|LETTER TO THE EDITOR
|Year : 2018 | Volume
| Issue : 5 | Page : 351-353
Unilateral facial telangiectasia macularis eruptiva perstansmimicking as rosacea
Preema Sinha, Anwita Sinha, Deep K Raman, Aradhana Sood
Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India
|Date of Web Publication||4-Sep-2018|
Department of Dermatology, Armed Forces Medical College, Pune - 411 040, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sinha P, Sinha A, Raman DK, Sood A. Unilateral facial telangiectasia macularis eruptiva perstansmimicking as rosacea. Indian Dermatol Online J 2018;9:351-3
|How to cite this URL:|
Sinha P, Sinha A, Raman DK, Sood A. Unilateral facial telangiectasia macularis eruptiva perstansmimicking as rosacea. Indian Dermatol Online J [serial online] 2018 [cited 2019 Nov 12];9:351-3. Available from: http://www.idoj.in/text.asp?2018/9/5/351/240524
A 23-year-old lady presented with a red itchy lesion on right cheek. The lesion started five years abck as a red pin head sized eruption which progressed to three times its original size over the next six months following which the size of the lesion remained stable. The lesion was associated with redness and itching which aggravated on touching or rubbing the lesion. She did not complaint of anypain or discharge from the lesion, numbness over the lesion, or any similar lesions elsewhere on the body. 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 with topical steroids.
No significant abnormality was detected in general physical and systemic examinations. Local examination revealed a partially defined solitary, normoaesthetic, erythematous, macule measuring 5 mm × 5 mm in diameter over right malar region adjoining right nasolabial fold [Figure 1] with positive Darier's sign [Figure 2]. Dermatoscopic examination revealed linear tortuous and polygonal vessels, red diffuse areas, and yellowish orange amorphous areas over the right cheek. White structureless area was also noted which can be explained be due to atrophy following topical steroid use [Figure 3]. Skin biopsy revealed orthokeratotic epidermis with collagenous fibrosis with dilated vascular channels in dermis. Toluidine blue stain demonstrated mast cells distributed in perivascular pattern and scattered in upper one-third of reticular dermis and within dermal appendages [Figure 4].
|Figure 3: Linear tortuous and polygonal vessels (black arrow), red diffuse areas and yellowish-orange amorphous areas (blue arrow), and white structureless areas (red arrow) demonstrated on dermoscopy: (Dermlite DL4; polarized mode, 10 × magnification, with pigment boost plus)|
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|Figure 4: Perivascular clustering of mast cells and dense collagenous fibrosis in the dermis- - Toluidine blue (400×)|
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No prominent dermal nerves, perineural inflammation, or granulomas were found to be present in dermis [Figure 5]. Mast cells were highlighted on Immunohistochemical stain for CD117 [Figure 6]. Serum tryptase level was within normal limits (6 ng/mL). Based on the above findings, a final diagnosis of telangiectatic variant of urticaria pigmentosa- was reached.
|Figure 5: H and E (200×) scanner image demonstrating minimal perivascular mono-nuclear infiltrates|
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|Figure 6: (IHC400×). Immunohistochemistry for CD117 highlighting the mast cells. HF: hair follicle; BV: blood vessel; arrows: mast cells positive for CD117|
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The patient was counseled regarding avoidance of precipitating factors such as stress, exercise, drugs, extremes of temperature, and alcohol. She was started on oral antihistamines, leukotriene receptor antagonists, and broad-spectrum sunscreens resulting in significant improvement in her symptoms. Mast cells can infiltrate multiple organs, including skin, spleen, bone marrow, liver, and lymph nodes, with skin being the most commonly involved site. Cutaneous mastocytosis (CM) is the proliferation of mast cells in the skin without evidence of involvement of other organs. It is known to havefour clinical variants: urticaria pigmentosa, isolated mastocytoma, diffuse cutaneous or erythrodermic mastocytosis, and telangiectasia maculariseruptiva perstans (TMEP). CM is associated with local and systemic symptoms resulting from release of several mediators such as histamine, leading to cutaneous flushing, dyspnea, rash, exacerbation of asthma, hypotension, and gastrointestinal disorders such as gastroesophageal reflux, ulcers, and diarrhea.
TMEP described by Parkes Weber in 1930 is a rare variant of cutaneous mastocytosis seen predominantly in adults. The lesions of TMEP typically appear as small, irregular reddish-brown telangiectatic macules. Lesions are typically non-pruritic and located on the trunk and proximal extremities in a symmetrical pattern. Palms, soles, and face are spared in most of the cases. Darier's sign is usually negative or weakly positive in patients with TMEP due to the relatively lower density of mast cellsin the lesions; although, it was positive in our patient. Sometimes, TMEP may involve unusual sites such as unilateral aspect of face or upper arms.,
The mechanism responsible for the telangiectasias and erythemaof the lesions seen inTMEP is local release of mast cell mediators and angiogenic factors from the activated mast cells which can leads to permanent vasodilation. Although most cases are limited to the skin, systemic involvement may be seen. Bone marrow, gastrointestinal tract, liver, spleen, and lymph node involvement is not uncommon.,
Dermoscopy in TMEP has been described by Akay et al. who described a reticular vascular pattern. Vano-Galvan et al. evaluated the dermoscopic findings of 127 patients with CM and described four distinct patterns: pigmented network, yellow-orange amorphous area, brown amorphous area, and telangiectasia with reticular pattern.,
In comparison, the dermoscopic hallmark of rosacea are the linear vessels characteristically arranged in vascular polygons.
There is no gold standard treatment for CM and the treatment is individualized according to the symptoms presented by the patient. Evidence from published literature favors an approach using a combination of avoidance of triggers such as alcohol, exercise, extreme temperatures, venoms, anaesthesia, aspirin, and nonsteroidal anti-inflammatory drugs thus limiting release of mast cell mediators alongwith treatment options such as antihistamines, phototherapy, mast cell stabilizers, and injectable epinephrine in case of anaphylaxis. A 585-nm flashlamp-pumped dye laser has also been reported to improve cutaneous symptoms. Yearly complete blood count, comprehensive metabolic panel, serum tryptase levels, 24-h urine histamine, and SCORMA index are recommended for disease surveillance and monitoring response to treatment.
This report highlights a case involving a young female with TMEP who was misdiagnosed for many years due to the unusual site of presentation.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]