COMMENTARY
TMEP or not TMEP: That is the question Nadine Marrouche, MD,a and Clive Grattan, MD, FRCPa,b Norwich and London, United Kingdom Key words: telangiectasia macularis eruptiva perstans; urticaria pigmentosa.
elangiectasia macularis eruptiva perstans (TMEP) has long been considered a rare telangiectatic subvariant of cutaneous mastocytosis with a generally good prognosis. Although TMEP is a well-known clinical term, it does not appear in the most recent World Health Organization classification of cutaneous mastocytosis.1 Moreover, TMEP has been used in the literature to describe patients with a spectrum of clinical presentations (Table I).2-8 In 1998, Sarkany et al8 proposed restricting the term TMEP to patients with the typical characteristic telangiectatic macules. Darier sign is usually negative in these patients. This corresponds to only a slight increase in the number of mast cells in lesional skin. Importantly, this definition excluded all cases previously described as having TMEP but actually presenting with classic urticaria pigmentosa (UP) lesions with associated telangiectasia. This separation was judged fundamental as it would have direct implications on prognosis. According to the above definition, TMEP is not associated with systemic mastocytosis.8 In view of the apparent conflict in terminology, we have traced the term TMEP to its original designation and looked at its chronological evolution. TMEP was first coined in 1930 by Weber and Hellenschmied2 in their description of a 60-yearold Russian woman who presented with 20-year history of an asymptomatic symmetric eruption over the chest and limbs, consisting of erythematous nonpurpuric macules with relatively illdefined borders. The lesions disappeared under diascopic pressure, occasionally leaving residual pigmentation. They noted that individual telangiectasia cannot be appreciated within the macules by naked-eye examination. However, they could not find an explanation for the lesions other than
T
‘‘telangiectatic hyperemia.’’ Later the same year, Weber examined a 26-year-old woman with typical UP lesions. Upon rubbing, some of the lesions urticated and were reminiscent of those seen in the initial case labeled as TMEP. This observation prompted Weber to entertain a relationship between UP and TMEP. To verify this association, he re-examined the initially described patient and noticed that a urticarial reaction was produced upon rubbing the lesions. Weber thus concluded that TMEP is a rare telangiectatic, relatively pigmentless variant of UP, occurring in older persons.2 In the following 2 decades, TMEP was rarely mentioned in the literature. It is understandable then that since its initial description by Weber, TMEP was thought to be a subvariant of UP, if not synonymous with it. It was not until 1964 that credit was again given to the entity TMEP by Cramer.5 Although the latter regarded TMEP as a form of UP, he spoke in favor of setting the entity apart from it on account of its characteristic morphology and the difficulty of classifying it histologically in the group of mastocytosis.5 Although TMEP has been considered to have little tendency for progression to systemic disease, significant systemic involvement has been described in those cases where an overlap with UP existed.9 If TMEP as defined by Sarkany et al8 is indeed a separate variant of cutaneous mastocytosis in which systemic disease is not observed, further studies will have to be undertaken to demonstrate biological and prognostic differences from UP and determine whether this pattern is less likely to become systemic than mixed TMEP with UP.
From the Department of Dermatology, Norfolk and Norwich University Hospital,a and St John’s Institute of Dermatology, London.b Funding sources: None. Conflicts of interest: None declared. Reprint requests: Nadine Marrouche, MD, Department of Dermatology, Norfolk and Norwich University Hospital,
Colney Lane, Norwich NR4 7UY, United Kingdom. E-mail: [email protected]. J Am Acad Dermatol 2014;70:581-2. 0190-9622/$36.00 Ó 2013 by the American Academy of Dermatology, Inc. http://dx.doi.org/10.1016/j.jaad.2013.09.006
581
J AM ACAD DERMATOL
582 Marrouche and Grattan
MARCH 2014
Table I. Selection of cases designated as telangiectasia macularis eruptiva perstans in the literature
Study
Weber and Hellenschmied,2 1930 Weber and Rast,3 1935 Moursund and Hirschmann,4 1951 Cramer,5 1964 Monheit et al,6 1979
No. of cases
1
Clinical presentation
Classic TMEP
Darier sign
Presence of mast cells on histopathology
1
Not described
11 1
Mixed TMEP and UP Mixed TMEP and UP
Variable 1
5/11 Numerous
1 1
Mixed TMEP and UP Mixed TMEP and UP
1 1
Numerous Numerous
Bonnefoy et al,7 1986
1
Classic TMEP
_
Numerous
Sarkany et al,8 1998
1
Classic TMEP
_
Few
Associated findings
Obesity, mild hypertension, and duodenal ulcer Arthralgia in 1 case Arthralgia and myalgia None Systemic mastocytosis (bone marrow, liver, lymph nodes), duodenal ulcers, flushing, palpitation, headache, nausea, diarrhea Loss of consciousness, dyspnea, flushing, diarrhea Flushing
TMEP, Telangiectasia macularis eruptiva perstans; UP, urticaria pigmentosa. REFERENCES 1. Pettigrew HD, Teuber SS, Kong JS, Gershwin ME. Contemporary challenges in mastocytosis. Clin Rev Allergy Immunol 2010;38:125-34. 2. Weber FP, Hellenschmied R. Telangiectasia macularis eruptiva perstans. Br J Dermatol Syphilol 1930;42: 374-82. 3. Weber FP, Rast H. Telangiectasia macularis eruptiva perstans. Acta Derm Venereol 1935;26:216. 4. Moursund MP, Hirschmann VR. Telangiectasia macularis eruptiva perstans; review of the literature, report of a case, and discussion of the etiology and pathology of generalized telangiectasia. AMA Arch Derm Syphilol 1951; 63:232-49.
