Coxiella burnetii Infection Associated with Thromboangiitis Obliterans-like Phenomena with Digital Auto-amputation: A Case Report and Review of Q fever Associated Autoimmunity

We present a case of a patient with chronic Q fever who presented with digital necrosis, auto-amputations, and positive anti-centromere antibody, mimicking a scleroderma vasculopathy or thromboangiitis obliterans. Coxiella burnetii infection has long been associated with the presence of auto-antibodies and autoimmune phenomena including vasculitis. Clinicians should consider Q fever testing in patients with new onset autoimmune diseases or auto-antibodies and appropriate exposure histories.

The historical differential diagnosis of the disease that afflicted Aleijadinho, the famous 18th century Brazilian sculptor

ABSTRACT Background: The famous Brazilian Baroque sculptor named Antônio Francisco Lisboa, known as “Aleijadinho” (1738–1814), suffered from a deforming disease of the lower and upper limbs. The condition was characterized by atrophy, paresis and amputation. His face was also affected, with inflammation of the eyelids, deviation of the labial commissure, drooping of the chin and lower lip, giving him a sinister expression. Despite the disabling and chronic illness, he produced several works of great expression in the 18th and 19th centuries. Some of them were declared World Heritage Sites by United Nations Educational, Scientific and Cultural Organization (UNESCO). Objective: To discuss the historical differential diagnosis of Aleijadinho’s disease through a narrative review of the literature. Methods: Scientific articles were searched in databases such as Google Scholar, Pubmed and Lilacs using the term “Aleijadinho”. Subsequently, data were collected in articles and books about the possible diseases of the sculptor. Results: Since the first clinical report on the sculptor, several historians and doctors have attempted to establish a diagnosis and several hypotheses have been proposed such as: syphilis, yaws (frambesia tropica), rheumatoid arthritis, Hansen’s disease (leprosy), thromboangiitis obliterans (Buerger disease), zamparina, cardina, porphyria cutanea tarda, stroke, amyloidosis, trauma and/or scurvy. Conclusions: Based on the literature, the authors conclude that the most likely diagnosis of Aleijadinho’s disease is consistent with leprosy.

Recent Updates and Advances in Winiwarter-Buerger Disease (Thromboangiitis Obliterans): Biomolecular Mechanisms, Diagnostics and Clinical Consequences

Thromboangiitis obliterans (TAO) or Buerger’s disease is a segmental inflammatory, thrombotic occlusive peripheral vascular disease with unknown aetiology that usually involves the medium and small-sized vessels of young male smokers. Due to its unknown aetiology and similarities with atherosclerosis and vasculitis, TAO diagnosis is still challenging. We aimed to review the status of biomolecular and laboratory para-clinical markers in TAO compared to atherosclerosis and vasculitis. We reported that, although some biomarkers might be common in TAO, atherosclerosis, and vasculitis, each disease occurs through a different pathway and, to our knowledge, there is no specific and definitive marker for differentiating TAO from atherosclerosis or vasculitis. Our review highlighted that pro-inflammatory and cell-mediated immunity cytokines, IL-33, HMGB1, neopterin, MMPs, ICAM1, complement components, fibrinogen, oxidative stress, NO levels, eNOS polymorphism, adrenalin and noradrenalin, lead, cadmium, and homocysteine are common markers. Nitric oxide, MPV, TLRs, MDA, ox-LDL, sST2, antioxidant system, autoantibodies, and type of infection are differential markers, whereas platelet and leukocyte count, haemoglobin, lipid profile, CRP, ESR, FBS, creatinine, d-dimer, hypercoagulation activity, as well as protein C and S are controversial markers. Finally, our study proposed diagnostic panels for laboratory differential diagnosis to be considered at first and in more advanced stages.

Nails Involvement in Winiwarter-Buerger Disease

<b><i>Introduction:</i></b> Buerger disease, or thromboangiitis obliterans, is an inflammatory and occlusive process involving small and medium size arteries and veins, which generally affects the lower limbs of young adult male with the habit of smoking. <b><i>Case Presentation:</i></b> This paper reports 2 patients who developed nail lesions as the first sign of Buerger disease. <b><i>Conclusion:</i></b> Signs and symptoms of Buerger’s disease are secondary to the inflammatory process and arterial occlusion which results in severe ischemia. Involvement of nails is not common, but we found 2 different clinical features which have not been previously reported in the literature: chronic paronychia, and proximal leukonychia or onycholysis and nail bed erosion.

MANAGEMENT OF THROMBOANGIITIS OBLITERANS - A CASE REPORT

Thromboangitis obliterans (TAOs) is a rare disease affecting arteries and veins of the upper and lower limbs. The condition has a strong association with the use of tobacco. Thromboangitis obliterans also known as Buerger's disease is found in the age group between 40 to 45 years, and men are most prone to get affected. The present case is a male aged 65 years complaining of a wound on the heel on the right foot, associated with pain, discharge, slough, foul smell, edema and discolouration of the skin for which he visited our hospital, the patient was previ- ously diagnosed as TAO, considering his clinical features at the time of admission, an intervention was planned based on the treatment principle of Dusta Vrana like Virechana, Basti and Raktamokshana. There was complete healing of the wound at the end of the treatment with no signs of recurrence during the follow-up suggesting the need for Shodhana in the effective management of TAO. Keywords: Dushta Vrana, Thromboangiitis Obliterans, Ayurveda, Panchakarma, Shodhana, Case report