| Table 3-23 Vaso-Occlusive Diseases |
| | Entity | | Clinical Findings | | Histology/Treatment |
| | Livedoid vasculopathy
(Atrophie blanche) | | Painful ulcerations on lower legs ± with surrounding livedo reticularis → heal with atrophic hypopigmented scars (atrophie blanche) | | Histology: superficial dermal vessels with hyalinized walls, thrombi, mild perivascular infiltrate
Treatment: antiplatelet, anticoagulant and fibrinocytic therapies |
| | Cholesterol emboli
(Figure 3.45E) | | Findings include livedo reticularis, peripheral gangrene, ulceration, nodules, cyanosis, retiform purpura | | Histology: elongated cholesterol clefts within small vessels, thrombi
Treatment: supportive treatment, ± antiplatelet agents |
| | Anti-phospholipid
syndrome (APS)
(Figure 3.45D) | | Findings include livedo reticularis, Raynaud’s phenomenon, vasculitis-like lesions, splinter hemorrhages
{Labs: anti-β2 glycoprotein, lupus anticoagulant, anti-cardiolipin antibodies} | | Histology: noninflammatory small vessel thombosis
Treatment: anticoagulation, antiplatelet agents |
| | Sneddon’s syndrome | | Persistent livedo reticularis associated with systemic arterial thrombi; labile hypertension, recurrent neurologic symptoms
{Can be manifestation of APS or distinct entity (skin/brain only)} | | Histology: partial or complete occlusion of small vessels
Treatment: warfarin |
| | Malignant atrophic
papulosis
(Degos disease)
(Figure 3.45F) | | Crops of small erythematous papules → porcelain white scars (similar to atrophie blanche); GI and CNS symptoms | | Histology: wedge-shaped dermal infarct, thrombosed arteriole (typically in subcutaneous fat)
Treatment; no proven treatment; aspirin ± pentoxifylline |
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