Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

Calcinosis Cutis Complicating Adult-Onset Dermatomyositis

Calcinosis Cutis Complicating Adult-Onset Dermatomyositis Calcinosis is a common complication of juvenile dermatomyositis, which can lead to extensive impairment of activities of daily living when it occursover the joints and limits or completely restricts movement.1 Lesions appear as hard nodules, commonly over bony prominences.1,2 Thesecalcifications occur in 10% to 40% of patients with juvenile dermatomyositis but are unusual in adults.2 Report of a Case. A 26-year-old woman presented for evaluation of multiple, hard subcutaneous nodules that had developed over a period of months in conjunction with worseningweakness and rash of dermatomyositis. Dermatomyositis had been diagnosed in 1998 at age 23 based upon the presence of characteristic dermatitis, including a heliotrope rash, Gottronpapules, a photodistributed poikiloderma, and typical periungual changes (Figure 1), and accompanied by proximal muscle weakness and elevated muscle-derived enzyme levels. A skin biopsy confirmedthe diagnosis by demonstrating the characteristic interface dermatitis with mucin deposition. Treatment at diagnosis consisted of various regimens oflow-dose corticosteroids, immunosuppressive drugs, and topical treatments. However, her skin was never without signs of dermatomyositis and her weaknessonly occasionally abated. Figure 1. View LargeDownload Photodistributed poikiloderma at initial consultative visit. In 1999, she was first sent for evaluation to 1 of us (J.P.C.). It was recommended that she use chloroquine phosphate, methotrexate, and, if responsewas inadequate, oral mycophenolate mofetil and/or intravenous immunoglobulin. Only slight improvement was noted and she became inconsistent with her treatmentsecondary to side effects. In 2002, she was again sent for an evaluation because of the development of multiple subcutaneous firm nodules. Physical examination revealed rock-hard subcutaneous nodules and plaques of the postaxillary, inframammary skin as well as the lateral part of thechest wall and arms. She was weak in the proximal muscles of both her upper and lower extremities. A 5-mm punch biopsy specimen taken from a nodule revealeda mixed lobular and septal calcifying panniculitis (Figure 2). Laboratory evaluation showed an elevated creatine kinase level of 300 U/L (normal, <179 U/L). Figure 2. View LargeDownload Subcutaneous tissue with sclerosis and calcification (hematoxylin-eosin, ×40). Recommendations at this time included administration of oral diltiazem and more aggressive management of dermatomyositis including higher doses oforal prednisone, monthly administration of intravenous immunoglobulin, and reintroduction of an immunosuppressive medication. Comment. Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous findings including the pathognomonic heliotrope rash.2 Itcan further be complicated by calcinosis, which is seen with a much higher frequency in children compared with adults.1,2 Calcinosis seen in connective tissue diseases is mostly of the dystrophic type and is believed to be a localized process rather than an imbalance ofcalcium homeostasis.3 One possible mechanism is the release of calcium from mitochondria in muscle cells damaged by themyopathy.3 Risk factors for the development of calcinosis other than young age include the presence of the tumor necrosisfactor α–308A allele, delay in diagnosis of dermatomyositis, and inconsistent and/or low-efficacy therapy among others.1,4,5 Onestudy found that patients with higher muscle-derived enzyme levels had a lower incidence of calcinosis, probably due to the more aggressive treatment regimenneeded for them.5 It has also been shown that a low socioeconomic status results in a delay in and less aggressiveapproach to therapy. This may have been a factor for our patient, although she was insured by a state program, she often failed to return for follow-upvisits to the physicians primarily in charge of her care. In addition, she may have been "undertreated" because of the low-grade activity of her disease. There are no controlled studies of the treatment of established calcinosis. However, the following therapies have been reported to be of benefit: bisphosphonates,calcium channel blockers, probenecid, warfarin, and aluminum hydroxide.1,3 In conclusion, our patient represents a rare complication of adult-onset dermatomyositis. It is possible that hadshe received early and aggressive and sustained therapy of the dermatomyositis, the calcinosis might have been prevented. The authors have no relevant financial interest in this article. References 1. Mukamel MHorev GMimouni M New insight into calcinosis of juvenile dermatomyositis: a study of composition and treatment J Pediatr. 2001;138763- 766PubMedGoogle ScholarCrossref 2. Callen JP Dermatomyositis Lancet. 2000;35553- 57PubMedGoogle ScholarCrossref 3. Vinen CSPatel SBruckner FE Regression of calcinosis associated with adult dermatomyositis following diltiazem therapy Rheumatology. 2000;39333- 334PubMedGoogle ScholarCrossref 4. Pachman LMLiotta-Davis MRHong DK et al. TNFα-308A allele in juvenile dermatomyositis: association with increased production of tumor necrosis factor α, disease duration, andpathologic calcifications Arthritis Rheum. 2000;432368- 2377PubMedGoogle ScholarCrossref 5. Fisler RELiang MGFuhlbrigge RC et al. Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis J Am Acad Dermatol. 2002;47505- 511PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Calcinosis Cutis Complicating Adult-Onset Dermatomyositis

Archives of Dermatology , Volume 140 (3) – Mar 1, 2004

Loading next page...
 
