Staphylococcal scalded skin syndrome (SSSS) is an exceedingly rare diagnosis in adults, with fewer than fifty cases reported in the literature. We report a case of septic shock that occurred with associated SSSS secondary to another uncommon diagnosis, bacterial pyomyositis.
A 64-year-old Caucasian male with a past medical history of diabetes mellitus type 2 and chronic renal insufficiency presented to the hospital complaining of loss of vision in the left eye that occurred after the development of a painful vesicular rash on the left forehead and temple. On exam, the patient had palsy of cranial nerves III, IV, and VI. His laboratory studies were unremarkable except for a creatinine of 2.7 mg/dl. He was admitted for treatment of herpes-zoster ophthalmicus and optic neuritis with intravenous acyclovir and methylprednisolone. On the fifth hospital day, the patient developed an exquisitely painful and rapidly expanding left neck mass with surrounding erythema, fever of 102°F, and white blood cell count of 40 x 109/L (93% segmented neutrophils). Despite vigorous hydration, the patient became hypotensive and required norepinephrine, and was empirically treated with vancomycin, clindamycin, and cefepime. His physical exam was significant for crusted lesions around the left eye and temple with a painful diffuse erythematous rash on the left lower face, neck, and chest with areas of skin sloughing. Nikolsky’s sign was present. The left neck was markedly tender with diffuse swelling and a 4 cm area of induration on the lateral aspect of sternocleidomastoid muscle (SCMM). A noncontrast CT scan of the neck revealed multiple locules of air with extensive surrounding phlegmonous changes of the left SCMM without mature abscess. The patient was taken emergently to the operating room for incision and drainage of the left SCMM. Operative findings were significant for an indurated left SCMM with extensive seropurulent discharge without discrete abscess. The infectious tract extended from the mastoid bone down to the anterior chest wall and anterior to the level of the clavicle and sternum. Blood and intraoperative wound cultures grew Staphylococcus aureus, supporting the diagnosis of bacterial pyomyositis with SSSS. As the patient exhibited some signs of toxic shock syndrome (TSS) and his condition was critical, he was given intravenous immunoglobulin (IVIG). He improved initially and no longer required pressor-support, however, the patient subsequently developed nosocomial infections that prolonged his hospital stay and led to ventilator dependence.
SSSS is caused by infection with an exfoliative exotoxin secreting S. aureus. SSSS is primarily a disease of children and neonates which carries a good prognosis. However, it is very rare in adults and has a reported mortality of 60%. SSSS is believed to be due to a genetic susceptibility to the toxins combined with inability to clear them in patients with impaired renal function, especially in those with underlying immunodeficiency. Features of SSSS in adults include: erythematous rash, fever, skin tenderness, positive Nikolsky’s sign, and positive blood cultures. Treatment consists of antibiotics directed toward the staphylococcal focus of infection. IVIG has shown efficacy in treating TSS and was administered on that basis. However, TSS shares clinical features with SSSS, and although not studied yet, IVIG may provide some benefit in SSSS as well. Toxin testing is ongoing.Our patient’s infectious source was bacterial pyomyositis, which led to SSSS due to his chronic renal insufficiency in conjunction with high dose corticosteroids.
This case highlights two unusual sources of septic shock, bacterial pyomyositis and SSSS. To our knowledge there have been no previous case reports due to this combination and none in which IVIG has been used.
Allen Blaivas, None.