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British Association of Dermatologists Richard L. Dobson, M.D.

Buffalo, N Y

The Fifty-eighth Annual Meeting of the British Association of Dermatologists was held in Nottingham, England, July 12-15, 1978. The meeting was ably chaired and organized by Dr. Patrick D. C. Kinmont, head of the Dermatology Department at the Queen's Medical Center in Nottingham. Prof. G. H. Elder of the National University of Wales reviewed recent advances in the cutaneous hepatic prophyrias. In hereditary coproporphyria, coproporphyrinogen oxidase is decreased by about 50%, resulting in an increased cellular content of coproporphyrinogen III and an increased excretion of this substance in the bile and feces. About 30% of patients with this disorder have skin lesions. When abdominal pain occurs, it is accompanied by increased porphobilinogen in the urine, probably as a result of decreased conversion to uroporphyrinogen III. In variegate porphyria, 80% of the patients have skin lesions. In this disorder, both copro- and protoporphyrins are increased due to a decreased conversion of coproporphyrinogen III to protoporphyrinogen IX. The disease is inherited as an autosomal-dominant, which leads to a marked reduction in coproporphyrinogen oxidase. In this disorder, affected but asymptomatic carriers can be detected by measuring porphyrins in cultured lymphocytes. In porphyria cutanea tarda, the distinguishing feature is an increase in fecal isocoproporphyrins. A uroporphyrinogen decarboxylase deficiency leads to an increased level of dehydroisocoproporphyrinogen. However, this enzyme defect, by itself, is insufficient to produce clinical disease, and liver cell damage must always be present, usually as a result of alcoholism. Recent studies indicate that there may be two types of porphyria cutanea tarda, one in which both hepatic and red blood cell enzymes are decreased and the other with only a hepatic deficiency. 0190-9622/79/010081+02500.20/0 9 1979 Am Acad Dermatol

N. B. Simpson of the University of Leeds reported that topical application of 0.5% progesterone in alcohol seems to reduce sebum output by competition with testosterone for 5-alpha reductase. However, this action is seen only in women and has no effect on ache. P. J. A. Holt et al of the National University of Wales reported on their findings in 15 patients with pyoderma gangrenosum. The disease is associated with an increased incidence of HLA-W40. Although there is no consistent evidence of depressed cell-mediated immunity, some patients show an inability to be sensitized with dinitrochlorobenzene (DNCB), a diminished response to mitogenic stimulation of cultured lymphocyte, and decreased neutrophil functions. N. Smith and G. C. Wells reported their observations on eosinophilic cellulitis, characterized by repeated episodes of edema. The dermis is infiltrated with eosinophils aggregated around collagen bundles. Later, histiocytes and giant cells engulf the debris, forming "flame figures." In addition, many eosinophilic granules can be observed lying loosely in the dermis. An interesting clinical feature is that there is a greenish color to the urticaria. Thus far, no immune complexes have been detected, but Ca has been observed on direct immunofluorescence. D. A. Norris of the University of Colorado, winner of the Stelwagen award at the 1977 meeting of the American Academy of Dermatology, discussed his findings in mycosis fungoides (MF). Normal lymphocytes correct the abnormal chemotaxis o f M F monocytes. Since no soluble inhibitors can be detected in MF serufn, this defect seems to be due to a lack of essential lymphocyte helper cell functions directed toward monocytes. L. G. Millard of the University of Leeds reported on the prognosis &cutaneous lupus erythematosus. In his experience, 44% of patients event81

Journal of the American Academy of Dermatology

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ually remitted after an average duration of 18 years. Lesions on the hands, feet, scalp, trunk, and especially preauricular plaques in men tend to persist. Patients with Raynaud's phenomenon tend to have a longer course. Of their 110 patients with discoid lupus erythematosus (DLE), 5 developed systemic lupus erythematosus (SLE) after 2 to 18 years. Prof. K. Wolff of Innsbruck, Austria, was guest lecturer and presented a magnificent summary of the current status of Langerhans cells. These cells are found in association with lymphocytes in DNCB-sensitized epidermis and are capable of phagocytizing metals that can produce contact dermatitis. Langerhans cells can form rosettes with sheep red blood cells, have C3, Fc, and lgG receptors, and thus have surface markers resembling those of macrophages. Current thinking is that the Langerhans cells process antigens for T cells and that this function can be inhibited by anti-Ia serum plus complement. Langerhans cells can also produce proliferation of T cells and stimulate the mixed lymphocyte reaction. Prof. A. Breathnach of St. Mary's Hospital reported results of his electron microscopic studies on the effect of dicarboxylic acids on melanocytes. These acids are products of the lipid metabolism of the organism causing tinea versicolor. When dicarboxylic acid compounds such as azelaic acid (Ca) are added to cultures of melanocytes in vitro, they seem to have a stimulatory effect on melanogenesis. However, in vivo, these compounds have no effect on normal melanocytes but seem to inhibit melanogenesis in "active" melanocytes. C. F. H. Vickers of the University of Liverpool presented a further 10-year follow-up on the prognosis of atopic dermatitis. He has been following a large group of patients for more than 20 years. Factors having no influence on prognosis include

severity at onset, position in family, type of infant feeding (specifically, breast feeding does not prevent the development of atopic dermatitis), or whether ichthyosis is present or not. Late onset adversely affects prognosis. If the disease begins after the age of 2 years, only 40% clear. Only children do somewhat worse than those with siblings. Interestingly, if both parents are affected, there is no effect on prognosis. If both extensor and flexural surfaces are involved in childhood, only 57% clear. If asthma is associated, there is a slightly worse prognosis, but rhinitis seems to have no influence. Acne and psoriasis occur much less fiequenfly in this group than in the general population. In Vickers' experience, only 25 of 2,000 patients developed cataracts. More than thirty patients were presented, Among the more interesting were a 35-year-old man with ichthyosis, poikiloderma of Jacobi, and an unclassified lymphoma, an extensive case of telangiectasia macularis eruptiva perstans, a 12-year-old b o y with dyskeratosis congenita with pigmentation on the neck suggesting a concomitant Fanconi's syndrome, a 22-year-old woman with reticular erythematous mucinosis originally described by Steigleder, 1 a 22-year-old woman with hereditary angioneurotic edema, and a 17-year-old girl with poikiloderma congenitale. One cannot help but comment on the magnificent hospitality of our British colleagues. The social events were a charming mixture of warmth and formality. To be announced formally to the Lord Mayor and the Sheriff of Nottingham by a beefeater-clad master of ceremonies will forever remain in the memory of all Americans privileged to attend this informative and weUorganized meeting. REFERENCE

1. SteiglederG: Br J Dermatol 91:19l, 1974.