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Potentially fatal interstitial lung disease can occur in clinically amyopathic dermatomyositis
Letters 797
J AM ACAD DERMATOL VOLUME 48, NUMBER 5
an eruption resembling mycosis fungoides. Arch Dermatol Syph 1940;42:53-8. 7. McCafferty. A case for diagnosis (mycosis fungoides). Arch Dermatol Syph 1928;17:132-3. 8. Wise F. Mycosis fungoides. Arch Dermatol Syph 1928;17:885-6. doi:10.1067/mjd.2003.248
Reply To the Editor: Dr Cribier’s letter and his recent article1 provide additional information about the connection of this histopathologic lesion with Jean Ferdinand Darier and Lucien Marie Pautrier. After more than half a century of misattribution, it is interesting that within a short time span, Drs Civatte,2 SchmidtSkrabs,3 Cribier, and I4 (my letter was submitted in October 2000) have independently brought up this issue. Perhaps it is time for the authors of dermatopathology textbooks and journal articles to call the lesion “Darier nest (Pautrier microabscess).” Then, before another half century elapses, the parenthetical component might be dropped. George A. Omura, MD 3621 Crestside Rd Birmingham, AL 35223
REFERENCES 1. Cribier BJ. Lucien Marie Pautrier (1876-1959): the man and the myth of the microabscesses. Dermatopathology: Practical and Conceptual 2001;7:135-47. 2. Civatte J. Darier and the French contribution to dermatopathology. Dermatopathology: Practical and Conceptual 2000;6:161-9. 3. Schmidt-Skrabs CC. Pautrier microabscesses (PA)– historical note. Am J Dermatopathol 2000;22:555. 4. Omura GA. Darier, Pautrier, and the microabscesses of mycosis fungoides. J Am Acad Dermatol 2002;46:320-1. doi:10.1067/mjd.2003.253
Potentially fatal interstitial lung disease can occur in clinically amyopathic dermatomyositis To the Editor: Interstitial lung disease (ILD) is a potentially fatal complication of the idiopathic inflammatory myopathies occurring in 5% to 40% of patients with classic dermatomyositis (DM).1,2 However, it is not generally appreciated that ILD can also occur in subsets of DM that lack hallmark clinical features such as muscle weakness (eg, clinically amyopathic DM [C-ADM]). C-ADM (synonym, DM sine´ myositis) is a subset of idiopathic inflammatory myopathies in which the hallmark cutaneous manifestations of DM (ie, DMspecific skin disease [DMSSD]) are present for 6 months or longer without the development of clinically evident muscle weakness.3-5 Although previ-
Table I. Summary of published cases of interstitial lung disease occurring in patients with dermatomyositisspecific skin disease but no muscle weakness Clinical features
Mean age, y (range) Median age, y Male/female ratio C-ADM subclassification† Mean interval (y) between onset of DMSSD and diagnosis of ILD (range) Median interval (y) between onset of DMSSD and diagnosis of ILD Fatal outcome from ILD (%) Antinuclear antibody (%) Myositis-specific antibodies (Jo-1) ESR mean value, mm/h Associated malignancy (%)
All cases (N ⴝ 34)
Japanese cases (n ⴝ 27)
Other cases (n ⴝ 7)*
53.4 (10-88) 52 14:20 ADM-14; HDM-4 1.49 (0.08-18.0)
54.5 (23-84) 52 11:16 ADM-11; HDM-3 1.06 (0.12-4.0)
49.0 (10-88) 47 3:4 ADM-3; HDM-1 3.42 (0.08-18.0)
0.83
0.83
0.65
18/34 (53) 6/27‡ (22) 0/26 44 (24 tested) 1/34 (3)
16/27 (59) 5/22 (23) 0/23 52 (19 tested) 1/27 (4)
2/7 (29) 1/5 (20) 0/3 35 (5 tested) 0/7
ADM, Amyopathic dermatomyositis (DM); C-, clinically; DMSSD, DM-specific skin disease; ESR, erythrocyte sedimentation rate; HDM, hypomyopathic DM; ILD, interstitial lung disease. *Countries from which non-Japanese cases were reported: Australia (1), Brazil (1), Malaysia (1), Korea (1), United States (1), West Indes (1), Yugoslavia (1). †C-ADM can be subclassified as follows: ADM (DMSSD for at least 6 months or longer without muscle weakness, normal muscle enzymes, and no other laboratory or radiologic evidence of muscle inflammation [eg, electromyography, muscle biopsy, muscle spectroscopy]); HDM (DMSSD for at least 6 months or longer without muscle weakness, normal muscle enzymes but some degree of other clinically silent laboratory or radiologic evidence of myositis). The key distinguishing feature of C-ADM is the fact that such patients display DMSSD for atypically prolonged periods of time without accompanying muscle weakness that is typical of classical DM. (Not all patients included in this analysis could be subclassified as either ADM or HDM since some had DMSSD for less than 6 months at the time of onset of their ILD.) ‡Number positive or abnormal/number tested.
