Abstract
Pityriasis rubra pilaris (PRP) is a rare, chronic erythematous squamous disorder of unknown etiology. It has been found in association with several autoimmune diseases, including thyroiditis, myositis, myasthenia gravis and vitiligo. Herein we report a case of systemic sclerosis in a patient with classic adult pityriasis rubra pilaris. A 38 year old woman with classic adult type 1 pityriasis rubra pilaris (PRP) developed progressive skin thickening of the trunk, face, upper and lower extremities after 2 years of PRP treatment with topical emollients and steroids. Clinical examination and immunological findings were consistent with SSc. Co-existence of these two rare conditions is documented for the first time.
Highlights
Pityriasis rubra pilaris (PRP) is a rare, chronic erythematous squamous disorder of unknown etiology
PRP has been found in association with several autoimmune diseases, including thyroiditis, myositis, myasthenia gravis and vitiligo
We report a case of systemic sclerosis in a patient with classic adult pityriasis rubra pilaris
Summary
Pityriasis rubra pilaris (PRP) is a rare, chronic erythematous squamous disorder of unknown etiology. A 38 years-old woman presented in June 2009 with a one year history of progressive widespread symmetrical cutaneous thickening of the skin of the proximal upper extremities, trunk and face, arthralgias, dyspnea on exertion and 10 kg weight loss over the previous 12 months. She had been diagnosed with type I adult-onset pityriasis rubra pilaris (PRP) at age 36 years, and had been treated with topical corticosteroids, emollients and cetirizine dichlorhydrate. Echocardiogram and electrogram were within normal limits but pulmonary function revealed moderate restrictive disease. Oral therapy with colchicine (1 mg per day) was instituted with partial improvement of skin manifestations
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