Skip to main content
Erschienen in: Die Dermatologie 2/2024

09.01.2024 | Dermatomyositis in der Hautheilkunde | CME

Aktuelle Aspekte zur Dermatomyositis

verfasst von: Dr. med. Julian Steininger, Prof. Dr. med. Claudia Günther

Erschienen in: Die Dermatologie | Ausgabe 2/2024

zum CME-Kurs Einloggen, um Zugang zu erhalten

Zusammenfassung

Dermatomyositis (DM) ist eine seltene Autoimmunerkrankung mit Haut- und Muskelsymptomen, die den idiopathisch inflammatorischen Myopathien zugeordnet wird. Neben Hautveränderungen und einer typischen Muskelschwäche und -atrophie können ebenfalls Herz und Lunge als wesentliche Organe betroffen sein. In 20 % der Fälle findet sich bei Erwachsenen eine Assoziation zu malignen Tumoren. Die Pathogenese der Erkrankung ist unvollständig verstanden. Sie basiert auf multifaktoriellen Ursachen, die genetische, umweltbezogene und immunologische Faktoren einschließen. Es resultiert eine Aktivierung angeborener und adaptiver Immunmechanismen, die durch eine Stimulation des Typ-I-Interferon-Signalweges und Immunantwort gegen körpereigene Strukturen charakterisiert ist. Myositis-spezifische Antikörper sind charakteristisch für DM und ermöglichen eine differenzierende Diagnose. Therapien umfassen Kortikosteroide, Antimalariamittel, Immunglobuline, Biologika wie Rituximab oder JAK(Januskinase)-Inhibitoren. Eine frühzeitige Diagnose und Behandlung sind entscheidend für eine bessere Prognose.
Literatur
1.
Zurück zum Zitat Wiendl H., Schmidt J. et al., Myositissyndrome, S2k-Leitlinie, 2022; in: Deutsche Gesellschaft für Neurologie (Hrsg.), Leitlinien für Diagnostik und Therapie in der Neurologie. Online: www.dgn.org/leitlinien (abgerufen am 2. Aug. 2023). Wiendl H., Schmidt J. et al., Myositissyndrome, S2k-Leitlinie, 2022; in: Deutsche Gesellschaft für Neurologie (Hrsg.), Leitlinien für Diagnostik und Therapie in der Neurologie. Online: www.​dgn.​org/​leitlinien (abgerufen am 2. Aug. 2023).
2.
3.
Zurück zum Zitat Lilleker JB, Vencovsky J, Wang G, Wedderburn LR, Diederichsen LP, Schmidt J et al (2018) The euromyositis registry: an international collaborative tool to facilitate myositis research. Ann Rheum Dis 77(1):30–39PubMedCrossRef Lilleker JB, Vencovsky J, Wang G, Wedderburn LR, Diederichsen LP, Schmidt J et al (2018) The euromyositis registry: an international collaborative tool to facilitate myositis research. Ann Rheum Dis 77(1):30–39PubMedCrossRef
4.
Zurück zum Zitat Aussy A, Boyer O, Cordel N (2017) Dermatomyositis and immune-mediated necrotizing myopathies: a window on autoimmunity and cancer. Front Immunol 8:992PubMedPubMedCentralCrossRef Aussy A, Boyer O, Cordel N (2017) Dermatomyositis and immune-mediated necrotizing myopathies: a window on autoimmunity and cancer. Front Immunol 8:992PubMedPubMedCentralCrossRef
5.
Zurück zum Zitat Furst DE, Amato AA, Iorga ŞR, Gajria K, Fernandes AW (2012) Epidemiology of adult idiopathic inflammatory myopathies in a U.S. managed care plan. Muscle Nerve 45(5):676–683PubMedCrossRef Furst DE, Amato AA, Iorga ŞR, Gajria K, Fernandes AW (2012) Epidemiology of adult idiopathic inflammatory myopathies in a U.S. managed care plan. Muscle Nerve 45(5):676–683PubMedCrossRef
6.
