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Dermatomyositis pp 125–133Cite as

Classic Dermatomyositis in Adults

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“Classic” DM in adults has various clinical and pathological features, which do not always appear simultaneously or with the same severity. Dermatomyositis is identified by a characteristic rash, which appeared simultaneously or, more commonly, preceded muscle weakness. In addition to manifesting clinical and laboratory evidence of myositis, adult patients with classic DM develop the hallmark cutaneous findings. Cutaneous manifestations of DM could be classified as photosensitive, hyperkeratotic, and vascular (see Table 6.2). Photosensitive lesions consist of heliotrope rash, a periorbital, dusky, violaceous erythema of one or both eyelids. Heliotrope rash could be a component of a more confluent erythema involving the entire face in many cases associated with edema, and erythematous exanthe-mas on discrete areas of the body: the neck, and anterior chest (in a V-sign) or the nape of the neck and the posterior aspect of the shoulders (shawl-sign), knees, elbows, and malleoli [1–3]. An erythematous rash may also be found on the face in a limited malar distribution, or more extensively with perioral sparing [4]. This erythema can extend to the ears and scalp [5]. The lesions are pruritic, and can be exacerbated after exposure to the sunlight. Gottron rash is a characteristic feature, with violaceous to dusky, red, flat-topped papules and plaques prominent on dorsal interphalangeal joints, elbows, and knees and, rarely, the malleoli. These papules evolve over time to have depressed, atrophic, porcelain white centers and prominent telangiectasias known as Gottron's sign over bony prominences.

A rare subgroup of patients have follicular hyperkeratosis, which may occur as a pit-yriasis rubra pilaris-like eruption (Wong-type DM). The lateral and palmar areas of the fingers may become rough with cracked, “dirty” horizontal lines, resembling “mechanic's hands”, and dilated capillary loops with punctate infarcts at the base of the fingernails with irregular, thickened, and distorted cuticles could be prominent. Scalp involvement is frequently evident as a diffuse, erythematous, scaly, atrophic dermatosis with mild-to-moderate alopecia. Cutaneous vasculitis is seen as palpable purpura and digital or oral ulcerations. Panniculitis may be a characteristic component of classic DM in adults.

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(2009). Classic Dermatomyositis in Adults. In: Dermatomyositis. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-79313-7_19

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  • DOI: https://doi.org/10.1007/978-3-540-79313-7_19

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