Presentation of telangiectasia macularis eruptiva perstans as. Telangiectasia macularis eruptiva perstans tmep is a rare form of cutaneous mastocytosis seen commonly in adults. Telangiectasia macularis eruptiva perstans tmep is a rare form of cutaneous mastocytosis and accounts for less than 1 percent of cases of mastocytosis. Abstract the authors present a case of telangiectasia macularis eruptiva perstans, an uncommon form of cutaneous mastocytosis, in a 53yearold man and discuss its clinics, pathophysiology, laboratory results, and treatment. The cosmetic treatment of urticaria pigmentosa with nd. Telangiectasia macularis eruptiva perstans an overview. Telangiectasia macularis eruptiva perstans primary care. Telangiectasia macularis eruptiva perstans is a rare form of cutaneous mastocytosis, occurring more frequently in middleaged adults and rarely in children. Treatment of telangiectasia macularis eruptiva perstans with the 585nm flashlamppumped dye laser. Telangiectasia macularis eruptiva perstans tmep is a rare type of mastocytosis that presents as multiple asymptomatic, flat, reddishbrown lesions on the skin. A 5yearold girl was referred to our department for further workup of asymptomatic disseminated reddishbrown macules with congenital onset and persistent course.
Ferrari b, spiner r, schroh r, alperovich r, hassan m. Cutaneous mastocytosis is a proliferation of masts cells on the skin without involvement of other organs. Visible small blood vessels that are blue in colour spider veins are called venulectasia because venules are involved. As the patient had no clinical symptoms, no medication was administered, and he was monitored periodically by a. Telangiectasia macularis eruptiva perstans tmep is an uncommon form of cutaneous mastocytosis that occurs exclusively in adults. It affects predominantly adults and is characterized by erythematous and or yellowbrownish macules with telangiectasias, preferably located on the trunk and upper. It appears most frequently in adults and only rarely affects young children or infants. Telangiectasia macularis eruptiva perstans telangiectasia macularis eruptiva perstans kalayciyan, ak. Pdf telangiectasia macularis eruptiva perstans ata. In some examples of the urticaria pigmentosa variant of mastocytosis, the increase in mast. Dermatoscopic findings in telangiectasia macularis eruptiva perstans 644 case report a 59 yearold male patient reported the appearance of erythematous skin lesions on the trunk and upper limbs 20 years ago, which regressed after a few months, leaving residual hyperchromic lesions.
Clinical and experimental dermatology, 1998 jan, 231. Telangiectasia macularis eruptiva perstans wikipedia. Telangiectasia macularis eruptiva perstans tmep is a rare form of cutaneous mastocytosis cm. It presents on the trunk and upper limbs as telangiectatic macules and patches due to concentrated mast cells degranulation which are identified as reticular vascular pattern in dermoscopy. Tmep is generally agreed to be cutaneous mastocytosis, an uncommon variant of urticaria pigmentosa.
The cutaneous forms of mastocytoses include urticaria pigmentosa, diffuse cutaneous mastocytosis, maculopapular mastocytosis, mastocytoma and telangiectasia macularis eruptiva perstans tmep. Courtesy of bryan anderson, md the presence of mast cell hyperplasia in the skin of mastocytosis patients can be confirmed clinically by firmly rubbing a characteristic lesion. Some researchers consider it a rare subtype of cutaneous mastocytosis. Jul 30, 2016 telangiectasia macularis eruptive perstans tmep is a very rare skin disease. What is the differential diagnosis of telangiectasia. Telangiectasia macularis eruptiva perstans clinical findings. Telangiectasia macularis eruptiva perstans tmep is a rare adult mastocytosis with prominent cutaneous manifestations including red to brown telangiectatic macules. Telangiectasia macularis eruptiva perstans presenting as. Telangiectasia macularis eruptive perstans genetic and. Telangiectasia macularis eruptiva perstans is a controversial clinical entity and was not recognized by the who. Two cases of telangiectasia macularis eruptiva perstans demonstrated by immunohistochemistry for ckit cd 117. Telangiectasia macularis eruptiva perstans is persistent, pigmented, asymptomatic eruption of macules usually less than 0. Dermatoscopy of the lesions show redtobrown, telangiectatic macules diffusely spread over the. Dermatoscopic findings in telangiectasia macularis.
