Wong-Type Dermatomyositis Showing Porokeratosis-Like Changes (Columnar Dyskeratosis): A Case Report and Review of the Literature

Background: Wong-type dermatomyositis (DM) exhibits simultaneous pityriasis rubra pilaris (PRP) features. Case Report: A 50-year-old woman presented with a heliotrope rash, Gottron's papules, and a poikilodermic, erythematous rash in shawl distribution without evidence of muscle weakness. Despite topical corticosteroids, the eruption progressed 9 months later to include generalized hyperkeratotic follicular papules, islands of sparing, and atrophic macules with a collarette of scale suggestive of porokeratosis. Mild dysphonia was the only sign of muscle weakness. Serology showed positive ANA. Histopathology revealed interface dermatitis with dermal mucin and melanophages, irregular psoriasiform hyperplasia, alternating mounds of para- and orthokeratosis, and tiers of dyskeratotic cells (columnar dyskeratosis). Systemic corticosteroid therapy was not tolerated; acitretin diminished the hyperkeratosis. While hyperpigmentation persisted, no progression of cutaneous or muscular symptoms has occurred after 22 months of follow-up and cessation of the therapy. Overall, her course did not differ from the natural history documented in the literature review of Wong-type DM. The most similar case also exhibited pseudocornoid lamella changes. Conclusion: Wong-type DM is a clinicopathologic DM-PRP hybrid that can also exhibit porokeratosis-like features best described as columnar dyskeratosis. Recognizing these types of lesions in DM is warranted in order to make an accurate assessment of their prognostic significance.