Histiocytoid giant cellulitis-like Sweet syndrome at the site of sternal aspiration: A case report and review of literature

BACKGROUND: Giant cellulitis-like Sweet syndrome (SS) is a rare subtype of SS, and reports of the combined histiocytoid type of pathology are scarce. Here, we report a case of SS with distinctive clinical presentations and which was difficult to distinguish from cellulitis. By sharing this case and a discussion of the related literature in detail, we aim to provide clinicians with new insights into the characteristics of histiocytoid giant cellulitis-like (HGC)-SS and the pathogenesis of SS. CASE SUMMARY: A 52-year-old male was admitted after experiencing progressive fatigue for 1 mo and tongue swelling with pain for 1 d. He was diagnosed with myelodysplastic syndrome (MDS) and angioneurotic edema of the tongue and floor of the mouth. However, 7 d after examination by sternal aspiration, a violaceous, tender, and swollen nodule developed at the site, with poorly demarcated erythema of the surrounding skin. Considering his profile of risk factors, the diagnosis of cellulitis was made and he was administered broad-spectrum antibiotics. When the lesion continued to worsen and he developed chills and fever, pathogenic and dermatopathological examination led to the diagnosis of HGC-SS. Treatment with prednisone led to the fever being relieved within 24 h and the skin lesion being resolved within 1 wk. The patient refused intensive treatment and was instead given thalidomide, erythropoietin, stanozolol, and supportive care. The prednisone was gradually tapered, with no signs of recurrence, but he died 2 mo later of severe pneumonia. CONCLUSION: HGC-SS demonstrates unique manifestation. SS and leukemia cutis share cytological origin. Myelofibrosis and SS are adverse prognostic factors for MDS.