Combination therapy with methylprednisolone,rituximab,and tofacitinib in antimelanoma differentiation-associated 5 gene dermatomyositis with rapidly progressive interstitial lung disease

To the Editor,Anti-melanoma differentiation-associated gene 5(anti-MDA5)antibody-associated dermatomyositis(DM)presenting with rapidly progressive interstitial lung disease(RP-ILD)is extremely challenging to treat.MDA5 is a cytosolic viral RNA sensor that normally triggers an innate response resulting in interferon I production.Upregulation of MDA5 expression can result in autoantibody formation,leading to overactivation of the type I interferon pathway,causing aberrant metabolic remodeling of peripheral B and T cells and a profibrotic response in the affected lungs.

Bullous eruption as a cutaneous manifestation of eosinophilic granulomatosis with polyangiitis

Cutaneous manifestations are variable in patients with eosinophilic granulomatosis with polyangiitis(EGPA).We report a case where bullous eruption was a presenting manifestation of EGPA.A 55-year-old Chinese man with a background of adult-onset asthma and chronic sinusitis was admitted for a 3-week history of lower limb weakness and a large bulla on his right calf with surrounding erythema.Skin biopsy showed a subepidermal blister with significant dermal eosinophilia,without vasculitis.The patient was diagnosed with EGPA with systemic involvement on the basis of a thorough medical history,eosinophilia,cardiac magnetic resonance imaging showing acute myocarditis,and nerve biopsy with eosinophilic vasculitic neuropathy.He responded well to corticosteroids and rituximab.Nonvasculitic bullous eruptions are rarely described as manifestations of EGPA.Prompt recognition of this entity is crucial for its early diagnosis.

Flagellate erythema in transcription intermediary factor 1 gamma dermatomyositis

A 52-year-old Chinese woman was diagnosed with transcription intermediary factor 1(TIF1)gamma dermatomyositis in November 2022,with manifestations of myositis,polyarthralgia,and low-grade fever.She had cutaneous manifestations of Gottron's sign,V-neck sign,shawl sign,and flagellate erythema(Figure 1A).No interstitial lung disease was detected by high-resolution computed tomography.Comprehensive malignancy screenings,encompassing pap smear and mammogram,endoscopic examination,and computed tomography scans of the thorax,abdomen,and pelvis,were performed.They returned negative,apart from a lobulated nodule with biopsy confirmed as sclerosing pneumocytoma at the left lower lobe(Figure 1C).The therapeutic regimen,consisting of high-dose steroids and mycophenolate mofetil,effectively ameliorated the patient's myositis and rash(Figure 1B).