What is refractory dermatomyositis?
Patients will be considered to have refractory disease if cutaneous manifestations exist despite treatment with steroids and at least one steroid-sparing systemic treatment commonly found to be useful in patients with dermatomyositis.
What is cutaneous dermatomyositis?
Dermatomyositis (dur-muh-toe-my-uh-SY-tis) is an uncommon inflammatory disease marked by muscle weakness and a distinctive skin rash. The condition can affect adults and children. In adults, dermatomyositis usually occurs in the late 40s to early 60s. In children, it most often appears between 5 and 15 years of age.
What are hypertrophic lesions?
Hypertrophic or verrucous DLE (hypertrophic LE) is a unique subset, in which unusual lesions occur. The thick, adherent scale is replaced by massive hyperkeratosis, and the lesions look like warts or squamous cell carcinomas. It was also described as verrucous lupus erythematosus or keratotic lupus erythematosus.
What is the hallmark skin lesion of SLE?
Acute cutaneous lupus erythematosus (ACLE) may be localized or generalized. The hallmark ACLE lesion is the malar rash or the butterfly rash, which is an erythematous raised pruritic rash involving the cheeks and nasal bridge. The rash may be macular or papular and spares the nasolabial folds (photoprotected).
What is refractory myositis?
Refractory myositis was defined as an inadequate response to at least two immunosuppressant/immunomodulatory drugs (cyclophophamide, azathioprine, methotrexate, cyclosporine, leflunomide, mycophenolate mofetil and/or intravenous human immunoglobulin, in their full-dose, for a minimum period of 3 months) given …
How does IVIg help myositis?
There are several theories on how the IVIg works in myositis patients, such as it acts as immunomodulatory drug/an immune booster, reduces the production of autoantibodies, works through complement fixation, neutralizes the assailant autoantibodies, or autoantigens, causes cytokines suppression or blockage [4].
What is heliotrope rash?
What is heliotrope rash? Heliotrope rash is caused by dermatomyositis (DM), a rare connective tissue disease. People with this disease have a violet or bluish-purple rash that develops on areas of the skin. They can also experience muscle weakness, fever, and joint aches.
What is lupus erythematosus Tumidus?
Lupus erythematosus tumidus (LET) is a photosensitive skin disease characterized by succulent, edematous, and non-scarring plaques. Histologic features include perivascular and periadnexal lymphocytic infiltration and interstitial mucin deposition.