Telangiectasia Macularis Eruptiva Perstans: Unusual Presentation and Treatment

Abstract

A 41-year-old woman presented with a 2-month history of pruritus and a generalized dermatitis that developed initially on the head and spread to the trunk, legs, and buttocks. The pruritus caused extreme discomfort and was not relieved by antihistamines or topical steroid treatment. The patient denied flushing, syncope, and vomiting. Her medical history included asthma treated with salmeterol/fluticasone propionate inhaler, and status post silicone breast augmentation. Physical examination revealed a papular dermatitis on the trunk and extremities composed of lesions up to 0.5 cm in diameter, surrounded by excoriation marks (Figure 1). There was no hepatosplenomegaly or lymphadenopathy. Darier's sign was negative. Results of complete blood count, peripheral blood film examination, and liver function tests were all with normal limits. A biopsy specimen taken from a lesion and stained with hematoxylin-eosin showed telangiectasias, with an increased number of mast cells around blood vessels (Figure 2). Positive Giemsa (Figure 3) and c-kit stain (Figure 4) indicated an increased number of mast cells. Bone marrow aspiration and total body CT performed to rule out systemic involvement showed no pathology. Protein electrophoresis was normal. Serum tryptase and histamine were within normal limits, and 24-hour urine collection for histamine was normal. Narrow-band UV-B treatment was begun 3 times weekly, reduced to twice weekly after 2 months, and then stopped. The first few treatments resulted in significant relief of the pruritus and regression of lesions. After 3 months without treatment, the patient remained free of pruritus and lesions.

Telangiectasia Macularis Eruptiva Perstans Treated with Narrow-Band UVB Phototherapy and Heliotherapy during the COVID-19 Pandemic

“…4 Although psoralen-UVA (PUVA) phototherapy is more widely used, NB-UVB phototherapy has proven effective in mastocytosis, TMEP, and urticaria pigmentosa; 2,3 it lightens lesions and decreases pruritus after 24 to 36 sessions. 2,5 While PUVA works faster, NB-UVB requires lower UV exposure and is thus safer and more tolerable. 4 Our patient improved after only five sessions of NB-UVB phototherapy; we can assume that more sessions would have even better results.…”

“…Telangiectasia macularis eruptiva perstans (TMEP) is an extremely rare variant, comprising less than 1% of mastocytosis cases. 1,2 Although it is generally confined to the skin, it can also manifest as headache, flushing, syncope, or diarrhea. 1,2 Symptomatic treatment is the mainstay of treatment (antihistamines and trigger avoidance).…”

“…1,2 Although it is generally confined to the skin, it can also manifest as headache, flushing, syncope, or diarrhea. 1,2 Symptomatic treatment is the mainstay of treatment (antihistamines and trigger avoidance). For purely cutaneous disease, no gold standard of treatment is recognized.…”

Telangiectasia Macularis Eruptiva Perstans: more than skin deep

“… 37 , 39 – 41 On occasion, TMEP may present as unexplained pruritis. 42 Some cases of TMEP may present in atypical locations such as unilaterally on the face 43 , 44 and/or localized to the upper arms. 26 Neurological complications such as prolonged loss of consciousness or the Kounis-like phenomenon have also been described.…”