Presentation
Mucosal involvement, as present in our patient, has not been described before. [clinicaldermatology.eu]
[…] an unusual clinical finding that presented persistent facial flushing in a patient with TMEP. [dovepress.com]
1 Unilateral telangiectasia macularis eruptiva perstans with an unusual clinical presentation. ( 29424045 ) 2018 2 Telangiectatic mastocytosis versus telangiectasia macularis eruptiva perstans. ( 30144139 ) Urbina F 2018 3 Telangiectasia Macularis Eruptiva [malacards.org]
IN REPORTING on a slide sent to him for an opinion as to the diagnosis in the case to be presented here, one of the leading dermatologic histopathologists in this country made this comment, "The series of words `telangiectasia macularis eruptiva perstans [jamanetwork.com]
Immune System
- Splenomegaly
[…] eruptiva perstans has been reported.[4] Most patients only have cutaneous involvement with pruritus of variable degree.[5] Systemic involvement is rare but can include episodic headaches, flushing, gastrointestinal complaints, palpitations, syncope, splenomegaly [escholarship.org]
Flushing, blistering, pruritus, cardiac arrhythmias, dyspnea, asthma exacerbations, hypotension, gastrointestinal upset, acid reflux, peptic ulcer disease, diarrhea, splenomegaly, increased numbers of mast cells in the bone marrow, abnormal skeletal radiographs [bloodjournal.org]
[…] tend to involute by adolescence, while lesions in adults are often persistent and at higher risk of being associated with systemic disease.[4] Symptoms of systemic involvement include flushing, dyspnea, abdominal pain, diarrhea, hypotension, syncope, splenomegaly [jcadonline.com]
Entire Body System
- Asymptomatic
Clinically, it is characterized by widespread, asymptomatic development of cutaneous telangiectasia in the absence of systemic symptoms. [readbyqxmd.com]
While most cutaneous mastocytoses occur in children and are asymptomatic, TMEP occurs predominantly in adults and is associated with systemic manifestations, requiring medical management. [pennstate.pure.elsevier.com]
We report a case of a 22-year-old female with an asymptomatic telangiectatic rash involving her left breast of 10 years' duration. Biopsies revealed cularis findings consistent with telangiectasia m a eruptiva perstans (TMEP). [ncbi.nlm.nih.gov]
Abstract We report a case of a 22-year-old female with an asymptomatic telangiectatic rash involving her left breast of 10 years' duration. Biopsies revealed cularis findings consistent with telangiectasia m a eruptiva perstans (TMEP). [unboundmedicine.com]
- Lymphadenopathy
There is no palpable lymphadenopathy or hepatosplenomegaly. He denies any fever, chills, abdominal pain or other skin concerns. Upon further questioning, the patient states that the symptoms have been evolving over the past 2 years. [plasticsurgerykey.com]
TMEP, accounting for [3] Systemic involvement may be present, and this is usually characterized by abdominal pain, diarrhea, flushing, dyspnea, hypotension, syncope, hepatosplenomegaly, lymphadenopathy, and bone changes. [4] Besides, elevated levels of [e-ijd.org]
Lymphadenopathy and hepatosplenomegaly were not present. Laboratory data A complete blood count and comprehensive metabolic panel were normal. Serum tryptase level was normal at 2 ng/mL. [escholarship.org]
- Fever
He denies any fever, chills, abdominal pain or other skin concerns. Upon further questioning, the patient states that the symptoms have been evolving over the past 2 years. [plasticsurgerykey.com]
The acute disease is often associated with fever, malaise, and arthralgias. [medical-dictionary.thefreedictionary.com]
Any systemic symptoms and similar family history were denied, including fever, joint pain, and only slight itching in the lesion area. [dovepress.com]
They can have infection, bleeding, and fevers from bone marrow failure engendered by too many mast cells. So, there can be a lot of different ways a person can come to medical attention with a mast cell disease. [onclive.com]
- Weight Loss
One year later she presented with weight loss, tiredness, and generalized pain particularly affecting her back. [jaad.org]
We present the case of a 51-year-old man with a history of pain with bowel movements and recent weight loss. Colonoscopy was performed, which revealed a rectal fissure with a lesion involving the rectum and anal canal. [medworm.com]
There was no associated fever, chestpain, abdominalpain, weight loss, diarrhea, syncope, bone pains, oral ulcers, joint pain., The patient was managed as a case of rosacea by local practitioners with topical treatments and she also self-medicated her [idoj.in]
Some of those patients may have extensive gastrointestinal tract infiltration resulting in low albumin, malabsorption, weight loss, and extensive diarrhea. [onclive.com]
- Constitutional Symptom
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]
Respiratoric
- Dyspnea
There was no history of any abdominal pain, diarrhea, flushing, dyspnea, hypotension, black-outs, and bone pain. Family history was unremarkable. [e-ijd.org]
TMEP can be a cause of morbidity with associated cutaneous findings (e.g. flushing, pruritus, ulcers) and extracutaneous symptoms (e.g. dyspnea, reflux, diarrhea, hypotension). [jofskin.org]
