Response to “two cases of ulcerative dermatomyositis with negative antimelanoma differentiation-associated gene 5 antibody”

Abstract

Full text

To the Editor: We read with great interest the letter by Choi et al, describing 2 additional cases of ulcerative dermatomyositis (DM) in the absence of anti-melanoma differentiation-associated gene 5 (MDA)-5 antibodies. Their findings build upon our previously published case of treatment-refractory, anti-MDA-5-negative DM with recurrent cutaneous ulcerations, which ultimately responded favorably to mycophenolate mofetil.1

Cutaneous ulceration is an uncommon but significant feature of DM, affecting approximately 3% to 19% of patients.2 These ulcers are often painful, debilitating, and challenging to manage, contributing to substantial morbidity.2^,^3 Anti-MDA-5 antibodies are strongly associated with ulceration in DM, and this combination is linked to an increased risk of rapidly progressive interstitial lung disease (ILD).2 The systemic vasculopathy observed in anti-MDA-5-positive DM has been proposed as a key factor in the connection between cutaneous ulcers and ILD, and skin biopsies from ulcerated lesions in these patients frequently reveal vasculopathic changes.2^,^3

Ulceration in DM without anti-MDA-5 antibodies, as observed in our case and the 2 reported by Choi et al, is less common and remains poorly defined. Unlike the vasculopathic findings typically seen in anti-MDA-5-positive DM, skin biopsies from our patients revealed subtle interface dermatitis compatible with a manifestation of collagen vascular disease. Cases of ulcerative DM without MDA-5 antibodies identified in the literature appear to support a similar clinical profile marked by refractory ulcerations and the absence of vasculopathy on biopsy (Table I). Among these patients, the occurrence of ILD, malignancy, and other serologic markers was more variable.Table IClinical and serologic features of ulcerative dermatomyositis in patients without anti-MDA-5 antibodiesAuthor(s)Tomb et al, 20024Al Awqati et al, 20165Ozer et al, 20241De Jesus et al, 20246Wald et al, 2025Case 17Wald et al, 2025Case 27Choi et al, 2025Case 1Choi et al, 2025Case 2Demographics34 F60 F53 F52 F33 F67 F37 M84 MVasculopathy on biopsyNo vasculopathy on biopsiesNot specifiedNo vasculopathy on biopsiesNot specifiedNot specifiedNot specifiedNo vasculopathy on biopsiesNo vasculopathy on biopsiesMuscle involvementTypical with myositis?Myositis present with EMGClinically amyopathic with elevated CK levelsClinically amyopathic but fulfill myositis criteriaMyositis with elevated CKFluctuant CKNormal CKNormal CKPresence of ILDUnknownNoNoYesNoNoYesNoAnti-MDA-5UnknownNegativeNegativeNegativeNegativeNegativeNegativeNegativeANAUnknownN/APositivePositiveNegativePositiveNegativeNegativeAnti-RO52UnknownN/APositiveNegativeN/AN/APositiveN/AAntibeta-2 glycoproteinUnknownN/APositiveN/AN/AN/AN/AN/AAnti-MI-2 antibodiesUnknownN/ANegativeN/AN/AN/AN/AN/ATIF1-yUnknownPositiveNegativeNegativeN/APositiveN/AN/AAnti-NXP2 (nuclear matrix protein 2)UnknownN/ANegativeNegativePositiveN/AN/AN/AAnti-JoUnknownN/ANegativeN/AN/AN/AN/AN/AMalignancyUnknownOvarian cancerNoNoNoBronchial carcinomaNoNoRefractory toRefractory oral steroidsN/AIV pulse steroids and IM methotrexateOral steroidsN/AN/AN/AN/AResponsive toRefractory to oral steroids and azathioprineIVIG, high-dose pulse corticosteroidsMycophenolate mofetilIV pulse steroidsIVIG, methylprednisolone pulse therapies, azathioprineIntravenous iloprost and bosentan, IVIG, oral steroidsN/AN/AANA, Anti-nuclear antibody; CK, creatinin kinase; EMG, electromyography; F, female; ILD, interstitial lung disease; IM, intramuscular; IVIG, intravenous immunoglobulin; IV, intravenous; M, male; MDA-5, melanoma differentiation-associated gene 5 antibody; N/A, not applicable.

Further research is needed to determine whether this subtype of DM has distinct pathogenic and prognostic implications. We appreciate the contributions of Choi et al8 in expanding the discussion on this less common presentation of DM and support continued investigation into its underlying mechanisms and optimal management.

Conflicts of interest

None disclosed.