Idiopathic atrophie blanche: treatment with low-dose heparin

Heine, K. G.

doi:10.1001/archderm.122.8.855

Cited by 9 publications

(12 citation statements)

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“…A similar fibrin occlusive vasculopathy is seen in biopsies of leg ulcers due to livedoid vasculopathy. Livedoid vasculopathy is associated with impaired fibrinolysis from a variety of genetic and acquired causes [5–7] and heparin an anticoagulant with profibrinolytic actions has been effective in some cases [8–10]. We postulate that dysregulation of the complement and coagulation cascades with inadequate fibrinolysis and angiogenesis may contribute to delayed healing in scleroderma- associated lower extremity ulcers.…”

Section: Introductionmentioning

confidence: 99%

Lower Extremity Ulcers in Systemic Sclerosis: Features and Response to Therapy

Shanmugam

Price

Attinger

et al. 2010

International Journal of Rheumatology

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Nondigital lower extremity ulcers are a difficult to treat complication of scleroderma, and a significant cause of morbidity. The purpose of this study was to evaluate the prevalence of nondigital lower extremity ulcers in scleroderma and describe the associations with autoantibodies and genetic prothrombotic states. A cohort of 249 consecutive scleroderma patients seen in the Georgetown University Hosptial Division of Rheumatology was evaluated, 10 of whom had active ulcers, giving a prevalence of 4.0%. Patients with diffuse scleroderma had shorter disease duration at the time of ulcer development (mean 4.05 years ± 0.05) compared to those with limited disease (mean 22.83 years ± 5.612, P value .0078). Ulcers were bilateral in 70%. In the 10 patients with ulcers, antiphospholipid antibodies were positive in 50%, and genetic prothrombotic screen was positive in 70% which is higher than expected based on prevalence reports from the general scleroderma population. Of patients with biopsy specimens available (n = 5), fibrin occlusive vasculopathy was seen in 100%, and all of these patients had either positive antiphospholipid antibody screen, or positive genetic prothrombotic profile. We recommend screening scleroderma patients with lower extremity ulcers for the presence of anti-phospholipid antibodies and genetic prothrombotic states.

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Section: Introductionmentioning

confidence: 99%

Lower Extremity Ulcers in Systemic Sclerosis: Features and Response to Therapy

Shanmugam

Price

Attinger

et al. 2010

International Journal of Rheumatology

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“…The deposition of fibrinoid material in dermal vessels with secondary ischemic change of the overlying skin, elevated serum fibrinopeptide A levels, 12 high incidence of antiphospholipid antibodies, defective release of tissue plasminogen activator, 13 and increased levels of plasminogen activator inhibitor in plasma 8 from patients with livedoid vasculitis support an underlying thrombo‐occlusive process. Clinical improvement obtained using fibrinolytics, 3 pentoxifylline, 5 anticoagulant, 6 and antithrombotic therapy also favors this pathogenetic mechanism. Specific histologic findings, such as thickened blood vessel walls, occasional deposition of immunoreactants and fibrin, and the possible association with immune‐mediated diseases, suggest a potential pathogenetic role of immunologic mechanisms 14,15 .…”

Section: Discussionmentioning

confidence: 99%

“…The current therapeutic approach to livedoid vasculitis is to use drugs that stimulate endogenous fibrinolytic activity, inhibit thrombus formation, or cause vasodilatation. Aspirin and dipyridamole, nicotinic acid, low‐dose heparin or warfarin, 6 dextran, 4 and pentoxifylline 5 have been used. Intravenous infusions of tissue plasminogen activator, which lyzes microvascular thrombi, restores circulation, and promotes wound healing, have been successfully used 8 .…”

