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August 16, 2010

Livedoid Vasculopathy in a Pediatric Patient With Elevated Lipoprotein(a) Levels: Prompt Response to Continuous Low-Molecular-Weight Heparin

Author Affiliations

Author Affiliations: Departments of Dermatology (Drs Goerge, Weishaupt, Metze, Sunderkötter, Steinhoff, and Schneider) and Pediatric Hematology (Dr Nowak-Göttl), University Hospital Münster, Münster, Germany.

Arch Dermatol. 2010;146(8):918-935. doi:10.1001/archdermatol.2010.177

A 14-year-old girl was seen in our department with ulcerations on the malleoli of both feet. Lesions had initially appeared 4 years earlier during the summer but had healed within weeks without treatment. In the following years, the intensity and duration of recurrent episodic ulcerations had increased with summer exacerbations. The patient described a localized sharp pain on the malleolar skin that always preceded the occurrence of lesions and led to sleeping disorders.

In the malleolar region, there was lightning-shaped livedo racemosa, partial necrosis, and several shallow ulcerations with crusts next to white scars (Figure 1A). The clinical findings and skin biopsy results led to the diagnosis of livedoid vasculopathy, which is the pathophysiologic consequence of either elevated thrombus formation or insufficient fibrinolysis in dermal capillaries due to endothelial, platelet, or coagulatory dysfunction. It is associated with prothrombotic disorders or with underlying systemic diseases (eg, scleroderma, lupus erythematosus).1,2 In our case, the findings of extensive laboratory testing for the established abnormalities associated with LV were all negative (Table). However, we found an increased level of lipoprotein(a) [Lp(a)] (69 mg/dL; normal, <30 mg/dL), an independent risk factor for cardiovascular events.3 [To convert Lp(a) to micromoles per liter, multiply by 0.0357.] The level of Lp(a) is genetically determined, and to our knowledge, no diet or medication can modify it.