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Acute Febrile Neutrophilic Dermatosis Clinical Presentation

  • Author: Yoon-Soo (Cindy) Bae, MD; Chief Editor: William D James, MD  more...
 
Updated: Mar 31, 2016
 

History

Fever typically precedes the appearance of each crop of lesions in acute febrile neutrophilic dermatosis (Sweet syndrome). The fever can precede the skin disease by several days to weeks; however, it may also occur simultaneously.[1] The crop of plaques or nodules in the classic form often appears abruptly and may persist for weeks or several months (days to weeks) if untreated.[1, 4]

Many patients report a febrile upper respiratory tract infection, tonsillitis, or flulike syndrome 1-3 weeks prior to onset of skin lesions. Vaccination or a gastrointestinal tract infection may also precede the eruption.[4, 11] Headache, malaise, and arthralgias are common.[1, 19]

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Physical

Skin manifestations of acute febrile neutrophilic dermatosis (Sweet syndrome)

Typical skin lesions are bright red, reddish blue, or violet papules, plaques, or nodules. Massive subepidermal edema sometimes produces a deceptively vesicular appearance. Lesions may be studded with pustules. Papules often coalesce into circinate or arcuate plaques.[1, 4, 7, 19] Plaques can cause pain and burning, but they are not pruritic. Lesions spontaneously resolve without scarring, or they resolve after treatment.[1] The face, neck, and extremities primarily are affected, characteristically in an asymmetric distribution.[1]

Atypical presentations in the external auditory canal and tympanic membrane are reported. Facial cellulitis and soft tissue infections of the extremities have also been described.[20, 21] Other atypical presentations include photodistributed violaceous plaques with heliotrope rash, malar erythema, scalp involvement, and Gottron-like papules.[22]

Ulcers and bullae are more common in malignancy-associated disease than in other forms. These lesions may be extensive and are generally hard to treat.[4]

Lesions on the dorsum of the hand are not uncommon. They sometimes appear vasculitic, which is not typically seen in Sweet syndrome.[19] The lesions are predominantly distributed over the dorsal aspects of the fingers and hands in a roughly symmetrical pattern. Other extensor surfaces may also be involved.[19] This is an anatomically limited form of Sweet syndrome.[10] Atypical pyoderma gangrenosum, bullous Sweet syndrome, and pustular vasculitis of the hands are considered by some to be variations of a single disease, neutrophilic dermatosis of the dorsal hands.[23] The vasculitis does not appear to be a primary immune-mediated process, as is seen in the primary leukocytoclastic vasculitides; rather, it is secondary vascular damage caused by toxic metabolites and proteases released from the extensive acute neutrophilic infiltrates in the skin.[10]

Note the images below:

Red nodules and plaques on the lateral aspect of t Red nodules and plaques on the lateral aspect of the hand.
Multiple lesions on the scalp. Multiple lesions on the scalp.
Close-up of a scalp lesion. Close-up of a scalp lesion.

Mucosal lesions of acute febrile neutrophilic dermatosis (Sweet syndrome)

Oral lesions can occur on the lips, buccal mucosa, and/or tongue. These lesions most commonly appear as ulcers in Sweet syndrome patients with hematologic disorders.[8] Conjunctivitis and episcleritis may also occur; these are the most common eye manifestations. Other ocular manifestations reported include uveitis, limbal nodules, glaucoma, subconjunctival hemorrhage, scleritis, iritis, and sudden visual loss.[24, 25, 26]

Extracutaneous manifestations of acute febrile neutrophilic dermatosis (Sweet syndrome)

Sweet syndrome can involve several organ systems.[1] Pulmonary manifestations can sometimes lead to substantial morbidity. Pulmonary involvement may manifest as dyspnea, chronic cough, or pulmonary infiltrates or effusions on chest radiograph. In rare cases, symptoms may become severe enough to cause respiratory failure or bronchiolitis obliterans organizing pneumonia. Fortunately, most cases of Sweet syndrome with pulmonary involvement tend to be highly responsive to glucocorticoid therapy.[27, 28, 29]

Other extracutaneous sites that have been reported include the bones, intestines, joints, bone marrow, liver, heart, muscles, spleen, and kidneys.[1, 30, 31, 32, 33, 34, 35, 36, 37, 38]

A few cases of systemic inflammatory response syndrome (SIRS) progressing to shock and organ dysfunction has been reported, with rapid improvement using high-dose intravenous methylprednisolone. The treatment efficacy highlights the association of SIRS with Sweet syndrome, which can be hard to distinguish from infection.[39, 40, 41]

