Inflammatory myopathies are a rare group of disorders that can cause significant disruption in the ability of an individual to adequately perform activities of daily living. In this case report, we present a case of a girl presenting with a substantial compromise of her ambulation with a muscle biopsy consistent with myonecrosis. She was subsequently diagnosed with an inflammatory myopathy and started on glucocorticoid and methotrexate therapy with minimal symptomatic improvement. Further in her clinical course, hypocalcemia and an undetectable 25-hydroxyvitamin D level were detected. Prompt institution of calcium and vitamin D supplementation significantly improved her myopathic condition. While there is evidence in the literature linking vitamin D deficiency with myopathy, there is a lack of data on the association between hypocalcemia and vitamin D deficiency with myonecrosis, which could represent comorbid states in myonecrosis. Therefore, vitamin D status should be established in all patients with myonecrosis, as vitamin D deficiency is easy to diagnose and treat, as exemplified in our patient\'s case, which shows that such treatment could lead to significant clinical improvement.

Diabetic myonecrosis is a rare and poorly understood complication of long-standing, inadequately controlled diabetes mellitus. Theoretical mechanisms contributing to the pathophysiology of diabetic myonecrosis include microvascular complications due to advanced glycation end-products, ischemia-reperfusion injuries, and dysregulated coagulation-fibrinolysis activity. Case reports of diabetic myonecrosis most commonly describe diabetic patients with chronically poor glycemic control who experience isolated swelling and severe pain in a unilateral lower limb with no signs of infection or systemic toxicity. Due to the rarity of this condition, there are currently no treatment guidelines. This case describes a 58-year-old male with a history of uncontrolled diabetes who presented with diabetic ketoacidosis with mixed hypovolemic and septic shock. Diabetic myonecrosis was incidentally discovered in the patient\'s right latissimus dorsi with CT imaging and subsequent surgical exploration. Spontaneous diabetic myonecrosis may mimic several other serious conditions and elicit suboptimal management strategies, particularly in the context of atypical presentations.

Clostridium septicum is a very rare cause of severe spontaneous pediatric enterocolitis and is often associated with underlying malignancy or immunocompromise. Likewise, cyclic neutropenia is a rare congenital immunodeficiency that is characterized by cyclical periods of neutropenia, often with more severe symptoms in the pediatric population. Here, we present a unique case of spontaneous C. septicum enterocolitis, sepsis, and myonecrosis in a child with undiagnosed cyclic neutropenia. Early recognition of pediatric sepsis, frequent reevaluation and identification of rapidly progressive infection, and early surgical intervention are critical for the effective management of a rare and severe infection.

There are many microvascular and macrovascular complications regarding uncontrolled diabetes mellitus (DM). Among them, diabetes myonecrosis is one of the complications but rarely seen in the uncontrolled DM patient population. Here, we present a rare case of DM myonecrosis in a patient with elevated hemoglobin A1c (HbA1c) of 18.2% and discuss the literature review of diabetes myonecrosis. A 48-year-old male with hypertension and uncontrolled type 2 diabetes mellitus (T2DM) with hemoglobin A1c of 18.2% presented with progressive swelling and pain in the right thigh for two days. Physical examination demonstrated swollen and tense tender right thigh with a circumference five inches larger than the left. Computed tomography (CT) and magnetic resonance imaging (MRI) results revealed severe myositis of the right leg, likely myonecrosis, and associated fascial edema/fasciitis. The patient was also complicated with diffuse anasarca, which was corrected with albumin transfusion and furosemide. Aspirin and lisinopril were also started for antithrombotic and cardioprotective effects. The right thigh swelling improved, and the patient could ambulate with supportive measures and regular physical therapy (PT). He was discharged home after 45 days of hospitalization. Diabetic myonecrosis is a rare condition and hence is underdiagnosed. In patients with uncontrolled diabetes, especially with diabetic complications, physicians should have high clinical suspicion to diagnose diabetic myonecrosis when patients present with an acute unilateral painful swollen limb. Our case highlights the complicated course of diabetes myonecrosis with anasarca, improved with supportive measures.

UNASSIGNED: Clostridial myonecrosis (CM), or gas gangrene, is a rare necrotizing muscle infection caused most often by Clostridium perfringens or C. septicum. Inoculation can occur either traumatically or spontaneously. CM has a high mortality rate if not treated promptly.
METHODS: A 64-year-old male presented to the emergency department (ED) with sudden onset left flank pain and fever. Repeated CT scans demonstrated progressive edema around the left iliopsoas muscle with gas formation and bleeding. The patient received intravenous fluids, meropenem, and clindamycin. Emergency laparotomy was performed on suspicion of necrotizing fasciitis and revealed a necrotic left iliopsoas muscle which was partially excised. Blood cultures were positive at 12 h with growth of C. septicum. Prolonged stay in the intensive care unit, and six additional surgical interventions to the abdomen, left thigh, and flank were needed. The patient was discharged after four months to a nursing home.
UNASSIGNED: C. septicum CM more often occurs spontaneously and is associated with colorectal malignancy. However, for our patient, CT colonography and proctoscopy did not reveal any pathology. Therefore, we believe the CM resulted from an injury the patient sustained while working in his backyard, either a cut from barbed wire on his arm or from soil contaminating his psoriatic lesions. Successful outcomes for patients with CM require a high index of suspicion, timely treatment with antibiotics, and repeated surgical debridements.
CONCLUSIONS: This case report describes the presentation and management of a presumably injury-related CM caused by C. septicum.