5. Cramer HJ. Telangiectasia macularis eruptiva perstans, a special form of urticaria pigmentosa. Hautarzt 1964;15:370-4. 6. Monheit GD, Murad T, Conrad M. Systemic mastocytosis and the mastocytosis syndrome. J Cutan Pathol 1979;6:42-52. 7. Bonnefoy M, Rouchouse B, Clavreul C, Decousus D, Claudy A. Prolonged losses of consciousness disclosing mastocytosis of the telangiectasia macularis eruptiva perstans type. Ann Dermatol Venereol 1986;113:259-62. 8. Sarkany RPE, Monk BE, Handfield-Jones SE. Telangiectasia macularis eruptiva perstans: a case report and review of the literature. Clin Exp Dermatol 1998;23:38-9. 9. Brunning RD, McKenna RW, Rosia J, Parkin JL, Risdall R. Systemic mastocytosis: extracutaneous manifestations. Am J Surg Pathol 1983;7:425-38.
TMEP or not TMEP: That is the question Nadine Marrouche, MD,a and Clive Grattan, MD, FRCPa,b Norwich and London, United Kingdom Key words: telangiectasia macularis eruptiva perstans; urticaria pigmentosa.
elangiectasia macularis eruptiva perstans (TMEP) has long been considered a rare telangiectatic subvariant of cutaneous mastocytosis with a generally good prognosis. Although TMEP is a well-known clinical term, it does not appear in the most recent World Health Organization classification of cutaneous mastocytosis.1 Moreover, TMEP has been used in the literature to describe patients with a spectrum of clinical presentations (Table I).2-8 In 1998, Sarkany et al8 proposed restricting the term TMEP to patients with the typical characteristic telangiectatic macules. Darier sign is usually negative in these patients. This corresponds to only a slight increase in the number of mast cells in lesional skin. Importantly, this definition excluded all cases previously described as having TMEP but actually presenting with classic urticaria pigmentosa (UP) lesions with associated telangiectasia. This separation was judged fundamental as it would have direct implications on prognosis. According to the above definition, TMEP is not associated with systemic mastocytosis.8 In view of the apparent conflict in terminology, we have traced the term TMEP to its original designation and looked at its chronological evolution. TMEP was first coined in 1930 by Weber and Hellenschmied2 in their description of a 60-yearold Russian woman who presented with 20-year history of an asymptomatic symmetric eruption over the chest and limbs, consisting of erythematous nonpurpuric macules with relatively illdefined borders. The lesions disappeared under diascopic pressure, occasionally leaving residual pigmentation. They noted that individual telangiectasia cannot be appreciated within the macules by naked-eye examination. However, they could not find an explanation for the lesions other than
T
‘‘telangiectatic hyperemia.’’ Later the same year, Weber examined a 26-year-old woman with typical UP lesions. Upon rubbing, some of the lesions urticated and were reminiscent of those seen in the initial case labeled as TMEP. This observation prompted Weber to entertain a relationship between UP and TMEP. To verify this association, he re-examined the initially described patient and noticed that a urticarial reaction was produced upon rubbing the lesions. Weber thus concluded that TMEP is a rare telangiectatic, relatively pigmentless variant of UP, occurring in older persons.2 In the following 2 decades, TMEP was rarely mentioned in the literature. It is understandable then that since its initial description by Weber, TMEP was thought to be a subvariant of UP, if not synonymous with it. It was not until 1964 that credit was again given to the entity TMEP by Cramer.5 Although the latter regarded TMEP as a form of UP, he spoke in favor of setting the entity apart from it on account of its characteristic morphology and the difficulty of classifying it histologically in the group of mastocytosis.5 Although TMEP has been considered to have little tendency for progression to systemic disease, significant systemic involvement has been described in those cases where an overlap with UP existed.9 If TMEP as defined by Sarkany et al8 is indeed a separate variant of cutaneous mastocytosis in which systemic disease is not observed, further studies will have to be undertaken to demonstrate biological and prognostic differences from UP and determine whether this pattern is less likely to become systemic than mixed TMEP with UP.
