Dermatology Online Journal

Telangiectasia macularis eruptiva perstans
Ahmet Altiner MD, Julia Tzu MD, Rishi Patel MD, Shane Meehan MD, Miguel Sanchez MD
Dermatology Online Journal 17 (10): 7

Department of Dermatology, New York University, New York, New York

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Abstract

We present a 31-year-old woman with a ten-year history of a progressive macular eruption; one of her macules demonstrated a wheal when rubbed. A biopsy was consistent with telangiectasia macularis eruptiva perstans. There were no signs and symptoms of systemic involvement despite the large body surface area of involvement.

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History

Figure 1	Figure 2

Figure 3	Figure 4

A 31-year-old woman presented to Bellevue Hospital Center with a ten-year history of blemishes that were distributed mostly on her limbs. The eruption started on the arms and began to involve her legs over the course of years. Recently, new lesions appeared on her back. The eruption is mildly pruritic and is fixed in place. She denied headache, irritability, flushing, rhinorrhea, bone pain, nasuea, vomiting, diarrhea, and abdominal pain. She denied symptoms when taking non-steroidal anti-inflammatory agents or while drinking alcohol. She did not recall having a reaction to any type of antibiotics and had never taken morphine. The patient had no family history of any skin disease.

Physical examination

On the arms, legs, abdomen, and back were brown macules and small patches without scale. Some of the lesions on the legs have prominent telangiectases. A brown macule on right arm demonstrated a wheal when rubbed. Lymphadenopathy and hepatosplenomegaly were not present.

Laboratory data

A complete blood count and comprehensive metabolic panel were normal. Serum tryptase level was normal at 2 ng/mL.

Histopathology

A Giemsa stain shows mast cells surrounding dilated, thin-walled blood vessels in the papillary dermis. There are about 11 to 17 mast cells per high-powered field.

Discussion

Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis and accounts for less than 1 percent of cases of mastocytosis [1]. It presents most often in adulthood and clinically is comprised of tan macules and small patches with telangiectases that most often involve the trunk [2]. On dermatoscopic examination, the telangiectases are aligned in a reticular pattern [3]. Most patients have only cutaneous findings with variable degrees of pruritus; the lesions typically do not urticate [4].

Mastocytosis is characterized by an increase in the number of mast cells in one or more organs. In the skin, more than 20 mast cells per high power field establishes a diagnosis. However, if lesional skin is shown to have more mast cells when compared to the normal skin, the diagnsosis of cutaneous mastocytosis also may be made. A mutation in the c-kit oncogene results in mast cell hyperplasia, but the role of c-kit mutations in TMEP are limited to case reports [5].

In the past five years, there have been case reports of TMEP that present in association with an underlying illness, such as myelodysplasia, Sjögren syndrome, and renal cancer [6]. It is unclear if these patients demonstrated the rare systemic symptoms that are associated with TMEP, such as flushing, diarrhea, headaches, and abnormal skeletal surveys. Although TMEP is a cutaneous manifestation of mastocytosis, systemic nvolvement may occur. Tryptase levels may serve as a guide to establish this involvement [7]. In the absence of systemic involvement, there is no need for the treatment of TMEP. Patients who experience burning and pruritus of their lesions should be counseled about avoiding agents that are known to be mast cell degranulators. Nonetheless, the literature highlights several successful modalities that are used to treat TMEP successfully. A recent publication reports the successful use of PUVA photochemotherapy in treating the stinging sensation associated with TMEP [8]. Montelukast also has been used [9]. For cosmetic improvement of lesions, a variety of lasers have been used, which include the 585 nm flash-pumped dye laser in two patients and total beam radiation in one patient [10, 11].

References

1. Cohn MS, Mahon MJ. Telangiectasia macularis eruptiva perstans. J Am Osteopathic Assoc 1994;94:246 [PubMed]

2. Tharp, M.D. Mastocytosis. In: Bolognia JJ, et al. Mastocytosis. Dermatology. London: Mosby Elsevier 2008:1848

3. Akay BN, et al. Dermatoscopic findings of cutaneous mastocytosis. Dermatology 2009;218:226 [PubMed]

4. Soter NA. The skin in mastocytosis. J Invest Dermatol 1991;96:32 [PubMed]

5. Lee HW, et al. Two cases of telangiectasia macularis eruptiva perstans demonstrated by immunohistochemistry for c-kit (CD 117). J Dermatol 2005;32:817 [PubMed]

6. Turchin I, Irani AM. Unusual cutaneous findings of urticaria pigmentosa and telangiectasia macularis eruptiva perstans associated with marked myelofibrosis. Int J Dermatol 2006;45:1215 [PubMed]

7. Schwartz LB, Irani AM. Serum tryptase and the laboratory diagnosis of systemic mastocytosis. Hematol Oncol Clin North Am 2000;14:641 [PubMed]

8. Sotiriou E, et al. Telangiectasia macularis eruptive perstans successfully treated with PUVA therapy. Photodermatol Photoimmunol Photomed. 2010;26:46 [PubMed]

9. Cengizlier R, et al. Treatment of telangiectasia macularis eruptive perstans with montelukast. Allergol Immunopathol (Madr). 2009;37:334 [PubMed]

10. Monahan TP, Petropolis AA. Treatment of telangiectasia macularis eruptiva perstans with total skin electron beam radiation. Cutis 2003; 71:357. [PubMed]

11. Ellis DL. Treatment of telangiectasia macularis eruptiva perstans with the 585-nm flashlamp-pumped dye laser. Derm Surg 1996; 22:33. [PubMed]

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