798 Letters
J AM ACAD DERMATOL MAY 2003
ously thought to be quite rare, more than 300 cases of C-ADM have now been published. Among these cases, we were surprised to find 34 published cases of often fatal ILD occurring in patients having DMSSD but no muscle weakness (Table I). Many of these reports have been published in languages other than English, especially Japanese. Not all of these cases meet the classification criteria for C-ADM because DMSSD in some cases had been present for less than 6 months when ILD presented clinically. However, these cases were included in the present analysis because they do represent ILD occurring in the context of DMSSD without the presence of muscle weakness. In addition to the 34 published cases summarized in Table I, we are aware of several other such cases reported in abstract form (5 cases), on Internet Web sites (2 cases, both fatal), and by personal communication (1 case, fatal). Thus, there can be little doubt that clinical evidence of muscle weakness need not be present for the development of potentially fatal, DM-associated ILD. Failure to recognize this clinical constellation can delay diagnosis and institution of proper treatment and, thereby, have disastrous consequences. Richard D. Sontheimer, MDa Sachiko Miyagawa, MD, PhDb Department of Dermatology University of Iowa Hospitals and Clinics 200 Hawkins Dr BT 2045-1 Iowa City, IA 52242-1090a Department of Dermatology Nara Medical University Nara, Japanb
REFERENCES 1. Hirakata M, Nagai S. Interstitial lung disease in polymyositis and dermatomyositis. Curr Opin Rheumatol 2000;12:501-8. 2. Douglas WW, Tazelaar HD, Hartman TE, Hartman RP, Decker PA, Schroeder DR, et al. Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 2001;164: 1182-5. 3. Sontheimer RD. Cutaneous features of classical dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol 1999;11:475-82. 4. Sontheimer RD. Would a new name hasten the acceptance of clinically amyopathic dermatomyositis (dermatomyositis sine´ myositis) as a distinctive subset within the idiopathic inflammatory dermatomyopathies spectrum of clinical illness? J Am Acad Dermatol 2002;46:626-36. 5. Caproni M, Cardinali C, Parodi A, Giomi B, Papini M, Vaccaro M, et al. Amyopathic dermatomyositis: a review by the Italian group of immunodermatology. Arch Dermatol 2002;138:23-7.
Published online March 11, 2003 doi:10.1067/mjd.2003.199
J AM ACAD DERMATOL VOLUME 48, NUMBER 5
an eruption resembling mycosis fungoides. Arch Dermatol Syph 1940;42:53-8. 7. McCafferty. A case for diagnosis (mycosis fungoides). Arch Dermatol Syph 1928;17:132-3. 8. Wise F. Mycosis fungoides. Arch Dermatol Syph 1928;17:885-6. doi:10.1067/mjd.2003.248
Reply To the Editor: Dr Cribier’s letter and his recent article1 provide additional information about the connection of this histopathologic lesion with Jean Ferdinand Darier and Lucien Marie Pautrier. After more than half a century of misattribution, it is interesting that within a short time span, Drs Civatte,2 SchmidtSkrabs,3 Cribier, and I4 (my letter was submitted in October 2000) have independently brought up this issue. Perhaps it is time for the authors of dermatopathology textbooks and journal articles to call the lesion “Darier nest (Pautrier microabscess).” Then, before another half century elapses, the parenthetical component might be dropped. George A. Omura, MD 3621 Crestside Rd Birmingham, AL 35223
REFERENCES 1. Cribier BJ. Lucien Marie Pautrier (1876-1959): the man and the myth of the microabscesses. Dermatopathology: Practical and Conceptual 2001;7:135-47. 2. Civatte J. Darier and the French contribution to dermatopathology. Dermatopathology: Practical and Conceptual 2000;6:161-9. 3. Schmidt-Skrabs CC. Pautrier microabscesses (PA)– historical note. Am J Dermatopathol 2000;22:555. 4. Omura GA. Darier, Pautrier, and the microabscesses of mycosis fungoides. J Am Acad Dermatol 2002;46:320-1. doi:10.1067/mjd.2003.253
Potentially fatal interstitial lung disease can occur in clinically amyopathic dermatomyositis To the Editor: Interstitial lung disease (ILD) is a potentially fatal complication of the idiopathic inflammatory myopathies occurring in 5% to 40% of patients with classic dermatomyositis (DM).1,2 However, it is not generally appreciated that ILD can also occur in subsets of DM that lack hallmark clinical features such as muscle weakness (eg, clinically amyopathic DM [C-ADM]). C-ADM (synonym, DM sine´ myositis) is a subset of idiopathic inflammatory myopathies in which the hallmark cutaneous manifestations of DM (ie, DMspecific skin disease [DMSSD]) are present for 6 months or longer without the development of clinically evident muscle weakness.3-5 Although previ-
