Characteristics of dermatomyositis patients with and without associated malignancy

Abstract

Background: Dermatomyositis belongs to the rare idiopathic, inflammatory myositis group. A previously postulated link between some cases of dermatomyositis and malignancy has been established in recent years. Criteria suggestive of a malignancy association are still being explored. Patients and Methods:We retrospectively analyzed data from 63 patients with dermatomyositis over a period of 15 years. Results:The following criteria argue for cancer-associated dermatomyositis: older age (> 52 years [P = 0.001], > 65 years [P = 0.002], ≥ 75 years [P = 0.002]), shorter time between manifestation and diagnosis of dermatomyositis (malignancy group: 59 days vs. non-malignancy group: 137 days [P = 0.022]), typical skin involvement such as Gottron sign (P = 0.045), centrofacial erythema (P = 0.036) and typical erythema on the upper arms and forearms (P = 0.019), oropharyngeal involvement (P = 0.015) and increased ALT (P = 0.031). The following criteria argue for non-cancer-associated dermatomyositis: younger age (≤ 52 years [P = 0.001], 40-65 years [P = 0.045]) and pruritus (P = 0.026). Conclusions:The aforementioned criteria have been documented in the literature, but reported findings are heterogenous concerning the suitability of their markers for malignancy association. Small study populations, few prospective controlled studies, summarization of different forms of myositis and inconsistent use nomenclature contribute to biased results. Our study aims to make an important contribution toward the identification of risk factors in cancer-associated dermatomyositis.

Clinical Characteristics of Anti-TIF-1γ Antibody-Positive Dermatomyositis Associated with Malignancy

“…Fiorentino et al reported that characteristic cutaneous features have been reported, including psoriasiform lesions or so called ‘red-on-white’ patches (hypopigmented macules/patches associated with focal, often follicular, telangiectatic erythema) [ 22 ]. Our patients’ cutaneous manifestations were similar to those in the reports [ 17 , 18 , 19 , 20 , 21 , 22 ], but these findings were severe compared with those in patients with TIF-1γ-negative DM. For example, skin lesions tended to be widespread, more dark-red in color, and scattered throughout the body.…”

“…One published series of patients with DM reported an incidence of cancer ranging from 9% to 42% [ 15 , 16 ]. The most frequent are ovarian, breast, lung, gastric, and colorectal tumors, as well as lymphomas in dermatomyositis, lung and urinary bladder cancers, and lymphomas in polymyositis [ 18 ]. Most cancers preceded, occurred concurrently, or occurred after the onset of DM, but is usually recognized within 3 years of DM diagnosis.…”

“…Most cancers preceded, occurred concurrently, or occurred after the onset of DM, but is usually recognized within 3 years of DM diagnosis. However, most of the cases may be diagnosed within 12 months [ 18 ]. In our study, cancer was recognized in one case after surgery for the primary lesion, and one patient was under anti-cancer therapy.…”

“…In our study, cancer was recognized in one case after surgery for the primary lesion, and one patient was under anti-cancer therapy. In general, clinical risk factors for malignancy in DM include older age, progressive disease, longer duration of symptoms before diagnosis, initiation of therapy after 24 months of muscle weakness, cardiac issues, pulmonary problems, male sex, severe skin manifestations, dysphagia, resistance to treatment, and a history of malignancy with risk of relapse and absence of ILD [ 14 , 18 ].…”

“…In general, typical skin lesions such as the Gottron sign, centrofacial erythema, and typical erythema on the upper arms and forearms were common in patients with DM and malignancy. The Gottron papules were distinctly hyperkeratotic and scaly [ 17 , 18 , 19 ]. Daly et al reported that facial dermatomyositis and the shawl sign were significantly more common in patients with TIF-1γ DM [ 20 ].…”