An alternate-day corticosteroid regimen for pemphigus vulgaris. A 13-year prospective study.

BACKGROUND: Pemphigus vulgaris (PV) at the early, usually oral and relatively stable stage, represents the majority of PV patients. Treatment modalities usually do not differ compared to those for the fully established disease. OBJECTIVES: To prospectively assess a standardized and effective therapeutic approach that aims at less morbidity due to adverse reactions. METHODS: The following regimen, also known as Lever's mini treatment (LMT), was used. Forty mg of oral prednisone on alternate days plus 100 mg azathioprine every day were administered until the complete healing of all lesions. A gradual monthly and later bimonthly decrease of prednisone was followed by the tapering of a second immunosuppressive agent, in a one-year period. RESULTS: Seventy-four patients suffering from early-stage-PV, and representing 70% of all PV patients seen through the years 1991-2003, were eligible in the study. Total follow-up period was 76 +/- 37 (26-180) months. During the 53 +/- 26 months of LMT, 6 (8%) patients dropped out of therapy, 9 (12%) required a change to another treatment, two (3%) died and 57 (77%) achieved a lesion-free condition. Forty-five (61%) patients were in complete remission for 27 +/- 29 months. Significant morbidity was estimated 4/74 (5.2%). Disease 'breakthroughs' necessitating treatment adjustments occurred in 30 patients, usually throughout the last phase of therapy and post-treatment follow-up. CONCLUSION: LMT may be a standardized therapeutic approach for the early and relatively stable stage of PV, resulting in high efficacy, safety and quality of life profile.

Intermittent vs. continuous 1-year cyclosporin use in chronic plaque psoriasis.

OBJECTIVE: To comparatively assess the impact of intermittent and continuous cyclosporine (CyA) administration in chronic plaque psoriasis. METHODS: A two-step procedure was followed for the 51 patients who were randomly assigned in the study. CyA in doses 2.5 mg kg(-1) day(-1) escalated up to 5 mg kg(-1) day(-1) for efficacy reasons, were given to all patients during the initial 12-week step. Only patients who achieved a minimum 50% reduction of baseline PASI could enter the 2nd step, which lasted for nine additional months. Subjects on the intermittent schedule stopped all therapy to receive other 12-week course(s) if and when they relapsed. Patients on the continuous schedule continued CyA therapy at the lowest, individualized, effective dose. RESULTS: 78% of all patients achieved a PASI-75, with subjects on continuous treatment manifesting greater improvement (92% vs 62%, P= 0.008). The decrease of DLQI was 89%, without any difference between the two schedules (P= 0.214). The median effective and maintenance doses were 3 (2.5-3.8) and 1.8 (0.7-3) mg kg(-1) day(-1), respectively for intermittent and continuous schedules. A significant correlation was observed between the clinical parameters and DLQI change at 1 year, for both groups of patients. Dose modifications for safety reasons were necessary in 5 patients, but no treatment discontinuation was required. Patients on the continuous regimen received 139% of the mean cumulative annual CyA dose of Intermittent therapy. CONCLUSION: The significantly higher annual cumulative dose required for the continuous CyA regimen compared to intermittent, leads to the suggestion that the first modality may offer higher physical improvement but must remain an option for those patients who do not respond or are uncooperative with intermittent CyA use.

Impetigo herpetiformis: menstrual exacerbations for 7 years postpartum.

Impetigo herpetiformis (IH) is a rare pustular dermatosis that shares striking clinical and histological similarities to generalized pustular psoriasis. The case of a 26-year-old primigravida with IH progressing in the 30th week of her pregnancy is presented. The peculiarity of our presentation relies on the reappearance of the disease postpartum. Monthly exacerbations during menstruation lasted for 7 years. Some probable clues regarding the pathogenesis of IH are suggested.

Comparison of chloroxylenol 0.5% plus salicylic acid 2% cream and benzoyl peroxide 5% gel in the treatment of acne vulgaris: a randomized double-blind study.

A 12-week double-blind randomized study was performed to compare benzoyl peroxide 5% (BP) gel and chloroxylenol 0.5% plus salicylic acid 2% (PCMX + SA) cream (Nisal cream) for efficacy and adverse reactions. Thirty-seven volunteers participated in the study, 19 in the BP group and 18 in the PCMX + SA group. The patients applied the medication twice daily to the entire face. Clinical evaluation and lesion counts were obtained at 0, 3, 6, 9 and 12 weeks. At week 12 both groups showed a marked improvement in both inflammatory and noninflammatory lesions (60% and 54% for the BP group and 62% and 56% for and 56% for the PCMX + SA group, respectively). Although PCMX + SA showed a slightly stronger keratolytic effect throughout the study period, there was no statistically significant difference in the reduction of the papulopustules or comedones between the two groups. Adverse effects such as erythema and photosensitivity were significantly fewer in the PCMX + SA group at week 12 (p = 0.0002 and p = 0.05, respectively). These results suggest that PCMX + SA cream is as effective as BP gel in the treatment of papulopustular and comedonal acne and that it is better tolerated.

Onychomycosis in Northern Greece during 1994-1998.

In the 5-year period 1994-1998, 13957 patients were examined in the Mycological Laboratory of the State Hospital for Skin and Venereal Diseases, in Thessaloniki, Greece. Of the 2766 patients presenting with onychomycoses (20%), 67% were women and 33% were men. In the toenail infections, dermatophytes were most often isolated (72.3%), especially in women, followed by moulds (9.6%) and yeasts (2%); 16.1% of the infections were mixed. In the fingernail infections mostly yeasts were isolated (72%), especially in women, followed by dermatophytes (10%) and moulds (5.6%); 12.4% of the infections were mixed.

