Presentation of Telangiectasia Macularis Eruptiva Perstans as a Long-Standing Solitary Plaque Associated with Renal Carcinoma

Pascual, José C.; Bañuls, José; Albares, M.P.; Vergara, Gloria; Belinchón, Isabel; Silvestre, Juan Francisco; Betlloch, Isabel

doi:10.1007/s10227-002-0144-z

Cited by 4 publications

(1 citation statement)

References 17 publications

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“…Some cases of TMEP have been described as familial, with a clustering within families. 27 , 31 There have been rare cases described in patients undergoing radiation, in patients with breast or renal carcinoma, 32 malignant melanoma, 33 or in patients with Sjogrens syndrome. 34 Other cases have been described in patients with multiple myeloma, 35 myeloproliferative disorders (such as Polycythemia Rubra Vera) 36 and in association with leukemia or myelofibrosis.…”

Section: Discussionmentioning

confidence: 99%

Telangiectasia Macularis Eruptiva Perstans: more than skin deep

Watkins

Bokor²,

Leicht³

et al. 2011

Dermatol Reports

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Systemic mastocytosis is a rare disease involving the infiltration and accumulation of active mast cells within any organ system. By far, the most common organ affected is the skin. Cutaneous manifestations of mastocytosis, including Urticaria Pigmentosa (UP), cutaneous mastocytoma or telangiectasia macularis eruptive perstans (TMEP), may indicate a more serious and potentially life-threatening underlying disease. The presence of either UP or TMEP in a patient with anaphylactic symptoms should suggest the likelihood of systemic mastocytosis, with the caveat that systemic complications are more likely to occur in patients with UP. TMEP can usually be identified by the typical morphology, but a skin biopsy is confirmative. In patients with elevated tryptase levels or those with frequent systemic manifestations, a bone marrow biopsy is essential in order to demonstrate mast cell infiltration. Further genetic testing for mutations of c-kit gene or the FIP1L1 gene may help with disease classification and/or therapeutic approaches. Rarely, TMEP has been described with malignancy, radiation therapy, and myeloproliferative disorders. A few familial cases have also been described. In this review, we discuss the clinical features, diagnosis and management of patients with TMEP. We also discuss the possible molecular pathogenesis and the role of genetics in disease classification and treatment.

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Section: Discussionmentioning

confidence: 99%

Telangiectasia Macularis Eruptiva Perstans: more than skin deep

Watkins

Bokor²,

Leicht³

et al. 2011

Dermatol Reports

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Treatment of telangiectasia macularis eruptiva perstans with montelukast

Cengizlier

Hücümenoğlu

Ozen

et al. 2009

Allergologia et Immunopathologia

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Telangiectasia Macularis Eruptiva Perstans: Unusual Presentation and Treatment

Rishpon

Matz

Gat

et al. 2006

Skinmed

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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.

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Presentation of Telangiectasia Macularis Eruptiva Perstans as a Long-Standing Solitary Plaque Associated with Renal Carcinoma

Cited by 4 publications

References 17 publications

Telangiectasia Macularis Eruptiva Perstans: more than skin deep

Telangiectasia Macularis Eruptiva Perstans: more than skin deep

Treatment of telangiectasia macularis eruptiva perstans with montelukast

Telangiectasia Macularis Eruptiva Perstans: Unusual Presentation and Treatment

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