1/63. hiv-associated pityriasis rubra pilaris responsive to triple antiretroviral therapy. hiv-associated pityriasis rubra pilaris (PRP) or PRP type VI designates a new distinctive entity reported in hiv patients. It is characterized by cutaneous lesions of PRP and variable association with lesions of acne conglobata, hidradenitis suppurativa and lichen spinulosus. We report a patient with hiv-associated PRP which was treated by triple antiretroviral therapy (zidovudine, lamivudin and saquinavir) with complete response. The patient has remained free from symptoms for 20 months of follow-up. We review the clinical features, pathology, evolution, treatment and possible aetiology of this recently described entity. ( info) |
| pityriasis rubra pilaris (PRP) refers to a group of erythematous, scaling dermatologic conditions that have been associated with seronegative arthritis. We report a case of polyarthritis in a young man with PRP in which magnetic resonance imaging suggested an entheseal-based pathology for the joint disease. The arthritis, but not the skin condition, demonstrated dramatic response to anti-tumor necrosis factor immunotherapy. ( info) |
| Acantholytic foci have been reported several times in pityriasis rubra pilaris (PRP). Lichenoid tissue reactions were also mentioned in the literature regarding PRP. We report a 58-year-old patient who, after having colon cancer, had PRP with biopsies showing acantholytic lesions and a heavy lichenoid lymphocytic infiltration. Investigation by serial sectioning of the acantholytic lesion suggested an involvement of the intraepidermal eccrine duct and further investigation with carcinoembryonic antigen (CEA) staining demonstrated a CEA-positive eccrine duct in the acantholytic foci. We suggest that acantholysis in PRP is induced by proteolytic enzymes, urea, and other substances in eccrine sweat in keratin-plugged acrosyringia. This patient had a combination of three relatively rare features of PRP-acantholysis, lichenoid reaction, and a cancer background. ( info) |
| Four patients with pityriasis rubra pilaris are reported. The diagnosis in each was based upon well-recognized clinical features. Two of them, a mother and son, had the disease since childhood and were marked by relative remission in spring and exacerbation in autumn. Moderate to severe pruritus was a common dominator. Erythroderma was a presenting feature in one case. Although histopathology was considered imperative, it only supplemented the clinical expression. vitamin a in heavy dosage, supplemented by vitamin e and stanozolol in tandem, was the mainstay of treatment. ( info) |
5/63. Three cases of pityriasis rubra pilaris successfully treated with cyclosporin A. BACKGROUND: pityriasis rubra pilaris (PRP) is reported to respond poorly to cyclosporin A (CsA). OBJECTIVE: We attempted to determine the efficacy of CsA in the treatment of classic adult-type PRP. methods: Three patients with classic adult-type PRP were treated with 5 mg/kg/day CsA. RESULTS: A sustained clinical response was achieved within 2-4 weeks of therapy. Relapses were noted when the CsA dose was decreased to 1.2 mg/kg/day. CONCLUSION: CsA should be considered in the treatment of classical adult-type PRP. ( info) |
6/63. An unusual presentation of dermatomyositis: the type Wong variant revisited. We describe a 53-year-old white woman with dermatomyositis (DM) who had additional clinical findings of pityriasis rubra pilaris (type Wong dermatomyositis) with histopathologic features of both pityriasis rubra pilaris (PRP) and porokeratosis. Type Wong dermatomyositis was originally described in 11 patients by Wong in 1969 and has been reported in 5 additional patients. This is a rarely described phenomenon in which patients with DM develop cutaneous hyperkeratotic lesions that resemble PRP and histologically show follicular hyperkeratosis and hair follicle destruction. Arrector pilorum muscles also show degenerative findings and myositis. We believe that this is the first reported case of a patient with type Wong DM who also has clinical and histologic features suggestive of porokeratosis. This is important because of the association of adult-onset dermatomyositis with internal malignancy and the well-documented association of porokeratosis with immunosuppression. These clinical and histologic findings serve as markers for malignancy in patients with DM. These patients warrant a complete review of systems and investigation for age-appropriate neoplasms as well as close long-term follow-up by dermatologists to ensure that these cutaneous eruptions are not overlooked. ( info) |
| Juvenile acute pityriasis rubra pilaris (JAPRP) is a form of pityriasis rubra pilaris (PRP) with particular clinical characteristics and course. It is usually preceded by an infectious condition. We report a boy, initially thought to have Kawasaki disease, and subsequently diagnosed as having JAPRP, who made a satisfactory recovery. In this case, prior antibiotic treatment made it impossible to show the presence of any microorganism. However, the clinical characteristics of these patients and the similarities with other disorders involving the skin that are mediated by superantigens led us to think that these antigens may be involved in the development of this disease. Also, in view of the obvious differences between JAPRP and the other PRP, we suggest that, in the future this disorder may be considered a separate entity as a reactive exanthem. ( info) |
8/63. Atypical pityriasis rubra pilaris associated with arthropathy and osteoporosis: a case report with 15-year follow-up. pityriasis rubra pilaris (PRP) is an idiopathic papulosquamous disease that clinically presents with palmoplantar keratoderma and follicular hyperkeratotic papules that coalesce into scaly erythematous plaques. We report a unique case of atypical PRP beginning at 1 year of age with associated severe arthropathy and osteoporosis. We further discuss the clinical and histopathologic aspects of PRP, its possible etiology, and other associated conditions. ( info) |
9/63. Merkel cell carcinoma and multiple cutaneous squamous cell carcinomas in a patient with pityriasis rubra pilaris. A 79-year-old female was diagnosed with Merkel cell carcinoma (MCC) and multiple cutaneous squamous cell carcinomas (SCC) occurring on a background of pityriasis rubra pilaris. At the time of initial diagnosis and treatment for upper limb MCC, axillary nodal metastases were clinically evident. In the ensuing months, she developed multiple rapidly progressing SCC and eventually a left arm soft tissue deposit of metastatic MCC. Treatment involved multiple courses of fractionated radiotherapy. The salient clinical features and supporting evidence for this case are presented. ( info) |
| pityriasis rubra pilaris is a chronic, papulosquamous dermatosis of unclear etiology. The case of a 61-year-old man with pityriasis rubra pilaris is presented. The clinical forms, histopathologic features, and treatment options of pityriasis rubra pilaris are reviewed. ( info) |