/lp/american-medical-association/calcinosis-cutis-complicating-adult-onset-dermatomyositis-xxqJk4zELB
Publisher
American Medical Association
Copyright
Copyright © 2004 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.140.3.365
Publisher site
See Article on Publisher Site

Abstract

Calcinosis is a common complication of juvenile dermatomyositis, which can lead to extensive impairment of activities of daily living when it occursover the joints and limits or completely restricts movement.1 Lesions appear as hard nodules, commonly over bony prominences.1,2 Thesecalcifications occur in 10% to 40% of patients with juvenile dermatomyositis but are unusual in adults.2 Report of a Case. A 26-year-old woman presented for evaluation of multiple, hard subcutaneous nodules that had developed over a period of months in conjunction with worseningweakness and rash of dermatomyositis. Dermatomyositis had been diagnosed in 1998 at age 23 based upon the presence of characteristic dermatitis, including a heliotrope rash, Gottronpapules, a photodistributed poikiloderma, and typical periungual changes (Figure 1), and accompanied by proximal muscle weakness and elevated muscle-derived enzyme levels. A skin biopsy confirmedthe diagnosis by demonstrating the characteristic interface dermatitis with mucin deposition. Treatment at diagnosis consisted of various regimens oflow-dose corticosteroids, immunosuppressive drugs, and topical treatments. However, her skin was never without signs of dermatomyositis and her weaknessonly occasionally abated. Figure 1. View LargeDownload Photodistributed poikiloderma at initial consultative visit. In 1999, she was first sent for evaluation to 1 of us (J.P.C.). It was recommended that she use chloroquine phosphate, methotrexate, and, if responsewas inadequate, oral mycophenolate mofetil and/or intravenous immunoglobulin. Only slight improvement was noted and she became inconsistent with her treatmentsecondary to side effects. In 2002, she was again sent for an evaluation because of the development of multiple subcutaneous firm nodules. Physical examination revealed rock-hard subcutaneous nodules and plaques of the postaxillary, inframammary skin as well as the lateral part of thechest wall and arms. She was weak in the proximal muscles of both her upper and lower extremities. A 5-mm punch biopsy specimen taken from a nodule revealeda mixed lobular and septal calcifying panniculitis (Figure 2). Laboratory evaluation showed an elevated creatine kinase level of 300 U/L (normal, <179 U/L). Figure 2. View LargeDownload Subcutaneous tissue with sclerosis and calcification (hematoxylin-eosin, ×40). Recommendations at this time included administration of oral diltiazem and more aggressive management of dermatomyositis including higher doses oforal prednisone, monthly administration of intravenous immunoglobulin, and reintroduction of an immunosuppressive medication. Comment. Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous findings including the pathognomonic heliotrope rash.2 Itcan further be complicated by calcinosis, which is seen with a much higher frequency in children compared with adults.1,2 Calcinosis seen in connective tissue diseases is mostly of the dystrophic type and is believed to be a localized process rather than an imbalance ofcalcium homeostasis.3 One possible mechanism is the release of calcium from mitochondria in muscle cells damaged by themyopathy.3 Risk factors for the development of calcinosis other than young age include the presence of the tumor necrosisfactor α–308A allele, delay in diagnosis of dermatomyositis, and inconsistent and/or low-efficacy therapy among others.1,4,5 Onestudy found that patients with higher muscle-derived enzyme levels had a lower incidence of calcinosis, probably due to the more aggressive treatment regimenneeded for them.5 It has also been shown that a low socioeconomic status results in a delay in and less aggressiveapproach to therapy. This may have been a factor for our patient, although she was insured by a state program, she often failed to return for follow-upvisits to the physicians primarily in charge of her care. In addition, she may have been "undertreated" because of the low-grade activity of her disease. There are no controlled studies of the treatment of established calcinosis. However, the following therapies have been reported to be of benefit: bisphosphonates,calcium channel blockers, probenecid, warfarin, and aluminum hydroxide.1,3 In conclusion, our patient represents a rare complication of adult-onset dermatomyositis. It is possible that hadshe received early and aggressive and sustained therapy of the dermatomyositis, the calcinosis might have been prevented. The authors have no relevant financial interest in this article. References 1. Mukamel MHorev GMimouni M New insight into calcinosis of juvenile dermatomyositis: a study of composition and treatment J Pediatr. 2001;138763- 766PubMedGoogle ScholarCrossref 2. Callen JP Dermatomyositis Lancet. 2000;35553- 57PubMedGoogle ScholarCrossref 3. Vinen CSPatel SBruckner FE Regression of calcinosis associated with adult dermatomyositis following diltiazem therapy Rheumatology. 2000;39333- 334PubMedGoogle ScholarCrossref 4. Pachman LMLiotta-Davis MRHong DK et al. TNFα-308A allele in juvenile dermatomyositis: association with increased production of tumor necrosis factor α, disease duration, andpathologic calcifications Arthritis Rheum. 2000;432368- 2377PubMedGoogle ScholarCrossref 5. Fisler RELiang MGFuhlbrigge RC et al. Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis J Am Acad Dermatol. 2002;47505- 511PubMedGoogle ScholarCrossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Mar 1, 2004

Keywords: dermatomyositis,calcinosis cutis

References