The key role of the dental practitioner in early diagnosis of periodontal Ehlers-Danlos syndromes: a rare case report of siblings.

Ehlers-Danlos syndromes (EDS) are a group of diverse hereditary connective tissue disorders. Various EDS subtypes present as different diseases. Periodontitis of early onset is a major criterion of periodontal EDS (pEDS). This article reports the clinical case of two siblings, young adults, who came to the clinic for diagnosis and treatment of periodontal disease. The patients had already been diagnosed with pEDS several months earlier after being referred for genetic testing by a dermatologist. It should be noted that in these siblings pEDS had been misinterpreted for years by health care specialists despite the patients' periodontal disease, which had appeared at the age of 3 years. The subsequent effects of periodontal disease in these patients jeopardized the survival prognosis of their teeth. It may be stated that, in spite of pEDS's status as a rare syndrome, the dental practitioner can play a key role in the early diagnosis by responding appropriately to periodontal manifestations at early stages. (Quintessence Int 2021;52:166-174; doi: 10.3290/j.qi.a45263)

Vasculopathies, cutaneous necrosis and emergency in dermatology.

Most emergencies in dermatology comprise a variety of entities with a usually benign course. However, vasculopathies and vasculitis are not common, but they could represent respectively 1.9% and 4.4% of these entities according to some studies of Emergency Dermatology Department. They become an important disease which has to be identified early to establish appropriate management and treatment. Some of them are well known, such as the leukocitoclastic vasculitis, Schölein-Henoch, panarteritis nodosa, antineutrophil cytoplasmic antibody associated vasculitis, giant cell arteritis, cryoglobulinemic vasculitis and antiphospholipid syndrome. More frequent vasculopathies are livedoid vasculopathy, pigmented purpuric dermatosis and calciphylaxis. Less common ones are caused by interferon and cholesterol crystal embolization. Others are very infrequent as Degos disease and Sneddon Syndrome. Among the more recently described ones there are deficiency of adenosine deaminase type 2 and crystalglobulinemia. The other group is composed of vasculopathies associated to microorganism as infective endocarditis, septic vasculopathy, aspergillosis, fusariosis, strongiloidosis, ecthyma gangrenosum, lucio phenomenon of leprosy and necrotic arachnidism. Finally, among these entities we can also find diseases associated with proinflammatory stages as disseminated intravascular coagulation, myeloproliferative disorders, intravascular lymphoma, metastasis intravascular. When we face cutaneous lesions characterized by reticulated violaceous lesions, palpable purpura or cutaneous necrosis, a careful clinico-pathological correlation as well as some laboratory or radiological tests are mandatory to further delineate a diagnosis and a proper first line empirical treatment.

Colitis ulcerosa asociada a pancreatitis autoinmune, sialoadenitis y vasculitis leucocitoclástica / Ulcerative colitis associated with autoimmune pancreatitis, sialadenitis and leukocytoclastic vasculitis

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