Sweet Syndrome with myofasciitis, characterized by giant cellulitis-like symptoms, observed in an imaging study, along with the discovery of a streptococcal trigger.
Rare Case of Sweet Syndrome with Muscle Involvement Causes Clinical Conundrum
A 34-year-old woman presented with a unique and challenging case of Sweet syndrome, a rare condition characterised by fever, skin lesions, and inflammation, accompanied by muscle involvement.
The patient initially presented with fever, leukocytosis, and a painful erythematous right thigh plaque, leading to an initial diagnosis of cellulitis. However, as her condition worsened with hypotension, altered mental status, neutrophilia, rising creatine kinase level, and elevated erythrocyte sedimentation rate, C-reactive protein, and lactate, the differential diagnosis expanded to include both infectious and non-infectious etiologies of myofasciitis.
The woman underwent a right thigh fasciotomy, but all tissue sampling and cultures remained negative for infection. A contrast-enhanced CT of the right thigh demonstrated geographic intramuscular fluid-attenuation along the fascial plane between the vastus lateralis and vastus intermedius, suspicious for evolving early myonecrosis. An ultrasound-guided aspiration of knee joint fluid yielded serous fluid with sparse white blood cells, negative Gram stain, and negative cultures, ruling out septic arthritis.
Despite the negative results, the patient was admitted to the ICU for presumed septic shock, with blood cultures drawn, IV fluids, vasopressors, and broad-spectrum antibiotics initiated. However, a skin biopsy revealed papillary dermal edema and extensive interstitial neutrophilia with leukocytoclasia, consistent with a diagnosis of giant cellulitis-like Sweet syndrome.
Giant cellulitis-like Sweet syndrome is a rare variant of Sweet syndrome, often associated with larger edematous lesions and potential clinical triggers such as morbid obesity, underlying malignancy, preceding bacteremia, and autoimmune disease. In this case, it appears the syndrome was likely triggered by a preceding group A streptococcal infection.
Clinical Features
Patients with Sweet syndrome often experience fever, malaise, and arthralgia. Cutaneous lesions typically present as tender, erythematous plaques or nodules primarily on the upper extremities, face, and neck. When muscles are affected, patients may exhibit localized muscle pain, swelling, and weakness, sometimes mimicking inflammatory myopathies or infectious myositis.
Diagnostic Imaging
MRI is the preferred diagnostic tool for detecting muscle involvement in Sweet syndrome, as it shows characteristic muscle edema and inflammation. Ultrasound may show muscle swelling and increased vascularity but is less sensitive and specific than MRI. Other imaging modalities, such as CT or PET scans, might be used to evaluate systemic involvement or rule out malignancy, as Sweet syndrome can be associated with hematologic cancers.
Histopathology
Skin biopsy typically shows dense neutrophilic infiltration without vasculitis. Muscle biopsy reveals neutrophilic infiltration within muscle fibers and interstitial tissue, sometimes accompanied by edema or necrosis.
In conclusion, Sweet syndrome with muscle involvement is a rare but important condition to consider in patients presenting with typical skin lesions plus muscle pain or weakness. MRI is the key diagnostic tool to detect and assess the extent of muscle inflammation. Histopathological confirmation from skin and/or muscle biopsy is essential to differentiate it from other causes of myositis or infectious processes.
This synthesis is based on general dermatological and neurological literature on Sweet syndrome and inflammatory myopathies, considering the absence of direct detailed search results on this rare manifestation.
- The rare case of Sweet syndrome with muscle involvement not only poses a clinical challenge but also requires careful evaluation of chronic diseases related to health and wellness, such as neurological disorders and mental health conditions.
- It is crucial for medical professionals to be aware of various therapies and treatments available for managing chronic diseases, including medications for treatment of mental health and skin care products for skin-related issues, as well as more invasive procedures like fasciotomies in severe cases of muscle involvement.
- Fitness and exercise regimens can play an essential role in managing symptoms of chronic diseases, as physical activity helps boost the immune system and overall well-being.
- In the management of Sweet syndrome with muscle involvement, collaboration between dermatologists, neurologists, rheumatologists, and infectious disease specialists may prove beneficial to ensure a comprehensive approach to diagnosis and treatment.
