Atrophie Blanche Synonyms: Atrophie alba, Atrophie blanche en plaque, Atrophie blanche of Milian, Hyalinizing segmental vasculitis, Livedoid vasculopathy, Livedoid vasculitis, Livedo reticularis with summer ulceration, PURPLE (Painful purpuric ulcers with reticular patterning on the lower extremities) ICD-9-CM code: 701.3
Are You Confident of the Diagnosis?
What you should be alert for in the history
The history may include painful, persistent, or recurrent ulcerations.
Characteristic findings on physical examination
Findings in early stages of the disease include painful purpuric ulcerating lesions, often with a punched-out appearance. Occasionally, blistering and crusting may precede the ulcerations. Later stages are marked by slightly depressed, white or ivory-colored scar-like plaques (Figure 1): the “atrophie blanche”), frequently with telangiectases at the edges; scars often appear to be stellate. Their location is usually on the distal aspects of the lower extremities, especially the ankles and dorsal surface of the feet, but occasionally on the face, hands, and trunk, as well.
Expected results of diagnostic studies
Histopathology reveals intravascular fibrin deposition in the superficial vessels, hyalinization of blood vessel walls, and extravasated red blood cells (Figure 2). Stasis dermatitis may show some similar histologic features but should not demonstrate fibrin deposition. There is no evidence of leukocytoclastic vasculitis.
ANA (antinuclear antibody), CBC (complete blood count), CMP (comprehensive metabolic panel), SPEP (serum protein electrophoresis), complement levels, and cryoglobulins are typically within normal limits. Vasculopathy laboratory studies may be positive or abnormal: factor V Leiden, prothrombin G20210A mutations, homocysteine levels, protein C and S levels, lupus anticoagulant, and anticardiolipin antibody.
The differential diagnosis includes:
Degos disease–also affects the gastrointestinal (GI) tract and central nervous system – histopathologic diagnosis
Atrophic scar – lack of other findings
Antiphospholipid antibody syndrome – laboratory findings
Hydroxyurea-induced atrophie-blanche-like lesions.- history
Other ulcerating conditions (eg, venous and arterial ulcers, diabetic ulcers)–Atrophie blanche does not have the preference for specific locations on the lower extremities (eg, the malleoli or at sites of trauma) – additional findings.
Who is at Risk for Developing this Disease?
The disease predominantly affects young to middle-aged women. There are no known geographic or racial predilections.
What is the Cause of the Disease?
The cause of atrophie blanche is unknown.
Fibrin deposition on histology, among other factors, suggests that the condition may result from a localized thrombo-occlusive process. Capillary hypertension may also play a role.
Systemic Implications and Complications
The condition has been associated in several case reports with systemic lupus erythematosus, antiphospholipid antibody syndrome, cutaneous polyarteritis nodosa, and various coagulation disorders such as Factor V Leiden mutation, Protein C deficiency, and antiphospholipid antibodies. Testing for each of these entities may be helpful in appropriate cases.
Treatment options are summarized in Table I.
|Medical Options||Surgical Options||Physical Modalities|
Dipyridomide and aspirin
Low-molecular weight heparin
Tissue plasminogen activator
|Excision with skin grafting
Psoralen with UVA (PUVA)
Optimal Therapeutic Approach for this Disease
Rule out or treat any possible associated infection with appropriate antimicrobials. Rule out venous or arterial disease as appropriate, including checking an ankle-brachial index, as well as ultrasound. Obtain a skin biopsy for histopathology; consider tissue culture, as well. Use blood work to screen for identifiable causes of vasculopathy.
-Dipyridamole (up to 75 mg four times a day) and aspirin (up to 325 mg) daily.
-Pentoxifylline 400 mg three times a day with meals.
-Phenformin and ethylestrenol were original first line therapy and reported to be effective, but phenformin was removed from the market in the US and Canada in 1977 due to its association with lactic acidosis.
-Appropriate wound care should be a part of any regimen, including appropriate, but conservative, debridement of necrotic tissue and use of occlusive dressings.
-Due to pain associated with lesions, pain management may also be necessary.
-Danazol 200 mg daily
-Low-dose heparin 5000U subcutaneously every 12 hours to once daily
-Low molecular weight heparin (enoxaparin) 1 mg/kg every 12 hours to once daily
-Nifedipine 10 mg orally three times a day
Agents, such as topical steroids, prednisolone, azathioprine, or intravenous immunoglobulin (IVIG), should be considered as third-line treatments; they have been reported as helping in case reports and case series, especially IVIG; however, a good explanation of their mechanism of action is lacking. Surgical options, primarily excision with skin grafting, are rarely considered in the literature.
There is no single best proven therapy for the disease, and evidence relies primarily on small case series. Relapses off therapy appear common.
Unusual Clinical Scenarios to Consider in Patient Management
In patients presenting with lesions of atrophie blanche, especially those with features of mononeuritis multiplex not confined to the lesion sites, polyarteritis nodosa should be ruled out. In these cases, it is essential that any skin biopsy not be too superficial.
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- Atrophie Blanche Synonyms: Atrophie alba, Atrophie blanche en plaque, Atrophie blanche of Milian, Hyalinizing segmental vasculitis, Livedoid vasculopathy, Livedoid vasculitis, Livedo reticularis with summer ulceration, PURPLE (Painful purpuric ulcers with reticular patterning on the lower extremities) ICD-9-CM code: 701.3
- Are You Confident of the Diagnosis?
- Who is at Risk for Developing this Disease?
- What is the Cause of the Disease?
- Systemic Implications and Complications
- Treatment Options
- Optimal Therapeutic Approach for this Disease
- Patient Management
- Unusual Clinical Scenarios to Consider in Patient Management