Zurück zum Zitat Bendewald MJ, Wetter DA, Li X, Davis MDP (2010) Incidence of dermatomyositis and clinically amyopathic dermatomyositis: a population-based study in olmsted county, minnesota. Arch Dermatol 146(1):26–30PubMedCrossRef Bendewald MJ, Wetter DA, Li X, Davis MDP (2010) Incidence of dermatomyositis and clinically amyopathic dermatomyositis: a population-based study in olmsted county, minnesota. Arch Dermatol 146(1):26–30PubMedCrossRef
7.
Zurück zum Zitat Mendez EP, Lipton R, Ramsey-Goldman R, Roettcher P, Bowyer S, Dyer A et al (2003) US incidence of juvenile dermatomyositis, 1995–1998: results from the national institute of arthritis and musculoskeletal and skin diseases registry. Arthritis Rheum 15;49(3):300–305CrossRef Mendez EP, Lipton R, Ramsey-Goldman R, Roettcher P, Bowyer S, Dyer A et al (2003) US incidence of juvenile dermatomyositis, 1995–1998: results from the national institute of arthritis and musculoskeletal and skin diseases registry. Arthritis Rheum 15;49(3):300–305CrossRef
8.
Zurück zum Zitat Zaba LC, Fiorentino DF (2012) Skin disease in dermatomyositis. Curr Opin Rheumatol 24(6):597–601PubMedCrossRef Zaba LC, Fiorentino DF (2012) Skin disease in dermatomyositis. Curr Opin Rheumatol 24(6):597–601PubMedCrossRef
9.
Zurück zum Zitat Márton K, Hermann P, Dankó K, Fejérdy P, Madléna M, Nagy G (2005) Evaluation of oral manifestations and masticatory force in patients with polymyositis and dermatomyositis. J Oral Pathol Med 34(3):164–169PubMedCrossRef Márton K, Hermann P, Dankó K, Fejérdy P, Madléna M, Nagy G (2005) Evaluation of oral manifestations and masticatory force in patients with polymyositis and dermatomyositis. J Oral Pathol Med 34(3):164–169PubMedCrossRef
10.
Zurück zum Zitat Manfredi A, Sebastiani M, Cassone G, Pipitone N, Giuggioli D, Colaci M et al (2015) Nailfold capillaroscopic changes in dermatomyositis and polymyositis. Clin Rheumatol 34(2):279–284PubMedCrossRef Manfredi A, Sebastiani M, Cassone G, Pipitone N, Giuggioli D, Colaci M et al (2015) Nailfold capillaroscopic changes in dermatomyositis and polymyositis. Clin Rheumatol 34(2):279–284PubMedCrossRef
11.
12.
13.
Zurück zum Zitat DeWane ME, Waldman R, Lu J (2020) Dermatomyositis: clinical features and pathogenesis. J Am Acad Dermatol 82(2):267–281PubMedCrossRef DeWane ME, Waldman R, Lu J (2020) Dermatomyositis: clinical features and pathogenesis. J Am Acad Dermatol 82(2):267–281PubMedCrossRef
14.
Zurück zum Zitat Fiorentino D, Chung L, Zwerner J, Rosen A, Casciola-Rosen L (2011) The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): a retrospective study. J Am Acad Dermatol 65(1):25–34PubMedPubMedCentralCrossRef Fiorentino D, Chung L, Zwerner J, Rosen A, Casciola-Rosen L (2011) The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): a retrospective study. J Am Acad Dermatol 65(1):25–34PubMedPubMedCentralCrossRef
15.
Zurück zum Zitat Gono T, Kawaguchi Y, Satoh T, Kuwana M, Katsumata Y, Takagi K et al (2010) Clinical manifestation and prognostic factor in anti-melanoma differentiation-associated gene 5 antibody-associated interstitial lung disease as a complication of dermatomyositis. Rheumatol (Oxford) 49(9):1713–1719CrossRef Gono T, Kawaguchi Y, Satoh T, Kuwana M, Katsumata Y, Takagi K et al (2010) Clinical manifestation and prognostic factor in anti-melanoma differentiation-associated gene 5 antibody-associated interstitial lung disease as a complication of dermatomyositis. Rheumatol (Oxford) 49(9):1713–1719CrossRef
16.
