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“Dysplastic nevus” syndrome: Does a survey make it real?
LETTERS NOTES & COMMENTS “Dysplastic nevus” syndrome: Does a survey make it real? To the Editor: In their article titled, “Management of Dysplastic Nevi: A Survey of Fellows of the American Academy of Dermatology” (J Am Dermatol 2002; 46:674-82), Tripp et al told that of 1216 fellows of the AAD queried, 456 responded and “almost all respondents (98%) accept the dysplastic nevus, or atypical mole, as an entity.” At the outset of their Discussion section, the coauthors made this declaration: “Currently, there is some controversy concerning the concept of dysplastic nevi. At one extreme, a few authors doubt what is commonly known as the dysplastic nevus or atypical mole even exists as a distinct clinical-histologic entity.13,14” Reference 13 cited is to an article by me nearly 15 years ago1 captioned, “What naevus is dysplastic, a syndrome, and the commonest precursor of malignant melanoma? A riddle and an answer.” And the answer, of course, is, “No nevus,” because the terms melanocytic dysplasia, dysplastic nevus, and dysplastic nevus syndrome never have been defined in a comprehensible, lucid, repeatable way. That fact is irrefutable.2 But never have I written that the dysplastic nevus does not exist morphologically, that is, clinically and histopathologically. Quite the contrary; not only does it exist—it is the most common nevus in humans! And that is the very reason that by far the most common diagnosis of melanocytic nevus issued in 2003 by pathologists to dermatologists is “dysplastic nevus,” “melanocytic nevus with severe dysplasia,” or “melanocytic nevus with architectural disorder and moderate melanocytic dysplasia,” or some variation on those themes. For more than 20 years, my stance about the matter of melanocytic dysplasia, dysplastic nevus, and dysplastic nevus syndrome has been very clear and very consistent. In short, all 3 concepts are bogus. That the nevus, itself, exists is incontrovertible. I aver that the term “melanocytic dysplasia” never has been defined by Clark and his coworkers in an understandable, clear, consistent way. Not only did they fail to define “melanocytic dysplasia” in the same way from article to article, they sometimes gave different definitions of it in the very same article!2 I assert that the so-called dysplastic nevus, as set forth by Clark and collaborators, never has been J AM ACAD DERMATOL
characterized, clinically and histopathologically, in a meaningful, intelligible, dependable way. In regard to clinical features, Clark and associates wrote, again and again, about the dysplastic nevus thus: “The lesions were flat, were often greater than 10 mm in diameter, and were haphazardly colored with areas that were black, blue, pink and sometimes depigmented.”3 Those features are the very same ones employed by dermatologists, worldwide, for diagnosis clinically of melanoma. In short, the criteria of Clark et al in regard to diagnosis clinically of “dysplastic nevus” simply do not work because they do not permit differentiation of that particular kind of nevus from melanoma. I contend that the criteria of Clark et al for diagnosis histopathologically of “dysplastic nevus” do not work either because they do not enable differentiation of it from melanoma. In 1989, for example, I wrote about the matter as follows4: “I do not use the term ‘dysplastic nevus’ because I am unable to identify a melanocytic nevus that fulfills Clark et al.’s criteria for histopathologic diagnosis of it, namely, (1) persistent lentiginous melanocytic hyperplasia; (2) atypical melanocytic hyperplasia (melanocytic nuclear atypia); (3) lamellar fibroplasia; (4) concentric eosinophilic fibroplasia; and (5) sparse patchy lymphocytic infiltrates. Furthermore, all five criteria, both individually and collectively, said by Clark et al. to be distinctive for dysplastic nevi, also may be seen in malignant melanomas.” In my judgment, criteria that really work for diagnosis of the most common presentation of the nevus they designate “dysplastic” are silhouette of a benign neoplasm (eg, symmetric, sharply circumscribed, relatively flat base), only slight, if any, elevation, in its center, and nests of melanocytes with small, oval, monomorphous nuclei confined to the dermoepidermal junction and the upper part of the dermis. I acknowledge, fully, the existence of the nevus pictured histopathologically by Clark et al in their many publications about the subject of “dysplastic nevi,” even though nearly every one of their photomicrographs is shown at relatively high magnification, which prohibits assessing the silhouette of it. To me, the nevus that they picture is the most common type of melanocytic nevus in man. For more than 15 years I have named that nevus eponymically for Clark, in the same manner that I do other comMARCH 2003 461
