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Telangiectasia Macularis Eruptiva Perstans

Presentation

[…] an unusual clinical finding that presented persistent facial flushing in a patient with TMEP. [dovepress.com]

Unusual presentation of more common disease/injury Ringed telangiectasias: an unusual presentation of telangiectasia macularis eruptiva perstans Laila Siddique1, Hadjh Ahrns2, Elizabeth Seiverling2,3 1Penn State College of Medicine, Penn State Hershey [casereports.bmj.com]

One year later she presented with weight loss, tiredness, and generalized pain particularly affecting her back. [jaad.org]

Case Presentation A 61-year-old Caucasian male was evaluated simultaneously in dermatology and hematology clinics for asymptomatic skin rash and pancytopenia, respectively. [austinpublishinggroup.com]

History Figure 1 Figure 2 Figure 3 Figure 4 A 31-year-old woman presented to Bellevue Hospital Center with a ten-year history of blemishes that were distributed mostly on her limbs. [escholarship.org]

Cardiovascular

  • Heart Disease

    Case Report This case reports a 58-year-old woman, BMI 38.6, nonsmoker, with no family history of mastocytosis or psoriasis, ischemic heart disease, osteoporosis, and hepatosplenomegaly. [karger.com]

    Precordial murmurs in high cardiac output states: differentiation from murmurs of congenital heart disease in infancy. J Pediatr 1965;66:729-36. 72. Sardeman H, Tygstrup I. Prolonged obstructive jaundice and haemangiomas: report of 2 cases. [hkjpaed.org]

Neurologic

  • Neglect

    It will help dermatologists recognize the common clinical manifestations of rare diseases and avoid the neglect and wrong diagnosis of this disease. [dovepress.com]

    Atopic dermatitis Lichen simplex chronicus Stasis dermatitis Seborrheic dermatitis stasis dermatitis-common complication of chronic lower leg edema due to either congestive heart failure, venous incompetence, or both. may be neglected by both the patient [quizlet.com]

Workup

They discuss the clinical features, diagnostic workup, and therapeutic options in the management of this relatively rare condition. [ncbi.nlm.nih.gov]

Diagnostic workup including tissue biopsy must be considered in such patients after a thorough history and physical have been performed and other telangiectatic processes have been ruled out. [pennstate.pure.elsevier.com]

Serum

  • Dyslipidemia

    He denied systemic symptoms and/or aggravating factors and was monitored for hypertension and dyslipidemia. [ncbi.nlm.nih.gov]

Biopsy

  • Liver Biopsy

    A liver biopsy was not performed because laboratory exams and physical examination were normal. [ncbi.nlm.nih.gov]

    A liver biopsy was not performed because laboratory exams and physical examination were normal. The suggestive clinical picture and the typical histopathology exam confirmed the diagnosis of telangiectasia macularis eruptiva perstans. [jcadonline.com]

Treatment

First-line treatment – phototherapy – had to be stopped, and systemic treatment with interferon alpha-2a was initiated. The treatment was successful with regression of skin lesions as well as mast cell infiltrates in the bone marrow. [karger.com]

Instrumental treatment. Physiotherapy. Reeducation. Rehabilitation, speech therapy, crenotherapy. [pascal-francis.inist.fr]

Because it is an infrequent disorder, controlled studies evaluating efficacy of treatment modalities cannot be carried out and treatment is usually based on data evolving from case reports. [elsevier.es]

Treatment of Telangiectasia Macularis Eruptiva Perstans with an Intense Pulsed Light Device. [jofskin.org]

Psoralen plus ultraviolet light A treatment results in improvement of symptoms and regression of skin lesions by inhibiting histamine release by mast cells. However, recurrence can occur after a variable interval from the end of treatment. [ncbi.nlm.nih.gov]

