Ulcerative Sweet syndrome accompanied by interstitial lung disease and myelodysplastic syndrome

ARTICLE

Auteur(s) : Hong-Hui Xu, Ting Xiao, Xing-Hua Gao, Hong-Duo Chen

Department of Dermatology, No. 1 Hospital of China Medical University, 155 North Nanjing Street, Shenyang 110001, China

Sweet syndrome (SS) is a reactive neutrophilic dermatosis characterized by tender plaques, nodules, fever and neutrophilia. SS is sometimes associated with medication, autoimmune diseases, malignancies and myelodysplastic syndrome (MDS) [1, 2]. MDS-associated SS may present with cutaneous ulceration and necrosis. Extracutaneous involvement includes lungs, muscle, heart, eyes and the central nervous system.

A 49-year-old Chinese man presented with a 4-week fever (39.5 °C), dyspnea, upper lip swelling and extensive tender plaques and nodules with ulceration. After a 3-week unsuccessful treatment of antibiotics and topical ointments, he was admitted. His past history included 5-year anemia and 2-year MDS-refractory anemia (MDS-RA) diagnosed by bone marrow examination and he had been treated with thalidomide, stanozol and transfusions. Physical examination revealed erythematous plaques around the swollen upper lip (figure 1A) and at injection sites; ulcers, 6.5 cm in diameter with ointment crusts on the knees (figure 1B) and around the anus; remarkable end-inspiratory pulmonary rales at the base of the lungs.

Laboratory examinations revealed leukocytes 1.3-2.8 × 10³/mm³, erythrocytes 1.69-2.07 × 10⁶/mm³, haemoglobin 3.8-9.0 g/dL, platelets 49-138 × 10³/mm³; erythrocyte sedimentation rate 70 mm at 1st hour. Serum ANA and anti-DNA antibodies, ANCA, and serology for HIV were negative. Biopsies from the upper lip and peri-ulcerative erythema revealed papillary dermal edema and intense neutrophil infiltrates in the dermis (figure 1C). Cultures and stains of the biopsy, blood and sputum were negative. Computed tomography (CT) revealed ground-glass opacities and reticular shadows in both lungs (figure 1D). Pulmonary function tests showed VC of 70% (normal range > 80%) and FEV1% of 85% (< 80%) indicating restrictive ventilatory impairment. Bronchoalveolar lavage fluid (BALF) disclosed 10% neutrophils (< 1%) with no pathogenic microorganisms.

A diagnosis of ulcerative SS and interstitial lung disease (ILD) was established. Intravenous methylprednisolone 56 mg/day and immunoglobulin (400 mg/kg/d for 5 days) were administered. The lip swelling and fever resolved within 1 week, but more painful erythematous plaques appeared on the face which cleared in 1 week (figures 1E, F). Dyspnea, pulmonary function and interstitial infiltration improved after 4 weeks. The ulcers healed with scars after about 6 weeks. Because of persistent pancytopenia, a bone marrow aspiration was taken which conformed to MDS-RA. Erythematous plaques sometimes relapsed and repeated fever, cough and dyspnea were encountered on tapering methylprednisolone. Three months later, he was admitted to the Department of Respiratory Medicine for high fever, cough and shortness of breath. Sputum cultures demonstrated Klebsiella pneumonie whereas blood cultures were negative. Chest CT scan showed interstitial infiltration and right lower lobe pneumonia. He died of respiratory failure 3 weeks later, despite treatment with sensitive antibiotics (meropenem, moxifloxacin and piperacillin) and corticosteroids.

To date, 4 patients with SS, MDS and pulmonary interstitial infiltration have been reported [3-6]; 2 died from respiratory failure and 1 from aspergillosis. The present patient developed SS 2 years after the diagnosis of MDS-RA. He presented with large skin ulcers mimicking pyoderma gangrenosum and the phenomenon of pathergy. The onset and relapse of respiratory symptoms coincided with the skin lesions and BALF contained increased number of neutrophils without microorganisms. Therefore, we considered the ILD was pulmonary involvement of SS. He died of respiratory failure 6 months after the onset, confirming the triad associated with a poor prognosis. Repeated fever and insufficient nutriment intake caused malnutrition which contributed to respiratory muscle weakness. Malnutrition, persistent leukopenia and systemic corticosteroids might reduce immune function, which resulted in unmanageable Klebsiella pneumonie infection. Furthermore, ILD impaired ventilation function and dispersion. The uncontrolled infection, disturbed gas exchange and respiratory muscle weakness contributed to the respiratory failure.

In summary, we report the fifth case of the triad of SS, ILD and MDS. To our knowledge, the distinctive ulcerative SS has not been reported.

Acknowledgement

References

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