1/10. 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.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
2/10. Inflammatory myopathy in a patient with cutaneous findings of pityriasis rubra pilaris: a case of Wong's dermatomyositis.We report the case of a 46-year-old woman presenting cutaneous lesions similar to those of pityriasis rubra pilaris, characterized by diffuse scaling erythroderma and palmoplantar keratoderma; skin biopsy showed follicular hyperkeratosis with a perivascular lymphocytic infiltrate. One year later she developed an inflammatory myopathy; on the basis of clinical features, a diagnosis of dermatomyositis was made. Treatment with prednisone and hydroxychloroquine led to complete control of the cutaneous and muscular involvement, which was maintained during prednisone tapering.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
3/10. Circumscribed juvenile pityriasis rubra pilaris.We report the case of a 20-year-old woman with a 10-year history of circumscribed juvenile-onset pityriasis rubra pilaris (PRP, type IV). Our patient had well-defined keratotic follicular papules on an erythematous base located on the extensor aspects of the extremities and dorsal aspects of the feet but no involvement of the palms and soles. Although most cases of type IV PRP follow a favourable course with spontaneous resolution of the lesions, this case demonstrates that circumscribed juvenile PRP can be more persistent and lasts several years.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
4/10. pityriasis rubra pilaris, type IV.A 4-year-old girl presented with a 3-year history of demarcated, salmon-pink, hyperkeratotic plaques, which were symmetrically distributed on the elbows, knees, ankles, and dorsal aspects of the hands and feet. A diffuse, orange-pink palmoplantar keratoderma was also evident. Clinical and histologic findings were consistent with a diagnosis of pityriasis rubra pilaris (PRP), type IV (circumscribed juvenile). Type IV PRP develops in prepubertal children, is typically localized to the distal aspects of the extremities, and has an unpredictable course. Although ultraviolet (UV) radiation can potentially exacerbate PRP, our patient has improved with broad-band UVB phototherapy.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
5/10. Successful treatment of type I adult-onset pityriasis rubra pilaris with infliximab.A 59-year-old woman presented with a painful, pruritic eruption that had commenced as an erythematous, dry patch on the upper back but progressed to erythroderma. Examination revealed orange-tinged erythroderma, scalp scaling, ectropion, palmoplantar keratoderma and nail changes. A diagnosis of type I adult-onset pityriasis rubra pilaris was made, and a subsequent skin biopsy was consistent with this. She was treated with a number of topical and systemic agents with minimal improvement or major side-effects. The patient was then treated with intravenous infliximab 5 mg/kg. She improved dramatically within 2 weeks and was no longer erythrodermic. Five further infusions resulted in additional improvement. methotrexate was briefly added to the regime, but was ceased owing to nausea. Topical tar and keratolytics were used on the scalp. The patient was left with minimal disease activity and was maintained on emollients.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
6/10. Childhood-onset pityriasis rubra pilaris with immunologic abnormalities.We examined a 6-year-old boy with seborrheic dermatitis like redness of the face, scaling of the scalp, and palmoplantar keratoderma. skin biopsy showed hyperkeratosis, parakeratosis, follicular plugging, and mild upper dermal perifollicular mononuclear inflammatory infiltrate, which are compatible with the diagnosis of pityriasis rubra pilaris. Immunologic studies revealed normal values of peripheral B and T lymphocytes. The spontaneous activity of T suppressor cells was activated and the function of T helper cells was impaired, however.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
7/10. pityriasis rubra pilaris and celiac sprue with malabsorption.A case is reported herein of a young man with pityriasis rubra pilaris, a disease of unknown cause, and malabsorption due to celiac bowel disease. The patient responded to high-dose vitamin a therapy (500,000 IU orally per day for fourteen days). There was complete exfoliation of the palmar-plantar keratoderma and a decrease in the peeling and follicular keratosis. He was then placed on a regimen of a gluten-free diet and 100,000 IU vitamin a per day. After seven months he had persistent erythema but minimal hyperkeratosis. The ways in which cutaneous lesions and malabsorption could be related are discussed.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
8/10. Changing forms of juvenile pityriasis rubra pilaris--a case report.pityriasis rubra pilaris (PRP) is a rare disease affecting both males and females. The aetiology is unknown, but it has an ill-defined relationship with psoriasis. Within the spectrum of PRP certain disease patterns are recognized, and regarded by many as helpful prognostic indicators. Griffiths has suggested a clinically based classification based on a series of 98 patients seen at St John's Hospital, london between 1950 and 1972 (Table 1). Classical type 1 PRP is an erythematous squamous disorder typically showing follicular hyperkeratosis, perifollicular erythema and sharply demarcated islands of unaffected skin. The palms and soles become hyperkeratotic and often exhibit a characteristic orange hue. Type III PRP is the juvenile counterpart of classical type I PRP. The following case report describes a patient who presented with type III PRP but later went on to develop the type IV or circumscribed, juvenile onset PRP.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
9/10. dermatomyositis with a pityriasis rubra pilaris-like eruption: a little-known distinctive cutaneous manifestation of dermatomyositis.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.- - - - - - - - - - ranking = 2keywords = palm (Clic here for more details about this article) |
10/10. pityriasis rubra pilaris: an unusual cutaneous complication of AIDS.pityriasis rubra pilaris is an uncommon hyperkeratotic, papulosquamous disorder that has been reported in patients infected by hiv. We recount a case of pityriasis rubra pilaris in an hiv-seropositive man. A 36-year-old man with a history of ulcerative colitis and recurrent otitis externa had diffuse psoriaform erythroderma. He was treated initially with methotrexate and isoretinoin without clinical improvement. skin examination showed large, erythematous, orange, scaly patches on the upper extremities and thickening of the nail beds. The palms and soles were hyperkeratotic. skin biopsy revealed changes that were consistent with pityriasis rubra pilaris. Six months before the onset of symptoms, results of an enzyme-linked immunosorbent assay (ELISA) and Western Blot assay for hiv were negative. Six months after symptoms, results of repeat enzyme-linked immunosorbent assay and Western blots for hiv were positive (CD4 T-cell count = 200 cells/ mm3). Clinical course had been complicated by episodes of staphylococcus aureus bacteremia, mucocutaneous candidiasis, and development of localized squamous cell carcinoma of the skin. The increased severity of pityriasis rubra pilaris should prompt clinicians to consider coinfection with hiv in patients who have disease that is refractory to treatment. Clinicians also should remain vigilant for the development of squamous cell carcinoma.- - - - - - - - - - ranking = 1keywords = palm (Clic here for more details about this article) |
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