Prognosis

Harpster Medicine Pediatric dermatology 2000 Telangiectasia macularis eruptiva perstans (TMEP) is a form of mastocytosis, an uncommon condition, particularly in children, characterized by telangiECTatic macules and generally has a good prognosis, with [semanticscholar.org]

The disorder is characterized by telangiectatic macules and generally has a good prognosis, with little tendency to urticate or cause constitutional symptoms. [ncbi.nlm.nih.gov]

In most cases the disease occurs in adults with clinical manifestations limited to the skin with a good prognosis. [scienceopen.com]

The overall prognosis of SM-AHNMD patients appears directly dependent upon the severity of the hematologic disease. [dermatologyadvisor.com]

Etiology

The etiology of pruritus is thought to be the accumulation of bile salts under the skin, whereas the pathogenesis of the telangiectatic damage is a result of a hyperdynamic circulation. [jaadcasereports.org]

[…] systemic mastocytosis. ( 3345092 ) 1988 40 Unilateral facial telangiectasia macularis eruptiva perstans. ( 3819061 ) Fried SZ...Lynfield YL 1987 41 Telangiectasia macularis eruptiva perstans; review of the literature, report of a case and discussion of the etiology [malacards.org]

Its etiology includes both congenital and acquired causes. PAV is characterized by one or a limited number of patches (composed of telangiectasia, atrophy, and hyper/hypopigmentation) especially over the breasts or buttocks (limb girdle area). [onlinelibrary.wiley.com]

Synonym: fifth disease Etiology The causative agent is human parvovirus B-19. [medical-dictionary.thefreedictionary.com]

Epidemiology

Epidemiology and Demographics Unlike urticaria pigmentosa, this disorder affects adults. Diagnosis Symptoms The rash is persistent, pigmented, asymptomatic. [wikidoc.org]

Articles in this issue include Human Mast Cell Signal Transduction; Mast cell tryptase role in homeostasis and coagulation; Mastocytosis: Current Classification and Diagnostic criteria; Epidemiology, risk factors and prognosis of mastocytosis; Mast cell [books.google.de]

[…] agammaglobulinemia Synonym(s): (no synonyms) Classification (Orphanet): - Rare allergic disease - Rare hematologic disease - Rare oncologic disease - Rare skin disease Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities - Epidemiological [csbg.cnb.csic.es]

The burden of liver disease in Europe: a review of available epidemiological data.J Hepatol. 2013; 58: 593-608 Weber F. Hellenschmied R. Telangiectasia macularis eruptiva perstans.Br J Dermatol. 1930; 42: 374-382 Marrouche N. Grattan C. [jaadcasereports.org]

The epidemiology of TMEP has yet to be fully defined. While urticaria pigmentosa, the most common of the cutaneous mastocytoses, is common in children, TMEP is very rare in children. Familial TMEP has been reported and can occur in children. [clinicaladvisor.com]

Pathophysiology

[…] form of cutaneous mastocytosis with potential systemic involvement that is often neglected in clinical practice, the classic clinical presentations of cutaneous lesions are usually as red-brown telangiectatic macules were shown.1,2 The pathogenesis and pathophysiology [dovepress.com]

We aimed to assess the clinical and histopathological characteristics of acquired bilateral TMEP, and the pathophysiological mechanism of acquired bilateral TMEP among these patients. [ncbi.nlm.nih.gov]

Pathophysiology is not completely understood and further studies are needed on a larger scale, especially on the molecular level. [austinpublishinggroup.com]

The authors present a case of telangiectasia macularis eruptiva perstans, an uncommon form of cutaneous mastocytosis, in a 53-year-old man and discuss its clinics, pathophysiology, laboratory results, and treatment. [scienceopen.com]

Prevention

The treatment of cutaneous mastocytosis aims at controlling symptoms and preventing mast cell degranulation. [pennstate.pure.elsevier.com]

TMEP is a benign condition, but special care should be taken in patients who have significant systemic symptoms to prevent complications. [aocd.org]

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