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Telangiectasia Macularis Eruptiva Perstans


Presentation

  • Therefore, TMEP should be considered when a patient presents with telangiectasia, even if the presentation is unilateral.[ncbi.nlm.nih.gov]
Short Stature
  • stature due to isolated growth hormone deficiency with X-linked hypogammaglobulinemia T-B severe combined immunodeficiency due to JAK3 deficiency X-linked agammaglobulinemia Synonym(s): (no synonyms) Classification (Orphanet): - Rare allergic disease[csbg.cnb.csic.es]
  • stature neurofibromatosis type II central neurofibromatosis; bilateral acoustic neuromas, meningioma, posterior subscapular cataracts, spinal glioma, unilateral schwanomma neuroma, acousticMEN type II, NF II neuroma, mucosal Sipple’s s. neutrophilic[dermadvocate.net]
Turkish
Rectal Urgency
  • He awakens with a "stomach ache" and rectal urgency, His palms itch, then his scalp groin and buttocks itch. He feels hot but does not sweat. Urticaria /- angioedema of the lips and face follow.[aaaai.org]
Petechiae
  • Physical examination demonstrated "cayenne pepper"-like brown macules, scattered petechiae, and multiple minute, blanching telangiectasias (Figure 1) that were more evident on dermatoscopy (Figure 2).[read.qxmd.com]
  • Notably, perifollicular lesions, petechiae, and ecchymosis occur more often below the hip, where there is greater hydrostatic pressure. [107] Prognostically, scurvy is fatal if left untreated.[emedicine.medscape.com]
  • […] lymphoma, melanoma, mesothelioma, sarcoma, TB, thyroid d. multiple myeloma alopecia, amyloidosis (primary), angioedema, cryoglobulinemia, follicular spicules of the nose, ichthyosiform dermatitis, leukocytoclastic vasculitis, necrobiotic xanthogranuloma, petechiae[dermadvocate.net]
  • Lesions are many and can coalesce) Langerhans cell histiocytosis (eruption may be extensive, involving scalp, face, trunk, buttocks, and intertriginous areas / closely set petechiae and scaly-crusty yellow-brown papules.[pcds.org.uk]
Papular Rash
  • Seen in UP Red-brown, maculo- papular rash, 0.5–3.5 mm, flushing, pruritis, Positive Darier's sign, dermatographism. Increased number of mature MC. Presents at any age, peak incidence in childhood and again in 3rd decade of life. Most common CM.[ncbi.nlm.nih.gov]
Axillary Freckling
  • Neurofibromas, axillary freckling and intraocular Lisch nodules were not detected on thorough whole-body examination. The lesions did not urticate on rubbing (negative Darier sign).[clinicaldermatology.eu]
Facial Pain
  • Although the occurrence of CM in the facial skin is rare, it may be a cause of chronic facial pain, and pain characteristics may mistakenly be interpreted as trigeminal nerve pathology.[read.qxmd.com]
  • A 75 year old woman complains to a physician of facial pain, headache, and intermittent visual symptoms. Both temporal arteries are biopsied.[quizlet.com]
Distractibility
  • The elusiveness of the diagnosis is enhanced at times by the presence in macular and papular lesions of eosinophils, a finding that may distract the observer from the fundamentally mast-cell character of the process.[derm101.com]
  • Discussion In our case, an uncommon disease manifestation as early as in the neonatal period distracted prior diagnosis.[clinicaldermatology.eu]
Trichotillomania
  • […] dermatitis and non-specific balanitis) and erythroplasia of Queyrat, inflammatory cicatricial alopecias (scalp discoid lupus erythematosus, lichen planopilaris, frontal fibrosing alopecia and folliculitis decalvans), nonscarring alopecias (alopecia areata, trichotillomania[read.qxmd.com]
  • Trichoblastoma (4 images) Trichodiscoma (0 images) Trichoepithelioma (7 images) Trichoepithelioma Papulosum Multiplex (8 images) Trichofolliculoma (1 images) Tricholemmal Carcinoma (0 images) Trichomoniasis (0 images) Trichomycosis Palmellina (1 images) Trichotillomania[dermis.net]
  • Trichotillomania Non-scarring alopecia with broken hairs; many with different lengths with excoriation involving the scalp. Treatment may involve behaviour modification.[icls.ca]
  • […] infection and kerion Staphylococcal folliculitis Syphilis Herpes simplex and zoster Lupus vulgaris Lichen planus Lupus erythematosus Sarcoid Scleroderma Basal cell carcinoma Non scarring alopecia occur in: Androgenic type Telogen effluvium Alopecia areata Trichotillomania[quizlet.com]
Agitation
  • Darier’s sign, a reaction to skin being rubbed/agitated resulting in redness and swelling, is usually absent, unlike other types of mastocytosis.[aocd.org]

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]

Treatment

  • Cosmetic treatment includes the use ofPsoralen and ultraviolet A (PUVA) therapy, total skin electron beam radiation and flashlamp pulsed-dye laser treatment.[ncbi.nlm.nih.gov]

Prognosis

  • 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]
  • 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]
  • Kagimoto, Yoshiko; Mizuashi, Masato; Iizawa, Osamu; Aiba, Setsuya 2012-01-01 00:00:00 Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis characterized by telangiectatic macules, with a generally good prognosis.[deepdyve.com]

Etiology

  • […] 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]
  • In the absence of systemic findings, however, a drug etiology must also be considered.[escholarship.org]
  • ., C1 stage of venous disease of legs as per CEAP (Clinical severity, Etiology, Anatomy, and Pathophysiology) classification. Superficial spider veins (reticular veins) are seen over the lower leg in this condition.[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 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • Source: Pathology Case Reviews - January 1, 2019 Category: Pathology Tags: Case Reports Source Type: research Fibromyalgia (FM) is characterized by chronic widespread pain whose pathophysiological mechanism is related to central and peripheral nervous[medworm.com]

Prevention

  • The treatment of cutaneous mastocytosis aims at controlling symptoms and preventing mast cell degranulation.[ncbi.nlm.nih.gov]
  • 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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