462 Letters
mon types of melanocytic nevi, such as for Unna, Miescher, and Spitz. Those 4 types of melanocytic nevi can be identified by their characteristic appearance clinically and by their distinctive silhouette histopathologically. I advise further that what was called by Clark et al the “dysplastic nevus syndrome” is not a syndrome at all. Irrespective of the number of “dysplastic nevi” that is deemed to be requisite for that “syndrome” (and there is no agreement whatsoever about the number), it still does not qualify as a syndrome because it is not a constellation of signs and symptoms that occur together and characterize a particular morbid state. It is a single finding, namely, one type of melanocytic nevus—and the most common type at that. Given these realities, I am baffled by the idea that 98% of respondents, all of them fellows of the AAD, not only give credence to the concept of dysplastic nevus “as an entity,” but, according to Tripp et al, “In agreement with the literature, accept the concept that patients with dysplastic nevus are at increased risk for melanoma. . . .” How is that conclusion tenable when Kopf et al have stated, repeatedly, that the criteria for diagnosing the dysplastic nevus clinically are the very same ones, the ABCDs, they introduced as a mnemonic for diagnosing melanoma clinically? This is what Kopf and collaborators5 wrote in 1985 about the matter of the ABCDs as a vehicle for diagnosis of “characteristic clinical features of early malignant melanoma”: “The characteristic clinical features of early malignant melanoma can be easily remembered by thinking of ABCD: ● Asymmetry ● Border irregularity ● Color variegation ● Diameter generally greater than 6 mm” And this is what Kopf and coworkers6 wrote in 1999 about the matter of the ABCDs as a vehicle for diagnosis of characteristic clinical features of dysplastic nevus: “One way to define the atypical moles in this [dysplastic nevus] syndrome is to use the same acronym—ABCD—that we first used to diagnose MM [malignant melanoma]. The same characteristics—asymmetry, border irregularity, color irregularity, and diameter of 6 mm or more—also correlate with the clinical characteristics of the atypical moles seen in patients with “classic’ dysplastic nevi.” It hardly is surprising, therefore, that in the article that precedes the one by Tripp et al in the same issue of the JAAD, Branstro¨ m et al, in commenting about “Laypersons’ Perceptual Discrimination of
J AM ACAD DERMATOL MARCH 2003
Pigmented Skin Lesions” (J Am Acad Dermatol 2002; 46:667-73) made this enigmatic statement: “The ABCD (asymmetry, border irregularity, color variegation, and diameter ⬎ 6 mm) criteria are a frequently-used guide introduced by the American Cancer Society. These criteria are important tools for clinical diagnosis of dysplastic nevi (DN) and melanomas in medical settings. These criteria have also been used in information campaigns in the United States to enhance the general public’s ability to distinguish benign lesions from melanoma.” How is it possible for a layperson to distinguish a “benign lesion” [dysplastic nevus] from a malignant neoplasm [melanoma] when leading dermatologists advocate the very same clinical criteria [ABCD] for identifying both of them? In sum and in short, there surely exists a nevus that today is known as the dysplastic nevus, alias the BK mole, the large atypical mole, the “funny looking mole,” etc; it happens to be the commonest nevus in man. The concept, however, that animates the notion of dysplastic nevus, namely, melanocytic dysplasia, is wholly without foundation and the syndrome predicated on the presence of that nevus is not a syndrome. By stating that “almost all respondents (98%) accept the dysplastic nevus or atypical mole, as an entity,” Tripp et al seem to give legitimacy not only to the concept of that nevus but to everything that flows from the concept, for example, precursor of melanoma, marker of persons at risk for melanoma, the precursor state. Had the coauthors of the article in 2002 in the JAAD been writing instead in the late 1940s and throughout the 1950s in the Archives of Dermatology and Syphilology about the “nevus” diagnosed by pathologists most often in those days, it