Morphoea with Myositis: A Rare Association

Abstract

In this case, we describe an unusual presentation of a young woman with a rash typical of morphoea (confirmed on biopsy), who went on to develop myositis in an atypical distribution. Although the association of myositis with diffuse systemic sclerosis is well described, the link with localised scleroderma (morphoea) and myositis has not been described.

Anti–3‐hydroxy‐3‐methylglutaryl‐coenzyme a reductase necrotizing myopathy masquerading as a muscular dystrophy in a child

“…The presence of anti‐HMGCR antibody in patients with linear scleroderma has not been evaluated or reported. Our literature search revealed a few case reports of adult patients with linear scleroderma and polymyositis or dermatomyositis . Similar to our patient, in most of these cases, linear scleroderma preceded the onset of muscle weakness.…”

Linear scleroderma with prominent multiple lymphadenopathy followed by the development of polymyositis: A case report and review of published work

“…Although it is generally accepted that adult cases of LSc develop other autoimmune diseases, 4,8 a PubMed search yielded only four reports on concomitant cases of dermatomyositis/polymyositis and LSc (summarized in Table 1). [9][10][11][12] In four out of five cases including our patient, the development of LSc preceded that of myositis. The interval between the onsets was at most 3 years, suggesting the presence of a common autoimmune background leading to the development of these two diseases.…”

“…Although it is generally accepted that adult cases of LSc develop other autoimmune diseases, a PubMed search yielded only four reports on concomitant cases of dermatomyositis/polymyositis and LSc (summarized in Table ) . In four out of five cases including our patient, the development of LSc preceded that of myositis.…”

Diagnostic criteria, severity classification and guidelines of localized scleroderma

“…On the other hand, localized scleroderma can be complicated by inflammatory myopathy, and to date there have been 10 case reports of localized scleroderma with inflammatory myopathy in which myositis findings were confirmed with muscle biopsy (linear scleroderma/limbs, n = 4; circumscribed morphea, n = 2; linear scleroderma/head, n = 4). [127][128][129][130][131][132][133][134] Muscle symptoms commonly include muscle weakness, fatigue and muscle atrophy, but muscle convulsions were reported in one out of 10 patients. 133 Therefore, if muscle contraction abnormalities are seen in patients with localized scleroderma, direct muscle injury must be considered in the pathology of this disease and tests must be implemented accordingly.…”

“…The most frequently used drugs are corticosteroids, used in 80% of previously reported cases with a reported 90% efficacy. 127,142,143,[145][146][147]153,154 However, only one case has been treated with corticosteroid monotherapy, 111 while four were treated with steroids and methotrexate, 142,143,146,154 three were treated with corticosteroids combined with azathioprine, 125,129,131 two were treated with corticosteroids combined with cyclophosphamide, 147 and one each were treated with corticosteroids combined with mycophenolate mofetil, 142 IFN-c 145 or high-dose Ig i.v. therapy.…”