Telangiectasia macularis eruptiva perstans involving the upper arms in an adult male

ARTICLE

The clinico-pathological pictures are consistent with telangiectasia macularis eruptiva perstans (TMEP).

Routine blood tests, chest x-ray, echography of the abdomen and bone scintigraphy taken to rule out systemic involvement, were normal.

After one year of follow up no sign of systemic involvement was observed.

We proposed laser treatment, but the patient refused because the lesions were symptomless.

Comments

Mastocytosis is a disease characterized by an abnormal increase in mast cells.

Cutaneous mastocytosis including urticaria pigmentosa, solitary mastocytoma, diffuse erythrodermic mastocytosis and telangiectasia macularis eruptiva perstans (TMEP) are usually distinguished from systemic mastocytosis.

In order to ameliorate the care for patients with mastocytosis, Metcalfe [1] proposed a classification in four categories (indolent mastocytosis, mastocytosis with associated hematological disorders, mast cell leukemia, lymphoadenopatic mastocytosis with eosinophilia) based on prognosis and associated hematological disorders.

The first category includes TMEP. It was originally described by Parker in 1930 [2]. Since then only a few cases have been described, associated or not with other cutaneous manifestations of mastocytosis, namely urticaria pigmentosa.

The descriptive term of TMEP is related to the macroscopic erythematous appearence of the skin lesions caused by the permanent vessel expansion secondary to local release of mast cell mediators and angiogenetic factors [3].

TMEP usually occurs in adults and the lesions involve principally the trunk and the extremities and very rarely the face [4]. Generally TMEP is considered a cutaneous mastocytosis only, but systemic symptoms and involvement have been described. In particular intestinal haemorrhages, gastric ulcers, abdominal cramps, malabsorption and diarrhoea [2, 5], dyspnea, tachycardia and headache [6], depression, pruritus, flushing, nausea and haematological alteration [5-8] and bone lesions [2, 5] have been described in patients with TMEP.

In a recent report [9] the confusion existing as to the meaning of the term TMEP, which has been used to describe two clinically different forms of cutaneous mastocytosis,was stressed.

According to the authors true TMEP had no associated lesions of urticaria pigmentosa [4, 7, 9], Darier's sign is usually absent or slight and the histological features very subtle with only a slight increase in mast cell number around the vessels of the superficial plexus and in the interstitial collagen, as in our case.

Others described cases which have a variant of urticaria pigmentosa with associated telangiectatic features.

Moreover Sarkany [9] proposed separating these two entities with telangiectatic component because in the cases of true TMEP there would not be systemic involvement in opposition to the largest number of reported cases of urticaria pigmentosa with associated telangectasia (previously described as TMEP) [2, 5, 6, 8].

On the other hand the described case of true TMEP [7] according to Sarkany, in which a malignant evolution was reported, casts a doubt on his assumption.

Undoubtedly the complexity of the relationship between urticaria pigmentosa and TMEP is further complicated by the observation of an inherited form of urticaria pigmentosa resolved during childhood and developing into TMEP as an adult [10].

Differential diagnosis of TMEP is considered with other conditions with telangiectasia [11]. The bilateral features in our case, the skin biopsy and the absence of associated conditions permits us to exclude the last syndrome.

Some pharmacological blockers of the effects of mast cell mediators have been used in TMEP but their value is uncertain.

True effective treatment of TMEP, although temporary, is surgery with 585 nm flashlamp-pumped Dye laser [4]. The effect of the laser appears to be secondary to reduction of the vasculature with no apparent effect on the mast cells.

Our patient refused this treatment because he was symptom-free.

The interest of this case in our opinion lies in the bilateral aspect of the lesions completely involving the upper arms and the peculiar very subtle telangiectatic appearance.

REFERENCES

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4. Ellis DL. Treatment of telangiectasia macularis eruptiva perstans with the 585-nm flashlamp-pumped dye laser. Dermatol Surg 1996; 22: 33-7.

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8. Travis WD, Li C-Y, Bergstralh EJ, et al. Systemic mast cell disease: analysis of 58 cases and literature review. Medicine 1988; 67: 345-68.

9. Sarkany RPE, Monk BE, Handfield-Jones SE. Telangiectasia macularis eruptiva perstans: a case report and review of the literature. Clin Exp Dermatol 1998; 23: 38-9.

10. Clark DP, Buesher L, Havey A. Familial urticaria pigmentosa. Arch Int Med 1990; 150: 1742-4.

11. Wilkin JK. Unilateral nevoid telangiectasia. Arch Dermatol 1977; 113: 486-8.