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Related Experiment Videos

Idiopathic atrophie blanche.

J K Shornick, B K Nicholes, P R Bergstresser

    Journal of the American Academy of Dermatology
    |June 1, 1983
    PubMed
    Summary

    Idiopathic atrophie blanche, a painful leg ulcer disorder, showed significant improvement with phenformin and ethylestrenol treatment. This vasculopathy, characterized by fibrin plugs, is distinct from vasculitis.

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    Area of Science:

    • Dermatology
    • Pathology
    • Vascular Medicine

    Background:

    • Idiopathic atrophie blanche presents as chronic, painful leg ulcerations in young to middle-aged women.
    • Characterized by purpuric macules/papules leading to ulceration and porcelain-white atrophic scars with ectatic vessels.
    • Previously misclassified as a form of cutaneous vasculitis.

    Purpose of the Study:

    • To investigate the pathophysiology of idiopathic atrophie blanche.
    • To evaluate the efficacy of phenformin and ethylestrenol in treating this condition.
    • To differentiate atrophie blanche from immune complex-mediated necrotizing vasculitis.

    Main Methods:

    • Studied twelve patients using immunofluorescence, light microscopy, and electron microscopy.
    • Extensive examinations to exclude other immunologic and vaso-occlusive disorders.
    • Ten patients received 1-12 years of combination therapy with phenformin and ethylestrenol.

    Main Results:

    • Microscopic examination revealed fibrin plugs occluding superficial dermal vessels, leading to necrosis and vessel obliteration.
    • New vessel formation observed in areas of fibrin deposition; leukocytes rarely involved.
    • Phenformin and ethylestrenol treatment resulted in rapid improvement of lesions, reduced pain, and fewer new lesions.
    • Treatment side effects were minimal and well-tolerated in most patients.

    Conclusions:

    • Idiopathic atrophie blanche is a vasculopathy characterized by vascular occlusion due to fibrin deposition, not vasculitis.
    • Phenformin and ethylestrenol demonstrate significant therapeutic benefit for this condition.
    • The absence of leukocytes and nuclear fragmentation distinguishes it from immune complex-mediated vasculitis.

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