Among children, cases of acquired cutis laxa have been reported in lesions consistent with Sweet syndrome.[42] A 2-year-old girl diagnosed with both Sweet syndrome and cutis laxa was found to have acute necrosis in the cardiac apex and interventricular septum and focal chronic inflammatory and granulation tissue in the aortic wall. The authors suggest that children with both Sweet syndrome and cutis laxa undergo complete cardiac evaluation by a pediatric cardiologist, as these findings can be fatal.[43]

Fewer than 30 cases of CNS involvement are reported in the literature. Encephalitis and meningitis are common neurologic manifestations in these cases. Peripheral neuropathy has also been reported. The most common symptoms are headaches, disturbed consciousness, and seizures.[44] HLA types B54 and CW1 are associated with Sweet syndrome with CNS involvement in Japanese patients.[44] Unlike Behçet syndrome, in which CNS involvement is progressive and severe, Sweet syndrome usually causes transient CNS involvement, but recurrences may occur.[44]

Proteinuria, hematuria, and decreased creatinine clearance have been reported.[16] See the Creatinine Clearance (measured) calculator. Cerebrospinal fluid pleocytosis also has been described, as has a sterile chronic recurrent multifocal osteomyelitis in children.[45]

Pathergy

Like pyoderma gangrenosum, Sweet syndrome is known to cause pathergy (also referred to as Köebner phenomenon), in which lesions occur in areas of minor trauma, such as sites of scratches, bites, and venipuncture.[46] The lesions may also be photodistributed or localized to the site of a previous phototoxic reaction (eg, sunburn).[1, 46]

Underlying disease

It is important to recognize and treat any associated or underlying systemic diseases or malignancies. Sweet syndrome may be a clue to the diagnosis of a systemic disorder or malignancy, and important clues should not be ignored. Imaging studies, such as ultrasonography, CT scanning, PET scanning, or MRI, may be helpful in identifying underlying malignancies.

Pregnancy

Acute neutrophilic dermatosis can occur during pregnancy and may recur in subsequent pregnancies. Presently, it is believed that the disease is not associated with fetal harm.

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Causes

Potential causes of acute febrile neutrophilic dermatosis (Sweet syndrome) are numerous, but some associations are well documented.[5] Classic or idiopathic Sweet syndrome is the most common presentation and accounts for more than 50% of cases.[47] Sweet syndrome associated with a (malignant) neoplasm accounts for approximately 11-54% of the cases. Most of these are hematopoietic malignancies (most commonly acute myeloid leukemia), but some are due to solid tumors, mostly those involving the genitourinary, breast, and gastrointestinal tract. Inflammatory (infectious) conditions are the next most frequently identified causes of Sweet syndrome.[5, 47]

Hematologic malignancy–related causes of febrile neutrophilic dermatosis (Sweet syndrome)

Myelodysplasia and chronic myelogenous leukemia may be associated with Sweet syndrome.[16, 48] Sweet syndrome can also be seen in association with acute myeloid leukemia (AML), including the promyelocytic (M3) variant of AML.[16, 49]

Other nonmyeloid hematologic malignancies that have occurred in association with acute febrile neutrophilic dermatosis include Hodgkin disease, cutaneous T-cell lymphoma, non-Hodgkin lymphoma, hairy cell leukemia, and multiple myeloma. (Patients with immunoglobulin G [IgG] secretion may be at increased risk for Sweet syndrome).[33, 50]

Nonhematologic malignancy has been associated with Sweet syndrome; rates of genitourinary, breast, and gastrointestinal cancers appear to be slightly increased in this group.[16]

Nonhematologic malignancy–related causes of febrile neutrophilic dermatosis (Sweet syndrome)

These are rare associations and include osteosarcoma, oral cancer/tonsil cancer, ovarian cancer, thyroid cancer, lung cancer, pheochromocytoma, and cervical and rectal carcinoma.[9, 50, 51]

Infection-related causes of febrile neutrophilic dermatosis (Sweet syndrome)

Multiple infections are described in association with Sweet syndrome, often involving the upper respiratory tract. Streptococcal pneumonia is the most commonly described infection.[46]

Other bacterial infections associated with Sweet syndrome also include those due to Salmonella or Staphylococcus species, Francisella tularensis, Yersinia enterocolitica, Entamoeba coli, Helicobacter pylori, Borrelia burgdorferi, nontuberculous organisms, (atypical), Tuberculous mycobacteria,Mycobacterium chelonae, and Penicillium species.[46, 52, 53]

Sweet syndrome may be a presenting feature of coccidiomycosis and associated with sporotrichosis.[28, 54] Viral agents such as HIV, cytomegalovirus (CMV), hepatitis A, hepatitis B, and parvovirus B19 has also been implicated.[55, 56]

Yersinia-associated Sweet syndrome has been noted to improve with antibiotics.[57]

Drug-related causes of febrile neutrophilic dermatosis (Sweet syndrome)

Multiple drugs have been reported to cause Sweet syndrome, with some of these reactions being noted in patients with underlying malignancy; therefore, the validity of these possible associations is unclear.[1]

Because the dominant cell in the dermal infiltrate is a neutrophil, drug-induced Sweet syndrome is not considered to be a drug hypersensitivity.