is likely they would have said that “almost all respondents (98%) accept the activated junctional nevus, or nevus with junctional activity, as an entity.” But where is the “activated junctional nevus” now, 50 years later? Reference to any photomicrograph published of it by the most vigorous proponent of it, Arthur Allen, a general pathologist, as was Clark, reveals at a glance that the so-called activated junctional nevus, in actuality, was a florid expression of melanoma in situ, and that doubtless is why the “nevus” purported to be “activated” now has joined the ash heap on which is strewn other woefully flawed concepts that, once upon a time, not too long ago, were embraced fervently by dermatologists, among them the Miescher (grenz ray) technique for treatment of “lentigo maligna,” “wide and deep” excision of primary melanoma in general, elective lymph node dissection, wholesale removal of nevi on sites especially susceptible to trauma, and BANS
Letters 463
J AM ACAD DERMATOL VOLUME 48, NUMBER 3
(a subject in the realm of melanocytic neoplasia that little more than a decade ago was “hot,” but that today evokes only a blank stare). The fate of the concept of the dysplastic nevus (not the reality of the nevus currently dubbed dysplastic) is inevitable, that is, the same as activated junctional nevus. A religion has been created about melanocytic dysplasia, dysplastic nevus, and dysplastic nevus syndrome, and that religion has its high priests, acolytes, and a laity of ardent (98%, it seems, of fellows of the AAD) devotees. But once a religion (such as that of ancient Greece) no longer has adherents, it is known as myth.2 In the not too distant future, melanocytic dysplasia, dysplastic nevus, and dysplastic nevus syndrome will be taken no more seriously than is Cerberus. A. Bernard Ackerman, MD Ackerman Academy of Dermatopathology New York, New York
challenge of existing and new theories and treatments. Science by consensus has a very bad history. Consider the prevailing geocentric theory at the time of Galileo, physics before Newton and Einstein, or the consensus views of proper excision margins for melanoma in the 1950s. Although the poll of the Academy members’ feelings about DNS may well serve trial lawyers trying to establish standard of care in a negligence case, it does nothing to advance the body of knowledge. Thomas J. Connelly, MD Connelly Skin Cancer Surgery Center 309 E Osceola St, Suite 205 Stuart, FL 34994 E-mail: [email protected] REFERENCE 1. Tripp JM, Kopf AW, Marghoob AA, Bart RS. Management of dysplastic nevi: a survey of fellows of the American Academy of Dermatology. J Am Acad Dermatol 2002;46:674-82. doi:10.1067/mjd.2003.209
REFERENCES 1. Ackerman AB. Why naevus is dysplastic, a syndrome, and the commonest precursor of malignant melanoma? A riddle and an answer. Histopathology 1988;13:241-56. 2. Ackerman AB, Massi D, Nielsen TA, et al. Dysplastic nevus. Atypical mole or typical myth? Philadelphia: Ardor Scribendi, Ltd; 1999. 3. Reimer RR, Clark WH Jr, Greene MH, Ainsworth AM, Fraumeni JF Jr. Precursor lesions in familial melanoma: a new genetic preneoplastic syndrome. JAMA 1978;239:744-6. 4. Ackerman AB. An exchange of views [with Wallace H. Clark, Jr.] regarding the dysplastic nevus controversy. Semin Dermatol 1989;8:229-50. 5. Friedman RJ, Rigel DS, Kopf AW. Early detection of malignant melanoma. CA 1985;35:130-51. 6. McBride A, Rivers JK, Kopf AW, Cockerell CJ, Bart RS, Grin CM, et al. Clinical features of dysplastic nevi. Dermatol Clin 1991;97:1721. doi:10.1067/mjd.2003.210
To the Editor: Like most members of the Academy I look to The Blue Journal to remain abreast of scientifically credible advancements in dermatology. Recently I was surprised and unhappy to see the publication of results of a survey of Academy members’ feelings about the so-called dysplastic nevus syndrome (DNS).1 I personally reject the concept of the dysplastic nevus syndrome because of its inability to survive scientific challenge. However, my problem is with the publication of the poll. Many members may mistakenly believe the consensus view somehow equates with scientific evidence for existence of the DNS. I believe that we should look to the Journal not for confirmation of the views of the Academy members but for scientific evaluation and
