Wong‐type dermatomyositis

A previously healthy 27‐year‐old woman presented to the dermatology department following a 3‐month history of an itchy erythema initially involving the face and which later progressed to her trunk and upper limbs. Physical examination revealed multiple hyperkeratotic follicular papules on the hands (Figure A). Laboratory examination revealed elevated levels of creatine kinase (487 U/L; reference interval, 40–200 U/L). Autoimmune antibody tests were negative. Electromyography revealed a myogenic pattern, and a myositis panel was positive for transcription intermediary factor 1‐γ (TIF1‐γ) antibody. The histopathological features revealed follicular hyperkeratosis and superficial lymphocytic perivascular and vacuolar interface alterations (Figure B [haematoxylin‐eosin stain, original magnification ×100]). A series of comprehensive malignancy workup was negative. The patient was diagnosed with Wong‐type dermatomyositis, a rare variant of dermatomyositis, characterised by pinkish, hyperkeratotic follicular papules with intervening areas of unaffected skin known as “islands of sparing”.1 To our knowledge, only 30 cases have been reported in the literature.2 The patient was successfully treated with systemic methylprednisolone (initially 40mg per day, subsequently transitioned to 10mg per day) and tacrolimus (initially 0.5mg twice a day, subsequently transitioned to 1mg twice a day) (Figure C).