2011
DOI: 10.4314/pamj.v6i1.69070
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Systemic sclerosis in a patient with pityriasis rubra pilaris

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 … Show more

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Cited by 4 publications
(2 citation statements)
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“…Radiographic evidence of acro-osteolysis is frequently described although only two cases of erosive arthritis have been reported [53,59]. Other rheumatological associations with PRP include myositis (specifically dermatomyositis and Wong-type dermatomyositis, with skin lesions characterized by the grouped keratotic follicular papules of PRP) [63,64], autoimmune thyroiditis [60], celiac sprue [65], systemic sclerosis [66] and myasthenia gravis [67].…”
Section: Pityriasis Rubra Pilarismentioning
confidence: 99%
“…Radiographic evidence of acro-osteolysis is frequently described although only two cases of erosive arthritis have been reported [53,59]. Other rheumatological associations with PRP include myositis (specifically dermatomyositis and Wong-type dermatomyositis, with skin lesions characterized by the grouped keratotic follicular papules of PRP) [63,64], autoimmune thyroiditis [60], celiac sprue [65], systemic sclerosis [66] and myasthenia gravis [67].…”
Section: Pityriasis Rubra Pilarismentioning
confidence: 99%
“…2 Immune dysregulation may play a role in the pathogenesis of PRP where there is an abnormal response to antigenic triggers, lymphocyte hypersensitivity to superantigens and T-lymphocyte abnormalities. 4 In addition, given the Small T-antigen of the TS-associated polyomavirus activates factors implicated in the MAPK pathway Editor Trichodysplasia Spinulosa (TS) is a rare, disfiguring skin condition that affects immunocompromised patients. The disease is characterized clinically by a papular eruption that can progress to leonine faces or alopecia and histologically by a proliferation of nucleated granular cells and dysplasia of inner root sheath cells.…”
Section: Pityriasis Rubra Pilaris-like Eruption With Dermatomyositis mentioning
confidence: 99%