G-CSF is a well-established factor.[1, 58]

Established factors include trimethoprim-sulfamethoxazole (Bactrim), all-trans retinoic acid, and minocycline, which have all appeared in more than 1 case report.[6]

Anecdotal or limited reports of drug or device associations include lithium, furosemide, hydralazine, carbamazepine, oral contraceptives, the Mirena intrauterine device, COX-2 inhibitors, azathioprine, doxycycline, diazepam, diclofenac, nitrofurantoin, propylthiouracil, lenalidomide, bortezomib, abacavir, imatinib, 5-azacytidine, decitabine, interleukin-2 (IL-2), clindamycin, mitoxantrone, and vaccinations (eg, for bacille Calmette-Guérin, smallpox, pneumococcal organisms, influenza).[11, 36, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68]

Systemic disorder–related causes of febrile neutrophilic dermatosis (Sweet syndrome)

Associated inflammatory disease can be identified in about 16% of patients with Sweet syndrome.[47]

The most common associated diseases are Crohn disease and ulcerative colitis, which some authors consider part of a continuum of neutrophilic dermatosis.[69]

Sjögren syndrome, Behçet disease, lupus erythematosus, rheumatoid arthritis, familial Mediterranean fever, and undifferentiated connective-tissue disease have been reported in association with acute neutrophilic dermatitis.[1, 70]

Miscellaneous causes of febrile neutrophilic dermatosis (Sweet syndrome)

Rare cases of acute neutrophilic dermatitis have occurred with spinal surgery, sarcoidosis, erythema nodosum, relapsing polychondritis, thyroiditis, and systemic lupus erythematosus.[1, 71, 72, 73, 74]

A few cases have been observed during pregnancy.[1, 5, 16]

Several cases of Sweet syndrome occurred with polycythemia vera.[75]

One patient had a mutation in the prothrombin gene (G20210A), but no conclusive association can be made at this time.[76]

A few cases of Sweet syndrome have been reported in the literature in transplantation patients, including those undergoing bone marrow and kidney transplantation. Although these patients are taking immunomodulatory medications, an “immune mediated state” has been described to explain the manifestation of Sweet syndrome in this patient population.[77]

Sweet syndrome as a manifestation of HIV-associated immune reconstitution inflammatory syndrome has been suggested.[78]

Diagnostic criteria

Diagnostic criteria (proposed by Su and Liu[79] and revised by von den Driesch[47] ) include the presence of 2 major and 2 minor clinical findings, as follows:

Major criteria are as follows:

  • Abrupt onset of tender or painful erythematous plaques or nodules, occasionally with vesicles, pustules, or bullae
  • Predominantly neutrophilic infiltration in the dermis without leukocytoclastic vasculitis

Minor criteria are as follows:

  • Preceding nonspecific respiratory or gastrointestinal tract infection or vaccination or associated with inflammatory disease, hemoproliferative disorders, solid malignant tumors, or pregnancy
  • Periods of general malaise and fever (body temperature >38°C)
  • Laboratory values during onset showing a erythrocyte sedimentation rate >20 mm, positive C-reactive protein (CRP) result, segmented nuclear neutrophils, bands >70% in peripheral blood smears, and leukocytosis (count >8000/µL) (meeting 3 of 4 of these values is necessary
  • Excellent response to treatment with systemic corticosteroids or potassium iodide

Diagnostic criteria for drug-induced Sweet syndrome (proposed by Walker and Cohen[6] ) include all 5 criteria below; all of the following are required for the drug eruptions to be considered a diagnosis of drug-induced Sweet syndrome:

  • Abrupt onset of painful erythematous plaques or nodules
  • Histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis
  • Pyrexia (38°C)
  • Temporal relationship between drug ingestion and clinical presentation, or temporally related recurrence after oral challenge
  • Temporally related resolution of lesions after drug withdrawal or treatment with systemic corticosteroids [6]
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Contributor Information and Disclosures
Author

Yoon-Soo (Cindy) Bae, MD Clinical Assistant Professor, Ronald O Perelman Department of Dermatology, New York University School of Medicine; Procedural Dermatologist, Laser and Skin Surgery Center of New York

Yoon-Soo (Cindy) Bae, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery, American Medical Association, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Massachusetts Medical Society, Women's Dermatologic Society, New England Dermatological Society

Disclosure: Nothing to disclose.

