Livedo Racemosa Associated with Epstein-Barr Virus-induced Post-transplant Lymphoproliferative Disorder / 대한피부과학회지

Livedo racemosa (LR) is characterized by a striking violaceous netlike patterning of the skin similar to the livedo reticularis, from which it differs by its localization (more generalized), and shape (irregular, broken circular segments). LR is probably caused by patchy impairment of cutaneous arteriolar circulation, resulting in venous dilatation and stasis of blood. LR is always associated with a pathological condition, including hematologic/hypercoagulable disease, vasculitis, connective tissue diseases, neoplasm, lymphoma, infection, cerebrovascular disease, adverse response to a drug, and etc. Hence, clinical, pathological and laboratory examinations are important for excluding these underlying diseases. To date, there have been few reports of LR secondary to posttransplant lymphoproliferative disease (PTLD) in dermatologic literatures. Herein, we report a case of LR associated with Epstein-Barr virus-induced PTLD of cervical lymph node in a 19-year-old female, who had generalized reticular erythematous to violaceous patch on the entire body after allogenic peripheral blood stem cell transplantation.

Sneddon syndrome associated with Protein S deficiency.

Sneddon syndrome (SS) is rare, arterio-occlusive disorder characterized by generalized livedo racemosa of the skin and various central nervous symptoms due to occlusion of medium-sized arteries of unknown. Seizure, cognitive impairment, hypertension, and history of repetitive miscarriages are the other symptoms seen in this disease. Livedo racemosa involves persisting irreversible skin lesions red or blue in color with irregular margins. Usually, SS occurs in women of childbearing age. Protein S deficiency is an inherited or acquired disorder associated with an increased risk of thrombosis. We present a 33-year-old woman with SS with diffuse livedo racemosa, recurrent cerebrovascular diseases, migraine-type headache, sinus vein thrombosis, and protein S deficiency. Protein S deficiency and with Sneddon syndrome rarely encountered in the literature.