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| CASE REPORT |
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| Year : 2014 | Volume
: 5
| Issue : 2 | Page : 69-70 |
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Sweet's syndrome with multiple myeloma
Khalid Alsaleh
Department of Medicine, Hematology and Oncology Division, College of Medicine, King Saud University, Riyadh, Saudi Arabia
| Date of Web Publication | 19-Jul-2014 |
Correspondence Address:
Khalid Alsaleh
Department of Medicine, Hematology and Oncology Division, College of Medicine, King Saud University, Riyadh
Saudi Arabia
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/1658-5127.137158
We are reporting a case of 70-yr-old gentleman who presented to dermatology clinic with painful red rash which was confirmed to be a sweet's syndrome by biopsy . Further work up showed IgA myeloma. The patient showed good response to systemic and topical steroid. This case illustrate the need of considering paraneoplastic phenomenon in elderly with sweet's syndrome . Sweet's syndrome has been rarely reported as paraneoplastic phenomenon in of multiple myeloma. Keywords: Leukemia, myeloma, Sweet′s syndrome
How to cite this article:
Alsaleh K. Sweet's syndrome with multiple myeloma. J Appl Hematol 2014;5:69-70 |
| Introduction | |  |
Sweet's syndrome is a dermatological condition, which has been attributed to idiopathic cause. A subset of the causes is linked to malignancy or drug reactions. [1] Most of the malignancies have been linked to leukemia and lymphoma. [2] Few cases only has been linked to multiple myeloma. [3]
We report a case, which has been referred to the clinic with minimal hematological abnormality, which turned up to be multiple myeloma.
| Case report | | |
This is a 70-year-old male referred to the hematology clinic from dermatology after he was assessed for progressive pruritic skin rash affecting both hand and forearms for 8 weeks [Figure 1]a. A biopsy was done, which confirmed the presence of dense neutrophilic infiltration for the epidermis with dilated blood vessels with the clinical picture consistent of neutrophilic dermatoses [Figure 1]b. No evidence of vasculitis or granuloma. | Figure 1: (a-c) This case illustrate the need to keep differential of hematological malignancy in case of sweet syndrome as this represent a paraneoplastic process
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Neutrophilic dermatoses are a clinically heterogeneous group of entities characterized histopathologically by the presence of a heavy dermal infiltrate of neutrophils and variable leukocytoclasis. This pattern has a wide differential, including Sweet's syndrome, bowel associated dermatosis, rheumatoid neutrophilic dermatosis, and Behcet's syndrome. [2]
Initial physical exam did confirm the presence of erythematous plaques, which coalesce to form plaques, which was a cover the dorsum of both hands. This was associated with desquamation of the superficial epidermis. This was tender; no other skin lesion has been noticed on the other part of the body. Patient denied any fever or arthralgia and he reported good general condition. Patient reported no symptoms of fatigue or bone ach.
Initial workup showed normal complete blood count with hemoglobin level of 90 g/L (normal 16.5-12.5), there was evidence of rouleaux on the peripheral blood smear. There was evidence of hypercalcemia with level up to 2.73 mm/L (normal 2.10-2.55). Serum protein electrophoresis showed monoclonal peak with immunoglobulin A level at 44.5 g/L (normal 0.7-3.12).
A bone marrow was done and confirmed the presence of excess mature plasma cells with 50% of the bone marrow with some in abnormal collection. That is interstially distributed with small areas of sheets pattern. Immunohistochemistry showed that plasma cells are positive for CD38, CD138, CD56 (partially positive) and lambda light chain [Figure 1]c. They are negative for CD20 and kappa light chain. No Bence-Jones protein was demonstrated. The skeletal survey confirmed the presence of lytic lesion in L5 and the pelvic bones.
Patient was started on treatment in the form of bortezomib and dexamethasone. He was also provided with dexamethasone cream to his skin lesion, there was a significant improvement after 2 weeks of the start of the treatment.
| Discussion | | |
Sweet's syndrome has been described for the 1 st time by Robert Sweet as a case of skin rash, neutrophilic infiltration and fever. Later on many reports have has been linked to malignancy, in the larges retrospective series malignancy was linked in 16% of the cases mostly hematological, of interest malignancy developed up to 1 year after the initial presentation in few cases. [3] Many cases improves with treatment of the underlining condition, of interest some cases of Sweet syndrome has been reported after treatment with bortezomib. [4]
Sweet syndrome presenting with painful plaque and classically associated with fever, arthralgia and leukocytosis, that's why the other term for Sweet syndrome, is acute febrile neutrophilic dermatosis. Less than 35 cases reported with associated multiple myeloma. [5] The pathophysiology has not been clear. A correlation with increased production of granulocyte colony-stimulating factor, which lead to increased recruitment of neutrophils and possible explaining the fever and skin rash. [6],[7] Overall many hematological malignancies has been linked to increased cytokines production. [6]
This case illustrate the need to keep differential of hematological malignancy in case of Sweet syndrome as this represent a paraneoplastic process. [3]
| References | | |
| 1. | Wolff K, Johnson R. Acute febrile neutrophilic dermatosis (Sweet syndrome). Ch. 32. Fitzpatricks Color Atlas and Synopsis of Clinical Dermatology. McGraw-Hill Professional. New York, USA; 2013. p. 1104. 
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| 2. | Cohen PR, Kurzrock R. Sweet′s syndrome revisited: A review of disease concepts. Int J Dermatol 2003;42:761-78.
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| 3. | Bourke JF, Keohane S, Long CC, Kemmett D, Davies M, Zaki I, et al. Sweet′s syndrome and malignancy in the U.K. Br J Dermatol 1997;137:609-13.
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| 4. | Knoops L, Jacquemain A, Tennstedt D, Theate I, Ferrant A, Van den Neste E. Bortezomib-induced Sweet syndrome. Br J Haematol 2005;131:142.
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| 5. | Bayer-Garner IB, Cottler-Fox M, Smoller BR. Sweet syndrome in multiple myeloma: A series of six cases. J Cutan Pathol 2003;30:261-4.
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| 6. | Paydas S, Sahin B, Seyrek E, Soylu M, Gonlusen G, Acar A, et al. Sweet′s syndrome associated with G-CSF. Br J Haematol 1993;85:191-2.
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| 7. | Reuss-Borst MA, Pawelec G, Saal JG, Horny HP, Müller CA, Waller HD. Sweet′s syndrome associated with myelodysplasia: Possible role of cytokines in the pathogenesis of the disease. Br J Haematol 1993;84:356-8.
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[Figure 1]
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