Diabetic myonecrosis is a rare complication of poorly controlled diabetes mellitus which commonly affects the thigh and is managed conservatively. Spontaneous ischemic necrosis of muscle is noted without a reduction in vascular supply. Pyomyositis caused by Staphylococcus aureus infection is another rare complication. Atypical presentation of myonecrosis and pyomyositis can occur in the form of simultaneous or sequential involvement of multiple muscle groups. We present a rare case of myonecrosis with pyomyositis in a 39-year-old male patient with a background of type 2 diabetes mellitus who presented with a 5-day history of worsening pain of the right thigh radiating to the right ankle, associated with groin swelling and fever. It is important for clinicians to have a low threshold of suspicion of this rare condition due to the other diverse and similar diagnoses, as well as to prevent further complications and morbidity.

BACKGROUND: Clostridium species are known to be the primary causative organism of gas gangrene. Non-clostridial gas gangrene (NCGG) is another rare necrotizing entity often associated with an underlying disease, particularly diabetes mellitus, and has a high mortality rate.
METHODS: A 16-year-old, immunocompetent male was referred to us after four days, following a roadside accident, with a degloving injury over the thigh and knee along with fractures around the knee. Although clinico-radiologically suspicious of gas gangrene, the initial smear report was negative for any Gram-positive bacilli. On the same day, he underwent aggressive debridement with an external fixator spanning the knee to salvage the limb. On post-operative day one, due to deteriorating general clinical condition and a strong clinical suspicion of gas gangrene, he underwent above-knee amputation (open stump) after discussion with microbiologists and physicians.
RESULTS: Polymicrobial non-clostridial infection was seen in culture reports taken serially at different stages of management. The latest follow-up showed a healed amputation stump following split skin grafting.
CONCLUSIONS: Although rare, polymicrobial infections can present as non-clostridial gas gangrene even in an immunocompetent patient. A high index of clinical suspicion with a multi-disciplinary approach helps in early decision-making to avoid a devastating outcome.

Diabetic myonecrosis is an uncommon complication related to long-standing poorly controlled diabetes. A 33-year-old Sudanese male patient with type one diabetes presented with progressive, severe bilateral thigh pain with low-grade fever. Laboratory results show hyperglycemia with ketonuria and elevated creatine kinase but normal white cell blood count. The patient was diagnosed initially with diabetic ketoacidosis with pyomyositis and received analgesic and insulin; the patient partially improved. After the second evaluation, bilateral thigh MRI was requested and shows diffuse edema involving the medial muscle group of the upper third of the right side with intramuscular facial edema, appearing as low signal in T1 and high signal in T2 and fat suppression images with no evidence of collection or abscess. Diagnosis of diabetic myonecrosis was made. The patient was managed conservatively and discharge on aspirin with full recovery.

Myonecrosis and compartment syndrome are rarely seen in sickle cell disease (SCD), and they have not been previously reported in HbSC disease. This case can potentially be recognized as the first case of a patient with HbSC presenting with spontaneous myonecrosis and compartment syndrome.

OBJECTIVE: Clostridial gas gangrene (GG) or clostridial myonecrosis is a very rare but life-threatening necrotizing soft tissue infection (NSTI) caused by anaerobic, spore-forming, and gas-producing clostridium subspecies. It is the most rapidly spreading and lethal infection in humans, also affecting muscle tissue. The high mortality, of up to 100%, in clostridial GG is mediated by potent bacterial exotoxins. Necrotizing fasciitis (NF) is an important differential diagnosis, most often caused by group A streptococci, primarily not affecting musculature but the subcutaneous tissue and fascia. In the early stages of the infection, it is difficult to distinguish between GG and NF. Therefore, we compare both infection types, identify relevant differences in initial clinical presentation and later course, and present the results of our patients in a retrospective review.
METHODS: Patients diagnosed with GG from 2008 to 2018 in our level one trauma center were identified. Their charts were reviewed retrospectively and data analyzed in terms of demographic information, microbiological and histological results, therapeutic course, outcome, and mortality rates. The laboratory risk indicator for NF (LRINEC) score was applied on the first blood work acquired. Results were compared to those of a second group diagnosed with NF.
RESULTS: Five patients with GG and nine patients with NF were included in the present study. Patients with GG had a mortality rate of 80% compared to 0% in patients with NF. In eight patients with NF, affected limbs could be salvaged; one NF underwent amputation. LRINEC did not show significant differences between the groups; however, C-reactive protein was significantly increased (P = 0.009) and hemoglobin (Hb) was significantly decreased (P = 0.02) in patients with GG. Interleukin-6 and procalcitonin levels did not show significant difference. Patients with GG were older (70.2 vs 50 years). Of the isolated bacteria, 86% were sensitive to the initial calculated antibiotic treatment with ampicillin-sulbactam or imipenem plus metronidazole plus clindamycin.
CONCLUSIONS: Both GG and NF need full-scale surgical, antibiotic, and intensive care treatment, especially within the first days. Among patients with NSTI, those with clostridial GG have a significantly increased mortality risk due to early septic shock caused by clostridial toxins. In the initial stages, clinical differences are hardly detectable. Immediate surgical debridement is the key to successful therapy for NSTI and needs to be performed as early as possible. However, patients should be treated in a center with an experienced interdisciplinary intensive care team based on a predetermined treatment plan.