| livedo reticularis is a mottled blue discoloration of the skin, which occurs in a netlike pattern. Livedoid vasculitis is a chronic disorder clinically manifested by recurrent painful ulcerations of the lower extremities and is characterized by the presence of smooth or depressed ivory-white lesions surrounded by hyperpigmentation and telangiectasia. We describe two patients with livedo reticularis and livedoid vasculitis who responded to puva therapy and propose that systemic PUVA with methoxsalen undergo further investigation as an alternative therapy for drug-resistant patients with livedo reticularis and livedoid vasculitis. ( info) |
| A 37-year-old Japanese male was admitted to Nagasaki University Hospital with abrupt onset of biphasic fever, general malaise and myalgia 9 days after coming back to japan from Manila. He developed a rubella like erythematous rash 3 days after admission and purpuric eruption one week after admission. A biopsied specimen from the purpura revealed lymphocytic vasculitis with T cell dominance and without immunoglobulin or complement deposition around the blood vessels. RT-PCR analysis on peripheral blood mononuclear cells using dengue virus specific primers confirmed the diagnosis of type 3 dengue fever. PCR analysis using virus specific primers is a rapid and valuable method for making a correct diagnosis of dengue fever. ( info) |
3/263. Oxalate kinetics and reversal of the complications after orthotopic liver transplantation in a patient with primary hyperoxalosis type 1 awaiting renal transplantation. We present the case of a young woman with end-stage renal disease secondary to primary hyperoxaluria type 1, who after 3 years and 6 months of maintenance hemodialysis, and despite intensification of the dialytic treatment, developed severe livedo reticularis in her extremities leading to ischemic cutaneous ulcerations, necessitating continuous intravenous infusion of narcotics for pain control. She received a liver transplant after native hepatectomy. However, due to positive crossmatch, she could not receive a kidney from that donor. After transplantation, following serial serum oxalate levels, the hemodialysis regimen was safely reduced from 4 h daily to 3 h three times weekly. Over the course of 6 weeks after liver transplantation, her livedo reticularis resolved, the ischemic ulcers markedly improved, she was weaned off all pain medications, and her erythropoietin-resistant anemia resolved. Our results suggest that in patients with primary hyperoxaluria type 1, who have received a liver transplant and are on maintenance hemodialysis, after serial serum oxalate determinations, some may safely be changed to a thrice-weekly maintenance hemodialysis regimen. Moreover, with this regimen the complications of systemic oxalosis can reverse. ( info) |
4/263. Percutaneous and combined percutaneous and intralesional Nd:YAG-laser therapy for vascular malformations. The numerous types of vascular abnormality are classified in groups according to their pathological and anatomical features. We present case histories of 2 patients who had vascular malformations of the face since birth or early childhood. Application methods, side-effects and complications of percutaneous and intra-lesional Nd:YAG-laser therapy are reviewed for these patients. A 54-year-old woman was treated percutaneously with the Nd: YAG-laser at 1064 nm, with 20 30 W, cw 1-5 s pulses and 2 - 3 mm spot size. A 59-year-old woman was treated with the combined percutaneous and intralesional laser therapy with 30 W, cw 1-5 s pulses and 2-3 mm spot size. In both cases, percutaneous or combined percutaneous and intra-lesional Nd: YAG-laser application resulted in a significant shrinking of the lesion. The Nd:YAG-laser radiation at 1064 nm presents an effective treatment of vascular malformations due to its deep penetration into the tissue. No standardized guidelines for Nd: YAG-laser therapy exist and the treatment parameters should be chosen individually according to the type of vascular malformation. ( info) |
5/263. Steroid-responsive pleuropericarditis and livedo reticularis in an unusual case of adult-onset primary hyperoxaluria. We present a case of a 54-year-old woman with rapidly progressive renal failure of uncertain origin, who developed pleuropericarditis and livedo reticularis 6 weeks after initiation of hemodialysis (HD). The presentation with acute renal failure, the development of serositis, and the dramatic clinical response to empiric steroid therapy initially suggested the diagnosis of a systemic inflammatory disorder or vasculitis. Renal biopsy, performed 3 days after presentation, suggested crystal deposition disease, and subsequent investigations, using both dialysate oxalate concentrations and liver biopsy, led to the diagnosis of primary hyperoxaluria (PH). We discuss this atypical adult presentation of PH and propose a role for the use of steroids in the management of the acute inflammatory symptoms of oxalosis. We also briefly discuss the current medical management of patients with PH, including transplantation. ( info) |
6/263. Acute abdominal pain as a leading symptom for Degos' disease (malignant atrophic papulosis). We report a case of a 16-yr-old white female patient with acute abdominal pain due to visceral involvement of Degos' disease that required extensive small bowel resection. skin manifestations of her disease had been present for 2 yr before the correct diagnosis. She died as a result of central nervous system involvement from Degos' disease. ( info) |
