1/21. Fibrosing alveolitis predating microscopic polyangiitis.A 65 year old male was diagnosed with "cryptogenic fibrosing alveolitis (CFA)" and treated successfully with prednisone. In the year following prednisone-tapering he presented with livedo reticularis, segmental pauci-immune glomerulonephritis and necrotizing vasculitis of the peripheral nerves, increased pulmonary fibrosis, and the presence of p-ANCA antibodies. Aggressive immunosuppressive treatment of this microscopic polyangiitis (MPA) was successful and also resulted in stabilization of the pulmonary fibrosis. This case illustrates that MPA may present itself monosymptomatic as CFA.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
2/21. Acral necrosis of the fingers as initial manifestation of cutaneous polyarteritis nodosa--a case report.Cutaneous polyarteritis nodosa (CPN) is a well-known entity showing subcutaneous tender nodules, livedo reticularis, and ulcerations as predominant features; arthralgia, myalgia, peripheral neuropathy, and general symptoms such as fever and malaise may also be present. Although the localization of the disease is not specific, the characteristic primary lesions are painful subcutaneous nodules on the lower extremities. Here we report the case of a 33-year-old man with blue-colored distal phalanges and necrosis of several fingertips on both hands as the initial manifestation of CPN.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
3/21. Fulminant and relentless cutaneous necrosis with excruciating pain caused by calciphylaxis developing in a patient undergoing peritoneal dialysis.A 50-year-old Japanese female with chronic renal failure who had been on continuous ambulatory peritoneal dialysis developed fulminant systemic cutaneous necrosis that began as painful livedo reticularis-like skin lesions on her thighs. Because of disseminated vascular calcification within the muscular layer of her lower limbs, we eventually diagnosed her with calciphylaxis. The skin necrosis progressed rapidly, and she died of sepsis and pneumonia on the 53rd hospital day. In addition to her long-lasting severe hyperparathyroidism and extremely elevated serum phosphorus and calcium levels, mechanical, frictional stimulation inflicted on the local skin and administration of corticosteroids were suspected to have precipitated the calciphylaxis. Our lack of awareness of this disease in its early stages resulted in our missing the chance to do a parathyroidectomy that might have changed the course. It is important to know the clinical features of this rare disease in order to make a diagnosis as early as possible.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
4/21. Primary hyperoxaluria: report of a patient with livedo reticularis and digital infarcts.Primary hyperoxaluria encompasses 3 rare genetic disorders of glyoxylate metabolism characterized by excessive urinary excretion of oxalic acid, resulting in oxalosis. patients typically have recurrent calcium oxalate nephrolithiasis and nephrocalcinosis, leading to chronic renal failure and death from uremia. Oxalate can deposit in extrarenal sites such as the heart, walls of arteries and veins, bone, and skin. We report a patient who presented with acute renal failure and later experienced livedo reticularis and peripheral gangrene before the diagnosis of primary hyperoxaluria was established. A skin biopsy specimen demonstrated numerous characteristic elongate to diamond-shaped, radially oriented, pale yellow translucent oxalate crystals within the vessels, and vessel walls of the subcutaneous fat that were strongly birefringent under polarized light.- - - - - - - - - - ranking = 5keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
5/21. Five cases of livedo-like dermatitis (Nicolau's syndrome) due to bismuth salts and various other non-steroidal anti-inflammatory drugs.The authors report five cases of Nicolau's syndrome observed over a period of about 25 years. The disease had occurred after intramuscular injections of different drugs (bismuth, diclofenac and ibuprofen). In all the described cases the clinical aspect was characterized by a livedoid pattern followed by a slow necrotizing evolution with scar formation; in some cases surgical debridement and plastic reconstructive surgery were performed. In the past Nicolau's syndrome was described after intramuscular injections of bismuth salts for the treatment of syphilis; now, although still rare, it is described after injections of various aqueous drug solutions. The pathogenesis of Nicolau's dermatitis appears to be more complex than the previous hypothesized embolism caused by oleous drugs.- - - - - - - - - - ranking = 0.37870715909608keywords = livedo (Clic here for more details about this article) |
6/21. An unusual case of calciphylaxis.BACKGROUND: Cutaneous calciphylaxis is a rare disorder that occurs most frequently in patients with end-stage renal disease (ESRD), those on hemodialysis, and renal transplant recipients. It is frequently associated with hyperparathyroidism and a markedly elevated calcium-phosphate product, and it carries a high mortality rate. The usual clinical presentation is of painful, stellate necrosis of the thighs or buttocks, often in the setting of livedo reticularis. death usually results from septicemia. OBJECTIVE: This report documents an unusual case of recurrent, self-limiting calciphylaxis in the setting of a patient with ESRD and discusses the clinical and pathologic features of this potentially very fatal disorder. methods AND RESULTS: A 52-year-old woman presented with a greater than one-year history of relapsing and remitting, exquisitely painful, necrotic, numular plaques on the abdomen, breast, and arm. This patient had a markedly elevated calcium-phosphate product and parathyroid hormone level. The diagnosis of calciphylaxis was made by wedge biopsy of the most recent plaque, revealing calcification of medium-sized subcutaneous vessels and lobular capillaries with associated epidermal necrosis. CONCLUSIONS: This case demonstrates an unusual clinical variant of calciphylaxis that presented without the characteristic stellate necrosis or livedo reticularis that normally marks this condition and spontaneous resolution without incurring septicemia. Regardless of morphology, calciphylaxis should be considered in the differential diagnosis of painful, necrotic lesions occurring in the setting of ESRD.