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

Abstract

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

Telangiectatic patches in a 10‐year‐old boy

“…Diagnosis is confirmed by a mast cell infiltrate in the upper‐third of the dermis around dilated venules and capillaries on histology 1 . The predominance of lymphocytes and the deeper infiltration on this patient's histology, while atypical, have been reported in other cases of TMEP 4‐6 . Giemsa stains, as in this case, are helpful to differentiate spindle‐shaped mast cells from histiocytes.…”

“…1 The predominance of lymphocytes and the deeper infiltration on this patient's histology, while atypical, have been reported in other cases of TMEP. [4][5][6] Giemsa stains, as in this case, are helpful to differentiate spindle-shaped mast cells from histiocytes.…”

“…1 Cases of pediatric TMEP are rarer than in adults, with <20 cases reported since 1937. 4,5,7,8 Differences between the pathogenesis of adult and pediatric CM are still an area of study, with pediatric CM less associated with systemic symptoms and more likely to regress than adult CM. The understanding of the genetic basis of CM is evolving.…”

Child case of linear variant of telangiectasia macularis eruptiva perstans

“…As far as we know, only six children with TMEP have been reported. 1 We herein report a child with TMEP with characteristic linear distribution following Blaschko lines.…”

“…Dear Editor, Rosai-Dorfman disease (RDD) is a rare histiocytic condition involving non-Langerhans cells, characterized by self-limited bilateral cervical lymphadenopathy. [1][2][3] Extranodal involvement has also been reported involving virtually all organ systems. [1][2][3][4] Annular elastolytic giant cell granuloma (AEGCG), a histiocytic granulomatous disease, is also rare and 40% of these cases were noted on non-sun-exposed skin.…”