Coauthor(s)

Sharon A Salter, MD Staff Physician, Department of Psychiatry, Tufts-New England Medical Center

Sharon A Salter, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Obstetricians and Gynecologists

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Timothy McCalmont, MD Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; Editor-in-Chief, Journal of Cutaneous Pathology

Timothy McCalmont, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society of Dermatopathology, California Medical Association, College of American Pathologists, United States and Canadian Academy of Pathology

Disclosure: Received consulting fee from Apsara for independent contractor.

Acknowledgements

Christina N Alavian, MD, Resident Physician, Division of Dermatology, University of Massachusetts Medical School

Disclosure: Nothing to disclose.

Alexa F Boer Kimball, MD, MPH Associate Professor of Dermatology, Harvard University School of Medicine; Vice Chair, Department of Dermatology, Massachusetts General Hospital; Director of Clinical Unit for Research Trials in Skin (CURTIS), Department of Dermatology, Massachusetts General Hospital

Alexa F Boer Kimball, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

References
  1. Cohen PR, Kurzrock R. Sweet's syndrome revisited: a review of disease concepts. Int J Dermatol. 2003 Oct. 42(10):761-78. [Medline].

  2. Sweet RD. An Acute Febrile Neutrophilic Dermatosis. Br J Dermatol. 1964 Aug-Sep. 76:349-56. [Medline].

  3. Vignon-Pennamen MD, Juillard C, Rybojad M, Wallach D, Daniel MT, Morel P, et al. Chronic recurrent lymphocytic Sweet syndrome as a predictive marker of myelodysplasia: a report of 9 cases. Arch Dermatol. 2006 Sep. 142 (9):1170-6. [Medline].

  4. Cohen PR, Kurzrock R. Sweet's syndrome: a neutrophilic dermatosis classically associated with acute onset and fever. Clin Dermatol. 2000 May-Jun. 18(3):265-82. [Medline].

  5. Cohen PR. Pregnancy-associated Sweet's syndrome: world literature review. Obstet Gynecol Surv. 1993 Aug. 48(8):584-7. [Medline].

  6. Walker DC, Cohen PR. Trimethoprim-sulfamethoxazole-associated acute febrile neutrophilic dermatosis: case report and review of drug-induced Sweet's syndrome. J Am Acad Dermatol. 1996 May. 34(5 Pt 2):918-23. [Medline].

  7. Callen JP. Neutrophilic dermatoses. Dermatol Clin. 2002 Jul. 20(3):409-19. [Medline].

  8. Arbetter KR, Hubbard KW, Markovic SN, Gibson LE, Phyliky RL. Case of granulocyte colony-stimulating factor-induced Sweet's syndrome. Am J Hematol. 1999 Jun. 61(2):126-9. [Medline].

  9. Dereure O, Hillaire-Buys D, Guilhou JJ. Neutrophil-dependent cutaneous side-effects of leucocyte colony-stimulating factors: manifestations of a neutrophil recovery syndrome?. Br J Dermatol. 2004 Jun. 150(6):1228-30. [Medline].

  10. Malone JC, Slone SP, Wills-Frank LA, et al. Vascular inflammation (vasculitis) in sweet syndrome: a clinicopathologic study of 28 biopsy specimens from 21 patients. Arch Dermatol. 2002 Mar. 138(3):345-9. [Medline].

  11. Carpentier O, Piette F, Delaporte E. Sweet's syndrome after BCG vaccination. Acta Derm Venereol. 2002. 82(3):221. [Medline].

  12. Kawakami T, Ohashi S, Kawa Y, et al. Elevated serum granulocyte colony-stimulating factor levels in patients with active phase of sweet syndrome and patients with active behcet disease: implication in neutrophil apoptosis dysfunction. Arch Dermatol. 2004 May. 140(5):570-4. [Medline].

  13. Kumar G, Bernstein JM, Waibel JS, Baumann MA. Sweet's syndrome associated with sargramostim (granulocyte-macrophage colony stimulating factor) treatment. Am J Hematol. 2004 Jul. 76(3):283-5. [Medline].

  14. Parsapour K, Reep MD, Gohar K, Shah V, Church A, Shwayder TA. Familial Sweet's syndrome in 2 brothers, both seen in the first 2 weeks of life. J Am Acad Dermatol. 2003 Jul. 49(1):132-8. [Medline].

  15. Tuerlinckx D, Bodart E, Despontin K, Boutsen Y, Godding V, Ninane J. Sweet's syndrome with arthritis in an 8-month-old boy. J Rheumatol. 1999 Feb. 26(2):440-2. [Medline].