From the Department of Dermatology, Norfolk and Norwich University Hospital,a and St John’s Institute of Dermatology, London.b Funding sources: None. Conflicts of interest: None declared. Reprint requests: Nadine Marrouche, MD, Department of Dermatology, Norfolk and Norwich University Hospital,
Colney Lane, Norwich NR4 7UY, United Kingdom. E-mail: [email protected]. J Am Acad Dermatol 2014;70:581-2. 0190-9622/$36.00 Ó 2013 by the American Academy of Dermatology, Inc. http://dx.doi.org/10.1016/j.jaad.2013.09.006
581
J AM ACAD DERMATOL
582 Marrouche and Grattan
MARCH 2014
Table I. Selection of cases designated as telangiectasia macularis eruptiva perstans in the literature
Study
Weber and Hellenschmied,2 1930 Weber and Rast,3 1935 Moursund and Hirschmann,4 1951 Cramer,5 1964 Monheit et al,6 1979
No. of cases
1
Clinical presentation
Classic TMEP
Darier sign
Presence of mast cells on histopathology
1
Not described
11 1
Mixed TMEP and UP Mixed TMEP and UP
Variable 1
5/11 Numerous
1 1
Mixed TMEP and UP Mixed TMEP and UP
1 1
Numerous Numerous
Bonnefoy et al,7 1986
1
Classic TMEP
_
Numerous
Sarkany et al,8 1998
1
Classic TMEP
_
Few
Associated findings
Obesity, mild hypertension, and duodenal ulcer Arthralgia in 1 case Arthralgia and myalgia None Systemic mastocytosis (bone marrow, liver, lymph nodes), duodenal ulcers, flushing, palpitation, headache, nausea, diarrhea Loss of consciousness, dyspnea, flushing, diarrhea Flushing
TMEP, Telangiectasia macularis eruptiva perstans; UP, urticaria pigmentosa. REFERENCES 1. Pettigrew HD, Teuber SS, Kong JS, Gershwin ME. Contemporary challenges in mastocytosis. Clin Rev Allergy Immunol 2010;38:125-34. 2. Weber FP, Hellenschmied R. Telangiectasia macularis eruptiva perstans. Br J Dermatol Syphilol 1930;42: 374-82. 3. Weber FP, Rast H. Telangiectasia macularis eruptiva perstans. Acta Derm Venereol 1935;26:216. 4. Moursund MP, Hirschmann VR. Telangiectasia macularis eruptiva perstans; review of the literature, report of a case, and discussion of the etiology and pathology of generalized telangiectasia. AMA Arch Derm Syphilol 1951; 63:232-49.
5. Cramer HJ. Telangiectasia macularis eruptiva perstans, a special form of urticaria pigmentosa. Hautarzt 1964;15:370-4. 6. Monheit GD, Murad T, Conrad M. Systemic mastocytosis and the mastocytosis syndrome. J Cutan Pathol 1979;6:42-52. 7. Bonnefoy M, Rouchouse B, Clavreul C, Decousus D, Claudy A. Prolonged losses of consciousness disclosing mastocytosis of the telangiectasia macularis eruptiva perstans type. Ann Dermatol Venereol 1986;113:259-62. 8. Sarkany RPE, Monk BE, Handfield-Jones SE. Telangiectasia macularis eruptiva perstans: a case report and review of the literature. Clin Exp Dermatol 1998;23:38-9. 9. Brunning RD, McKenna RW, Rosia J, Parkin JL, Risdall R. Systemic mastocytosis: extracutaneous manifestations. Am J Surg Pathol 1983;7:425-38.
