Skin Manifestation in Dermatomyositis
Dermatomyositis (DM) is a systemic autoimmune disorder with skin, muscle involvement, and other manifestations including polyarthritis, interstitial lung fibrosis, and, in 20–25 % of the cases, a coexistent malignancy. Muscle symptoms include proximal limb weakness, myalgia, and tenderness. In classic dermato-/polymyositis, cutaneous manifestation occurs in 30–40 % of the adult and 95 % of the juvenile cases. 4–8.2 % of the patients with DM show exclusively skin symptoms and never develop muscle involvement. Photosensitivity is an important clinical feature of DM.
Skin symptoms include a violaceous erythema, Gottron’s papules appearing over the metacarpophalangeal, interphalangeal, elbow, and knee joints. DM-related skin symptoms also include periorbital (heliotrope) edematous rash overlying of the eyelids and periorbital tissue. A diffuse flat purple-red skin violaceous erythema may also be present on the dorsal surface of the arms and fingers; over the deltoids, posterior shoulders, and neck (“shawl sign”); V area of anterior neck and upper chest; and central aspect of the face. Periungual telangiectasia also belongs to the typical DM-related skin signs. Further secondary changes of DM include scale, follicular hyperkeratosis, pigmentary changes, subepidermal bullous change, and ulceration. Poikiloderma atrophicans vasculare (poikilodermatomyositis) that is a circumscribed violaceous erythema with associated telangiectasia, hypopigmentation, hyperpigmentation, and superficial atrophy may be present in certain parts of the body including the V area of anterior neck and upper chest, back, flanks, and buttocks. Occasionally, calcinosis cutis may also appear.
The term amyopathic DM (ADM) (synonymous with “DM sine myositis”) and furthermore a closely related form of DM, “hypomyopathic DM,” (HDM) are characterized by the appearance of typical skin disease for at least 6 months without having any muscle weakness.
Cancer-associated DM is probably much more prevalent in adults than it has previously been suspected. Almost all children with DM also develop cutaneous manifestations usually before the diagnosis of myositis.
KeywordsInclusion Body Myositis Cutaneous Manifestation Membrane Attack Complex Anterior Neck Skin Symptom
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