She also experienced flushing, dizziness, tinnitus, dyspnea, wheezing, cough, arthralgia and daily abdominal cramps with diarrhea. She was referred for bone marrow biopsy and further evaluation, and was diagnosed with TMEP on skin biopsy. [bloodjournal.org]
- Aspiration
[…] and get into the aspirate smear. [onclive.com]
Yes, in my opinion, you do need to do a bone marrow biopsy (rather than aspirate) on your patient. [aaaai.org]
Serum tryptase, bone marrow aspirate/biopsy, and complete blood count were negative for systemic mastocytosis. References 1. Kotz EA, Anderson D, Thierst BH. Cutaneous T cell lymphoma. J Eur Acad Dermatol Venereol. 2003;17: 131–137. 2. [jcadonline.com]
An aspiration removes a sample of fluid with a needle. The sample(s) are then analyzed by a pathologist. A common site for a bone marrow biopsy and aspiration is the pelvic bone, which is located in the lower back by the hip. [mastocytosisaustralasia.com]
Gastrointestinal
- Diarrhea
There was no history of any abdominal pain, diarrhea, flushing, dyspnea, hypotension, black-outs, and bone pain. Family history was unremarkable. [e-ijd.org]
TMEP can be a cause of morbidity with associated cutaneous findings (e.g. flushing, pruritus, ulcers) and extracutaneous symptoms (e.g. dyspnea, reflux, diarrhea, hypotension). [jofskin.org]
It is unclear if these patients demonstrated the rare systemic symptoms that are associated with TMEP, such as flushing, diarrhea, headaches, and abnormal skeletal surveys. [escholarship.org]
- Abdominal Pain
There was no history of any abdominal pain, diarrhea, flushing, dyspnea, hypotension, black-outs, and bone pain. Family history was unremarkable. [e-ijd.org]
He denies any fever, chills, abdominal pain or other skin concerns. Upon further questioning, the patient states that the symptoms have been evolving over the past 2 years. [plasticsurgerykey.com]
She denied headache, irritability, flushing, rhinorrhea, bone pain, nasuea, vomiting, diarrhea, and abdominal pain. She denied symptoms when taking non-steroidal anti-inflammatory agents or while drinking alcohol. [escholarship.org]
Cardiovascular
- Hypotension
There was no history of any abdominal pain, diarrhea, flushing, dyspnea, hypotension, black-outs, and bone pain. Family history was unremarkable. [e-ijd.org]
TMEP can be a cause of morbidity with associated cutaneous findings (e.g. flushing, pruritus, ulcers) and extracutaneous symptoms (e.g. dyspnea, reflux, diarrhea, hypotension). [jofskin.org]
Liver, Gall & Pancreas
- Hepatosplenomegaly
Neither osteolytic lesion nor hepatosplenomegaly were found. Though he was treated with azelastine hydrochloride for 4 months, the skin lesions were not improved. [jstage.jst.go.jp]
There is no palpable lymphadenopathy or hepatosplenomegaly. He denies any fever, chills, abdominal pain or other skin concerns. Upon further questioning, the patient states that the symptoms have been evolving over the past 2 years. [plasticsurgerykey.com]
TMEP, accounting for [3] Systemic involvement may be present, and this is usually characterized by abdominal pain, diarrhea, flushing, dyspnea, hypotension, syncope, hepatosplenomegaly, lymphadenopathy, and bone changes. [4] Besides, elevated levels of [e-ijd.org]
Musculoskeletal
- Bone Pain
There was no history of any abdominal pain, diarrhea, flushing, dyspnea, hypotension, black-outs, and bone pain. Family history was unremarkable. [e-ijd.org]
She denied headache, irritability, flushing, rhinorrhea, bone pain, nasuea, vomiting, diarrhea, and abdominal pain. She denied symptoms when taking non-steroidal anti-inflammatory agents or while drinking alcohol. [escholarship.org]
diarrhea, hypotension, syncope, splenomegaly, and bone pain.[3–5] Cutaneous mastocytosis can be divided into the following variants: urticaria pigmentosa, mastocytoma, diffuse and erythrodermic cutaneous mastocytosis, and TMEP.[3] TMEP is a rare form [jcadonline.com]
Skin
- Urticaria
Andrews, 1 in describing urticaria pigmentosa, stated briefly, "Persistent red macules with scarcely any pigmentation are observed in some cases. To these, Weber, Barber, and others have given the name telangiectasia macularis eruptiva perstans." [jamanetwork.com]
2017 9 Telangiectasia macularis eruptiva perstans (TMEP): A form of cutaneous mastocytosis with potential systemic involvement. ( 26899198 ) Severino M....Bulai Livideanu C. 2016 10 Telangiectasia macularis eruptiva perstans or highly vascularized urticaria [malacards.org]
Urticaria pigmentosa is a benign neoplastic process with a wide range of features clinical and findings histopathologic. In newborns and infants, lesions of urticaria pigmentosa may be plaques, nodules, and tumors, and bullae may develop on them. [derm101.com]
- Skin Lesion
Dermoscopic features of skin lesions in patients with mastocytosis. Arch Dermatol. 2011;147:932-40. [scielo.br]
Neither osteolytic lesion nor hepatosplenomegaly were found. Though he was treated with azelastine hydrochloride for 4 months, the skin lesions were not improved. [jstage.jst.go.jp]
Mastocytosis presents in two forms—cutaneous and systemic.[3] Mast cells most commonly localize to the skin, accounting for the frequent presentation of skin lesions in patients affected by either form. [jcadonline.com]
- Flushing
Although TMEP is a rare disease, facial flushing secondary TMEP should be emphasized in clinical practice. [dovepress.com]
