Dermatomyositis paraneoplastic M33.1

Dermatomyositis combined with or caused by a malignancy, see below. Paraneoplastic syndromes.

Tumor associations: There is an increased risk of lung, ovarian, breast and colorectal carcinoma. Furthermore, increased bladder carcinomas, pancreatic carcinomas, renal carcinomas are found.

General: General fatigue with muscle weakness and muscle soreness. Patients can perform their normal activities (e.g., climbing stairs, combing hair) with difficulty or not at all.

Note: Patient appears tired at consultation, leans on chair when standing up, hand clasp is feeble, gait sluggish, voice quiet to broken.

Integument (often first sign of dermatomyositis: skin symptoms precede muscle weakness in 1/3 of cases). Skin symptoms see below. Dermatomyositis.

Other organ manifestations:

• Muscles: Increasing, symmetrical, painful (sore) muscle weakness, especially of proximal limb segments. Calcifications of the muscles are possible.
• Skeletal system: Arthralgias and arthritides occur in about 25% of pat. with inflammatory muscle diseases. Partly type of symmetrical polyarthritis, also as oligo- or monarthritis. Rarely mutilating arthritis (DD: antisynthetase syndrome; see overlap syndrome below). Further: Severe osteoporosis, more extensive calcification of soft tissues (tendons, muscles, aponeuroses), and significant joint deformities (joint space is preserved!).
• Lung: Lung involvement (primary interstitial pneumonitis) in 15-30% of patients.
• Vessels: Concomitant Raynaud's phenomenon or scleroderma-like edema (hands) suggest overlap syndrome. This symptomatology is rarely associated with a malignant tumor!
• Adipose tissue: Rare but clinically established manifestation of dermatomyositis with indurated, painful nodules or plaques on the abdomen, buttocks, and arms. Ulceration and lipodystrophy may occur.
• Internal organs: involvement of heart, intestine and kidneys. Dysphagia due to weakness of oropharyngeal muscles. Associated: Bronchopneumonia (aspiration due to dysphagia), hepato-splenomegaly, nephritis, tenderness of large nerve trunks, psychic changes, focal retinitis peripapillosa, cotton-wool exudate, small streaky hemorrhages in the nerve fiber layer, papilledema, rarely episcleritis and scleritis.

Serum: serum creatinine phosphokinase (especially MM type), aldolase, CK (up to 50-fold increased), GOT, GPT, LDH increased. Creatinine excretion increased during relapse.

Antibodies: Detection of antinuclear antibodies (ANA) is possible in about 33% of cases. The following antibody profile is also possible:

TIF1-gamma antibodies positive in 40-75% of cases;

Cutaneous necroses, necroses in the muscle biopsy, SPA > 40 mm/hour, age > 60 years, asthenia all speak for a malignoma-associated dermatomyositis.

1. Chairatchaneeboon M et al (2015) Calcific panniculitis and nasopharyngeal cancer-associated adult-onset dermatomyositis: a case report and literature review. Springerplus 4:201
2. Di Rollo D et al (2014) Cancer risk in dermatomyositis: a systematic review of the literature. G Ital Dermatol Venereol 149: 525-537
3. Dobloug GC et al (2015) Survival and cancer risk in an unselected and complete Norwegian idiopathic inflammatory myopathy cohort. Semin Arthritis Rheum doi: 10.1016/j.semarthrit.2015.06.005.
4. Lu X et al (2014) Factors predicting malignancy in patients with polymyositis and dermatomyositis: a systematic review and meta-analysis. PLoS One 9: e94128
5. Olazagasti JM et al (2015) Cancer risk in dermatomyositis: a meta-analysis of cohort studies. At J Clin Dermatol 16:89-98
6. Requena C et al (2014) Paraneoplastic dermatomyositis: a study of 12 cases. Actas Dermosifiliogr 105: 675-682