Photoessay of the cutaneous manifestations of the idiopathic inflammatory myopathies.

Adult and juvenile dermatomyositis, polymyositis and myositis overlapping with another connective tissue disease are rare systemic autoimmune diseases with a primary feature of weakness and muscle inflammation. Cutaneous findings specific to the underlying condition are present in many patients with these disorders. Some lesions are highly characteristic of the idiopathic inflammatory myopathies (IIM), especially in dermatomyositis. Some cutaneous findings are common but not specific to the IIM and others are less frequently observed in patients with these illnesses. Many of these manifestations also have different grades of disease activity or damage. This photoessay reviews the classification and assessment of the cutaneous manifestations of the IIM and presents example photographs of many of the lesions of adult and juvenile IIM accumulated from the clinical experience of international experts in these conditions. The purpose of this work is to facilitate better recognition of the diverse cutaneous manifestations associated with these inflammatory myopathies.

Review of the classification and assessment of the cutaneous manifestations of the idiopathic inflammatory myopathies.

Adult and juvenile dermatomyositis, polymyositis and myositis overlapping with another connective tissue disease are rare systemic autoimmune diseases with a primary feature of weakness and muscle inflammation. Cutaneous findings specific to the underlying condition are present in many patients with these disorders. Some lesions are highly characteristic of the idiopathic inflammatory myopathies (IIM), especially in dermatomyositis. Some cutaneous findings are common but not specific to the IIM and others are less frequently observed in patients with these illnesses. Many of these manifestations also have different grades of disease activity or damage. This photoessay reviews the classification and assessment of the cutaneous manifestations of the IIM and presents example photographs of many of the lesions of adult and juvenile IIM accumulated from the clinical experience of international experts in these conditions. The purpose of this work is to facilitate better recognition of the diverse cutaneous manifestations associated with these inflammatory myopathies.

Alternative scoring of the Cutaneous Assessment Tool in juvenile dermatomyositis: results using abbreviated formats.

OBJECTIVE: The Cutaneous Assessment Tool (CAT) is a comprehensive, semiquantitative tool for the assessment of skin disease in juvenile dermatomyositis (DM). The goal of this study was to determine whether alternative scoring methods would shorten the CAT without compromising its measurement characteristics. METHODS: A total of 113 children with juvenile DM were assessed at baseline; 94 were assessed again 7-9 months later. Interrater reliability, internal consistency, construct validity, and responsiveness were obtained using the original scoring method and 2 alternative methods: the maximum and binary scoring methods. RESULTS: Spearman's correlations of the maximum and binary methods with the original were both 0.98 (P < 0.0001) for the CAT activity score, and 0.96 and 0.98, respectively (P < 0.0001), for the CAT damage score. Values obtained for interrater reliability, internal consistency, construct validity, and responsiveness were similar for all 3 scoring methods. Although there was a trend toward the maximum method having higher interrater reliability and the binary method having higher responsiveness, the confidence intervals were overlapping and no statistically significant differences were observed. Correlation coefficients for the 3 scoring methods with other measures of myositis disease activity and damage were very similar. CONCLUSION: The maximum and binary methods of scoring the CAT have measurement characteristics similar to the original method. Adoption of one of these abbreviated scoring methods should increase its acceptability to clinicians and researchers.

Preliminary validation and clinical meaning of the Cutaneous Assessment Tool in juvenile dermatomyositis.

OBJECTIVE: To provide preliminary validation of the Cutaneous Assessment Tool (CAT), a new tool to assess cutaneous manifestations of juvenile dermatomyositis (DM), and to explore the clinical meaning of CAT scores. METHODS: Children with juvenile DM (n = 113) were assessed at baseline and 7-9 months later (n = 94). Internal consistency, redundancy, construct validity, and responsiveness of the CAT were examined. CAT scores corresponding to ordinal global assessments were determined. RESULTS: Item-total correlations ranged from 0.27-0.67 for activity lesions present in > or =10% of patients; item-domain and domain-total correlations ranged from 0.25-0.99. Cronbach's alpha was 0.79 for the CAT activity score and 0.74 for the CAT damage score. As predicted, the CAT activity score correlated strongly with both global disease activity and skin disease activity and moderately with the Childhood Myositis Assessment Scale, whereas the CAT damage score correlated moderately with the physician global disease and skin disease damage scores. Median CAT activity scores of 1, 7, 13, 18, and 31 corresponded to absent, mild, moderate, severe, and extremely severe skin disease activity, respectively. Median CAT damage scores of 0, 1, 2, and 5 correlated with the same descriptions of damage (severe and extremely severe combined). CONCLUSION: Preliminary validation of the CAT demonstrated good internal consistency, nonredundancy, good construct validity, and appropriate responsiveness. The CAT is a comprehensive, semiquantitative assessment tool for skin disease in juvenile DM.

Skin involvement in dermatomyositis.

PURPOSE OF REVIEW: Dermatomyositis (DM) is a rare multisystem autoimmune disorder of adults and children that primarily affects skin and skeletal muscle. Classification systems of dermatomyositis, polymyositis, and the other idiopathic inflammatory myopathies focus primarily on features of muscle involvement. However, cutaneous disease does not always parallel muscle disease in its onset, activity, or response to therapy. This review will describe the distinct cutaneous clinical and histopathologic presentation of DM and the relation between these cutaneous findings, pathogenesis of DM, and serological subsets of DM. RECENT FINDINGS: This review discusses recent findings that have begun to elucidate the pathogenesis of DM, including polymorphism of tumor necrosis factor-alpha 308A allele and maternal fetal microchimerism. The recent description of other systemic diseases and drugs causing DM-like eruptions and the recognition that DM can resemble other common dermatoses highlights the need for a cutaneous biopsy to diagnose and distinguish the cutaneous features of DM. Once diagnosed, a number of noninvasive imaging modalities and new cutaneous assessment instruments can be used to follow and evaluate patients with DM. SUMMARY: Recognition of cutaneous and histopathologic findings in DM is essential for prompt and accurate diagnosis and treatment of DM.

Primary cutaneous fungal infections in solid organ transplantation: a case series.

Cutaneous fungal infections in solid-organ transplant patients present in a variety of nonspecific ways, requiring a high index of suspicion to diagnose correctly. In the present series of four transplant recipients, subsequent primary cutaneous fungal infections presented as papules, plaques, ulcers and subcutaneous nodules. Transplantations included one cardiac, two renal and one renal-pancreatic transplant. Fungal infections were limited to the skin; there was no evidence of disseminated disease in any case. The pathogens isolated were Scedosporium apiospermum (Pseudallescheria boydii), Alternaria species, Aspergillus fumigatus, and a coelomycete in the Coniothyrium-Microsphaeropsis complex of dark molds. Individuals were successfully treated with surgical debridement, antifungal agents, and reduction of immunosuppressive therapy. All patients and allografts survived. Accurate diagnosis, aggressive surgery and appropriate antifungal therapy, combined with close outpatient follow-up, optimize the likelihood of a cure in a transplant population.