Telangiectasia macularis eruptiva perstans

Ak, Kalayciyan; Kotoğyan, Agop

doi:10.1046/j.1468-3083.2001.00247.x

Cited by 9 publications

(6 citation statements)

References 10 publications

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“…Of the five reported cases of TMEP in children, no medical treatment was required, as none had any systemic complaints 1–5 . The degree of symptomatic complaints in mastocytosis is probably related to the number of mast cells and mediators they produce, but the number of mast cells in the TMEP lesion showed only a slight increase as in our case 10 .…”

Section: Discussionmentioning

confidence: 43%

“…TMEP is usually characterized by scattered multiple telangiectatic macules primarily involving the trunk, extremities, or face. To our knowledge, five prepubescent children with TMEP have been reported in the literature 1–5 . The first case was a 2‐year‐old girl with macules on the extremities and face 1 .…”

Section: Discussionmentioning

confidence: 99%

“…Second and third cases were 10‐year‐old girls with macules on the face and extremities or the upper trunk and face 2,3 . The fourth was a 9‐year‐old girl with macules on the face, legs, and sacral area 4 . The fifth case was a 23‐month‐old boy with macules and patches on the anterior trunk and extremities 5 .…”

Section: Discussionmentioning

confidence: 99%

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Linear telangiectasia macularis eruptiva perstans mimicking acquired nevoid telangiectasia in a 5‐year‐old girl

et al. 2011

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Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis characterized by telangiectatic macules, with a generally good prognosis. 1 TMEP rarely affects young children and infants and so far has been reported as scattered or unilateral telangiectatic macules, but we report here an unusual case of linear TMEP in a five-year-old girl.A 5-year-old girl visited our hospital for pruritic eruptions that had developed on the right side of the neck, right upper arm, right hand, and left lower leg two years before ( Fig. 1a-c). There was neither a family history of the same symptom nor personal medical history.Closer examination showed linearly distributed telangiectases on the neck, right arm ( Fig. 1d), right hand, and left lower leg in a contralateral and dermatomal pattern, which seemed to be in the distribution of right C3-5, C7, T1, and left L5. The lesions were smooth to the touch on palpation. Stroking the lesion did not stimulate Darier's sign. There was no splenomegaly or lymphadenopathy.The laboratory findings, including the basophil level of the peripheral blood smear, liver transaminases, alkaline phosphatase, serous estradiol, and thyroid hormones, were within normal limits.Suspecting the possibility of acquired nevoid telangiectasia (ANT), morphea, or TMEP (linear mastocytosis), we obtained a biopsy from part of a lesion. A punch biopsy specimen from her right upper arm showed thin and markedly dilated capillaries with an abundant perivascular infiltrate of lymphocytes in the papillary dermis, without hyperpigmentation in the basal layer ( Fig. 2a). There was no remarkable sclerosis in the dermis. Both Toluidine blue stain (Fig. 2b) and Giemsa stain demonstrated scattered spindle-shaped mast cells, mainly around the dilated vessels and follicles. Based on her clinical appearance and histopathological findings, a diagnosis of TMEP was made. She was treated with beclomethasone dipropionate ointment for three months, but it failed to improve her eruptions. DiscussionTMEP is usually characterized by scattered multiple telangiectatic macules primarily involving the trunk, extremities, or face. To our knowledge, five prepubescent children with TMEP have been reported in the literature. 1-5 The first case was a 2-year-old girl with macules on the extremities and face. 1 Second and third cases were 10-year-old girls with macules on the face and extremities or the upper trunk and face. 2,3 The fourth was a 9-yearold girl with macules on the face, legs, and sacral area. 4 The fifth case was a 23-month-old boy with macules and patches on the anterior trunk and extremities. 5 To the best of our knowledge, this young girl is the first case of pediatric linear TMEP to be reported in the literature.Several adults with unilateral facial TMEP have been reported, which may mimic ANT. 6,7 ANT is usually 69 ª

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Section: Discussionmentioning

confidence: 43%

Section: Discussionmentioning

confidence: 99%

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Linear telangiectasia macularis eruptiva perstans mimicking acquired nevoid telangiectasia in a 5‐year‐old girl

et al. 2011

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“…Genellikle erişkinlerde görülen hastalık, sıklıkla orta yaşlı obez kadınları etkilemektedir (1,6). Bununla birlikte literatürde bebek ve çocuk olgulara da rastlanmaktadır (7,8). Klinik olarak eritemli zeminde birbiri ile birleşme eğiliminde çok sayıda, asemptomatik, kahverengi hiperpigmente maküller görülür.…”

Section: Discussionunclassified

Telenjiektazia Makülaris Eruptiva Perstans Olgusu

Çölgeçen¹,

Okur²,

Erdoğan³

et al. 2014

tdd

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Telenjiektazia makülaris eruptiva perstans (TMEP), mastositozların nadir görülen bir formu olup, genellikle erişkinlerde görülür. Sıklıkla gövdede yuvarlak, oval, kahverengi kırmızı maküler lezyonlarla seyreder. Sistemik tutulum görülebilir. Bu makalede, klinik ve histopatolojik olarak TMEP tanısı konulan 51 yaşında kadın hasta, TMEP'in nadir görülmesi nedeni ile sunulmaktadır.

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“…There have been reports of TMEP in children, infants and familial cases but these are extremely rare. 27 , 28 TMEP is traditionally thought to be restricted to the skin, however, it has been reported in association with SM. 10 , 29 Bone marrow, gastrointestinal tract, liver, spleen and lymph node involvement have all been described in patients with TMEP.…”

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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Telangiectasia macularis eruptiva perstans

Cited by 9 publications

References 10 publications

Linear telangiectasia macularis eruptiva perstans mimicking acquired nevoid telangiectasia in a 5‐year‐old girl

Linear telangiectasia macularis eruptiva perstans mimicking acquired nevoid telangiectasia in a 5‐year‐old girl

Telenjiektazia Makülaris Eruptiva Perstans Olgusu

Telangiectasia Macularis Eruptiva Perstans: more than skin deep

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