An unusual presentation of dermatomyositis: The type Wong variant revisited

We describe a 53-year-old white woman with dermatomyositis (DM) who had add itional clinical findings of pityriasis rubra pilaris (type Wang dermatomyo sitis) with histopathologic features of both pityriasis rubra pilaris (PRP) and porokeratosis. Type Wong dermatomyositis was originally described in 1 1 patients by Wrong in 1969 and has been reported in 5 additional patients. This is a rarely described phenomenon in which patients with DM develop cu taneous hyperkeratotic lesions that resemble PRP and histologically show fo llicular hyperkeratosis and hair follicle destruction. Arrector pilorum mus cles also show degenerative Findings and myositis. We believe that this is the first reported case of a patient with type Wong DM who also has clinica l and histologic features suggestive of porokeratosis. This is important be cause of the association of adult-onset dermatomyositis with internal malig nancy and the well-documented association of porokeratosis with immunosuppr ession. These clinical and histologic findings serve as markers for maligna ncy in patients with DM. These patients warrant a complete review of system s and investigation For age-appropriate neoplasms as well as close long-ter m follow-up by dermatologists to ensure that these cutaneous eruptions are not overlooked.