Unique challenges to diagnosing sweet syndrome following induction chemotherapy for relapsed Acute Myeloid Leukemia (AML): A case and brief-review

Abstract

Background: Sweet Syndrome (SS) is a rare inflammatory skin condition characterized by the sudden appearance of tender, erythematous or violaceous papules, plaques, and nodules typically found on the face, neck, shoulder, upper extremities, and trunk. Often, SS is difficult to diagnose because of its various non-specific manifestations, including fever, arthralgia, myalgia and ocular involvement. In most cases described in literature, cutaneous and pulmonary symptoms of SS present in a concomitant manner. Several reported cases of pulmonary SS have shown that if left untreated, acute respiratory distress syndrome can ensue and progress to fatal respiratory failure.

Case report: A 58-year-old female with acute myeloid leukemia (AML) secondary to chronic lymphocytic leukemia (CLL) presented with new nodular lesions, dyspnea, and fevers. Chest X-ray revealed pulmonary infiltrates. The patient developed new facial lesions and worsening hypoxic respiratory failure. Further infectious workup was negative. She was found to have SS with pulmonary involvement and initiated on high-dose intravenous (IV) steroids with marked clinical improvement.

Conclusions: Major and minor criteria for the diagnosis of lung-associated SS should be carefully evaluated, especially when a biopsy is unavailable. The following case report describes the clinical course and outcomes from treatment for this patient.

Keywords: Acute Myeloid Leukemia; Neutrophilic dermatosis; Pulmonary sweet syndrome.

Fig. 1

Patient's maxillofacial CT scan without contrast, where a green arrow indicates a left sided solitary nodule extending from the dermal to subcutaneous regions, with little epidermal change and minor follicular accentuation. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Fig. 2

Photograph of the nodular lesions seen on the patient's face and extremities.

Fig. 3

Patient's coronal maxillofacial CT scan with contrast, where a green arrow indicates complete opacification of the maxillary sinuses with hyperdense material on the left side and fat stranding along the left orbit. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Fig. 4

Patient's axial chest CT scan with contrast, where two green arrows reveal ground glass opacities of the bilateral upper and middle lobes with consolidative opacities of the lower lobes. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Fig. 5

Patient's maxillofacial CT with contrast, where a green arrow indicates chronic polypoid mucosal thickening in the maxillary sinuses, but no evidence of acute fungal sinus disease is present. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Fig. 6

Summary of the patient's clinical course.