THE PROGNOSIS OF GENERALIZED PUSTULAR PSORIASIS TERENCE

Summary.— Data concerning 155 patients with all types of generalized pustular psoriasis have been reviewed. Generalized pustular psoriasis of Yon Zumbusch type is a severe disease. Thirty-four of 106 patients followed up have died. In 26 of these, death was attributable to the disease or its treatment. G.P.P. developing from acral pustulosis (acrodermatitis continua) has the worst prognosis and is likely to prove fatal. G.P.P. evolving from ordinary, pustular palmo-plantar or atypical fiexural forms of psoriasis has a better prognosis and may remit spontaneously.
The use of steroids systemically for ordinary or erythrodermic psoriasis may precipitate G.P.P. and perpetuate the severe phase of psoriasis. Methotrexate is advocated for the treatment of the severe acute phase; but, in some patients, systemic steroid therapy cannot be avoided.     

Supernatants from cultured human epidermal keratinocytes stimulate angiogenesis

Conditioned media from three different strains of human epidermal keratinocytes in culture were assayed on the chorioallantoic membrane of the chick embryo. Vascular responses were examined 4 days later stereo-microscopically and compared with controls—unconditioned medium and medium incubated with 3T3 cells. Specimens were also collected after 1,2,3 and 4 days for serial histological examination. Media from all three strains of keratinocytes stimulated statistically significant vascular growth relative to controls. Leukocytic infiltration was not demonstrated histologically at any time. In addition, quantitation of ectodermal epithelial hyperplasia in experimental groups did not reveal any significant difference when compared with controls. These results have demonstrated that epidermis may directly encourage vascular growth in the absence of significant inflammation.     

Developmental milestones or conceptual millstones? Some practical and theoretical limitations in infant assessment procedures.

There is a wide gap between recent research models of infant development and available clinical procedures. This paper considers the limitations of current assessment instruments, with special reference to motor-impaired infants. It also examines the theoretical and conceptual limitations imposed on our understanding of the developmental process by the models of development and measurement on which the instruments are based. These limitations are especially important for assessment and intervention strategies with impaired infants (0-30 months). It is suggested that new assessment methods are needed to tap the processes underlying developmental change and more accurately reflect the complexity of factors affecting development.     

Evidence for altered cell-mediated immunity in postmastectomy lymphoedema

Patients with chronic lymphoedema are prone to develop chronic infections and various tumours in the lymphoedematous limb, suggesting that regional immune surveillance is impaired. To test the hypothesis that cutaneous cell-mediated immunity is impaired. 35 women with postmastectomy lymphoedema were investigated using dinitrochlorobenzene to test the afferent and efferent loops of the allergic contact immune response. The results suppot the role of lymphatics as an important component of the immune response to allergens by the demonstration of impairment of both the afferent and efferent loops of the allergic contact dermatitis reaction, and confirm that there is suppression of immune competence in a lymphoedematous limb.     

Contact sensitivity in community-based leg ulcer patients

We performed standard epicutaneous patch testing on 59 community-based patients with leg ulcers of predominently venous aetiology. Allergic reactions occurred in a total of 30 of 59 (50.8%) of patients and were significantly more common in patients with coexisting eczema (62%) than in those without (38%) (P<0.05). In 26 of 30 (86.7%) patients with reactions, the allergies were related to leg ulcer therapy and the treatment was changed accordingly. This study demonstrates that contact sensitivities are common among community-based leg ulcer patients and we suggest that identification of allergens by patch testing should reduce the morbidity, in particular in those patients with complicating eczema.     

WEGENER'S GRANULOMATOSIS: CLINICAL FEATURES AND OUTCOME IN SEVENTEEN PATIENTS

Seventeen patients with Wegener's granulomatosis are reviewed. Eleven males and six females, with a mean age of 46.9± 4.5 years, were followed for 35.7 ± 9.0 months. Mean duration from time of onset of symptoms to diagnosis was 8.5 ±3.1 months. Constitutional symptoms (100%), lower respiratory tract involvement (93%), renal involvement (87%), and upper respiratory tract involvement (80%) were the most frequent clinical manifestations. Arthritis (60%), dermal vasculitis (60%), and inflammatory ocular disease (40%) were also common. Elevated ESR (94%), anemia (70%), and lymphopenia (77%) were frequent laboratory findings prior to treatment. Five patients had renal failure at presentation and two patients progressed from no renal involvement at presentation to renal failure at diagnosis, while five patients progressed from renal involvement without impairment at diagnosis to end-stage renal failure. Seven patients died; six of these deaths were related to active Wegener's granulomatosis. The patients with a severe systemic vasculitis, and renal involvement had a poor outcome while predominant respiratory disease had a good prognosis.     

MICROVASCULAR PATTERN AND BLOOD STASIS IN SKIN DISEASE

SUMMARY.— Some of the factors contributing to severe intravascular stasis in the blood vessels supplying the epidermis are discussed. Changes in blood viscosity result from a variety of factors which act maximally at the peak and in the venous arm of the papillary vessel. The influence of cold on blood viscosity in the papillary vessel is particularly emphasized.
The importance of aggregation of blood cells in the superficial vessels and of factors such as platelet aggregation, which may be irreversible and may influence disaggregation are discussed.
The presence of "preferential channels" in the capillary meshwork of the skin is illustrated and their significance as passages permitting redistribution of blood away from the epidermis is emphasized.
Studies of platelet stickiness in a group of patients suffering from sensitivity to cold and studies on blood flow in one patient with acrocyanosis are presented.     

Computer microtechnology for a severely disabled preschool child

The developments and considerations essential to providing microtechnological aids for a preschool severely disabled child confined to a bed in a hospital ward are described. This single case report illustrates the needs of a quadriplegic boy dependent on a ventilator via a tracheostomy. Provision of a computer, a variety of specially designed switches and software together with a self-driven powered wheelchair maximized expression of his developmental needs. The decision-making process of providing the equipment is described as well as the problems encountered. His development in language and cognitive skills is discussed in the context of his change in experience and competence as he learned to control the technological aids.