Resolution of Telangiectasia Macularis Eruptiva Perstans with Successful Treatment of Synchronous Large Granular Lymphocytic Leukemia

We present a case of an uncommon manifestation of a rare type of cutaneous mastocytosis, Telangiectasia Macularis Eruptiva Perstans (TMEP). The diagnosis of TMEP was made simultaneously with pancytopenia caused by large granular lymphocytic leukemia (LGL). Successful treatment for LGL with a six-month course of oral cyclophosphamide and prednisone led to complete resolution of TMEP skin lesions, in addition to recovery of counts. The World Health organization (WHO) classification of hematopoietic disorders did not include telangiectasia macularis eruptiva perstans as subtype of cutaneous mastocytosis. On the other hand, the classification acknowledged a subtype of systemic mastocytosis that is associated with clonal hematological non-mast cell lineage disease. This case sheds a light on common association of mast cell disorders with other clonal hematological disorders. Pathophysiology is not completely understood and further studies are needed on a larger scale, especially on the molecular level.