Section: Discussionmentioning

confidence: 99%

“…The etiology of livedoid vasculitis is still obscure and therapy remains very difficult. Some therapeutic methods, such as bed rest with leg elevation, saline soaking, adequate compression, lumbar sympathectomy, systemic steroids, vasodilators (nicotinic acid, nifedipine), sulfasalazine, fibrinolytic agents (phenformin, ethylestrenol), antiplatelet agents (aspirin, dipyridamole), dextran infusion, pentoxifylline, minidose heparin, prostacyclin, low‐dose danazol, and tissue plasminogen activator, have been tried 3,4 , 5,6 , 7,8 . Nevertheless, there is no consistently appropriate modality, and thus a new, safe, and effective treatment for this condition is needed.…”

Section: Introductionmentioning

confidence: 99%

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Livedoid vasculitis responding to PUVA therapy

et al. 2001

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“…Since impaired hemostasis and fibrinolysis play an important role in the etiology, therapy should modulate or interfere with microcirculatory disturbances. Three major groups of drugs are used in the treatment of AB: 1) drugs that stimulate endogenous fibrinolytic activity (combination of phenformin and ethylestrenol, tissue plasminogen activator, aspirin, and/or warfarin) 4, 7 ; 2) drugs that inhibit thrombus formation (antiplatelet and anticoagulant drugs: aspirin, dipyridamole, prostaglandin E 1 , pentoxifylline, heparin) 8–10 ; and 3) vaso‐dilating drugs (nifedipine, sulfasalazine) 1, 3 …”

Section: Classification Of Atrophie Blanchementioning

confidence: 99%

Idiopathic Atrophie Blanche

Amato

Chiarini²,

Berti³

et al. 2006

Skinmed

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A 41-year-old woman presented with a 3-year history of purpuric lesions followed by superficial, painful ulcers and development of lesions on the lower legs and on the dorsa of the feet, particularly in the summer. The patient was asymptomatic during the winter months. On physical examination she had irregular, scleroatrophic, white-ivory, coalescent lesions on a livedoid basis, with purpuric and, in some lesions, pigmented borders with numerous telangiectatic capillaries. These lesions were localized on the medial sides of the lower legs and on the dorsa of the feet (Figure 1). Laboratory investigations were normal or negative, including complete blood cell count, platelets, coagulation indexes, erythrocyte sedimentation rate, serum immunoglobulins, antinuclear antibodies, anti-double-stranded DNA, anticardiolipin, antiphospholipids, antineutrophilic cytoplasmic antibodies, circulating immunocomplexes, complement fractions (C3, C4), cryoglobulins, rheumatoid factor, and Rose-Waaler reaction. The only laboratory abnormality was an elevated fibrinogen level (472 mg/dL). Doppler velocimetry excluded a chronic venous insufficiency. Thoracic x-ray and abdominal ultrasound were normal. A digital photoplethysmograph revealed functional Raynaud's phenomenon. A biopsy specimen taken from a purpuric lesion showed an atrophic epidermis with parakeratosis and focal spongiosis. An increased number of small-sized vessels were observed within a sclerotic dermis. Most of the vessels in the upper dermis were dilated and showed endothelial swelling; some were occluded due to amorphous hyaline microthrombi (Figure 2). There were fibrinoid deposits around the vessels with thickening of the vessel walls. Extravasated erythrocytes were found throughout the upper and mid-dermis. There was a sparse perivascular lymphocytic infiltrate but no vasculitis. Direct immunofluorescence showed a perivascular microgranular deposit of IgM (+), C3 (++), and fibrinogen/fibrin (+++). On the basis of clinical, serologic, histopathologic, and immunopathologic findings, a diagnosis of idiopathic atrophie blanche was made. The patient was treated with dapsone (50 mg p.o. q.d.) and pentoxifylline (400 mg p.o. t.i.d.) with pain relief and complete resolution of the ulcerations after 6 weeks of therapy.

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Idiopathic atrophie blanche: treatment with low-dose heparin

Cited by 9 publications

References 0 publications

Lower Extremity Ulcers in Systemic Sclerosis: Features and Response to Therapy

Lower Extremity Ulcers in Systemic Sclerosis: Features and Response to Therapy

Livedoid vasculitis responding to PUVA therapy

Idiopathic Atrophie Blanche

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