To the Editor: The survey1 of attitudes of fellows of the American Academy of Dermatology (AAD) concerning dysplastic nevi represents a milestone in dermatology, but not a happy one. Using opinion polling rather than reliance solely on experiment and observation is not acceptable as validation of a medical or scientific concept. Nor can ignoring the elemental requirements of science— consistent observability, measurability, predictability and reproducibility upon which dermatology as well as the rest of medicine is based—and substituting theology or mythology for rationality be considered a suitable basis for patient care. The survey demonstrates one point clearly: nearly all dermatologists hold a deep quasireligious conviction that the dysplastic nevus exists and that anything resembling dysplasia, whatever that means, is to be extirpated from the body. Is there any doubt or controversy about this? Hardly worth mentioning, according to the authors. “At one extreme” are just two contrarian reports2,3 by nonbelievers to be compared with the opinion of the “vast majority of respondents in our survey (98%) [who] believe that the entity does indeed exist.” In any other field of science or medicine a concept this unproven would long ago have been discarded. Never mind that the dysplastic nevus as a precursor of melanoma was born more of wishful thinking than rigorous observation. Never mind that no carefully controlled studies were ever performed which might have delayed or stopped this train be-
characterized, clinically and histopathologically, in a meaningful, intelligible, dependable way. In regard to clinical features, Clark and associates wrote, again and again, about the dysplastic nevus thus: “The lesions were flat, were often greater than 10 mm in diameter, and were haphazardly colored with areas that were black, blue, pink and sometimes depigmented.”3 Those features are the very same ones employed by dermatologists, worldwide, for diagnosis clinically of melanoma. In short, the criteria of Clark et al in regard to diagnosis clinically of “dysplastic nevus” simply do not work because they do not permit differentiation of that particular kind of nevus from melanoma. I contend that the criteria of Clark et al for diagnosis histopathologically of “dysplastic nevus” do not work either because they do not enable differentiation of it from melanoma. In 1989, for example, I wrote about the matter as follows4: “I do not use the term ‘dysplastic nevus’ because I am unable to identify a melanocytic nevus that fulfills Clark et al.’s criteria for histopathologic diagnosis of it, namely, (1) persistent lentiginous melanocytic hyperplasia; (2) atypical melanocytic hyperplasia (melanocytic nuclear atypia); (3) lamellar fibroplasia; (4) concentric eosinophilic fibroplasia; and (5) sparse patchy lymphocytic infiltrates. Furthermore, all five criteria, both individually and collectively, said by Clark et al. to be distinctive for dysplastic nevi, also may be seen in malignant melanomas.” In my judgment, criteria that really work for diagnosis of the most common presentation of the nevus they designate “dysplastic” are silhouette of a benign neoplasm (eg, symmetric, sharply circumscribed, relatively flat base), only slight, if any, elevation, in its center, and nests of melanocytes with small, oval, monomorphous nuclei confined to the dermoepidermal junction and the upper part of the dermis. I acknowledge, fully, the existence of the nevus pictured histopathologically by Clark et al in their many publications about the subject of “dysplastic nevi,” even though nearly every one of their photomicrographs is shown at relatively high magnification, which prohibits assessing the silhouette of it. To me, the nevus that they picture is the most common type of melanocytic nevus in man. For more than 15 years I have named that nevus eponymically for Clark, in the same manner that I do other comMARCH 2003 461
462 Letters
mon types of melanocytic nevi, such as for Unna, Miescher, and Spitz. Those 4 types of melanocytic nevi can be identified by their characteristic appearance clinically and by their distinctive silhouette histopathologically. I advise further that what was called by Clark et al the “dysplastic nevus syndrome” is not a syndrome at all. Irrespective of the number of “dysplastic nevi” that is deemed to be requisite for that “syndrome” (and there is no agreement whatsoever about the number), it still does not qualify as a syndrome because it is not a constellation of signs and symptoms that occur together and characterize a particular morbid state. It is a single finding, namely, one type of melanocytic nevus—and the most common type at that. Given these realities, I am baffled by the idea that 98% of respondents, all of them fellows of the AAD, not only give credence to the concept of