Self-healing generalized infantile myofibromatosis with elevated urinary bFGF.

We report a case of generalized infantile myofibromatosis with favorable outcome despite systemic involvement. Elevated urinary bFGF levels during the active phase of the disease suggested an angiogenic stimulation in the pathogenesis of myofibromatosis.

Pulse methylprednisolone therapy for severe alopecia areata: an open prospective study of 45 patients.

BACKGROUND: Oral corticosteroids may be effective in the treatment of severe alopecia areata (AA), but the side effects of prolonged therapy limit their use. The benefit of a single intravenous pulse of methylprednisolone has not been evaluated in patients with ongoing hair loss of less than 12 months' duration. OBJECTIVE: Our purpose was to determine the effectiveness of an intravenous pulse of methylprednisolone at 1, 3, 6, and 12 months in patients with active severe AA of less than 12 months' duration. METHODS: Forty-five patients were included in this open study. All had rapid and extensive hair loss for less than 1 year (first occurrence or relapse), with the bald area exceeding 30% of the scalp. There were 20 multifocal, 10 ophiasic, 9 universalis, and 6 totalis cases. Intravenous methylprednisolone, 250 mg, was administered twice a day on 3 successive days. Follow-up for at least 12 months (up to 29 months) was performed. The percentage of pretreatment bald area covered by hair regrowth at 1,3,6, and 12 months was measured. RESULTS: No major side effects were observed. Patients with multifocal AA (n = 20) showed the best response rate, with 9, 12, 13, and 12 showing 100% or 50% to 100% regrowth at 1, 3, 6, and 12 months, respectively. Relapse occurred at 3 months in 1 patient, at 6 months in 2, and at 12 months in 4. A second pulse was tried in 2 patients with relapse with 100% regrowth that was stable at 12 and 28 months. In patients with ophiasic AA (n = 10), no total regrowth was observed; 6 had no response, 4 showed 20% to 70% regrowth at 1 month with relapse at 3 and 6 months. A second series of pulses was given to the 4 initial responders 3 to 13 months after the first series; the response rate to this second treatment was better than the first. In patients with universalis and totalis AA (n = 15), no total regrowth was observed initially; 8 patients had no response, and 3 showed 50% to 90% regrowth at I month, with subsequent improvement at 3 and 6 months. In 4 patients who did not show an initial response, a significant (90% to 100%) delayed regrowth was observed between 9 and 16 months after the pulse therapy. CONCLUSION: A single series of intravenous pulse of methylprednisolone appears to be well tolerated and effective in patients with rapidly progressing extensive multifocal AA, but not those with ophiasic and universalis AA.

Upregulation of connexin 26 between keratinocytes of psoriatic lesions.

To assess whether the expression of connexins (Cx) by keratinocytes is altered under conditions of abnormal epidermal differentiation, we have compared Cx26, Cx32, Cx37, Cx40, and Cx43 in the epidermis of 11 psoriatic patients who had not been treated for at least 1 mo and of seven healthy individuals. In all samples of fully mature psoriatic plaques, we have observed a massive expression of Cx26, as judged at both the transcript level (northern blot) and the protein level (immunofluorescence). This protein became consistently detected between keratinocytes of the basal and granular layers at the periphery of psoriatic plaques and in all layers of fully developed psoriatic epidermis, except in regions of parakeratosis. None or a minimal amount of Cx26 was observed in both control and nonlesional regions of psoriatic epidermis. Psoriatic plaques also contained Cx43, the prominent gap junction protein in the interfollicular epidermis of normal human skin. The levels of this protein appeared to be slightly higher in psoriatic than in control skin, as judged at both the transcript level (northern blot) and the protein level (immunofluorescence). Three other connexins (Cx32, Cx37, and Cx40), which are not observed in control interfollicular epidermis, were not induced in either nonlesional or lesional regions of psoriatic skin. The data indicate that selective changes in the normal expression of connexins by keratinocytes are associated with the changes in the proliferation and differentiation program that these cells undergo in psoriasis.

Cutaneous manifestations of relapsing polychondritis in a patient receiving goserelin for carcinoma of the prostate.

