High risk of death in elderly patients with extensive bullous pemphigoid.

OBJECTIVE: To evaluate survival and factors predicting death in bullous pemphigoid. DESIGN: Retrospective analysis of cohort. SETTING: Three referral centers (university hospitals). PATIENTS: Among 237 patients recruited between January 1, 1985, and December 31, 1992, 20 were excluded because of doubtful diagnosis. The 217 remaining patients were 79+/-11 years old (mean+/-SD); 120 were women and 97 were men; and 79% had been treated with oral corticosteroids, 40 to 90 mg/d. INTERVENTIONS: Missing information on follow-up was minimized by letters and/or telephone calls to patients, families, nursing homes, and physicians. MAIN OUTCOME MEASURES: Actuarial survival curve, compared with the expected curve as derived from census data, and evaluation of prognostic factors by comparing initial characteristics between patients alive at 6 months and these who died before that point. RESULTS: Survival curve demonstrated an early increased mortality: 17% at 3 months and 31% at 6 months, mainly from sepsis and cardiovascular diseases. Of the factors related to bullous pemphigoid activity (duration; pruritus; and number and extent of blisters, eosinophilia, and serum antibodies) only generalized pemphigoid was predictive of death in comparison with localized forms. In multivariate analysis, age of 86 years or more (relative risk, 7.1; 95% confidence interval [CI], 2.0-25.4; P < or = .01), poor general condition (relative risk, 8.2; 95% CI, 3.0-22.4; P < or = .001), female sex (relative risk, 2.4; 95% CI, 1.1-5.4; P < or = .05), and generalized disease (relative risk, 4.4; 95% CI, 1.4-13.7; P < or = .01) were associated with increased risks of death at 6 months. CONCLUSION: In this series, generalized bullous pemphigoid had a poor prognosis especially in older patients and those in poor general condition.

[Inguinal mastocytosis with histiocytic infiltrate]. / Mastocytose inguinale avec infiltrat histiocytaire.

INTRODUCTION: The diagnosis of mastocytosis is usually not difficult in cases with typical skin lesions. Intertrigo is exceptional. CASE REPORT: An 83-year-old patient developed inguinal intertrigo bilaterally. The lesions contained maculopapulous elements without Darier's sign. The histology examination revealed a mixed mast cell infiltration predominated by histiocytes. The ultrastructure confirmed the diagnosis of cutaneous mastocytosis. No extension was observed. DISCUSSION: Skin folds are rarely involved in infiltrating diffuse mastocytosis. Two cases have been reported in the literature including one doubtful case. Mastocytosis should thus be included in the possible diagnoses in patients with macupopapulous intertrigo. In addition, the mastocytic and histiocytic polymorphism observed here is rare.

[Survival prognosis in pemphigoid. A cohort analysis of 78 patients]. / Pronostic vital de la pemphigoide. Analyse d'une cohorte de 78 malades.

INTRODUCTION: Bullous pemphigoid (BP) is the most frequent autoimmune bullous skin disease and usually affects elderly patients. Despite some conflicting data, vital prognosis is poor in BP, of which mortality rate after one year of treatment varies between 10 and 40 p. 100. To date, the factors influencing this prognosis remain to be determined. PATIENTS AND METHODS: A cohort of 78 consecutive patients with BP (mean age: 80 +- 4 years) has been prospectively selected on a six-year period (1987-1992) in Limoges. The diagnosis of BP was made on clinical criteria (using a standardized questionnaire), direct immunofluorescence (IF) findings (linear deposits of IgG and/or C3 along the basement membrane zone) and confirmed by direct immunoelectron microscopy and/or Western immunoblotting on epidermal extracts. RESULTS: The follow-up analysis (mean duration of follow-up: year of treatment (22 p. 100 in the first three months) with an actuarial survival curve inclined to be horizontal by the end of this first year of treatment. Therefore, we have studied the clinical or immunologic factors susceptible to influence the vital prognosis of BP according to this main criterion: death (or survival) at the end of the first year of treatment. None of the following factors has been found to be significantly linked to the vital prognosis in BP: age, sex, extent of skin lesions at presentation, presence of mucosal lesions, blood eosinophilia, presence of circulating basement membrane zone autoantibodies by indirect IF. The clinical factors of bad prognosis were an altered general condition and a history of coronaropathy. The presence of circulating autoantibodies against BP180 antigen (but not the one of autoantibodies against BP230) as detected by immunoblotting on epidermal extracts was found to be significantly more frequent (50 vs 22 percent) in BP patients who died within the first year of treatment (p < 0.02). DISCUSSION: Although modestly discriminating, the presence of circulating autoantibodies against BP180 represents the first vital prognosis factor demonstrated in BP. This result confirms the growing pathophysiologic importance of the anti-BP180 autoantibodies of which the pathogenic role has been recently formally demonstrated in BP using an animal model.

[Cerebral atrophy of vascular origin in the course of neurofibromatosis]. / Atrophie cérébrale d'origine vasculaire au cours d'une neurofibromatose.

The authors report the case of a 39-year-old woman with type I neurofibromatosis who presented a right incomplete proportional hemiplegia which progressively worsened over a 6-month period. Left hemispheric atrophy with heterogeneous features, predominant in the temporoparietal region, was revealed by computerized tomography. Atrophy was associated with diffuse vascular lesions in the distal part of the left sylvian and anterior cerebral arteries, leading to major cortical hypoperfusion. Vascular examination showed no hypertension nor any sign of arterial involvement in another region. This case illustrates the nature of vasculopathy associated with neurofibromatosis. Its expression is polymorphous, with lesions inducing stenosis (the most common ones), aneurysmal lesions or veritable angiodysplasias (either hypo- or hyperplastic). The vascular expression of neurofibromatosis is often overlooked. However, in the presence of an inexplicable occlusive or aneurysmal vasculopathy, it is advisable to search for signs of neurofibromatosis since ill-defined forms exist.

[Disulone hypersensitivity syndrome]. / Syndrome d'hypersensibilité a la Disulone.

Hypersensitivity to dapsone (Disulone) is a rare side effect of sulfone therapy. The diagnosis is based on variable clinical manifestations and laboratory findings dominated by hyperthermia, skin eruptions and a mixed form of hepatitis. We observed a case of hypersensitivity to dapsone in a 57-year-old woman who was treated with Disulone for cicatricaial pemphigoid. Clinical manifestations and laboratory results returned to normal 3 weeks after drug withdrawal. This case is interesting because the skin eruptions classically described were not observed. In addition this is apparently the first case reported of such a hypersensitivity syndrome occurring during treatment of autoimmune bullous dermatosis.