Telangiectasia macularis eruptiva perstans

Cohn, Monique S.; Mahon, Michael J.

doi:10.7556/jaoa.1994.94.3.246

Cited by 22 publications

(5 citation statements)

References 5 publications

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“…The mastocytosis syndrome is known to appear slightly more often in men than in women (1.5:1) 11 . Our review of the literature on TMEP cases reported since 1990, in contrast, revealed reports on 15 men and 14 women, 2,4,8–10,12–29 not including our report. The equal prevalence of TMEP among men and women differs from the slightly greater prevalence among men of the mastocytosis group to which TMEP belongs.…”

contrasting

confidence: 55%

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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contrasting

confidence: 55%

Telangiectasia Macularis Eruptiva Perstans: Unusual Presentation and Treatment

Rishpon

Matz

Gat

et al. 2006

Skinmed

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“…Although UP is generally well‐recognized when presenting with its classical appearance, unusual macroscopic presentations of cutaneous mastocytosis are often misdiagnosed given the potential variability in clinical presentation and subtlety with establishing the histological diagnosis in the context of a low pre‐test probability 14 . Adult patients with UP often have a high likelihood of an underlying systemic mast cell disorder 15 . One case series described systemic involvement in up to 47% of cases with cutaneous mastocytosis 16 with aggressive (non‐indolent) systemic mastocytosis in up to 9% of those cases.…”

Section: Discussionmentioning

confidence: 99%

“… 14 Adult patients with UP often have a high likelihood of an underlying systemic mast cell disorder. 15 One case series described systemic involvement in up to 47% of cases with cutaneous mastocytosis 16 with aggressive (non‐indolent) systemic mastocytosis in up to 9% of those cases. Therefore, any patient presenting with characteristic skin findings should be investigated as having a cutaneous manifestation of systemic mastocytosis.…”

Section: Discussionmentioning

confidence: 99%

Urticaria pigmentosa and systemic mastocytosis

Keow,

Chin‐Yee,

Hsia

et al. 2023

Clinical Case Reports

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Key Clinical MessageAdditional investigations for systemic involvement should be initiated once the diagnosis of cutaneous mastocytosis has been established in an adult patient. A serum tryptase can serve as a screening test for systemic mastocytosis, and persistent elevations should prompt further investigations, such as bone marrow studies.AbstractUrticaria pigmentosa (UP) is the most common form of cutaneous mastocytosis, presenting as a wide variety of macroscopic appearances. Cutaneous mastocytosis in pediatric patients usually does not present with systemic involvement, but more than half of adult patients with cutaneous mastocytosis demonstrate systemic involvement. Currently, there is no guidance surrounding systemic testing in patients with UP. A 50‐year‐old Caucasian male was referred to the Clinical Immunology and Allergy clinic with a history of a rash. He initially presented to hospital 12 years prior with group A beta hemolytic streptococcus bacteremia treated with multiple different antibiotics. One week following discharge, he developed erythematous brown spots on his right leg which were flat, non‐pruritic, and not painful. The rash later expanded to his trunk and extremities. A skin biopsy performed 2 years prior to referral to our clinic demonstrated urticaria pigmentosa. The CD117 immunohistochemical stain showed increased perivascular and interstitial mast cells in the superficial dermis. Darier's sign was negative on physical examination, and venom testing was also negative. Although he had no symptoms of systemic involvement, his serum tryptase was elevated at 47.6 ng/mL in the context of normal kidney and liver function. A skeletal survey was normal, and an abdominal ultrasound ruled out splenomegaly. Bone marrow biopsy demonstrated a mild increase in paratrabecular and perivascular atypical mast cells, in keeping with systemic mastocytosis. Adult patients with cutaneous mastocytosis have a high likelihood of having an underlying systemic mast cell disorder. Therefore, any patient presenting with characteristic skin findings should be investigated as having a cutaneous manifestation of systemic mastocytosis. This case demonstrates the utility of serum tryptase and its role in triggering additional investigations and guiding appropriate therapy.

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“…TMEP is a rare manifestation of cutaneous mastocytosis and is often overlooked or misdiagnosed given its variable clinical presentation [15] and subtlety with establishing the histological diagnosis [16]. Unlike other forms of cutaneous mastocytosis, patients with TMEP have a high likelihood of an underlying systemic mast cell disorder [17,18]. One case series described systemic involvement in up to 47% of cases with TMEP [6] with aggressive (non-indolent) systemic mastocytosis in up to 9% of those cases.…”

Section: Discussionmentioning

confidence: 99%

Telangiectasia macularis eruptiva perstans with systemic involvement: a case report

Keow

Ayoub

Chin‐Yee

et al. 2022

Preprint

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Background: Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis which usually presents as ill-defined reddish brown telangiectatic macules. Although cutaneous mastocytosis rarely presents with systemic involvement, almost half of patients with TMEP demonstrate systemic mastocytosis. Currently, there is no guidance surrounding systemic testing in patients with TMEP. Case presentation: A 50-year-old Caucasian male was referred to the Clinical Immunology and Allergy clinic with a history of a rash. He initially presented to hospital 12 years prior with group A beta hemolytic streptococcus bacteremia treated with multiple different antibiotics. One week following discharge, he developed erythematous brown spots on his right leg which were flat, non-pruritic and not painful. The rash later expanded to his trunk and extremities. A skin biopsy performed two years prior to referral to our clinic demonstrated telangiectasia macularis eruptiva perstans. The CD117 immunohistochemical stain showed increased perivascular and interstitial mast cells in the superficial dermis. Darier’s sign was negative on physical examination, and venom testing was also negative. Although he had no symptoms of systemic involvement, a serum tryptase was elevated at 47.6 ng/mL in the context of normal kidney and liver function. A skeletal survey was normal and an abdominal ultrasound ruled-out splenomegaly. Bone marrow biopsy demonstrated a mild increase in paratrabecular and perivascular atypical mast cells, in keeping with systemic mastocytosis.Conclusions: Unlike other forms of cutaneous mastocytosis, patients with TMEP have a high likelihood of an underlying systemic mast cell disorder. Therefore, any patient presenting with characteristic skin findings should be investigated as having a cutaneous manifestation of systemic mastocytosis. This case demonstrates the utility of serum tryptase, and its role in triggering additional investigations and guiding appropriate therapy.

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Telangiectasia macularis eruptiva perstans

Cited by 22 publications

References 5 publications

Telangiectasia Macularis Eruptiva Perstans: Unusual Presentation and Treatment

Telangiectasia Macularis Eruptiva Perstans: Unusual Presentation and Treatment

Urticaria pigmentosa and systemic mastocytosis

Telangiectasia macularis eruptiva perstans with systemic involvement: a case report

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