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

Case Report

Ann Hematol Oncol. 2015;2(7): 1052.

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

Saud Alsubait¹, Deeb A3,4, Zimmermann N3,5, Bhaskaran J3, Salkeni MA1,2*

¹Department of Medicine, West Virginia University, USA

²Mary Babb Randolph Cancer Center, West Virginia University, USA

³Cincinnati Veteran Affairs Hospital Medical Center, USA

4Department of Medicine, University of Cincinnati, USA

5Department of Pathology, University of Cincinnati, USA

*Corresponding author: Mohamad A Salkeni, Department of Medicine, Mary Babb Randolph Cancer Center, West Virginia University, Morgantown, USA

Received: July 10, 2015; Accepted: August 09, 2015; Published: August 11, 2015


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.

Keywords: Cutaneous mastocytosis; Telangiectasia Macularis Eruptiva Perstans; Pancytopenia


Cutaneous mastocytosis is defined by the abnormal accumulation of mast cell in the skin. This may or may not lead to significant symptoms. The treatment is generally directed toward symptom control; hence, if asymptomatic, patient may be observed. While there are ample recommendations to guide the treatment of CM, literature guiding therapy for TMEP is scarce. We present a case of a TMEP which completely resolved with the treatment of the associated clonal hematological disorder, which, in our patient, was large granular lymphocytic leukemia.

Case Presentation

A 61-year-old Caucasian male was evaluated simultaneously in dermatology and hematology clinics for asymptomatic skin rash and pancytopenia, respectively. His rash started on his feet approximately seven years earlier and spread gradually to involve his calves, shins, and knees, in addition to chest and abdomen. He reported no associated pruritus or other symptoms of irritation. He has recently noticed increased dyspnea on exertion and fatigue which he attributed to deconditioning. No other constitutional symptoms were reported. A complete blood count performed by patient’s primary care physician revealed pancytopenia. On physical examination, there were numerous scarlet-colored macular and telangiectatic spots that measured 1-3 mm in size. The lesions were confluent, too numerous to count and were mainly located on the shins, knees, lower medial thighs, lower abdomen (peri-umbilical), and the chest (periareolar) (Figures 1A,1B,1C). Patient was found to have a palpable splenic tip and palpable liver 2 cm below costal margin. Laboratory work up on initial presentation revealed mild anemia with inadequate reticulocytosis, thrombocytopenia (hematocrit 29.3%, hemoglobin 10.3 g/dl, MCV 98.3 fL, platelets 102,000 per μL), and significant leukopenia and neutropenia (WBC 1,300 per μL, absolute neutrophil count 488 per μL, and absolute lymphocyte count 447 per μL). On further testing, patient was found to have elevated conjugated and total bilirubin (0.5 and 2.1 mg/dL. Normal range ≤ 0.3 and 0.2- 1.2 for direct and total bilirubin, respectively), elevated alkaline phosphatase (249 U/L. Normal range35-110 U/L) and slight elevation in ALT and AST (51 U/L and 47 U/L. Normal range 0-50 U/L and 5-40 U/L for ALT and AST, respectively). Review of peripheral blood smear was most significant for the presence of occasional large lymphocytes with abundant cytoplasm that contained few large granules (Figure 2). Anemia work up ruled out the presence of any nutritional deficiency.