Roth, PE; Grosshans, E; Bergoend, H
In a retrospective study we tried to evaluate the number of severe psoriasis with a lethal outcome observed in France in a 20-year period from 1965 to 1985. Among 992 psoriatic in-patients on care during this period in the Dermatology Clinic of Strasbourg, 7 died of different complications directly related to the skin disease or its therapy; 39 further cases could be gathered through different departments of dermatology of France. Patients who died had generalized psoriasis (13 cases), psoriatic erythroderma (15 cases) and generalized pustular psoriasis (18 cases); 18 (39 p. 100) also had psoriatic polyarthritis. Circumstances leading to death (table I) were metabolic disorders, related to erythroderma in most cases, non-specific complications (infections, amyloidosis) or complications of specific treatments (methotrexate, etretinate, corticosteroids, mechlorethamine). A comprehensive review of the literature over a century showed that only 72 lethal psoriasis cases have been reported: this rather low number may be due to the fact that some rare pathologies, such as visceral amyloidosis (12 cases) (table III) and fatal complications of methotrexate therapy (38 cases) (table V), paradoxically are more often published than non-specific complications occurring in severe psoriasis, such as cardiovascular failure or cachexy in erythrodermic patients. However, the review of the literature shows, as our own inquiry, the poor prognosis of generalized pustular forms and of psoriasis-associated polyarthropathies: among 42 lethal cases where enough data were available, 23 (55 p. 100) had psoriatic polyarthritis. Death due to psoriasis has to be distinguished from general causes of death among psoriatics: an increased mortality by lung cancer or heart ischemia has been observed in Sweden in female psoriatic patients and related to their style of life; in the USA, in a retrospective survey of psoriatics treated by photochemotherapy, an increased mortality by liver cirrhosis, cancer of colon and tumors of central nervous system has been reported. Such studies do not mention the death cases directly related to the evolution of psoriasis. The most severe form of psoriasis remains the generalized pustular form, and progress in therapy has not improved the mortality rate: 17.5 p. 100 in 1965 to 16.5 p. 100 in 1985. On the other hand, the prognosis of psoriatic erythroderma in now less severe and progress in therapy and nursing has lowered the mortality rate. The association of pustular psoriasis and psoriatic arthritis occurring in young adults is the clinical presentation with the worst prognosis according to our own study and to the review of literature. The mean age of deceased patients with arthritis is significantly lower than the mean age of all patients whose death is related to psoriasis (46 years versus 71 years). Metabolic and haemodynamic disturbances are responsible for death in 26 to 41 p. 100 of cases. Sepsis is the cause of death in 31 to 32.5 p. 100 respectively of observed and published cases. Secondary amyloidosis accounts for death in 17 p. 100 of all fatal cases, but this is probably an overestimate, these cases being preferentially published. Neurological complications intervene in 3.5 p. 100 and renal failure in 2 p. 100 of the cases; 25 p. 100 can be ascribed to therapy, and the number of iatrogenic lethal complications has been increasing since 1964. More information is available for methotrexate (table V) than for other antipsoriatic systemic efficient treatments, but it is impossible to decide between deaths due to methotrexate and deaths related to the severity of psoriasis in methotrexate-treated patients. Suicide is exceptional. In 13.5 p. 100 of the patients, cachexy was the terminal state. In spite of progress in therapy (photochemotherapy, retinoids, methotrexate, cyclosporin A), few patients (0.7 p. 100) with severe psoriasis will have an intractable evolution and may come to exist.