Illustrations
Figure 1
Clinical spectrum of cutaneous dermatomyositis. A ) Erythematous, infiltrated papules at the metacarpophalangeal and interphalangeal joints (Gottrońs papules). B ) Periungueal telangiectasia with associated cuticular overgrowth (Keining‘s sign) and small haemorrhagic infarctions of the nail-folds. C ) Dusky red erythema on the upper chest (V-sign). D ) Dusky red erythema on the upper back (Shawl-sign).
Figure 1
Figure 2
Muscular involvement in dermatomyositis. A ) H&E staining of severely affected skeletal muscle with a dense perifascicular lymphocytic infiltrate (asterisk) and macrophages (circle). The subfascial compartment harbours many atrophic muscle fibres (arrows). B ) C5b9 immunohistochemistry shows complement deposits on abundant capillaries (brown dots), while immunoreactivity on large vessels is normal (arrow). Note the necrotic fibres with immunoreactivity to C5b9 (asterisk). Scale bar: 100 μm. C ) Fat atrophy of both iliopsoases and both glutei maximi muscles with focal enhanced signal intensity. D ) Enhanced signal intensity of both glutei maximi muscles.
Figure 2
Figure 3
Relationship between laboratory parameters in patients with anti-TIF-1 gamma and anti-MDA-5 antibodies using Spearman's correlation coefficient. A ) Positive correlation between GOT and CK in patients with anti-TIF-1 gamma antibodies (p = 0.0167, r = 1) but no correlation in patients with anti-MDA-5 antibodies (p = 0.6583, r = 0.2571). B -D ) No correlation between LDH and CK (anti-TIF-1 gamma antibody group: p = 0.0833, r = 0.9; anti-MDA-5 antibody group: p = 0.1750, r = 0.6571), LDH and GOT (anti-TIF-1 gamma antibody group: p = 0.0833, r = 0.9; anti-MDA-5 antibody group: p = 0.2, r = 0.5714), or LDH and GPT (anti-TIF-1 gamma antibody group: p = 0.0833, r = 0.9; anti-MDA-5 antibody group: p = 0.9635, r = 0.03571).
Figure 3
Figure 4
Proposed update of the criteria of Sontheimer et al . for amyopathic dermatomyositis.
Figure 4
Tableaux
Auteurs
1 Department of Dermatology and Allergology,
2 Institute of Neuropathology, Philipps-University, MarburgGermany
Background Dermatomyositis (DM) is a group of autoimmune diseases characterized by a variable degree of skin symptoms and myopathy. An amyopathic form of DM (ADM) has been described, and more recently, an anti-TIF-1 gamma-positive subtype, characterized by poikiloderma and associated with a relatively high risk of cancer.