Zurück zum Zitat Ceribelli A, Fredi M, Taraborelli M, Cavazzana I, Tincani A, Selmi C et al (2014) Prevalence and clinical significance of anti-MDA5 antibodies in European patients with polymyositis/dermatomyositis. Clin Exp Rheumatol 32(6):891–897PubMed Ceribelli A, Fredi M, Taraborelli M, Cavazzana I, Tincani A, Selmi C et al (2014) Prevalence and clinical significance of anti-MDA5 antibodies in European patients with polymyositis/dermatomyositis. Clin Exp Rheumatol 32(6):891–897PubMed
17.
Zurück zum Zitat Rogers A, Chung L, Li S, Livia-Casciola-Rosen, Fiorentino DF (2017) The cutaneous and systemic findings associated with nuclear matrix protein‑2 antibodies in adult dermatomyositis patients. Arthritis Care Res (Hoboken) 69(12):1909–1914PubMedCrossRef Rogers A, Chung L, Li S, Livia-Casciola-Rosen, Fiorentino DF (2017) The cutaneous and systemic findings associated with nuclear matrix protein‑2 antibodies in adult dermatomyositis patients. Arthritis Care Res (Hoboken) 69(12):1909–1914PubMedCrossRef
18.
Zurück zum Zitat Cruellas MGP, Viana V dos ST, Levy-Neto M, de Souza FHC, Shinjo SK (2013) Myositis-specific and myositis-associated autoantibody profiles and their clinical associations in a large series of patients with polymyositis and dermatomyositis. Clin (Sao Paulo) 68(7):909–914CrossRef Cruellas MGP, Viana V dos ST, Levy-Neto M, de Souza FHC, Shinjo SK (2013) Myositis-specific and myositis-associated autoantibody profiles and their clinical associations in a large series of patients with polymyositis and dermatomyositis. Clin (Sao Paulo) 68(7):909–914CrossRef
19.
Zurück zum Zitat Meisterfeld S, Röber N, Conrad K, Beissert S, Aringer M, Günther C (2017) A chronic recurrent disease course of dermatomyositis is associated with autoantibodies against transcriptional intermediary factor 1‑γ. Br J Dermatol 177(2):590–593PubMedCrossRef Meisterfeld S, Röber N, Conrad K, Beissert S, Aringer M, Günther C (2017) A chronic recurrent disease course of dermatomyositis is associated with autoantibodies against transcriptional intermediary factor 1‑γ. Br J Dermatol 177(2):590–593PubMedCrossRef
20.
Zurück zum Zitat Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A et al (2001) Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet 357(9250):96–100PubMedCrossRef Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A et al (2001) Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet 357(9250):96–100PubMedCrossRef
21.
Zurück zum Zitat Uruha A, Nishikawa A, Tsuburaya RS, Hamanaka K, Kuwana M, Watanabe Y et al (2017) Sarcoplasmic MxA expression: a valuable marker of dermatomyositis. Neurology 88(5):493–500PubMedCrossRef Uruha A, Nishikawa A, Tsuburaya RS, Hamanaka K, Kuwana M, Watanabe Y et al (2017) Sarcoplasmic MxA expression: a valuable marker of dermatomyositis. Neurology 88(5):493–500PubMedCrossRef
22.
Zurück zum Zitat Suzuki S, Uruha A, Suzuki N, Nishino I (2017) Integrated diagnosis project for inflammatory myopathies: an association between autoantibodies and muscle pathology. Autoimmun Rev 16(7):693–700PubMedCrossRef Suzuki S, Uruha A, Suzuki N, Nishino I (2017) Integrated diagnosis project for inflammatory myopathies: an association between autoantibodies and muscle pathology. Autoimmun Rev 16(7):693–700PubMedCrossRef
23.