Relapsing polychondritis is a chronic rheumatologic disorder of unknown etiology. Cutaneous manifestations occur in nearly half of the patients and often precede cartilaginous involvement. We present the case of a man with a history of prostatic adenocarcinoma who underwent monthly injections of goserelin (Zoladex), an LH-RH analogue. Five months after the beginning of the treatment, he presented cutaneous manifestations, which then recurred monthly, after each goserelin injection. After goserelin interruption and replacement with another treatment (cyproterone acetate), the patient was asymptomatic for 2 months. A cutaneous relapse then occurred followed by a typical cartilaginous involvement. In our observation, goserelin seems to have triggered the cutaneous manifestations of relapsing polychondritis. An hormonal precipitating factor in relapsing polychondritis has already been suggested by reports of patients whose disease worsened under chorionic gonadotropin treatment or during pregnancy.

[Ulcers of the tongue, pityriasis lichenoides and primary parvovirus B19 infection]. / Ulcerations de la langue, parapsoriasis en gouttes et primo-infection à parvovirus B19.

INTRODUCTION: We report a case of parapsoriasis en gouttes (or pityriasis lichenoides) which presents two peculiarities. First, the patient had lingual ulcerations and second, the eruption appeared during a seroconversion for Parvovirus B19. OBSERVATION: A 25 old woman presented a first episode of characteristic parapsoriasis en gouttes associated with purpuric palmoplantar lesions and lingual ulcerations, reaching deep muscular in histology. DISCUSSION: This observation of parapsoriasis en gouttes, peculiar because of lingual ulcerations, is mostly interesting because of its association with a primo-infection to Parvovirus B19. The receptor of the virus is localised on endothelial cells and that could explain purpuric lesions and ulcerations observed.

[Prevalence of hypothyroidism and hyperthyroidism in temporal arteritis and rhizomelic pseudopolyarthritis. A controlled study of 104 cases]. / Prevalence de l'hypothyroidie et de l'hyperthyroidie dans la maladie de Horton et la pseudopolyarthrite rhizomélique. Etude contrôlée portant sur 104 cas.

The aim of this study was to assess the prevalence of hyperthyroidism and hypothyroidism in giant cell arteritis and polymyalgia rheumatica. The prevalence of thyroid dysfunction in giant cell arteritis and polymyalgia rheumatica patients was determined retrospectively from 1976 through 1984 and prospectively from 1984 through 1991. A control group was composed of patients over 55 years of age consecutively admitted to the same hospital department for another condition. Patients were screened for thyroid dysfunction using a thyrotropin assay. Abnormal results were evaluated by T3 and T4 assays and, if needed, a TRH test. Among the 68 giant cell arteritis patients (mean age 72.6 +/- 7 years), of which 41 were included in the prospective arm of the study, 6 had hypothyroidism and 3 had hyperthyroidism. Corresponding figures were 4 and 4 among the 36 patients with polymyalgia rheumatica (mean age 71.7 +/- 8.3 years), of which 18 were evaluated prospectively. Among the 305 controls (mean age 71.6 +/- 9.4 years), 16 had hypothyroidism and 10 had hyperthyroidism. Prevalences of hypothyroidism, hyperthyroidism, and antithyroid antibodies were not significantly different in the control and case groups. Data fail to support previous suggestions that giant cell arteritis or polymyalgia rheumatica patients may be an increased risk for hypothyroidism or hyperthyroidism. They lend no indirect support to the hypothesis that giant cell arteritis and polymyalgia rheumatica may be autoimmune disorders.

Pulse of methylprednisolone in alopecia areata.

In an attempt to stop the evolution of recent-onset severe alopecia areata (AA), we tested pulse corticotherapy on 9 patients. Acceptance into the study was based on the following criteria: recent-onset AA (< 1 year), AA in an active state, bald surface > 30% of the scalp, no contraindication to pulse corticotherapy. Each patient was given 250 mg i.v. of methylprednisolone twice a day on 3 successive days. In 8 patients the course of the ongoing episode of AA was stopped. At the 6-month follow-up, a regrowth on 80-100% of the bald surface was observed in 6 patients. One patient did not respond to treatment, and 2 had less than 50% of regrowth. This open study suggests that pulse corticotherapy: (1) can stop the course of severe AA in an active state, (2) is well tolerated without major side effects and (3) does not permit a stable control of AA of more than 1 year duration. This treatment seems to be indicated for severe AA of recent onset.