  16. Bourke JF, Keohane S, Long CC, et al. Sweet's syndrome and malignancy in the U.K. Br J Dermatol. 1997 Oct. 137(4):609-13. [Medline].

  17. Boatman BW, Taylor RC, Klein LE, Cohen BA. Sweet's syndrome in children. South Med J. 1994 Feb. 87(2):193-6. [Medline].

  18. Barr KL, O' Connell F, Wesson S, Vincek V. Nonbullous neutrophilic dermatosis: Sweet's syndrome, neonatal lupus erythematosus, or both?. Mod Rheumatol. 2009. 19(2):212-5. [Medline].

  19. Cohen PR. Skin lesions of Sweet syndrome and its dorsal hand variant contain vasculitis: an oxymoron or an epiphenomenon?. Arch Dermatol. 2002 Mar. 138(3):400-3. [Medline].

  20. Crum NF, Higginbottom PA, Fehl FC, Graham BS. Sweet's syndrome masquerading as facial cellulitis. Cutis. 2003 Jun. 71(6):469-72. [Medline].

  21. Saliba WR, Goldstein LH, Habib GS, Elias MS. Sweet's syndrome affecting the external auditory canal and tympanic membrane. J Laryngol Otol. 2004 Jan. 118(1):48-9. [Medline].

  22. Owen CE, Malone JC, Callen JP. Sweet-like dermatosis in 2 patients with clinical features of dermatomyositis and underlying autoimmune disease. Arch Dermatol. 2008 Nov. 144(11):1486-90. [Medline].

  23. Walling HW, Snipes CJ, Gerami P, Piette WW. The relationship between neutrophilic dermatosis of the dorsal hands and sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum. Arch Dermatol. 2006 Jan. 142(1):57-63. [Medline].

  24. Levy J, Schneck M, Erlich Y, Lifshitz T. Sweet's syndrome presenting as acute episcleritis. Can J Ophthalmol. 2005 Feb. 40(1):90-2. [Medline].

  25. Kato T, Kawana S, Takezaki S, Kikuchi S, Futagami A. Case of Sweet's syndrome with extensive necrosis and ulcers accompanied by myelodysplastic syndrome. J Nippon Med Sch. 2008 Jun. 75(3):162-5. [Medline].

  26. Song WK, Bang D, Choi YJ, Lee SC, Oh SH. Sudden visual loss due to occlusive venous vasculitis associated with Sweet syndrome. Arch Dermatol. 2009 Feb. 145(2):216-8. [Medline].

  27. Astudillo L, Sailler L, Launay F, et al. Pulmonary involvement in Sweet's syndrome: a case report and review of the literature. Int J Dermatol. 2006 Jun. 45(6):677-80. [Medline].

  28. DiCaudo DJ, Ortiz KJ, Mengden SJ, Lim KK. Sweet syndrome (acute febrile neutrophilic dermatosis) associated with pulmonary coccidioidomycosis. Arch Dermatol. 2005 Jul. 141(7):881-4. [Medline].

  29. Robbins CM, Mason SE, Hughey LC. Sweet syndrome with pulmonary involvement in a healthy young woman. Arch Dermatol. 2009 Mar. 145(3):344-6. [Medline].

  30. Hospach T, von den Driesch P, Dannecker GE. Acute febrile neutrophilic dermatosis (Sweet's syndrome) in childhood and adolescence: two new patients and review of the literature on associated diseases. Eur J Pediatr. 2009 Jan. 168(1):1-9. [Medline].

  31. Fain O, Mathieu E, Feton N, et al. Intestinal involvement in Sweet's syndrome. J Am Acad Dermatol. 1996 Dec. 35(6):989-90. [Medline].

  32. Levin J, Werth VP. Skin disorders with arthritis. Best Pract Res Clin Rheumatol. 2006 Aug. 20(4):809-26. [Medline].

  33. Bayer-Garner IB, Cottler-Fox M, Smoller BR. Sweet syndrome in multiple myeloma: a series of six cases. J Cutan Pathol. 2003 Apr. 30(4):261-4. [Medline].

  34. Shinojima Y, Toma Y, Terui T. Sweet syndrome associated with intrahepatic cholangiocarcinoma producing granulocyte colony-stimulating factor. Br J Dermatol. 2006 Nov. 155(5):1103-4. [Medline].

  35. Dorenkamp M, Weikert U, Meyer R, Schwimmbeck PL, Morguet AJ. Heart failure in acute febrile neutrophilic dermatosis. Lancet. 2003 Oct 25. 362(9393):1374. [Medline].

  36. Marie I, Levesque H, Joly P, et al. Neutrophilic myositis as an extracutaneous manifestation of neutrophilic dermatosis. J Am Acad Dermatol. 2001 Jan. 44(1):137-9. [Medline].