Zurück zum Zitat Rothwell S, Cooper RG, Lundberg IE, Miller FW, Gregersen PK, Bowes J et al (2016) Dense genotyping of immune-related loci in idiopathic inflammatory myopathies confirms HLA alleles as the strongest genetic risk factor and suggests different genetic background for major clinical subgroups. Ann Rheum Dis 75(8):1558–1566PubMedCrossRef Rothwell S, Cooper RG, Lundberg IE, Miller FW, Gregersen PK, Bowes J et al (2016) Dense genotyping of immune-related loci in idiopathic inflammatory myopathies confirms HLA alleles as the strongest genetic risk factor and suggests different genetic background for major clinical subgroups. Ann Rheum Dis 75(8):1558–1566PubMedCrossRef
24.
Zurück zum Zitat Love LA, Weinberg CR, McConnaughey DR, Oddis CV, Medsger TA, Reveille JD et al (2009) Ultraviolet radiation intensity predicts the relative distribution of dermatomyositis and anti-Mi‑2 autoantibodies in women. Arthritis Rheum 60(8):2499–2504PubMedPubMedCentralCrossRef Love LA, Weinberg CR, McConnaughey DR, Oddis CV, Medsger TA, Reveille JD et al (2009) Ultraviolet radiation intensity predicts the relative distribution of dermatomyositis and anti-Mi‑2 autoantibodies in women. Arthritis Rheum 60(8):2499–2504PubMedPubMedCentralCrossRef
25.
Zurück zum Zitat Christensen ML, Pachman LM, Schneiderman R, Patel DC, Friedman JM (1986) Prevalence of coxsackie B virus antibodies in patients with juvenile dermatomyositis. Arthritis Rheum 29(11):1365–1370PubMedCrossRef Christensen ML, Pachman LM, Schneiderman R, Patel DC, Friedman JM (1986) Prevalence of coxsackie B virus antibodies in patients with juvenile dermatomyositis. Arthritis Rheum 29(11):1365–1370PubMedCrossRef
26.
Zurück zum Zitat Kissel JT, Mendell JR, Rammohan KW (1986) Microvascular deposition of complement membrane attack complex in dermatomyositis. N Engl J Med 314(6):329–334PubMedCrossRef Kissel JT, Mendell JR, Rammohan KW (1986) Microvascular deposition of complement membrane attack complex in dermatomyositis. N Engl J Med 314(6):329–334PubMedCrossRef
27.
Zurück zum Zitat Baechler EC, Bauer JW, Slattery CA, Ortmann WA, Espe KJ, Novitzke J et al (2007) An interferon signature in the peripheral blood of dermatomyositis patients is associated with disease activity. Mol Med 13(1–2):59–68PubMedPubMedCentralCrossRef Baechler EC, Bauer JW, Slattery CA, Ortmann WA, Espe KJ, Novitzke J et al (2007) An interferon signature in the peripheral blood of dermatomyositis patients is associated with disease activity. Mol Med 13(1–2):59–68PubMedPubMedCentralCrossRef
28.
Zurück zum Zitat Greenberg SA, Pinkus JL, Pinkus GS, Burleson T, Sanoudou D, Tawil R et al (2005) Interferon-alpha/beta-mediated innate immune mechanisms in dermatomyositis. Ann Neurol 57(5):664–678PubMedCrossRef Greenberg SA, Pinkus JL, Pinkus GS, Burleson T, Sanoudou D, Tawil R et al (2005) Interferon-alpha/beta-mediated innate immune mechanisms in dermatomyositis. Ann Neurol 57(5):664–678PubMedCrossRef
29.
Zurück zum Zitat Liao AP, Salajegheh M, Nazareno R, Kagan JC, Jubin RG, Greenberg SA (2011) Interferon β is associated with type 1 interferon-inducible gene expression in dermatomyositis. Ann Rheum Dis 70(5):831–836PubMedCrossRef Liao AP, Salajegheh M, Nazareno R, Kagan JC, Jubin RG, Greenberg SA (2011) Interferon β is associated with type 1 interferon-inducible gene expression in dermatomyositis. Ann Rheum Dis 70(5):831–836PubMedCrossRef
30.
