Scurvy. A forgotten pseudovasculitis.

Scurvy is a nutritional disease caused by ascorbic acid (vitamin C) deficiency. Althought currently it is a rare disease, we should considerer it in the differential diagnosis of purpura and arthritis in patients with restrictive diets. We present the case of a 49-year-old man with a history of a nutritional disorder presented to our hospital with generalized purpura and hemarthros. Following the anamnesis and laboratory findings, rheumatological, infectious and hematological etiologies were excluded. Finally, the diagnosis of scurvy was made upon demostration poor levels of vitamin C and a spectacular response to nutritional supplements. We compare this case with 19 similar cases reported in the medical literature.

Pachydermodactyly: a systematic review.

INTRODUCTION/OBJECTIVES: Pachydermodactyly is a rare, benign fibromatosis located around the proximal interphalangeal joints. It is often misdiagnosed as juvenile idiopathic arthritis and may cause unnecessary treatments and anxiety in patients. The goal of this paper is to describe this condition through all the existing information in the scientific literature. METHOD: A systematic review and a descriptive study have been conducted. A systematic research was performed in PubMed, Embase, Cochrane Library and WOS. RESULTS: Pachydermodactyly was four times more frequent in male subjects and usually started in adolescence. Bilateral presentation was more frequent. History of microtrauma in both hands due to digital manipulation was found in almost half of the patients, many of them showed some neuropsychiatric disorder. In women, the onset happened later, unilateral involvement and family history were more frequent. Swelling of soft tissue without joint implication was found in imaging tests. The progression was usually positive and the treatment included stopping the microtrauma, administrating intralesional corticoids and/or surgery. CONCLUSIONS: Diagnosis can be established in asymptomatic young patients through a congruent physical exam, regular analytic results and imaging tests that simply show swelling of soft tissue-a biopsy is generally not required for diagnosis. As pachydermodactyly's course is asymptomatic and benign, knowledge about this condition is limited, which increases the likelihood of its underdiagnosis-it is important that clinicians know of pachydermodactyly in order to avoid misdiagnosis.

Recommendations of the Spanish Rheumatology Society for Primary Antiphospholipid Syndrome. Part I: Diagnosis, Evaluation and Treatment. / Recomendaciones de la Sociedad Española de Reumatología sobre síndrome antifosfolípido primario. Parte I: Diagnóstico, evaluación y tratamiento.

OBJECTIVE: The difficulty in diagnosis and the spectrum of clinical manifestations that can determine the choice of treatment for primary antiphospholipid syndrome (APS) has fostered the development of recommendations by the Spanish Society of Rheumatology (SER), based on the best possible evidence. These recommendations can serve as a reference for rheumatologists and other specialists involved in the management of APS. METHODS: A panel of four rheumatologists, a gynaecologist and a haematologist with expertise in APS was created, previously selected by the SER through an open call or based on professional merits. The stages of the work were: identification of the key areas for drafting the document, analysis and synthesis of the scientific evidence (using the Scottish Intercollegiate Guidelines Network [SIGN] levels of evidence) and formulation of recommendations based on this evidence and formal assessment or reasoned judgement techniques (consensus techniques). RESULTS: 46 recommendations were drawn up, addressing five main areas: diagnosis and evaluation, measurement of primary thromboprophylaxis, treatment for APS or secondary thromboprophylaxis, treatment for obstetric APS and special situations. These recommendations also include the role of novel oral anticoagulants, the problem of recurrences or the key risk factors identified in these subjects. This document reflects the first 21, referring to the areas of: diagnosis, evaluation and treatment of primary APS. The document provides a table of recommendations and treatment algorithms. CONCLUSIONS: An update of the SER recommendations on APS is presented. This document corresponds to partI, related to diagnosis, evaluation and treatment. These recommendations are considered tools for decision-making for clinicians, taking into consideration both the decision of the physician experienced in APS and the patient. A partII has also been prepared, which addresses aspects related to obstetric SAF and special situations.