Progression of peripheral joint disease in psoriatic arthritis: a 5-yr prospective study.
OBJECTIVE: To assess the evolution of disease subgroups and the frequency of progression of peripheral joint disease in a prospectively studied cohort of patients with psoriatic arthritis (PsA). METHODS: The cohort was identified as the first consecutive 100 patients attending a psoriatic arthritis clinic and who had been the subject of a previously published cross-sectional retrospective study. Nine of the 100 patients had died, three declined follow-up and one could not be traced. The remaining 87 patients (49 females, 38 males) completed the study proforma at a median follow-up interval of 65 months (range 39-90). An analysis of initial plasma viscosity compared with rates of progression of joint score was performed. RESULTS: Eighteen patients changed subgroup; 11 had an increase in the number of joints involved, six a decrease, and one changed from an oligoarticular pattern to predominant spondylitis. Within the polyarticular group 37/51 patients had an increase in the number of joints involved. For the whole population, there were significant increases in the number of joints involved (median 6 vs 11, P < 0.001 Wilcoxon signed rank) and Health Assessment Questionnaire scores (median 0.375 vs 0.5, P < 0.001). The median rate of joint progression was 0.42 peripheral joints per year (range 0-7.2). However, the rate of peripheral joint involvement was highest in the first year of arthritis (median 4.0 joints/yr) as measured in 13 patients who had onset within 12 months of baseline assessment. There were no significant differences in skin and nail scores although nine more patients had developed nail disease. There was a significant correlation between the initial viscosity and rate of progression of joint damage (Spearman correlation, P < 0.011). CONCLUSIONS: Peripheral joint disease is progressive in the majority of patients with PsA and reinforces the need for effective monitoring and treatment. (+info)
Progressive patterned scalp hypotrichosis, with wiry hair, onycholysis, and intermittently associated cleft lip and palate: clinical and genetic distinction from Marie Unna.
Marie Unna hereditary hypotrichosis has been described in over a dozen families since 1924. Features include scant or no eyebrows at birth, the development of firm wiry hair in the first few years of life followed by a progressive patterned scalp alopecia in the second or third decade. This is associated with generalized hypotrichosis of the body and the condition is nonsyndromic. We have identified a novel form of autosomal dominant ectodermal dysplasia that resembles Marie Unna hereditary hypotrichosis in a family of 23 members over four generations. Affected individuals have patterned hair loss and associated hair shaft dystrophy similar to that seen in Marie Unna hereditary hypotrichosis. It differs from Marie Unna hereditary hypotrichosis by an absence of signs of affectation at birth, relative sparing of body hair, distal onycholysis, and intermittent cosegregation with autosomal dominant cleft lip and palate. Linkage studies to the known Marie Unna locus at 8p21 near the Hairless gene were performed. Linkage analysis using markers D8S298, D8S560, D8S258, and D8S282 revealed significant exclusion of this locus (Z = -2.0 or lower) at theta = 0.1. This demonstrates that this novel ectodermal dysplasia is both phenotypically and genetically distinct from Marie Unna hereditary hypotrichosis. (+info)
Nail toxicity related to taxanes.
A 70-year-old woman with non-small-cell lung cancer developed severe nail toxicity while she was being treated with docetaxel at three-week intervals. Docetaxel is a chemotherapeutic agent of the taxane family. Taxanes are well known to cause nail changes, but mainly when used on a weekly basis. (+info)
Onychomycosis of the feet; treatment with griseofulvin.
Griseofulvin, a new orally administered antifungal antibiotic which has proved to be effective for the treatment of a wide variety of superficial fungus infections of man, was used in the treatment of 51 patients with infections of the toenails due to T. rubrum. Thirty-four of the patients were treated with griseofulvin alone and seven were treated with griseofulvin combined with surgical avulsion of all involved toenails. The remaining ten had bilateral infections, and avulsion was done on one foot but not the other before griseofulvin therapy was begun. Of 34 patients who were treated with griseofulvin alone, few had complete cure even after prolonged treatment. Some nails showed improvement for a time, then no further gain; some showed no improvement; some showed resistant wedges of infection which penetrated proximally toward the posterior nail fold.In the instances of surgical avulsion, clinically normal nails regrew during griseofulvin therapy. This simple procedure, with thorough removal of all underlying keratinous debris, apparently did away with foci of possible reinfection. The results of the study indicated that surgical avulsion of the toenails in combination with griseofulvin therapy is an effective and practical method of treating onychomycosis of the toenails due to T. rubrum. (+info)
Acrokeratosis paraneoplastica of Bazex.
Bazex disease is one of the rarer cutaneous paraneoplastic syndromes. It is characterized by psoriasiform changes on the digits, and in some patients spread to the ears, nose and in later stages to the limbs and trunk. The associated malignancy is typically a squamous cell carcinoma of the upper aerodigestive tract. We review the literature regarding acrokeratosis paraneoplastica of Bazex and report three cases which illustrate both the typical and some of the less common changes that are seen in the condition. (+info)
Psoriasis confined to the nails.
A case is presented of a 49-year-old man with psoriasis limited to the nails for 12 years. The patient had no evidence of psoriasis elsewhere. He had been treated with a year-long course of griseofulvin and 2 months of weekly fluconazole without effect before the diagnosis of psoriasis was made by biopsy of the lateral nail fold. Options for the treatment of nail psoriasis are reviewed. (+info)
Pachyonychia congenita, type II.
A 5-year-old girl presented with extensor hyperkeratotic papules and subungual hyperkeratosis with nail-plate discoloration affecting all twenty nails. The mother reported that her daughter had natal teeth. By report, the father has a similar history and constellation of clinical findings. The patient's clinical presentation and history was consistent with pachyonychia congenita, which is a genodermatosis linked to mutations in the genes encoding keratins 6, 16, and 17. (+info)
Surgical aspects of subungual malignant melanomas. The Scottish Melanoma Group.
The incidence, treatment, and survival of subungual malignant melanomas in Scotland is reviewed from the Scottish Melanoma Group database. Between 1979 and 1989, 100 cases of subungual melanoma were identified (2.8% of all malignant melanomas in Scotland). The tumors tended to be locally advanced at the time of presentation (mean Breslow depth, 4.7 mm +/- 3.0 mm), and this is reflected in an overall 5-year survival of 41%. There was no difference in the survival of patients treated with local/proximal interphalangeal (PIP) joint amputation compared with those having more proximal amputations. Because nearly 70% of these tumors arose on the thumb or hallux, it is concluded that, provided adequate clearance could be obtained, less radical excision should be performed for these lesions to maintain maximum function. (+info)