Massive Sweet Syndrome with Myofasciitis and Streptococcal Trigger: Examining Various Imaging Results
A 34-year-old woman presented with fever, leukocytosis, and a painful erythematous right thigh plaque, marking an unusual case of Giant Cellulitis-Like Sweet Syndrome (GCLSS) with muscle involvement. This condition, while rare, is recognized to sometimes manifest as neutrophilic myofasciitis, characterized by the infiltration of neutrophils in the muscle fascia and muscle tissue itself.
Typical imaging findings of neutrophilic myofasciitis in GCLSS include muscle and fascial edema, often seen on MRI as increased signal on T2-weighted or STIR sequences. Diffuse involvement of the muscle fascias and adjacent muscle fibers, sometimes with fascial thickening, may also be present. Contrast enhancement may be observed due to inflammation.
In this case, the initial differential diagnosis included infectious and noninfectious etiologies of myofasciitis, including early pyomyositis, necrotizing fasciitis, and toxic shock syndrome. However, the MRI showed nonspecific small right knee joint effusion and mild asymmetric synovial hyperenhancement, which, along with the patient's symptoms, led to the presumption of cellulitis.
The woman was initially treated for cellulitis with broad-spectrum antibiotics. However, she developed hypotension, altered mental status, neutrophilia, rising creatine kinase level, and elevated erythrocyte sedimentation rate, C-reactive protein, and lactate, suggesting a systemic inflammatory response syndrome.
Subsequent imaging, including CT and ultrasound of the right thigh, revealed early myonecrosis in the setting of inflammatory myofasciitis. Two fasciotomies were performed, but all tissue sampling and cultures remained negative for infection. A contrast-enhanced CT on hospital day 7 demonstrated geographic intramuscular fluid-attenuation along the fascial plane between the vastus lateralis and vastus intermedius, suspicious for evolving early myonecrosis.
The MRI also showed milder diffuse T2 hyperintense muscle edema in the biceps femoris and semitendinosis, indicating the involvement of other muscles. Arthritis and arthralgias were seen in 33% of patients in the same study, with the knees and wrists being the most frequently involved joints.
Treatment with high dose intravenous corticosteroids produced rapid clinical improvement, underscoring the sterile nature of the muscle inflammation in GCLSS, which represents an extension of neutrophilic dermatosis into deeper tissues.
This case highlights the importance of considering GCLSS in the differential diagnosis of patients with atypical presentations of cellulitis, especially when other imaging findings suggest muscle involvement. The rarity of this condition means that specific, detailed data on the incidence and exact imaging patterns of neutrophilic myofasciitis in GCLSS are not extensively available, reflecting the specialty of the topic. However, understanding the typical imaging findings can aid in diagnosing and managing such cases.
- Despite the initial diagnosis of cellulitis, the MRI revealed skin conditions such as mild diffuse T2 hyperintense muscle edema in the biceps femoris and semitendinosis, indicating the involvement of other muscles in the case, which is a characteristic of chronic diseases like Giant Cellulitis-Like Sweet Syndrome (GCLSS).
- The woman's case showcased the importance of considering GCLSS in the differential diagnosis of patients with atypical presentations of medical-conditions like cellulitis, particularly when there are signs of muscle involvement, such as the infiltration of neutrophils in the muscle fascia and muscle tissue itself.
- As GCLSS can sometimes manifest with neurological disorders like altered mental status and hypotension, it's crucial to monitor a patient's health-and-wellness beyond just skin care aspects, especially since specific, detailed data on the incidence and exact imaging patterns of such conditions are not widely available.
- Skin care is vital in managing psoriasis, but understanding the typical imaging findings and recognition of unusual presentations, like the current case of Giant Cellulitis-Like Sweet Syndrome (GCLSS) with muscle involvement, can aid in diagnosing and managing such cases that may lead to chronic diseases.
