ABSTRACT
IgA vasculitis and IgA nephropathy are characterized by IgA deposition in blood vessels and glomerular mesangium, respectively. The former is far more common in the pediatric population, while the latter presents more often in adulthood. A consensus regarding whether these two conditions are manifestations of the same disease that occurs on a spectrum has not yet been reached, and, to our knowledge, no clinical trials to address this question have been conducted. Here, we describe a 27-year-old patient who presented to the emergency department multiple times before being diagnosed with IgA vasculitis with no identifiable trigger and soon after developed IgA nephropathy. This case highlights the importance of ruling out these conditions, especially IgA vasculitis, in adults presenting with a petechial rash, but also the need for studies that investigate whether and how these conditions are related so that patients can be appropriately diagnosed and treated as efficiently as possible.
ABSTRACT
Lung cancer is one of the leading causes of cancer-related death worldwide. Lung cancer commonly metastasizes to the liver, bone, and brain, but metastasis to skeletal muscles is rare. The development of metastasis in skeletal muscles indicates stage IV disease with a poor prognosis. The most effective treatment strategy is unclear. Palliative radiotherapy is often used to treat skeletal muscle metastases, and patient survival is poor, with an average survival of one year. Here we discuss the case of a 76-year-old female diagnosed with lung adenocarcinoma with metastasis to the trapezius muscle. Initially, she was treated with stereotactic body radiotherapy for stage T1 lung adenocarcinoma. Her follow-up surveillance positron emission tomography (PET) scan in 11 months showed an abnormal focal area of increased activity localizing to the long head of the right triceps muscle. The diagnosis was confirmed with an ultrasound-guided biopsy of the trapezius muscle. Following that, the patient underwent wedge resection of the right middle and upper lobe of the lung and partial right trapezius resection. Afterward, she was given radiation therapy at the tricep resection site. She remained disease-free for four years after excision and radiation therapy.
ABSTRACT
BACKGROUND: Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome is a rare, hereditary, autoinflammatory disease. However, there are few cases reported in the literature. Therefore, we conduct this systematic review to summarize current evidence. METHODS: We conducted a systematic search in July 2021 using 11 different electronic databases. The included articles were screened according to our inclusion and exclusion criteria and assessed using an appropriate quality assessment tool. Then, the relevant data were extracted and summarized in tables accordingly. Each step of the previous one was done by 3 independent reviewers, and the conflicts were resolved by discussion and sometimes by counseling a senior member. RESULTS: The final included studies were 18 articles with 34 cases (mean age = 8 years, male/female = 19/15). The most reported symptoms and signs were fever 97.1%, erythematous plaques 76.5%, arthralgia 67.6%, hepatomegaly 61.8%, violaceous hue 61.8%, lipodystrophy in extremities 53.1% in addition to low weight and height. Rare features were reported too. The laboratories were not specific, which may be explained by a systemic inflammatory response. Vasculitis was the dominant feature in the skin biopsy, whereas the calcification in the basal ganglia was a prominent sign in many cases. CONCLUSIONS: Fever, skin lesions, and systemic inflammatory response were the prominent features of chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome. The clinical picture is the main guide in addition to the pathological findings. Mutation detection is the confirmatory test. Prednisolone is the most effective reported treatment for acute presentations in the literature.