Cutaneous mastocytosis, including urticaria pigmentosa, solitary mastocytoma, diffuse cutaneous mastocytosis, and telangiectasia macularis eruptiva perstans tmep, is usually distinguished from systemic mastocytosis. The most frequent site of organ involvement of mastocytosis is the skin. Dermatoscopic findings in telangiectasia macularis eruptiva. Telangiectasia macularis eruptiva perstans tmep is a rare adult mastocytosis with prominent cutaneous manifestations including red to brown telangiectatic macules typically on the chest or limbs. The disorder is characterized by telangiectatic macules and. Telangiectasia macularis eruptive perstans successfully treated with puva therapy. A clinical approach to diagnose patients with localized. It presents most often in adulthood and clinically is comprised of tan macules and small patches with telangiectases that most often involve the trunk 2. Telangiectasia macularis eruptiva perstans tmep is a rare form of cutaneous mastocytosis. A new insight over telangiectasia macularis eruptiva perstans. Recent advances in the understanding and therapeutic.
Histologically, tmep presents with scattered mast cells lined up around the dilated capillaries and venules of. Tmep is a very rare cutaneous finding seen in less than one percent of patients with mastocytosis. We present a 31yearold woman with a tenyear history of a progressive macular eruption. The term telangiectasia macularis eruptiva perstans tmep was originally used to describe a rare form of cutaneous mastocytosis cm that was limited to the skin with lesions consisting of irregular, telangiectatic macules ranging in color from red to brown. Please refer to the related chapter on mastocytosis. Mastocytosis urticaria pigmentosa, telangiectasia macularis. Telangiectasia macularis eruptive perstans tmep is a very rare skin disease. The diagnosis in this case is telangiectasia macularis eruptive perstans tmep, one of the classic clonal mast cell dyscrasias. To these, weber, barber, and others have given the name telangiectasia macularis eruptiva perstans. Telangiectasia macularis eruptiva perstans tmep, is a rare variant of cutaneous mastocytosis characterized by pruritic telangiectatic macules. Telangiectasia macularis eruptiva perstans tmep is a rare subtype of cutaneous mastocytosis that tends to appear during adulthood. Telangiectatic macularis eruptiva perstans tmep is the rarest type of cutaneous mastocytosis cm. Pdf telangiectasia macularis eruptiva perstans aylin. Two cases of telangiectasia macularis eruptiva perstans demonstrated by.
Telangiectasia macularis eruptiva perstans in the presence. At birth or during the first 3 months of life may present with flushing attacks due to high histamine content. A majority of the pictures are of skin manifestations of mastocytosis. Jan 31, 2011 telangiectasia macularis eruptiva perstans. Clinically, it present as erythematous or pigmented macules and papules, dotted with telangiectasias, located on the trunk and limbs. In telangiectasia macularis eruptive perstans, features may be subtle, with increased mast cells around dilated superficial capillaries, basal cell hyperpigmentation of overlying epidermis, superficial lymphohistiocytic infiltrate. Telangiectasia macularis eruptiva perstans american. Telangiectasia macularis eruptiva perstans tmep is a rare type of mastocytosis that i occasionally encounter in both my the woodlands dermatology and conroe dermatology offices.
Clinical and molecular categorization of progressive. Either urticaria pigmentosa, solitary mastocytoma, diffuse and erythrodermic cutaneous mastocytosis or telangiectasia macularis eruptiva perstans clinical features. Most cutaneous mast cell disorders have a good prognosis mastocytosis. Telangiectasia macularis eruptiva perstans, british journal. Telangiectasia macularis eruptiva perstans tmep is a form of mastocytosis. Telangiectasia is a condition in which there are visible small linear red blood vessels broken capillaries. Mastocytosis is a rare disease characterized by a primary pathological increase in mast cells in different tissues. The severity of symptoms depends on the number of mast cells in the tissues. Telangiectasia macularis eruptiva perstans tmep is a rare form of cutaneous mastocytosis with distinctive semiology and histopathology, but that is not free of controversy regarding its individualization as a nosological entity. Telangiectasia macularis eruptiva perstans ahmet altiner md, julia tzu md, rishi patel md, shane meehan md, miguel sanchez md dermatology online journal 17 10. Two cases of telangiectasia macularis eruptiva perstans. Telangiectasia macularis eruptiva perstans tmep is a much rarer form of cutaneous mastocytosis that affects adults. Telangiectasia macularis eruptiva perstans and anaphylaxis. It affects predominantly adults and is characterized by.
Systemic mastocytosis was present in 47% of these patients. Clinically, there are tantobrown macules and patchy erythema with telangiectases that most often. Tmep may show systemic manifestations and may be associated with myelodysplasia, myeloproliferative disorders, acute myeloid leukemia, andor lymphoproliferative disease, but is not well recognized by hematologists. He presented with a diffuse, telangiectasic macules within a tanbrown background. Telangiectasia macularis eruptiva perstans in an indian. Telangiectasia macularis eruptiva perstans in an indian man. Telangiectasia macularis eruptiva perstans, journal of the. For some authors, the presence of exclusively telangiectatic macules, along with superficial vascular plexus dilation and low density of mast.