  37. Quilichini R, Mazzerbo F, Baume D, Carsuzaa F, Burtey S. [Sweet's syndrome and aseptic abscess of the spleen]. Rev Med Interne. 1996. 17(12):1029-31. [Medline].

  38. Alper Y, Sprecher E, Bergman R, Birnbaum RF. Sweet's syndrome-like neutrophilic dermatosis resulting from exposure to a radiocontrast agent. J Am Acad Dermatol. 2008 Mar. 58(3):488-9. [Medline].

  39. Shugarman IL, Schmit JM, Sbicca JA, Wirk B. Easily missed extracutaneous manifestation of malignancy-associated Sweet's syndrome: systemic inflammatory response syndrome. J Clin Oncol. 2011 Aug 20. 29(24):e702-5. [Medline].

  40. Otheo E, Ros P, Vázquez JL, et al. Systemic inflammatory response syndrome associated with Sweet's syndrome. Pediatr Crit Care Med. 2002 Apr. 3(2):190-193. [Medline].

  41. Sawicki J, Morton RA, Ellis AK. Sweet syndrome with associated systemic inflammatory response syndrome: an ultimately fatal case. Ann Allergy Asthma Immunol. 2010 Oct. 105(4):321-3. [Medline].

  42. Timmer-DE Mik L, Broekhuijsen-VAN Henten DM, Oldhoff JM, DE Geer DB, Sigurdsson V, Pasmans SG. Acquired cutis laxa in childhood Sweet's syndrome. Pediatr Dermatol. 2009 May-Jun. 26(3):358-60. [Medline].

  43. Guhamajumdar M, Agarwala B. Sweet syndrome, cutis laxa, and fatal cardiac manifestations in a 2-year-old girl. Tex Heart Inst J. 2011. 38(3):285-7. [Medline].

  44. Hisanaga K, Iwasaki Y, Itoyama Y. Neuro-Sweet disease: clinical manifestations and criteria for diagnosis. Neurology. 2005 May 24. 64(10):1756-61. [Medline].

  45. Nobeyama Y, Kamide R. Sweet's syndrome with neurologic manifestation: case report and literature review. Int J Dermatol. 2003 Jun. 42(6):438-43. [Medline].

  46. Fett DL, Gibson LE, Su WP. Sweet's syndrome: systemic signs and symptoms and associated disorders. Mayo Clin Proc. 1995 Mar. 70(3):234-40. [Medline].

  47. von den Driesch P. Sweet's syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol. 1994 Oct. 31(4):535-56; quiz 557-60. [Medline].

  48. Ayirookuzhi SJ, Ma L, Ramshesh P, Mills G. Imatinib-induced sweet syndrome in a patient with chronic myeloid leukemia. Arch Dermatol. 2005 Mar. 141(3):368-70. [Medline].

  49. Melinkeri SR, Gupta RK, Dabadghao S. A Sweet-like syndrome manifesting as gingival hyperplasia and myositis without cutaneous involvement. Ann Hematol. 2002 Jul. 81(7):397-8. [Medline].

  50. Dereure O, Ebrard-Charra S, Guillon F, Baldet P, Guilhou JJ. Sweet's syndrome associated with pheochromocytoma. Dermatology. 2004. 208(2):175. [Medline].

  51. Loehberg L, Simon M. Sweets syndrome: a rare first cutaneous diagnostic sign in cervical cancer. Eur J Dermatol. 2009 Jan-Feb. 19(1):87-8. [Medline].

  52. Neoh CY, Tan AW, Ng SK. Sweet's syndrome: a spectrum of unusual clinical presentations and associations. Br J Dermatol. 2007 Mar. 156(3):480-5. [Medline].

  53. Polat M, Mualla P, Parlak AH, et al. Erythema nodosum and Sweet's syndrome in patients with glandular tularemia. Int J Dermatol. 2011 Jul. 50(7):866-9. [Medline].

  54. Freitas DF, Valle AC, Cuzzi T, et al. Sweet syndrome associated with sporotrichosis. Br J Dermatol. 2012 Jan. 166(1):212-3. [Medline].

  55. Oskay T, Karademir A, Kutluay L. Sweet's syndrome associated with cytomegalovirus infection. Int J Dermatol. 2004 Jan. 43(1):57-9. [Medline].

  56. Fortna RR, Toporcer M, Elder DE, Junkins-Hopkins JM. A case of sweet syndrome with spleen and lymph node involvement preceded by parvovirus B19 infection, and a review of the literature on extracutaneous sweet syndrome. Am J Dermatopathol. 2010 Aug. 32(6):621-7. [Medline].

