SUMMARY.—
A series of 104 cases of generalized pustular psoriasis has been studied. The syndrome occurs predominantly in the second half of life, affecting both sexes. Two quite distinct sub‐groups were discernible. In the first the pre‐pustular phase of psoriasis began early in life, was typical through‐out and was prolonged. At least one third of these cases were apparently precipitated by the withdrawal of systemic corticosteroid therapy. Others were provoked by pregnancy or infection. It is likely that this type is usually extraneously provoked. In the second, the psoriasis was of late onset and atypical, acral or flexural patterns predominating in the pre‐pustular phase. In these progress to generalized pustular disease was rapid and apparently spontaneous.
Four clinical patterns of generalized pustular phase were apparent and have been named the Zumbusch, annular, localized and exanthematic types. The Zumbusch type was characterized by widespread fiery erythema, sheeted pustulation and scarlatiniform peeling, accompanied by malaise, fever and often leucocytosis. The annular type was a more low‐grade sub‐acute affection characterized by gyrate and annular pustular lesions and little systemic disturbance. The exanthematic type arose de novo, usually as a single short‐lived episode following infection or drug exposure. In the localized type restricted areas of pustular psoriasis developed in and around ordinary psoriatic plaques.
Two thirds were erythrodermic at some stage and one third had polyarthritis. Oral lesions occurred in 5 and hypocalcaemia in 5 during pustular phases.
The different clinical patterns of pustular psoriasis have been interpreted in terms of the balance between vascular hypertrophy, epidermopoiesis and leucocytic immigration into the epidermis.
SUMMARY.— Treatment with systemically administered corticosteroids and folio acid antagonists was evaluated in 104 patients with generalized pustular psoriasis.
Seventy‐three were given steroids; the remainder were not. Seventeen patients died. There were more deaths and fewer lasting remissions in the steroid‐treated group. Although steroids can control the pustular phase, the incidence and severity ot side effects seriously reduced their long‐term value. Steroid treated patients tended to suffer increasingly severe generalized pustular relapses often precipitated by attempted withdrawal of the steroid. Satisfactory control with minimal or no side effects was achieved in only 12 out of 73 patients.
Forty‐seven patients received folic acid antagonists, predominantly methotrexate. In 37 the pustules were cleared by the drug at some stage. Response to methotrexate was less satisfactory in patients previously treated with steroids, but it was of particular value in covering steroid withdrawal in patients whose disease had become refractory to the latter or in whom serious steroid side effects had developed.
There were 8 deaths among the methotrexate treated patients, 3 of which were directly attributable to the drug. In 16 other patients treatment had to be discontinued because of side effects. Methotrexate w as responsible mainly for the minor short‐term complications and steroids for the more serious long‐term ones.
The outlook for spontaneous and long‐lasting remission was much greater in younger patients and in those with a previous history of long‐standing ordinary psoriasis.
Transepidermal water losses (TEWL) from psoriatic and eczematous skin were measured while skin temperature was varied between 29 degrees C and 37 degrees C. The relationship of TEWL to skin temperature in these diseases is different from that obtaining in normal skin. These differences are thought to be due to the altered filtration properties of diseased stratum corneum. Formulae were devised to allow TEWL rates in psoriasis and eczema to be expressed at a standard temperature for comparative purposes.
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