International Journal of Rheumatic Diseases 2013; 16: 777–779


Histiocytoid Sweet syndrome and cutaneous polyarteritis nodosa secondary to myelodysplastic syndrome

Dear Editor, In 1964 R.D. Sweet described a clinical picture affecting young women, characterized by fever, neutrophilia, raised plaques in the limbs, face and neck and a dense neutrophilic infiltrate quickly reversed with corticosteroids.1 This corresponded to the classical form of Sweet syndrome (SS), by far the most common variant (80–90% of cases), but pharmacological, gestational or paraneoplastic types of SS have been described,2 representing the hematologic neoplasms the 85% of the latter group, which has specific clinical and epidemiological characteristics: no female predominance, elderly patient affectation, relapsing clinical course, atypical lesions and frequent extracutaneous involvement.2 On the other hand, histiocytoid SS (HSS), a histologic variant of SS characterized by a mononuclear histiocytoid infiltrate, was described by Requena et al. in 2005.3 Additionally, in 1931, Lindberg first recognized cutaneous polyarteritis nodosa (cPAN), a rare form of vasculitis of unknown etiology limited to skin, affecting small-to-medium-sized arteries.4 We report a patient with HSS and cPAN, both secondary to a myelodysplastic syndrome (MS). A 75-year-old patient presented with a history of polymyalgia rheumatica 5 years before admission, idiopathic pleuropericarditis 3 years before, prostate neoplasm successfully treated with radiation and hormone therapy 1 year before and fluctuating leukopenia, thrombocytopenia and anemia of unknown origin during the last 6 years, with no transfusion requirements until the time of admission. The patient requested a second opinion in our center because of recurrent episodes over the last year of fever (39°C), arthralgias and slightly burning, maculo-papular, erythematous, sharp-edged lesions, affecting arms, face, abdomen and back, with a previous biopsy showing a monocytic infiltrate in superficial and deep dermis. The patient was treated with corticosteroids (prednisone 0.3–0.5 mg/kg/day), initially with a good response, but recurrence when prednisone was tapered below 0.2 mg/

kg/day. For this reason other immunosuppressive drugs (azathioprine and cyclophosphamide) were added to the treatment without improvement. On admission, the patient reported the appearance of sporadic nodules in the anterior and lateral lower extremities over the last 2 years, usually unilateral, of sudden onset and gradual spontaneous resolution, leading to mild residual hyperpigmentation, with previous biopsies showing an intense inflammatory infiltrate in the hypodermis and deep dermis affecting medium vessel walls, associated with segmental areas of fibrinoid necrosis. Physical examination revealed residual nodular lesions in the anterior surface of the legs, and maculopapular lesions on the back, arms and abdomen, scarcely representative. Fever of 39°C and a flare of cutaneous lesions developed immediately after the withdrawal of corticosteroids (Fig. 1). However, the leg nodules remained unchanged.

Figure 1 Maculo-papular, erythematous, sharp edged lesions secondary to histiocytoid Sweet syndrome.

© 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd


Laboratory tests showed a slight pancytopenia (hemoglobin 10.6 g/dL, leukocytes 4.2 9 109/L, neutrophils 2.8 9 109/L, lymphocytes 700 9 109/L, platelets 113 9 109/L), antinuclear antibodies (ANA) 1/320 with homogeneous pattern, and anti-DNA of 17 IU/mL (normal reference values up to 15 IU/L). Virological, serological tests and blood cultures were negative. Thorax and abdominal CT scan showed no abnormalities. A skin biopsy of one of the back lesions showed a perivascular and interstitial infiltrate of medium-sized mononuclear cells in the upper and deep dermis and connective septa of the subcutaneous tissue. The cells showed a vesicular nuclei with scant amphophilic cytoplasm. Immunohistochemical studies were consistent with monomyelocyte lineage with immunoreactivity for CD15, CD43, MAC387, lysozime, CD68/KP1 and myeloperoxidase. Inconspicuous lymphocitic infiltrates were also present and were predominantly composed of T lymphocytes. Biopsy specimen was thought to be consistent with the diagnosis of HSS. Specific cutaneous infiltrates associated with myelogenous leukemia were ruled out. Innmunofluorescence examination of skin was negative for immunoreactants in the walls of the vasculature. Reviews of both the biopsy at the onset of the disease and the most recent one, there were no differences in the hematoxylin-eosin appearance. Review of the initial biopsy of leg nodules in our center showed compatibility with cPAN. A bone marrow aspirate revealed a refractory anemia with excess of blasts type I with an intermediate-1 prognostic index (IPSS). Treatment with azacitidine and corticosteroids (0.2 mg/kg/day) was started, controlling both the hematologic and the skin disease, having just one more flare in the next 10 months, when corticosteroid dosage was decreased below 0.15 mg/kg/day. This flare was controlled after returning to the previous dose. In summary, we report a patient with HSS and cPAN, both secondary to a MS. The fact that the clinical manifestations of MS occurred prior to the development of HSS and cPAN may suggest MS as the origin of the other two diseases. The association between MS and neutrophilic vasculitis is well known, being one of the most frequent skin manifestations of this disease.5 What is not so frequent is the relationship between MS and HSS, probably due to the recent description of HSS.6,7 In a similar way, PAN is among the most common of vasculitis associated with MS8 but cPAN associated with MS has not been reported to date, as far as the authors know.


