An unusual presentation of dermatomyositis: The type Wong variant revisited

• Published in: Journal of the American Academy of Dermatology Vol. 43; no. 5; pp. 908 - 912
• Main Authors: Lupton, Jason R., Figueroa, Patricio, Berberian, Brenda J., Sulica, Virginia I.
• Format: Journal Article
• Language: English
• Published: New York, NY Mosby, Inc 01-11-2000; Elsevier
• Subjects: Biological and medical sciences; Dermatomyositis - complications; Dermatomyositis - pathology; Female; Humans; Medical sciences; Middle Aged; Pityriasis Rubra Pilaris - pathology; Porokeratosis - complications; Porokeratosis - pathology; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Skin - pathology; Case study; Human; Immunopathology; Connective tissue disease; Striated muscle disease; Skin disease; Systemic disease; Dermatomyositis; Clinical form; Autoimmune disease
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1067/mjd.2000.102648

We describe a 53-year-old white woman with dermatomyositis (DM) who had additional clinical findings of pityriasis rubra pilaris (type Wong dermatomyositis) with histopathologic features of both pityriasis rubra pilaris (PRP) and porokeratosis. Type Wong dermatomyositis was originally described in 11 patients by Wong in 1969 and has been reported in 5 additional patients. This is a rarely described phenomenon in which patients with DM develop cutaneous hyperkeratotic lesions that resemble PRP and histologically show follicular hyperkeratosis and hair follicle destruction. Arrector pilorum muscles 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 clinical and histologic features suggestive of porokeratosis. This is important because of the wassociation of adult-onset dermatomyositis with internal malignancy and the well-documented association of porokeratosis with immunosuppression. These clinical and histologic findings serve as markers for malignancy in patients with DM. These patients warrant a complete review of systems and investigation for age-appropriate neoplasms as well as close long-term follow-up by dermatologists to ensure that these cutaneous eruptions are not overlooked. (J Am Acad Dermatol 2000;43:908-12.)