- - - - - - - - - - ranking = 2keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
7/21. Cutaneous necrosis by calcific uremic arteriolopathy.BACKGROUND: Calcific uremic arteriolopathy is a rare and serious disorder characterized by systemic medial calcification of the arteries and tissue ischemia. Most often it is found in patients with chronic renal failure on dialysis and in renal transplant recipients with secondary hyperparathyroidism. methods: We report six patients with end-stage renal disease [five on hemodialysis (one with a nonfunctioning renal graft) and one on peritoneal dialysis] who developed painful livedo reticularis and skin necrosis of the limbs. All had secondary hyperparathyroidism and elevated calcium-phosphorus product. Our patients presented with the following clinical features: white race (six patients), hypoalbuminemia (three patients), diabetes (one patient), and obesity (four patients). RESULTS: Subtotal parathyroidectomy was performed in three cases. Despite this procedure, two patients died; one patient survived and his lesions healed. One patient was treated with aggressive wound care and hemodialysis (with low dialysate calcium concentration and Renagel phosphorus binders) and one patient received only local wound care, both with improvement of their lesions. In one case, no therapy was performed because the patient died immediately after diagnosis. CONCLUSIONS: The three patients who survived (Cases 4, 5 and 6) had distal lesions, normal serum albumin, and an early diagnosis. There was a relationship between the outcome of the patients and these factors, rather than the type of treatment received.- - - - - - - - - - ranking = 1keywords = livedo reticularis, reticularis, livedo (Clic here for more details about this article) |
8/21. Embolia cutis medicamentosa.Embolia cutis medicamentosa or Nicolau syndrome is an acute necrotic condition of skin and subcutis that follows intramuscular administration of certain medicines. A young lady from rural area presented with an irregular-contoured ulceration with livedoid features on left deltoid region which she developed after receiving intramuscular injection of diclofenac sodium. The histopathological examination of the biopsied tissue demonstrated dead keratinocytes with degenerated epidermis and dermis. The condition improved on local management of wound with a residual scar.- - - - - - - - - - ranking = 0.075741431819216keywords = livedo (Clic here for more details about this article) |
9/21. Digital skin necrosis in congenital afibrinogenaemia associated with hepatitis c virus infection, mixed cryoglobulinaemia and anticardiolipin antibodies.Congenital afibrinogenaemia is a rare genetic disorder transmitted as an autosomal recessive trait and characterized by the complete absence of fibrinogen in the plasma. We report a 41-year-old woman who suffered from congenital afibrinogenaemia and hepatitis c viral infection and presented with ischaemic necrosis and livedo of the toes. Laboratory investigations showed the presence of mixed cryoglobulinaemia and anticardiolipin antibodies. Resolution occurred with plasmapheresis. We discuss the pathophysiology of this unusual condition and review the literature for skin manifestations associated with this rare haemostasis disorder.- - - - - - - - - - ranking = 0.075741431819216keywords = livedo (Clic here for more details about this article) |
10/21. Nicolau syndrome following intramuscular diclofenac administration: a case report.Nicolau syndrome (livedoid dermatitis) is a rare adverse reaction of a still largely unidentified pathogenesis at the site of intramuscular drug injection. The typical presentation is pain around the injection site soon after injection, followed by erythema, livedoid patch, haemorrhagic patch, and finally necrosis of skin, subcutaneous fat, and muscle tissue. The phenomenon has been related to the administration of a variety of drugs, including non-steroidal anti-inflammatory drugs, corticosteroids, and penicillin. We report a case of Nicolau syndrome following an intramuscular injection of diclofenac. The large ulceration over the right buttock was positive for pseudomonas aeruginosa, and histology revealed subcutaneous fat necrosis and non-specific inflammation with no evidence of malignancy or vasculitis. The lesion required multiple debridements and a partial-thickness skin graft. Subcutaneous injection, rather than intramuscular injection, was found to be a determining factor in this case. Clinicians must be cautious in the use of proper injection procedures, including appropriate needle length, in order to minimise complications.- - - - - - - - - - ranking = 0.15148286363843keywords = livedo (Clic here for more details about this article) |
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