| Livedoid vasculitis, otherwise known as segmental hyalinizing vasculitis or livedo reticularis with summer ulceration, is a chronic disease with lesions affecting the feet and lower legs. Early lesions show petechiae, but characteristic features are recurrent, bizarrely shaped ulcers that heal to leave hyperpigmentation and atrophie blanche. The aetiology of the disorder is unknown, but the histology shows fibrin deposition within both the wall and lumen of affected vessels. The absence of a sufficient perivascular infiltrate or leucocytoclasia argues against a vasculitis, being more in keeping with a thrombo-occlusive process. Four patients with livedoid vasculitis with ulceration are described, all of whom had associated raised anticardiolipin antibodies but no other evidence of systemic disease. We suggest that livedoid vasculitis may be a manifestation of the antiphospholipid syndrome and recommend that all patients are screened for this. We also discuss treatment options for this often resistant condition. ( info) |
| Of 11 patients with primary or secondary antiphospholipid syndrome (APS), four exhibited papules or nodules on the finger, sole or leg as the initial cutaneous manifestation. Histological examination demonstrated thrombosed vessels or vessels containing organized thrombi in the dermis or in the subcutaneous fat tissue. Cutaneous papules and nodules should be recognized as skin manifestations of APS. Screening tests for antiphospholipid antibodies and lupus anticoagulant are required in patients with cutaneous papules or nodules of unknown aetiology. In cases of positive antiphospholipid antibodies and/or lupus anticoagulant, histological examination is critical in the establishment of the diagnosis of APS. ( info) |
9/263. Intravascular and diffuse dermal reactive angioendotheliomatosis secondary to iatrogenic arteriovenous fistulas. Reactive angioendotheliomatosis is a rare benign process that has been mainly described in patients with systemic infections, such as subacute bacterial endocarditis or tuberculosis, and in association with intravascular deposition of cryoproteins. Histopathologically, it is characterized by a proliferation of endothelial cells within vascular lumina resulting in the obliteration of the involved vessels. Another rare variant of reactive angioendotheliomatosis has been described in the lower extremities of patients with severe peripheral vascular atherosclerotic disease. It consists of violaceous and purpuric plaques histopathologically characterized by diffuse proliferation of endothelial cells interstitially arranged between collagen bundles of the reticular dermis. This second variant has been named diffuse dermal reactive angioendotheliomatosis. We report two patients with reactive cutaneous angioendotheliomatosis appearing distally to arteriovenous fistulas used for hemodialysis because of chronic renal failure. The first patient showed intravascular reactive angioendotheliomatosis, while the second one had purpuric plaques that were characterized histopathologically by diffuse dermal angioendotheliomatosis. Both patients showed an arteriovenous "steal" syndrome with distal ischemia, and it is possible that a local increase of vascular endothelial growth factor, as is the case in hypoxia situations, induces the endothelial proliferation. To the best of our knowledge, cutaneous reactive angioendotheliomatosis has not been previously described in association with arteriovenous shunts. ( info) |
10/263. angiomatosis of skin with local intravascular immunoglobulin deposits, associated with monoclonal gammopathy. A potential cutaneous marker for B-chronic lymphocytic leukemia. A report of unusual case with immunohistochemical and immunofluorescence correlation and review of the literature. Reactive cutaneous vascular proliferation or angiomatosis is associated with various conditions, but is rarely seen secondary to vascular occlusion. We report an unusual case of a 79-year-old female who presented with 8 month history of purpuric facial plaques, with painful crusted ulceration of the nose, later developing similar eruptions on hands, thighs and trunk. Biopsies showed marked angioproliferation, with intravascular (IV) hyaline deposits (PAS , fibrin /-; IgM , fibrinogen , and C3 ), associated with endothelial hyperplasia (factor viii , vimentin ). Immunofluorescence showed IV IgM, fibrinogen, and granular C3 deposits within vessel walls. Initially, extensive investigations only showed minimal monoclonal gammopathy of undetermined significance (MGUS) and repeatedly negative cryoglobulins. After a 3-year follow-up, the patient developed chronic lymphocytic leukemia (B-CLL). This case illustrates a difficult diagnostic challenge. Although this condition resembles other forms of reactive angiomatosis, it shows distinct features and should be considered in the differential diagnosis of unusual vascular proliferations of the skin. The cutaneous lesions are also considered a potential marker for an underlying systemic condition, which may require prolonged clinical follow-up. We believe this condition to be related to other rare cutaneous vascular proliferations associated with plasma cell and lymphoproliferative disorders. Furthermore, we suggest a common pathogenetic pathway resulting from the IV immunoglobulin deposits causing vascular injury, finally leading to the angiomatosis. ( info) |