Table I. Summary of published cases of interstitial lung disease occurring in patients with dermatomyositisspecific skin disease but no muscle weakness Clinical features
Mean age, y (range) Median age, y Male/female ratio C-ADM subclassification† Mean interval (y) between onset of DMSSD and diagnosis of ILD (range) Median interval (y) between onset of DMSSD and diagnosis of ILD Fatal outcome from ILD (%) Antinuclear antibody (%) Myositis-specific antibodies (Jo-1) ESR mean value, mm/h Associated malignancy (%)
All cases (N ⴝ 34)
Japanese cases (n ⴝ 27)
Other cases (n ⴝ 7)*
53.4 (10-88) 52 14:20 ADM-14; HDM-4 1.49 (0.08-18.0)
54.5 (23-84) 52 11:16 ADM-11; HDM-3 1.06 (0.12-4.0)
49.0 (10-88) 47 3:4 ADM-3; HDM-1 3.42 (0.08-18.0)
0.83
0.83
0.65
18/34 (53) 6/27‡ (22) 0/26 44 (24 tested) 1/34 (3)
16/27 (59) 5/22 (23) 0/23 52 (19 tested) 1/27 (4)
2/7 (29) 1/5 (20) 0/3 35 (5 tested) 0/7
ADM, Amyopathic dermatomyositis (DM); C-, clinically; DMSSD, DM-specific skin disease; ESR, erythrocyte sedimentation rate; HDM, hypomyopathic DM; ILD, interstitial lung disease. *Countries from which non-Japanese cases were reported: Australia (1), Brazil (1), Malaysia (1), Korea (1), United States (1), West Indes (1), Yugoslavia (1). †C-ADM can be subclassified as follows: ADM (DMSSD for at least 6 months or longer without muscle weakness, normal muscle enzymes, and no other laboratory or radiologic evidence of muscle inflammation [eg, electromyography, muscle biopsy, muscle spectroscopy]); HDM (DMSSD for at least 6 months or longer without muscle weakness, normal muscle enzymes but some degree of other clinically silent laboratory or radiologic evidence of myositis). The key distinguishing feature of C-ADM is the fact that such patients display DMSSD for atypically prolonged periods of time without accompanying muscle weakness that is typical of classical DM. (Not all patients included in this analysis could be subclassified as either ADM or HDM since some had DMSSD for less than 6 months at the time of onset of their ILD.) ‡Number positive or abnormal/number tested.
798 Letters
J AM ACAD DERMATOL MAY 2003
ously thought to be quite rare, more than 300 cases of C-ADM have now been published. Among these cases, we were surprised to find 34 published cases of often fatal ILD occurring in patients having DMSSD but no muscle weakness (Table I). Many of these reports have been published in languages other than English, especially Japanese. Not all of these cases meet the classification criteria for C-ADM because DMSSD in some cases had been present for less than 6 months when ILD presented clinically. However, these cases were included in the present analysis because they do represent ILD occurring in the context of DMSSD without the presence of muscle weakness. In addition to the 34 published cases summarized in Table I, we are aware of several other such cases reported in abstract form (5 cases), on Internet Web sites (2 cases, both fatal), and by personal communication (1 case, fatal). Thus, there can be little doubt that clinical evidence of muscle weakness need not be present for the development of potentially fatal, DM-associated ILD. Failure to recognize this clinical constellation can delay diagnosis and institution of proper treatment and, thereby, have disastrous consequences. Richard D. Sontheimer, MDa Sachiko Miyagawa, MD, PhDb Department of Dermatology University of Iowa Hospitals and Clinics 200 Hawkins Dr BT 2045-1 Iowa City, IA 52242-1090a Department of Dermatology Nara Medical University Nara, Japanb
REFERENCES 1. Hirakata M, Nagai S. Interstitial lung disease in polymyositis and dermatomyositis. Curr Opin Rheumatol 2000;12:501-8. 2. Douglas WW, Tazelaar HD, Hartman TE, Hartman RP, Decker PA, Schroeder DR, et al. Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 2001;164: 1182-5. 3. Sontheimer RD. Cutaneous features of classical dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol 1999;11:475-82. 4. Sontheimer RD. Would a new name hasten the acceptance of clinically amyopathic dermatomyositis (dermatomyositis sine´ myositis) as a distinctive subset within the idiopathic inflammatory dermatomyopathies spectrum of clinical illness? J Am Acad Dermatol 2002;46:626-36. 5. Caproni M, Cardinali C, Parodi A, Giomi B, Papini M, Vaccaro M, et al. Amyopathic dermatomyositis: a review by the Italian group of immunodermatology. Arch Dermatol 2002;138:23-7.
Published online March 11, 2003 doi:10.1067/mjd.2003.199