Sweet’s syndrome with pulmonary involvement

Abstract

Sweet’s syndrome is an acute febrile neutrophilic dermatosis with classical clinical features. Systemic manifestations in Sweet’s syndrome including fever, arthralgia, myalgia and ocular involvement are common. Pulmonary involvement is a rare manifestation that has been reported previously in 34 cases and can be fatal if left untreated. We report a striking case of Sweet’s syndrome with respiratory failure secondary to bilateral pulmonary interstitial infiltrates, which rapidly responded to intravenous corticosteroid therapy. This case is an important reminder of the systemic manifestations of Sweet’s syndrome and highlights the value of collaboration between different medical specialities to optimise patient management and outcomes.

New Practical Aspects of Sweet Syndrome

“…Involvement of lungs in SS is a rare phenomenon yet can be associated with significant morbidity and mortality. Manifestations of pulmonary involvement in SS include bronchiectasis [91], diffuse alveolar hemorrhage [92], ground glass opacities mimicking pneumonia [67,91,93,94], obstructive lung disease [91], and pleural effusion [95]. Pulmonary involvement may follow skin involvement [67,91,92,94,95] or the pulmonary manifestations may appear concurrently with dermatologic lesions [93].…”

“…Manifestations of pulmonary involvement in SS include bronchiectasis [91], diffuse alveolar hemorrhage [92], ground glass opacities mimicking pneumonia [67,91,93,94], obstructive lung disease [91], and pleural effusion [95]. Pulmonary involvement may follow skin involvement [67,91,92,94,95] or the pulmonary manifestations may appear concurrently with dermatologic lesions [93]. Clinically, a patient with pulmonary involvement may present with pulmonary symptoms such as dyspnea [67,91,94,95] or hemoptysis [92], or without any obvious pulmonary symptoms such that pulmonary involvement only becomes evident after analysis of imaging studies [93].…”

Sweet syndrome with pulmonary involvement in a patient with myelodysplastic syndrome

“…In this case, our patient was found to have violaceous papulonodules prior to having a screening chest CT, which demonstrated bilateral lower lobe infiltrates and ground glass opacities. Although our patient recovered lung function with significant improvement of cutaneous lesions on systemic Dermatology Online Journal || Case Presentation corticosteroids, Sweet syndrome with pulmonary involvement has, in five cases, rapidly progressed to acute respiratory distress syndrome and death [7]. Furthermore, as seen in our patient, of 34 previous reported cases of pulmonary involvement in Sweet syndrome, 18 cases involved the presence of hematologic disorders, including MDS, myeloproliferative disorder, and agnogenic myeloid metaplasia [9,10].…”

“…Bronchoalveolar lavage often reveals a high neutrophil content (>50%) with negative cultures, whereas transbronchial biopsies show dense, sterile neutrophilic alveolar infiltrates. In 16 of 34 cases of Sweet syndrome with pulmonary involvement the skin manifestations presented first, whereas in 12 cases the two occurred simultaneously [7,8]. In this case, our patient was found to have violaceous papulonodules prior to having a screening chest CT, which demonstrated bilateral lower lobe infiltrates and ground glass opacities.…”

“…Symptoms of pulmonary Sweet typically involve progressive dyspnea and a dry nonproductive cough that typically present concomitantly with cutaneous lesions [7]. Chest X-ray and chest CT show diffuse unilateral or bilateral interstitial infiltrates, with some cases demonstrating pulmonary ground glass opacities and pleural effusions.…”