dysplastic nevus “as an entity,” but, according to Tripp et al, “In agreement with the literature, accept the concept that patients with dysplastic nevus are at increased risk for melanoma. . . .” How is that conclusion tenable when Kopf et al have stated, repeatedly, that the criteria for diagnosing the dysplastic nevus clinically are the very same ones, the ABCDs, they introduced as a mnemonic for diagnosing melanoma clinically? This is what Kopf and collaborators5 wrote in 1985 about the matter of the ABCDs as a vehicle for diagnosis of “characteristic clinical features of early malignant melanoma”: “The characteristic clinical features of early malignant melanoma can be easily remembered by thinking of ABCD: ● Asymmetry ● Border irregularity ● Color variegation ● Diameter generally greater than 6 mm” And this is what Kopf and coworkers6 wrote in 1999 about the matter of the ABCDs as a vehicle for diagnosis of characteristic clinical features of dysplastic nevus: “One way to define the atypical moles in this [dysplastic nevus] syndrome is to use the same acronym—ABCD—that we first used to diagnose MM [malignant melanoma]. The same characteristics—asymmetry, border irregularity, color irregularity, and diameter of 6 mm or more—also correlate with the clinical characteristics of the atypical moles seen in patients with “classic’ dysplastic nevi.” It hardly is surprising, therefore, that in the article that precedes the one by Tripp et al in the same issue of the JAAD, Branstro¨ m et al, in commenting about “Laypersons’ Perceptual Discrimination of
J AM ACAD DERMATOL MARCH 2003
Pigmented Skin Lesions” (J Am Acad Dermatol 2002; 46:667-73) made this enigmatic statement: “The ABCD (asymmetry, border irregularity, color variegation, and diameter ⬎ 6 mm) criteria are a frequently-used guide introduced by the American Cancer Society. These criteria are important tools for clinical diagnosis of dysplastic nevi (DN) and melanomas in medical settings. These criteria have also been used in information campaigns in the United States to enhance the general public’s ability to distinguish benign lesions from melanoma.” How is it possible for a layperson to distinguish a “benign lesion” [dysplastic nevus] from a malignant neoplasm [melanoma] when leading dermatologists advocate the very same clinical criteria [ABCD] for identifying both of them? In sum and in short, there surely exists a nevus that today is known as the dysplastic nevus, alias the BK mole, the large atypical mole, the “funny looking mole,” etc; it happens to be the commonest nevus in man. The concept, however, that animates the notion of dysplastic nevus, namely, melanocytic dysplasia, is wholly without foundation and the syndrome predicated on the presence of that nevus is not a syndrome. By stating that “almost all respondents (98%) accept the dysplastic nevus or atypical mole, as an entity,” Tripp et al seem to give legitimacy not only to the concept of that nevus but to everything that flows from the concept, for example, precursor of melanoma, marker of persons at risk for melanoma, the precursor state. Had the coauthors of the article in 2002 in the JAAD been writing instead in the late 1940s and throughout the 1950s in the Archives of Dermatology and Syphilology about the “nevus” diagnosed by pathologists most often in those days, it is likely they would have said that “almost all respondents (98%) accept the activated junctional nevus, or nevus with junctional activity, as an entity.” But where is the “activated junctional nevus” now, 50 years later? Reference to any photomicrograph published of it by the most vigorous proponent of it, Arthur Allen, a general pathologist, as was Clark, reveals at a glance that the so-called activated junctional nevus, in actuality, was a florid expression of melanoma in situ, and that doubtless is why the “nevus” purported to be “activated” now has joined the ash heap on which is strewn other woefully flawed concepts that, once upon a time, not too long ago, were embraced fervently by dermatologists, among them the Miescher (grenz ray) technique for treatment of “lentigo maligna,” “wide and deep” excision of primary melanoma in general, elective lymph node dissection, wholesale removal of nevi on sites especially susceptible to trauma, and BANS
Letters 463
J AM ACAD DERMATOL VOLUME 48, NUMBER 3
