A peculiar clinical spectrum, associated with anti-NXP2 antibodies, characterized by DM rash, in particular Gottron’s papules, heliotropic rash, peripheral oedema and periungual telangiectasias [2, 6, 8, 10–12], dysphagia and calcinosis has been described [6, 10, 11, 33–35]. The gene discussed is MORC3; the disease is dermatomyositis.