Telangiectasia macularis eruptiva perstans tmep has not been fully characterized. These conditions generally run a benign course, but rarely, those with systemic involvement can evolve into a more serious haematological disorder. Clinically, tmep appears as very small red brown macules on the trunk and extremities. May 01, 2001 a diagnosis of telangiectasia macularis eruptiva perstans tmep was suspected. Telangiectasia macularis eruptiva perstans dermnet nz. Telangiectasia macularis eruptiva perstans request pdf. Tmep is typically characterised by scattered redbrown macules on the. The lesions of tmep typically appear as small, irregular red spots and brown widened blood vessels on the skin telangiectasia mostly located on the trunk, legs, and arms in a symmetrical pattern. It is an uncommon condition, particularly in children. Sutton and sutton 2 devoted a brief but comprehensive paragraph to the disorder and agreed to its interrelation with urticaria pigmentosa. A case presentation and discussion between 2 mm and 4 mm in diameter and are commonly found on the trunk and proximal extremities, symetrically.
Lee hw, jeong yi, choi jc, lee mw, choi jh, moon kc, et. Four years previously subcutaneous nodules that had appeared on her knees and the occipital area were. Cutaneous mastocytosis is a proliferation of mast cells limited to the skin that spares other organs. Some authors suggest a fourth subtype of cm, telangiectasia macularis eruptive perstans tmep, should be considered a distinct entity from up. We present the case of a 58yearold woman who was diagnosed as having telangiectasia macularis eruptiva perstans tmep and was successfully treated with puva photochemotherapy. Tmep is a rare disease, found in less than 1% of patients with cutaneous mastocytosis 1. If you would like a highquality copy or to use the image for commercial purposes, contact the owner. Trevisan summary we present a case of telangiectasia macularis eruptiva perstans tmep that occurred in an asymptomatic, otherwise healthy 15 yearold boy. Treatment and prognosis of cutaneous mastocytosis uptodate. A 41yearold woman presented with a 2month history of pruritus and a generalized dermatitis that developed initially on the head and spread to. Tmep can be a cause of morbidity with associated cutaneous findings e. As the patient had no clinical symptoms, no medication was administered, and he was monitored periodically by a general practitioner and a dermatologist.
While most cutaneous mastocytoses occur in children and are asymptomatic, tmep occurs predominantly in adults and is associated with systemic manifestations, requiring medical management. Although tmep has been traditionally thought to be restricted to the skin, a recent retrospective multicentric study established a diagnosis with systemic involvement of mastocytosis in 47% patients affected by tmep and aggressive systemic mastocytosis in 9%. Telangiectasia macularis eruptiva perstans tmep is a variant of cutaneous mastocytosis that typically arises in adults, characterized by numerous telangiectatic macules, usually on the trunk. Page 48 telangiectasia macularis eruptiva perstans. Telangiectasia macularis eruptiva perstans europe pmc. Telangiectasia macularis eruptiva perstans is a rare form of cutaneous mastocytosis and is observed in less than one percent of the patients with mastocytosis. Phenotypic and genotypic characteristics of mastocytosis according to the age of onset. Mastocytosis is the term for a diverse group of conditions where a single or clonal population of mast cells accumulate in one or more tissues, for example, skin, bone marrow, liver, spleen, gastrointestinal tract and lymph nodes. We sought to estimate the frequency and clinical characteristics of tmep in a cohort of adult patients with cutaneous mastocytosis, and to assess the presence of systemic involvement. Telangiectasia macularis eruptiva perstans accounted for 14% of adult patients with cutaneous mastocytosis at 2 referral centers for mastocytosis. Dariers sign urtication on scratching is variably elicited. While cutaneous mastocytosis can include maculopapular cutaneous mastocytosis mpcm, formerly known as urticaria pigmentosa up, telangiectasia macularis eruptiva perstans. This chapter considers the terminologies of mastocytoma, maculopapular cutaneous mastocytosis previously referred to as urticaria pigmentosa, and telangiectasia macularis eruptiva perstans. Visual guide to skin lesions tms the mastocytosis society.
The findings were characteristic for cutaneous mastocytosis with classic morphologic features of telangiectasia macularis eruptiva perstans. There is limited information on systemic involvement in telangiectasia macularis eruptiva perstans, a form of cutaneous mastocytosis. Telangiectasia macularis eruptiva perstans tmep is a clinical variant of cutaneous mastocytosis which is rarely observed in childhood. This skin disorder is caused by an increased number of mast cells entering the skin. This condition is benign and there is limited treatment available. Treatment of telangiectasia macularis eruptiva perstans. Telangiectasia macularis eruptive perstans genetic and rare. Visual guide to skin lesions in mast cell diseases the following pages are a photo journal of examples of how mast cell diseases can present.