Value of routine diagnostic criteria of bullous pemphigoid.

BACKGROUND AND DESIGN: The clinical, histologic, and direct (DIF) and indirect (IIF) immunofluorescence findings are used in a critical, although arbitrary, manner in the routine diagnostic process of bullous pemphigoid (BP). Our purpose was to estimate their relative value. In the present retrospective study, a follow-up of at least 18 months was used as a prerequisite for the final diagnosis of BP (63 patients) and controls (n=159). RESULTS: The clinical, histologic, DIF, and IIF diagnostic criteria of BP were found to vary independently of each other. Positive DIF was the most sensitive (90.5%) typical for BP histology and positive IIF were the most specific (99%). Immunopathologic tests were the most valuable, especially in the atypical varieties of BP. Nearly 25% of patients in this group would have been misdiagnosed if IF tests had not been performed. Atypical cases (40%) seemed to represent a clinical continuum over the whole spectrum of the disease. Patients with exclusively immunoglobulin G (IgG) and C3 basal membrane zone (BMZ) deposits were significantly more often seropositive than the rest of the DIF-positive cases; however, the class of BMZ immunoreactants varied according to the site of biopsy. C3 was almost invariably deposited at the BMZ of DIF-positive patients. When Igs were also present, they were only exceptionally (5% of cases) of greater fluorescence intensity than C3. CONCLUSIONS: The combination of clinical data plus one positive immunopathologic test provide the best combination of sensitivity and specificity (98%), and seem to be most appropriate in defining patient populations for study purposes. The relationship between the classes of immunoreactants should be better evaluated with reference to the site of skin biopsy. It may be suggested, however, that the likelihood of BP existence is very low when in vivo C3 is absent or of lower intensity of fluorescence than the concomitant Ig(s).

Plasmapheresis in toxic epidermal necrolysis.

BACKGROUND: The treatment of toxic epidermal necrolysis (TEN) is usually based on the removal of the offending drug(s), fluid replacement, nutritional support, and local management. The mortality and morbidity, however, remain high and the death rate may be reduced to 10% only in special centers that use biologic dressings. Plasma exchange (PE) was proven efficacious in small series of patients and of no particular value in others. METHODS: Seven patients suffering from severe TEN covering 30%-80% of body surface area and having two or four mucous membranes involved, were included in this open study. Malignancy (Hodgkin's disease, brain tumor) and a variety of medicaments (carbamazepine, allopurinol, diphenylhydantoin, cefaclor, amoxicyllin with clavullanic acid) were considered as causally implicated. One to four PEs of 2.5 L were given on alternate days in six patients and on a daily basis in the seventh. RESULTS: All patients recovered successfully from their disease. No new lesions appeared after the first PE in four patients. Neither adverse reactions from this therapy nor sequelae from TEN were observed after a long follow-up lasting up to 8 years. CONCLUSIONS: Although PE is expensive and requires easy venous access to be performed, it could be listed in the first line of TEN therapy. The method is safe and efficacious, providing prompt relief from pain and rapid cessation of necrolysis. The alternate day PEs are considered preferable to the everyday regimen.

Cutaneous alternariosis in an immunocompromised patient.

A case of cutaneous alternariosis in a 68-year-old male farmer is presented. The patient had been treated with prednisone and azathioprine for thrombocytopenic purpura, and two months later he noted multiple ulcerated and slightly infiltrated granulomatous lesions on exposed areas. The diagnosis was confirmed by results of direct examination, culture of biopsy material, and histologic examination. A concurrent herpetic infection healed without sequelae. The protracted course of our patient's condition and a review of the literature suggest the tapering or stopping of steroids as the first step in the successful and permanent treatment of cutaneous alternariosis.

The treatment of pemphigus vulgaris. Experience with 48 patients seen over an 11-year period.

We present a retrospective analysis of 48 patients with pemphigus vulgaris (PV) who were seen between 1978 and 1988. They were divided into three treatment groups: 25 (group A) received 40-100 mg of oral prednisone daily; eight (group B) received > 100 mg of prednisone daily; 15 (group C) received 40 mg of oral prednisone every other day and azathioprine 100 mg daily. A second immunosuppressive agent was subsequently added to the treatment regimen of three patients in group A and eight patients in group B. By 1989, 10% of the patients had been able to discontinue all therapy, and were in complete remission. Sixty-five per cent of patients were on maintenance therapy, but in clinical remission. Twenty-five per cent of the patients had died [eight in group A (31%) and four in group B (50%)] either as a consequence of the disease or its treatment. None of the patients in group C had died. Most of the deaths occurred during the first 2-3 months of therapy. Morbidity and mortality were related to the severity of the disease, to the maximum dose of prednisone required to induce remission, and to the presence of other diseases. Patients needing a total of 5 g or more of prednisone to induce a remission during the acute stage had a high mortality rate.

Eosinophils in oral and cutaneous lesions of erythema multiforme.

The purpose of this retrospective clinicopathologic study was to investigate the presence of eosinophils in oral and skin lesions of erythema multiforme. Seventeen cases were selected which fulfilled clinical and histopathologic criteria for the disease. Twelve out of 13 sections taken from skin biopsies and 4 out of 5 sections taken from oral lesions contained eosinophils in varying densities. The occurrence of eosinophils in the lesions of erythema multiforme is parallelized and discussed with the existence of certain clinical and histopathologic aspects. Therefore, we suggest that eosinophils occur in cutaneous and oral lesions of erythema multiforme and are occasionally numerous.