Cutaneous polyarteritis nodosa was diagnosed following the diagnostic criteria recently proposed by Nakamura et al.9 and systemic PAN was reasonably excluded according to the classification criteria of the American College of Rheumatology. Several autoimmune features have been related with MS.10 Our patient had nodular lesions on the legs histologically and clinically consistent with cPAN. He had been also diagnosed as having polymyalgia rheumatica, and the presence of pleuropericarditis, lymphopenia, high ANA titers and slightly positive anti-DNA suggested lupus erithematosus according to the American College of Rheumatology. We were able to confirm cPAN diagnosis after reviewing the initial biopsy performed in another center. However, clinical data supporting polymyalgia and lupus were taken from the previous clinical chart, so the authors could not confirm these diagnoses. In conclusion, we describe the first case, as far as the authors know, of HSS and cPAN secondary to MS. MS should be suspected in patients with hematologic abnormalities and cutaneous lesions suggestive of cPAN or neutrophilic vasculitis like SS. Iago PINAL-FERNANDEZ,1 Berta FERRER FABREGA, Marc RAMENTOL SINTAS1 and Roser SOLANS LAQUE1 2

Departments of Internal Medicine, and 2 Pathology, Vall d′Hebron Hospital, Passeig de la Vall d’Hebron 119-129, Barcelona, Spain 08035


Correspondence: Dr Iago Pinal-Fernandez, email: [email protected]

REFERENCES 1 Sweet RD (1964) An Acute Febrile Neutrophilic Dermatosis. Br J Dermatol 76, 349–56. 2 Cohen PR (2007) Sweet’s syndrome–a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2, 34. 3 Requena L, Kutzner H, Palmedo G et al. (2005) Histiocytoid Sweet syndrome: a dermal infiltration of immature neutrophilic granulocytes. Arch Dermatol 141(7), 834–42. 4 Lindberg K (1931) Ein Beitrag zur Kenntnis der Periarteritis Nodosa. Acta Med Scand 76(1-2), 183–225. 5 Farah C, Bulai Livideanu C et al. (2010) Prevalence and prognostic value of cutaneous manifestations in patients with myelodysplastic syndrome. J Eur Acad Dermatol Venereol 24(10), 1171–5. 6 Kaiser R, Connolly K, Linker C, Maldonado J, Fye K (2008) Stem cell transplant for myelodysplastic syn-

International Journal of Rheumatic Diseases 2013; 16: 777–779


drome-associated histiocytoid Sweet’s syndrome in a patient with arthritis and myalgias. Arthritis Rheum 59 (12), 1832–4. 7 Ten Oever J, Kuijper PH, Kuijpers AL, Dercksen MW, Vreugdenhil G (2009) Complete remission of MDS RAEB following immunosuppressive treatment in a patient with Sweet’s syndrome. Neth J Med 67 (8), 347–50. 8 Fain O, Hamidou M, Cacoub P et al. (2007) Vasculitides associated with malignancies: analysis of sixty patients. Arthritis Rheum 57(8), 1473–80.

International Journal of Rheumatic Diseases 2013; 16: 777–779

9 Nakamura T, Kanazawa N, Ikeda T et al. (2009) Cutaneous polyarteritis nodosa: revisiting its definition and diagnostic criteria. Arch Dermatol Res 301 (1), 117–21. 10 Giannouli S, Voulgarelis M, Zintzaras E, Tzioufas AG, Moutsopoulos HM (2004) Autoimmune phenomena in myelodysplastic syndromes: a 4-yr prospective study. Rheumatology (Oxford) 43 (5), 626–32.


Histiocytoid Sweet syndrome and cutaneous polyarteritis nodosa secondary to myelodysplastic syndrome.

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