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).

[Perforating necrobiosis lipoidica: 2 cases]. / Nécrobiose lipoïdique perforante: deux observations.

INTRODUCTION: Perforating necrobiosis lipoidica is a rare clinical form of necrobiosis lipoidica and is almost always associated with diabetes. Necrotized tissue is usually eliminated via transfollicular perforations. CASE REPORT: We report two cases of perforating necrobiosis lipoidica associated with type II diabetes mellitus. The characteristic clinical feature was the presence of keratotic plugs around the periphery of the lesions. Transfollicular perforation predominated in the first case. The second also presented epidermal perforation. DISCUSSION: There have been only 6 cases of perforating necrobiosis lipoidica in the literature. To our knowledge, this is the first case report of transepidermal elimination. We confirmed the constant association between this perforation type and diabetes mellitus.

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.

[Multinodular plaque-type blue nevus, superficial and neuroid]. / Naevus bleu multinodulaire en plaque, superficiel et neuroïde.

We report an exceptional case of multinodular plaque-type blue nevus in a 63-year-old woman, located on the presternal region. The lesions appeared at the age of 25 years on an achromic area. The probable clinical diagnosis was that of a pigmentary nevus. Histopathological examination showed a partially cellular blue nevus with a superficial topography and a neuroid arrangement. On this occasion, a review of the literature and a study of this peculiar form of blue nevus are made.