Acquired bilateral telangiectasia macularis eruptiva perstans: A unique clinical feature of photodamaging rather than a subtype of cutaneous mastocytosis

Abstract

Telangiectasia macularis eruptiva perstans (TMEP) is a rare subtype of cutaneous mastocytosis, characterized by telangiectatic tan to brown macules on the trunk and extremities. Although TMEP has been descried as an uncommon disease in the literature, we often encounter patients with TMEP lesions in the outpatient clinic. We aimed to assess the clinical and histopathological characteristics of acquired bilateral TMEP, and the pathophysiological mechanism of acquired bilateral TMEP among these patients. We retrospectively reviewed 30 patients (28 men and 2 women) with acquired bilateral TMEP; multiple telangiectatic dark red to brown macules that were symmetrically distributed. The clinical characteristics and general histopathological findings of lesional skin were investigated. The number of mast cells was evaluated using immunohistochemical analysis with an antibody directed against c-kit (CD117). Acquired bilateral TMEP was predominantly localized on the sun-exposed area: the upper arm in 30 patients (100%), forearm in 19 patients (63.3%) and anterior chest in 15 patients (50%). A total of 16 patients (53.3%) showed at least one aggravating factor, including UV irradiation, alcohol use and heat exposure. Compared with the mast cell numbers in 19 age- and biopsy site-matched healthy controls (91 ± 29.0/mm ), the number of mast cells in the papillary dermal skin of acquired bilateral TMEP patients was significantly increased (159 ± 37.2/mm , P < 0.01). In addition, a significant difference in vessel numbers in the papillary dermis was observed between acquired bilateral TMEP patients and healthy controls (10.5 ± 1.9 vs 5.4 ± 1.0/mm , P < 0.01). Acquired bilateral TMEP is a relatively common disorder in middle-aged Asian men. An increased number of mast cells and dilated vessels might be a photoaging-related reactive process of chronic sun-exposure, which consequently leads to the formation of characteristic telangiectatic hyperpigmentary macules through certain melanogenic mediators.

Multinucleate cell angiohistiocytoma: A clinicopathologic study of 62 cases and proposed diagnostic criteria

“…With CD117 immunostaining we were able to show that certain cases did indeed show increased mast cells in the dermis and increased mast cells around the vessels, yet this did not constitute a majority of cases. Also, when compared with the mast cell count of normal or inflamed human skin (50‐100 mast cells per mm 2 or 5‐10 mast cells per blood vessel), only two cases showed more than 10 mast cells per vessel and none more than 100 per mm 2 . It remains important to be aware that MCAH may have subjectively elevated mast cell counts; our case of generalized MCAH was originally misdiagnosed as TMEP.…”

Unilateral telangiectasia macularis eruptiva perstans with an unusual clinical presentation

“…TMEP is usually symmetrically distributed on the trunk and extremities, and do not exhibit Darier's sign or mast cell activation symptoms. 1 Here, we present a rare unilateral TMEP, which differs from previously reported TMEP.…”

Telangiectatic mastocytosis versus telangiectasia macularis eruptiva perstans

“…Mast cell number varied from 15 to 30 per high-power view (9400) (Fig. 4 As Moon et al 1 have stated, there is no cut-off value for the increased number of mast cells; it would be useful to know their average count of mast cells (9400) because it has been the initial methodology used in many previous reports. Most patients presented two or more varied symptoms attributed to mast cell mediators, including recurrent epistaxis, spontaneous tachycardia, itching of affected zones after sun exposure, nau- sea and matinal epigastric pain (disabling breakfast), abdominal pain, spontaneous ecchymosis, headaches and ear rubefaction (exceptionally described).…”

“…Dear Editor, I read with interest the proposal of Moon et al 1 about excluding telangiectasia macularis eruptiva perstans (TMEP) from the mastocytosis group, reclassifying it as a unique clinical feature of photoaging. In their series, they describe a subset of patients with symmetrical red to brown macules on sun-exposed areas.…”

“…Memorable ones were a female diagnosed with rosacea: while being treated with an antihistaminic for acute urticaria she told me that doing so had ceased a 1-year persistent diarrhea; and another woman with telangiectases, under psychiatric control for repeated fainting when nervous (sign exceptionally described). 4 As Moon et al 1 have stated, there is no cut-off value for the increased number of mast cells; it would be useful to know their average count of mast cells (9400) because it has been the initial methodology used in many previous reports.…”