  57. Escallier F, Gaudard S, Courtois JM, Dalac S, Collet E, Lambert D. [Sweet's syndrome and Yersinia enterocolitica infection]. Ann Dermatol Venereol. 1990. 117(11):858-60. [Medline].

  58. Kemmett D, Hunter JA. Sweet's syndrome: a clinicopathologic review of twenty-nine cases. J Am Acad Dermatol. 1990 Sep. 23(3 Pt 1):503-7. [Medline].

  59. Hamill M, Bowling J, Vega-Lopez F. Sweet's syndrome and a Mirena intrauterine system. J Fam Plann Reprod Health Care. 2004 Apr. 30(2):115-6. [Medline].

  60. Hoverson AR, Davis MD, Weenig RH, Wolanskyj AP. Neutrophilic dermatosis (Sweet syndrome) of the hands associated with lenalidomide. Arch Dermatol. 2006 Aug. 142(8):1070-1. [Medline].

  61. Knoops L, Jacquemain A, Tennstedt D, Theate I, Ferrant A, Van den Neste E. Bortezomib-induced Sweet syndrome. Br J Haematol. 2005 Oct. 131(2):142. [Medline].

  62. Smith KJ, Skelton H. Acute onset of neutrophilic dermatosis in patients after therapy with a COX-2-specific inhibitor. Int J Dermatol. 2003 May. 42(5):389-93. [Medline].

  63. El-Azhary RA, Brunner KL, Gibson LE. Sweet syndrome as a manifestation of azathioprine hypersensitivity. Mayo Clin Proc. 2008 Sep. 83(9):1026-30. [Medline].

  64. Jovanovic M, Poljacki M, Vujanovic L, Duran V. Acute febrile neutrophilic dermatosis (Sweet's syndrome) after influenza vaccination. J Am Acad Dermatol. 2005 Feb. 52(2):367-9. [Medline].

  65. Alencar C, Abramowtiz M, Parekh S, et al. Atypical presentations of Sweet's syndrome in patients with MDS/AML receiving combinations of hypomethylating agents with histone deacetylase inhibitors. Am J Hematol. 2009 Oct. 84(10):688-9. [Medline].

  66. Rondina A, Watson AC. Bullous Sweet's syndrome and pseudolymphoma precipitated by IL-2 therapy. Cutis. 2010 Apr. 85(4):206-13. [Medline].

  67. Kandula S, Burke WS, Goldfarb JN. Clindamycin-induced Sweet syndrome. J Am Acad Dermatol. 2010 May. 62(5):898-900. [Medline].

  68. Kümpfel T, Gerdes LA, Flaig M, Hohlfeld R, Wollenberg A. Drug-induced Sweet's syndrome after mitoxantrone therapy in a patient with multiple sclerosis. Mult Scler. 2011 Apr. 17(4):495-7. [Medline].

  69. Rappaport A, Shaked M, Landau M, Dolev E. Sweet's syndrome in association with Crohn's disease: report of a case and review of the literature. Dis Colon Rectum. 2001 Oct. 44(10):1526-9. [Medline].

  70. Oskay T, Anadolu R. Sweet's syndrome in familial Mediterranean fever: possible continuum of the neutrophilic reaction as a new cutaneous feature of FMF. J Cutan Pathol. 2009 Aug. 36(8):901-5. [Medline].

  71. Propst DA, Bossons CR, Sutterlin CE 3rd. Sweet's syndrome associated with spinal surgical intervention. A case report. Spine (Phila Pa 1976). 1998 Aug 1. 23(15):1708-10. [Medline].

  72. Dadban A, Hirschi S, Sanchez M, Lagrange B. Association of Sweet's syndrome and acute sarcoidosis: report of a case and review of the literature. Clin Exp Dermatol. 2009 Mar. 34(2):189-91. [Medline].

  73. Nakayama H, Shimao S, Hamamoto T, Munemura C, Nakai A. Neutrophilic dermatosis of the face associated with aortitis syndrome and Hashimoto's thyroiditis. Acta Derm Venereol. 1993 Oct. 73(5):380-1. [Medline].

  74. Tabache F, El Kartouti A, Abdelilah T, et al. Systemic lupus erythematosus revealed by sweet syndrome. Joint Bone Spine. 2011 Jul. 78(4):420-1. [Medline].

  75. Mahajan VK, Sharma NL, Sharma RC. Sweet's syndrome from an Indian perspective: a report of four cases and review of the literature. Int J Dermatol. 2006 Jun. 45(6):702-8. [Medline].

  76. Tursen U, Bocekli E, Kaya TI, Dusmez D, Ikizoglu G. Sweet's syndrome in a woman with a prothrombin gene (G20210A) mutation. Int J Dermatol. 2002 Sep. 41(9):596-7. [Medline].