Zurück zum Zitat Wong D, Kea B, Pesich R, Higgs BW, Zhu W, Brown P et al (2012) Interferon and biologic signatures in dermatomyositis skin: specificity and heterogeneity across diseases. Plos One 7(1):e29161PubMedPubMedCentralCrossRef Wong D, Kea B, Pesich R, Higgs BW, Zhu W, Brown P et al (2012) Interferon and biologic signatures in dermatomyositis skin: specificity and heterogeneity across diseases. Plos One 7(1):e29161PubMedPubMedCentralCrossRef
31.
Zurück zum Zitat Burdick LM, Somani N, Somani AK (2009) Type I IFNs and their role in the development of autoimmune diseases. Expert Opin Drug Saf 8(4):459–472PubMedCrossRef Burdick LM, Somani N, Somani AK (2009) Type I IFNs and their role in the development of autoimmune diseases. Expert Opin Drug Saf 8(4):459–472PubMedCrossRef
32.
Zurück zum Zitat Chen KL, Patel J, Zeidi M, Wysocka M, Bashir MM, Patel B et al (2021) Myeloid dendritic cells are major producers of IFN‑β in dermatomyositis and may contribute to hydroxychloroquine refractoriness. J Invest Dermatol 141(8):1906–1914.e2PubMedPubMedCentralCrossRef Chen KL, Patel J, Zeidi M, Wysocka M, Bashir MM, Patel B et al (2021) Myeloid dendritic cells are major producers of IFN‑β in dermatomyositis and may contribute to hydroxychloroquine refractoriness. J Invest Dermatol 141(8):1906–1914.e2PubMedPubMedCentralCrossRef
33.
Zurück zum Zitat McNiff JM, Kaplan DH (2008) Plasmacytoid dendritic cells are present in cutaneous dermatomyositis lesions in a pattern distinct from lupus erythematosus. J Cutan Pathol 35(5):452–456PubMedCrossRef McNiff JM, Kaplan DH (2008) Plasmacytoid dendritic cells are present in cutaneous dermatomyositis lesions in a pattern distinct from lupus erythematosus. J Cutan Pathol 35(5):452–456PubMedCrossRef
34.
Zurück zum Zitat Crow YJ, Stetson DB (2022) The type I interferonopathies: 10 years on. Nat Rev Immunol 22(8):471–483PubMedCrossRef Crow YJ, Stetson DB (2022) The type I interferonopathies: 10 years on. Nat Rev Immunol 22(8):471–483PubMedCrossRef
35.
Zurück zum Zitat Vuong V, Duong TA, Aouizerate J, Authier FJ, Ingen-Housz-Oro S, Valeyrie-Allanore L et al (2016) Dermatomyositis: factors predicting relapse. J Eur Acad Dermatol Venereol 30(5):813–818PubMedCrossRef Vuong V, Duong TA, Aouizerate J, Authier FJ, Ingen-Housz-Oro S, Valeyrie-Allanore L et al (2016) Dermatomyositis: factors predicting relapse. J Eur Acad Dermatol Venereol 30(5):813–818PubMedCrossRef
36.
Zurück zum Zitat Efremova M, Finotello F, Rieder D, Trajanoski Z (2017) Neoantigens generated by individual mutations and their role in cancer immunity and immunotherapy. Front Immunol 8:1679PubMedPubMedCentralCrossRef Efremova M, Finotello F, Rieder D, Trajanoski Z (2017) Neoantigens generated by individual mutations and their role in cancer immunity and immunotherapy. Front Immunol 8:1679PubMedPubMedCentralCrossRef
37.
Zurück zum Zitat Rosen A, Casciola-Rosen L (2016) Autoantigens as partners in initiation and propagation of autoimmune rheumatic diseases. Annu Rev Immunol 20;34:395–420CrossRef Rosen A, Casciola-Rosen L (2016) Autoantigens as partners in initiation and propagation of autoimmune rheumatic diseases. Annu Rev Immunol 20;34:395–420CrossRef
38.
Zurück zum Zitat Mohassel P, Rosen P, Casciola-Rosen L, Pak K, Mammen AL (2015) Expression of the dermatomyositis autoantigen transcription intermediary factor 1γ in regenerating muscle. Arthritis Rheumatol 67(1):266–272PubMedPubMedCentralCrossRef Mohassel P, Rosen P, Casciola-Rosen L, Pak K, Mammen AL (2015) Expression of the dermatomyositis autoantigen transcription intermediary factor 1γ in regenerating muscle. Arthritis Rheumatol 67(1):266–272PubMedPubMedCentralCrossRef
