Avery H. LaChance

We describe radiological and symptomatic improvement in two patients with dermatomyositis with recalcitrant muscle disease following off-label treatment with anifrolumab, a monoclonal antibody targeting type I interferon receptor recently approved by the FDA and EMA for the treatment of systemic lupus erythematosus.

Dear Editor, Acquired cutis laxa (ACL) is a rare dermatological condition with numerous proposed aetiologies including medications, malignancy, infection, enzyme disorders, autoimmunity and renal disease, and although the pathogenesis of inherited CL is somewhat understood, little is known about ACL.¹ ACL is presumed to be a sequela of environmental exposures leading to elastic fibre destruction.² ACL typically presents in adulthood, and diagnosis is based on history and clinical examination followed by histopathology revealing reduction in elastic fibres or elastic fibre fragmentation.^2,3 Patients have localized or generalized wrinkled skin, and disease is either insidious or associated with preceding inflammation.^1,2 This study aimed to better understand potential presentations and triggers of ACL. Following approval by the Institutional Review Board at Mass General Brigham (MGB), patients diagnosed with CL from January 1989 to April 2021 at MGB hospitals were identified using the Research Patient Data Registry. Inherited CL was excluded. Data were then extracted from medical records.

Eosinophilic fasciitis (EF) is a fibrosing disorder of the fascia characterized by symmetric erythema, edema, and induration of the extremities. The largest known EF cohort previously published found that 16% of patients have monoclonal gammopathy (MG).¹ Further characterization of EF-associated MG is limited to the case report level.^2-4 This represents a knowledge gap compared with other MG-associated dermatologic conditions, such as scleromyxedema and scleredema, which are associated with immunoglobulin (Ig) G–λ and IgG-κ MG, respectively. This study sought to systematically analyze the characteristics and outcomes of EF-associated MG. Identify all potential conflicts of interest that might be relevant to your comment. Conflicts of interest comprise financial interests, activities, and relationships within the past 3 years including but not limited to employment, affiliation, grants or funding, consultancies, honoraria or payment, speaker's bureaus, stock ownership or options, expert testimony, royalties, donation of medical equipment, or patents planned, pending, or issued. Err on the side of full disclosure. If you have no conflicts of interest, check "No potential conflicts of interest" in the box below. The information will be posted with your response. Not all submitted comments are published. Please see our commenting policy for details.