burn; typically, they are not pruritic.3,4 Clinical symptoms, such asarthralgias and uveitis, are variableand nonspecific. An elevated ESRand neutrophilia of at least 70% maybe present.3

CAUSES OFSWEET SYNDROME
Sweet syndrome may be idiopathic,drug-induced, or associated with anunderlying medical disorder.4-7 The idiopathicsyndrome occurs most often;however, the presentation, diseasecourse, and treatment are the same forall forms of the disorder.

The syndrome is a marker ofmalignancy; approximately 10% to20% of patients with the syndromehave cancer. Acute myelogenousleukemia is the most common underlyingmalignancy.5,7,8 Other associatedsystemic disorders includeinflammatory bowel disease, autoimmunedisorders, and infectionscaused by Streptococcus pneumoniae,Salmonella typhi, Yersinia enterocolitica,and other organisms.4-6,9,10

The syndrome may occur aftertrauma-as in our patient-or chemicalirritation of the skin (the Koebnerphenomenon).3 Several drugs havebeen implicated in the disorder;among these are hydralazine, nitrofurantoin,minocycline, trimethoprimsulfamethoxazole,and granulocytecolony-stimulating factor.11-15

WORKUPAND DIAGNOSIS
Perform a thorough assessmentof a patient who presents with lesionsof recent onset that suggest Sweetsyndrome. Obtain an in-depth history,and conduct a careful physical examination;order a complete blood cellcount, peripheral blood smear, andchest radiograph.

Histopathologic examination of aspecimen from a Sweet syndrome lesionusually reveals a dense, dermal,perivascular, polymorphonuclear infiltratewithout vasculitis.1-4Two major and 4 minor diagnosticcriteria were established in 1986.16To make the diagnosis, the 2 majorand 2 of the minor criteria must bepresent.16

The major criteria are:

  • The abrupt onset of tender or painfulplaques or nodules.
  • Neutrophilic dermal infiltrationwithout vasculitis.

The minor criteria include:

  • A preceding fever or infection.
  • A constitutional manifestation, suchas fever, arthralgias, conjunctivitis, ormalignancy.
  • Leukocytosis.
  • A good response to corticosteroidsbut not to antibiotics.

A fifth minor criteria-an elevatedESR-was added in 1989.

17

TREATMENT
Untreated Sweet syndrome lesionsresolve spontaneously within l to3 months; however, corticosteroids-the standard therapy-can amelioratesymptoms and lesions within 2 to 5days.3,7 The usual dose of prednisoneis 0.5 to 1.5 mg/kg/d3,4,17 tapered over4 to 6 weeks to prevent recurrences.7

When the syndrome is drug-induced,discontinue the culprit agentimmediately. Successful treatment ofan underlying disease can resolveSweet syndrome as well. However, ifthe associated disease remains, Sweetsyndrome is likely to recur despitecorticosteroid therapy.

Regardless of an often positiveinitial response to therapy, the disorderrecurs in approximately 30% of patients.17 For those whose disease is refractoryto corticosteroids, second-lineagents-such as dapsone, NSAIDs,and colchicine-may be effective.3,7

References:

REFERENCES:1. Sweet RD. An acute febrile neutrophilic dermatosis.Br J Dermatol. 1964;76:349-356.
2. Gunawardena DA, Gunawardena KA, RatnayakaRM, Vasanthanathan NS. The clinical spectrum ofSweet's syndrome (acute febrile neutrophilic dermatosis):a report of eighteen cases. Br J Dermatol.1975;92:363-373.
3. von den Driesch P. Sweet's syndrome (acutefebrile neutrophilic dermatosis). J Am Acad Dermatol.1994;31:535-560.
4. Fitzgerald RL, McBurney EI, Nesbitt LT Jr.Sweet's syndrome. Int J Dermatol. 1996;35:9-15.
5. Cohen PR, Kurzrock R. Sweet's syndrome andmalignacy. Am J Med. 1987;82:1220-1226.
6. Jordaan HF. Acute febrile neutrophilic dermatosis:a histopathological study of 37 patients and a reviewof the literature. Am J Dermatopathol. 1989;11:99-111.
7. Cohen PR, Talpaz M, Kurzrock R. MalignancyassociatedSweet's syndrome: review of the worldliterature. J Clin Oncol. 1988;6:1887-1897.
8. Deguchi M, Tsunoda T, Yuda F, Tagami H.Sweet's syndrome in acute myelogenous leukemiashowing dermal infiltration of leukemic cells.Dermatology. l997;194:182-184.
9. Zillikens D, Goldstein RK, Elsner P, et al. Sweet'ssyndrome associated with Salmonella typhimuriuminfection. Acta Derm Venereol. 1991;71:77-79.
10. Elsner P, Hartmann AA, Lechner W. Sweet'ssyndrome associated with Yersinia enterocolitica infection.Dermatologica. 1986;173:85-89.
11. Gilmour E, Chalmers RJ, Rowlands DJ. DruginducedSweet's syndrome (acute febrile neutrophilicdermatosis) associated with hydralazine.Br J Dermatol. 1995;133:490-491.
12. Retief CR, Malkinson FD. Nitrofurantoin-associatedSweet's syndrome. Cutis. 1999;63:177-179.
13. Thibaut MJ, Billick RC, Srolovitz H. Minocycline-induced Sweet's syndrome. J Am Acad Dermatol.1992;27:801-804.
14. Walker DC, Cohen PR. Trimethoprim-sulfamethoxazole–associated acute febrile neutrophilicdermatosis: case report and review of drug-inducedSweet's syndrome. J Am Acad Dermatol. 1996;34:918-923.
15. Prevost-Blank PL, Shwayder TA. Sweet's syndromesecondary to granulocyte colony-stimulatingfactor. J Am Acad Dermatol. 1996;35:995-997.
16. Su WP, Liu HN. Diagnostic criteria for Sweet'ssyndrome. Cutis. 1986;37:167-174.
17. von den Driesch P, Gomez RS, Kiesewetter F,Hornstein OP. Sweet's syndrome: clinical spectrumand associated conditions. Cutis. 1989;44:193-200.