39.
Zurück zum Zitat Dunn GP, Old LJ, Schreiber RD (2004) The three Es of cancer immunoediting. Annu Rev Immunol 22:329–360PubMedCrossRef Dunn GP, Old LJ, Schreiber RD (2004) The three Es of cancer immunoediting. Annu Rev Immunol 22:329–360PubMedCrossRef
40.
Zurück zum Zitat Fiorentino DF, Mecoli CA, Rosen MC, Chung LS, Christopher-Stine L, Rosen A et al (2022) Immune responses to CCAR1 and other dermatomyositis autoantigens are associated with attenuated cancer emergence. J Clin Invest 132(2):e150201PubMedPubMedCentralCrossRef Fiorentino DF, Mecoli CA, Rosen MC, Chung LS, Christopher-Stine L, Rosen A et al (2022) Immune responses to CCAR1 and other dermatomyositis autoantigens are associated with attenuated cancer emergence. J Clin Invest 132(2):e150201PubMedPubMedCentralCrossRef
41.
Zurück zum Zitat Heinen A, Schnabel A, Brück N, Smitka M, Wolf C, Lucas N et al (2021) Interferon signature guiding therapeutic decision making: ruxolitinib as first-line therapy for severe juvenile dermatomyositis? Rheumatol (Oxford) 60(4):e136–e138CrossRef Heinen A, Schnabel A, Brück N, Smitka M, Wolf C, Lucas N et al (2021) Interferon signature guiding therapeutic decision making: ruxolitinib as first-line therapy for severe juvenile dermatomyositis? Rheumatol (Oxford) 60(4):e136–e138CrossRef
42.
Zurück zum Zitat Fischer K, Aringer M, Steininger J, Heil J, Beissert S, Abraham S et al (2022) Improvement of cutaneous inflammation and panniculitis in patients with dermatomyositis by the janus kinase inhibitor baricitinib. Br J Dermatol 187(3):432–435PubMedCrossRef Fischer K, Aringer M, Steininger J, Heil J, Beissert S, Abraham S et al (2022) Improvement of cutaneous inflammation and panniculitis in patients with dermatomyositis by the janus kinase inhibitor baricitinib. Br J Dermatol 187(3):432–435PubMedCrossRef
43.
Zurück zum Zitat Higgs BW, Zhu W, Morehouse C, White WI, Brohawn P, Guo X et al (2014) A phase 1b clinical trial evaluating sifalimumab, an anti-IFN‑α monoclonal antibody, shows target neutralisation of a type I IFN signature in blood of dermatomyositis and polymyositis patients. Ann Rheum Dis 73(1):256–262PubMedCrossRef Higgs BW, Zhu W, Morehouse C, White WI, Brohawn P, Guo X et al (2014) A phase 1b clinical trial evaluating sifalimumab, an anti-IFN‑α monoclonal antibody, shows target neutralisation of a type I IFN signature in blood of dermatomyositis and polymyositis patients. Ann Rheum Dis 73(1):256–262PubMedCrossRef
Metadaten
Titel
Aktuelle Aspekte zur Dermatomyositis
verfasst von
Dr. med. Julian Steininger
Prof. Dr. med. Claudia Günther
Publikationsdatum
09.01.2024

Weitere Artikel der Ausgabe 2/2024

Die Dermatologie 2/2024 Zur Ausgabe

One Minute Wonder

One Minute Wonder

Dermatologischer Schnappschuss

„Salt and pepper sign“

Leitlinien kompakt für die Dermatologie

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Neu im Fachgebiet Dermatologie

09.04.2024 | Sexuell übertragbare Erkrankungen | Podcast | Nachrichten

Wer kümmert sich um STI bei Frauen?

mit Dr. med. Angela Schuster und Pauline Meurer, Institut für Allgemeinmedizin, Charité Berlin
In Kooperation mit:
Zeitschrift für Allgemeinmedizin, DEGAM

Update Dermatologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.