(a subject in the realm of melanocytic neoplasia that little more than a decade ago was “hot,” but that today evokes only a blank stare). The fate of the concept of the dysplastic nevus (not the reality of the nevus currently dubbed dysplastic) is inevitable, that is, the same as activated junctional nevus. A religion has been created about melanocytic dysplasia, dysplastic nevus, and dysplastic nevus syndrome, and that religion has its high priests, acolytes, and a laity of ardent (98%, it seems, of fellows of the AAD) devotees. But once a religion (such as that of ancient Greece) no longer has adherents, it is known as myth.2 In the not too distant future, melanocytic dysplasia, dysplastic nevus, and dysplastic nevus syndrome will be taken no more seriously than is Cerberus. A. Bernard Ackerman, MD Ackerman Academy of Dermatopathology New York, New York
challenge of existing and new theories and treatments. Science by consensus has a very bad history. Consider the prevailing geocentric theory at the time of Galileo, physics before Newton and Einstein, or the consensus views of proper excision margins for melanoma in the 1950s. Although the poll of the Academy members’ feelings about DNS may well serve trial lawyers trying to establish standard of care in a negligence case, it does nothing to advance the body of knowledge. Thomas J. Connelly, MD Connelly Skin Cancer Surgery Center 309 E Osceola St, Suite 205 Stuart, FL 34994 E-mail: [email protected] REFERENCE 1. Tripp JM, Kopf AW, Marghoob AA, Bart RS. Management of dysplastic nevi: a survey of fellows of the American Academy of Dermatology. J Am Acad Dermatol 2002;46:674-82. doi:10.1067/mjd.2003.209
REFERENCES 1. Ackerman AB. Why naevus is dysplastic, a syndrome, and the commonest precursor of malignant melanoma? A riddle and an answer. Histopathology 1988;13:241-56. 2. Ackerman AB, Massi D, Nielsen TA, et al. Dysplastic nevus. Atypical mole or typical myth? Philadelphia: Ardor Scribendi, Ltd; 1999. 3. Reimer RR, Clark WH Jr, Greene MH, Ainsworth AM, Fraumeni JF Jr. Precursor lesions in familial melanoma: a new genetic preneoplastic syndrome. JAMA 1978;239:744-6. 4. Ackerman AB. An exchange of views [with Wallace H. Clark, Jr.] regarding the dysplastic nevus controversy. Semin Dermatol 1989;8:229-50. 5. Friedman RJ, Rigel DS, Kopf AW. Early detection of malignant melanoma. CA 1985;35:130-51. 6. McBride A, Rivers JK, Kopf AW, Cockerell CJ, Bart RS, Grin CM, et al. Clinical features of dysplastic nevi. Dermatol Clin 1991;97:1721. doi:10.1067/mjd.2003.210
To the Editor: Like most members of the Academy I look to The Blue Journal to remain abreast of scientifically credible advancements in dermatology. Recently I was surprised and unhappy to see the publication of results of a survey of Academy members’ feelings about the so-called dysplastic nevus syndrome (DNS).1 I personally reject the concept of the dysplastic nevus syndrome because of its inability to survive scientific challenge. However, my problem is with the publication of the poll. Many members may mistakenly believe the consensus view somehow equates with scientific evidence for existence of the DNS. I believe that we should look to the Journal not for confirmation of the views of the Academy members but for scientific evaluation and
To the Editor: The survey1 of attitudes of fellows of the American Academy of Dermatology (AAD) concerning dysplastic nevi represents a milestone in dermatology, but not a happy one. Using opinion polling rather than reliance solely on experiment and observation is not acceptable as validation of a medical or scientific concept. Nor can ignoring the elemental requirements of science— consistent observability, measurability, predictability and reproducibility upon which dermatology as well as the rest of medicine is based—and substituting theology or mythology for rationality be considered a suitable basis for patient care. The survey demonstrates one point clearly: nearly all dermatologists hold a deep quasireligious conviction that the dysplastic nevus exists and that anything resembling dysplasia, whatever that means, is to be extirpated from the body. Is there any doubt or controversy about this? Hardly worth mentioning, according to the authors. “At one extreme” are just two contrarian reports2,3 by nonbelievers to be compared with the opinion of the “vast majority of respondents in our survey (98%) [who] believe that the entity does indeed exist.” In any other field of science or medicine a concept this unproven would long ago have been discarded. Never mind that the dysplastic nevus as a precursor of melanoma was born more of wishful thinking than rigorous observation. Never mind that no carefully controlled studies were ever performed which might have delayed or stopped this train be-