There was no history of any abdominal pain, diarrhea, flushing, dyspnea, hypotension, black-outs, and bone pain. Family history was unremarkable. [e-ijd.org]
TMEP can be a cause of morbidity with associated cutaneous findings (e.g. flushing, pruritus, ulcers) and extracutaneous symptoms (e.g. dyspnea, reflux, diarrhea, hypotension). [jofskin.org]
- Pruritus
Two months after his initial presentation, he reported that he was feeling better on the prescribed regimen with significantly diminished pruritus. [jaadcasereports.org]
The patient had complete resolution of both his cutaneous lesions and pruritus, which has continued through one year of follow-up. [ncbi.nlm.nih.gov]
TMEP can be a cause of morbidity with associated cutaneous findings (e.g. flushing, pruritus, ulcers) and extracutaneous symptoms (e.g. dyspnea, reflux, diarrhea, hypotension). [jofskin.org]
The patient was started on topical steroids for two weeks and antihistamine therapy, with a noted decrease in pruritus but no change in cutaneous lesions. [actamedicaphilippina.upm.edu.ph]
H1 receptor antagonists can be helpful for reducing pruritus or flushing. [escholarship.org]
- Darier's Sign
The lesions did not urticate on rubbing (negative Darier sign). Of note, within most of the lesions conspicuous small arborizing capillary vessels which could be even better visualized on dermoscopy were noted ( Figure 1C ). [clinicaldermatology.eu]
Darier's sign was negative. There was no history of any abdominal pain, diarrhea, flushing, dyspnea, hypotension, black-outs, and bone pain. Family history was unremarkable. [e-ijd.org]
Darier sign was negative. Additionally, mild palmar erythema was noted bilaterally. [jaadcasereports.org]
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]
Due to the small number of mast cells, Darier's sign is usually absent. Histologic changes are very subtle, showing only a slight increase in mast cell numbers around capillaries and venules of the superficial plexus. We report two cases of TMEP. [koreascience.kr]
Neurologic
- Stroke
[…] skin releases histamine, causing hives Dermatographism: dermal edema resembling hives due to stroking with pointed instrument Description: Solitary lesion, or small group as part of urticaria pigmentosa Affect extremities and trunk, not palms and soles [pathologyoutlines.com]
Stroking the lesion did http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png International Journal of Dermatology Wiley http://www.deepdyve.com/lp/wiley/linear-telangiectasia-macularis-eruptiva-perstans-mimicking-acquired-rD7LxwBbmm [deepdyve.com]
Unlike other forms of cutaneous mastocytosis, Darier sign (wheal and flare reaction upon rubbing/stroking the lesion) is absent. The diagnosis requires demonstration of increased mast cells in skin biopsies in appropriate clinical context. [webpathology.com]
Stroking of lesion resulted in a wheal with flare (Darier's sign) in both cases. Systemic examination was within normal limits in both cases. [bioline.org.br]
The lesions blanched with diascopy but did not urticate, blister, or itch after a brisk stroke. There was no hepatosplenomegaly or lymphadenopathy. [escholarship.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
Treatment of Telangiectasia Macularis Eruptiva Perstans with an Intense Pulsed Light Device. [jofskin.org]
Unusual cutaneous findings of urticaria pigmentosa and telangiectasia macularis eruptiva perstans associated with marked myelofibrosis. ( 17040445 ) Turchin I....Schloss E. 2006 21 Telangiectasia macularis eruptiva perstans: unusual presentation and treatment [malacards.org]
In addition, treatment response criteria for the various clinical conditions, disease-specific symptoms, and specific pathologies are provided. [scienceopen.com]
The flushing patient: differential diagnosis, workup, and treatment. J Am Acad Dermatol. 2006;55(2):193–208. doi:10.1016/j.jaad.2005.07.057 [dovepress.com]
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
In most cases the disease occurs in adults with clinical manifestations limited to the skin with a good prognosis. [scienceopen.com]
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]
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
3345092 ) Parks A....Camisa C. 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]
Synonym: fifth disease Etiology The causative agent is human parvovirus B-19. [medical-dictionary.thefreedictionary.com]
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]
Epidemiology
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]
Epidemiology and Demographics Unlike urticaria pigmentosa, this disorder affects adults. Diagnosis Symptoms The rash is persistent, pigmented, asymptomatic. [wikidoc.org]
[…] 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]
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]
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]
Pathophysiology is not completely understood and further studies are needed on a larger scale, especially on the molecular level. [austinpublishinggroup.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]
Gastrocrom (Cromolyn) | A prescription medication called “Gastrocrom” (Cromolyn) s used in people with a food allergy to prevent serious reactions to foods. [santamariamedicine.com]