  77. Osband AJ, Laskow DA, Mann RA, Berkowicz T, Ianosi-Irimie M, Boruchoff S. Sweet syndrome after kidney transplantation. Transplant Proc. 2009 Jun. 41(5):1954-6. [Medline].

  78. Haddow LJ, Lehloenya R, Mosam A, Malaka S, Moosa MY. Sweet syndrome: adverse drug reaction or novel manifestation of HIV-associated immune reconstitution inflammatory syndrome?. J Am Acad Dermatol. 2011 Jul. 65(1):e23-5. [Medline].

  79. Su WP, Liu HN. Diagnostic criteria for Sweet's syndrome. Cutis. 1986 Mar. 37(3):167-74. [Medline].

  80. Ayoub N, Charuel JL, Diemert MC, et al. Antineutrophil cytoplasmic antibodies of IgA class in neutrophilic dermatoses with emphasis on erythema elevatum diutinum. Arch Dermatol. 2004 Aug. 140(8):931-6. [Medline].

  81. Krug B, Sonet A, Pirson AS, Mahy N, Bosly A, Borght TV. FDG PET utility in paraneoplastic sweet syndrome. Clin Nucl Med. 2004 Feb. 29(2):91-2. [Medline].

  82. Vignon-Pennamen MD, Aractingi S. Sweet's syndrome and leukemia cutis: a common skin homing mechanism?. Dermatology. 2003. 206(2):81-4. [Medline].

  83. Jeanfils S, Joly P, Young P, Le Corvaisier-Pieto C, Thomine E, Lauret P. Indomethacin treatment of eighteen patients with Sweet's syndrome. J Am Acad Dermatol. 1997 Mar. 36(3 Pt 1):436-9. [Medline].

  84. Maillard H, Leclech C, Peria P, Avenel-Audran M, Verret JL. Colchicine for Sweet's syndrome. A study of 20 cases. Br J Dermatol. 1999 Mar. 140(3):565-6. [Medline].

  85. Amouri M, Masmoudi A, Ammar M, Boudaya S, Khabir A, Boudawara T, et al. Sweet's syndrome: a retrospective study of 90 cases from a tertiary care center. Int J Dermatol. 2016 Mar 9. [Medline].

  86. Aram H. Acute febrile neutrophilic dermatosis (Sweet's syndrome). Response to dapsone. Arch Dermatol. 1984 Feb. 120(2):245-7. [Medline].

  87. Traczewski P, Rudnicka L. Adalimumab in dermatology. Br J Clin Pharmacol. 2008 Nov. 66(5):618-25. [Medline]. [Full Text].

  88. Gupta AK, Skinner AR. A review of the use of infliximab to manage cutaneous dermatoses. J Cutan Med Surg. 2004 Mar-Apr. 8(2):77-89. [Medline].

  89. Haliasos E, Soder B, Rubenstein DS, Henderson W, Morrell DS. Pediatric Sweet syndrome and immunodeficiency successfully treated with intravenous immunoglobulin. Pediatr Dermatol. 2005 Nov-Dec. 22(6):530-5. [Medline].

  90. Yamauchi PS, Turner L, Lowe NJ, Gindi V, Jackson JM. Treatment of recurrent Sweet's syndrome with coexisting rheumatoid arthritis with the tumor necrosis factor antagonist etanercept. J Am Acad Dermatol. 2006 Mar. 54(3 Suppl 2):S122-6. [Medline].

  91. Ambrose NL, Tobin AM, Howard D. Etanercept treatment in Sweet's syndrome with inflammatory arthritis. J Rheumatol. 2009 Jun. 36(6):1348-9. [Medline].

  92. Browning CE, Dixon JE, Malone JC, Callen JP. Thalidomide in the treatment of recalcitrant Sweet's syndrome associated with myelodysplasia. J Am Acad Dermatol. 2005 Aug. 53(2 Suppl 1):S135-8. [Medline].

  93. Kluger N, Gil-Bistes D, Guillot B, Bessis D. Efficacy of anti-interleukin-1 receptor antagonist anakinra (Kineret®) in a case of refractory Sweet's syndrome. Dermatology. 2011. 222(2):123-7. [Medline].

  94. Hashemi SM, Fazeli SA, Vahedi A, Golabchifard R. Rituximab for refractory subcutaneous Sweet's syndrome in chronic lymphocytic leukemia: A case report. Mol Clin Oncol. 2016 Mar. 4 (3):436-440. [Medline].

 
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Red nodules and plaques on the lateral aspect of the hand.
Multiple lesions on the scalp.
Close-up of a scalp lesion.
 
 
 
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