Dermatomyositis with a pityriasis rubra pilaris-like eruption: a little-known distinctive cutaneous manifestation of dermatomyositis

Abstract

A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. These patients showed generalized follicular hyperkeratosis and diffuse thickening of the palms and soles. Histopathological findings consisted of keratotic plugging of the follicular infundibulum and features of erector pili myositis. We report on an 18-year-old woman with dermatomyositis. The diagnosis was established by characteristic enzymatic alterations, electromyographic pattern of myositis and the findings in a muscle biopsy, although the patient had no evidence of muscular weakness during a follow-up of 14 years. She developed an erythematosus and squamous eruption associated with diffuse palmoplantar keratoderma. Histopathological features consisted of a papillomatous epidermis with spicules of compact eosinophilic hyperkeratosis over the tips of papillae that were not related to hair follicles. Pityriasis rubra pilaris-like eruption seems to be a characteristic although uncommon cutaneous manifestation in dermatomyositis.

Papules and Pustules of the Elbows and Knees: An Uncommon Clinical Sign of Dermatomyositis in Oriental Children

“…He also had a symmetrical, hyperkeratotic, follicular eruption on his knees ( Fig. 2) that appeared almost identical to that reported by Requena et al (2). The differential diagnosis at this stage included juvenile circumscribed pityriasis rubra pilaris, follicular psoriasis, and papular eczema.…”

Palmoplantar Hyperkeratosis: A Rare Cutaneous Manifestation of Juvenile Dermatomyositis

“…Wong-type dermatomyositis (4) is present in a small group of patients with dermatomyositis who present with pityriasis rubra pilaris (PRP)-like eruptions, including diffuse palmoplantar hyperkeratosis and follicular hyperkeratosis. A literature review revealed six cases of JDM preceded by PRP-like eruptions (5-9), three of which (5,6,9), as in our case, had predominantly palmoplantar changes. We therefore wish to alert clinicians to this rare cutaneous manifestation of JDM, particularly because it may be the first sign of this rare and serious condition.…”

Seborrheic Area Erythema as a Common Skin Manifestation in Japanese Patients with Dermatomyositis

“…Some patients with follicular distribution of MVE in the face (fig. 1b) should be distinguished from cases of Wong-type DM, which is DM with a pityriasis-rubra-pilaris-like eruption [18]. They did not have hyperkeratotic papules or keratoderma on the palms and soles.…”