SKIN LESIONS IN PERIARTERITIS NODOSA

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854 single daily dose of 5-10 mg. may rescue a patient from a severe addisonian crisis, and as little as 025 mg. daily or on alternate days may maintain electrolyte balance thereafter. But it can readily induce excessive sodium retention, hypertension, and cedema, so it must be given with care. Although some patients may remain well on fluorohydrocortisone alone, just as some patients did on deoxycortone alone, others do not feel as fit as they might unless cortisone is given too. Thorn et al. found that a daily supplement of 2-5 mg. of cortisone was usually required. Fluorohydrocortisone is also potent as a suppressor of endogenous corticotrophin secretion. Doses of 1-10 mg. daily have reduced the urinary 17-ketosteroids to normal in adrenogenital virilism secondary to bilateral adrenal hyperplasia. A constant watch must be kept for excessive sodium retention, and if there is evidence of this the patient should have a low-sodium diet and extra potas- sium chloride. The anti-inflammatory action of 1 mg. of fluorohydrocortisone is similar to that of 20 mg. of cortisone or hydrocortisone. The intense sodium-retain- ing effect, however, precludes the use of fluorohydro- cortisone in this way, except in the topical treatment of skin diseases. Here fluorohydrocortisone ointment has been effective; and it is relatively cheap, because a smaller amount of hormone is needed. 10. Nuzum, J. W. jun., Nuzum, J. W. Arch. intern. Med. 1954, 94, 6. 11. Ketron, L. W., Bernstein, J. E. Arch. Derm. Syph., Chicago, 1939, 40, 929. 12. Bureau, Y., Barrière, H. Ann. Derm. Syph., Paris, 1954, 81, 601. 13. Gaté, J., Vayre, J., Saint-Cyr, M. Bull. Soc. franç. Derm. Syph. 1953, 60, 313. SKIN LESIONS IN PERIARTERITIS NODOSA SKIN lesions are found in about 25% of cases of periarteritis nodosa. The best-known manifestation is the subcutaneous nodule, though this is found in only 16% of all cases.1o Ketron and Bernstein 11 doubted whether the diagnosis could always be made histologically from the more superficial erythematous or papular eruptions of periarteritis nodosa, because the vessels involved in these changes were too small to show the distinctive lesions of the disease. That it may never- theless be useful to make a biopsy examination of such lesions is shown by the experience of Bureau and Barrièrep who describe a fatal case of periarteritis nodosa with various skin lesions including a " new " manifesta- tion-an apparently superficial circumscribed red scaly eruption resembling tinea, which showed clearer histo- logical evidence of periarteritis than the grosser skin lesions elsewhere. Nodules, as Bureau and Barriere point out, are most commonly found along the course of small arteries, particularly on the legs but also on the wrist, forearm, angle of the jaw, forehead, and neck. There may be more superficial papular elements, sometimes with central necrosis, reproducing the appearance of some forms of tuberculides. Erythematous macules are common, particularly on the limbs ; they may become purpuric or lead to a secondary blister with an appearance resemb- ling erythema multiforme. Purpura, which is said to be the most common lesion, may be massive. Nearly always it is associated with other skin changes-erythema, nodules, or blisters. Occasionally there is dull erythema- tous or cyanotic reticulation of the skin-so-called livedo racemosa. Telangiectatic plaques have also been seen. Necrosis of the skin may develop either in a way resemb- ling familiar forms of gangrene of the extremities or secondary to blisters which break, leading to the formation of sharply outlined ulcers. These may be severe and resemble those in the " phagadenic " ulceration 13 which occasionally accompanies ulcerative colitis. The mucosse of the mouth, nose, and rectum may show papular or nodular lesions, transient blisters, necrotic ulcers, or massive infiltration 14 Nasal infiltration with a tuberculoid granuloma was described by Wegener 15 in patients who died and in whom necropsy revealed widespread visceral lesions of this type and in addition the vascular changes of periarteritis nodosa. It is a long clinical jump to Miescher’s 16 benign case in which skin infiltration resembling Bazin’s disease showed histological changes interpreted as periarteritis nodosa leading into a tuberculoid process. Other workers have reported cases in which skin lesions of the clinical and histological type described in the systemic disease have been found in patients who remained otherwise well. Perhaps until the significance of the histological lesion of periarteritis nodosa is understood, such cases should not be grouped with those of the major disease. It is not yet clear what types of necrotising arteritis are in fact setiologically identical, even if their pathogenesis and histological appearance seem similar. Bohrod 17 sees enough similarity between them to suggest that they do form a group, whereas Zeek 18 continues to discourage the grouping together of all forms of necrotising arteritis, among which she recognises as many as five categories: hypersensitivity arteritis, allergic granulomatous arteritis, rheumatic arteritis, temporal arteritis, and classical periarteritis nodosa. Dermatologists are aware of puzzling cases of skin lesions which have in common two main features-first, histological evidence of vascular inflammation and, secondly, a perplexing lack of evidence of any general physical disease. Many of the nodular and necrotising lesions formerly diagnosed as tuberculides are now less confidently regarded as manifestations of tuberculosis. Some are called " nodular vasculitis," whereas Gougerot’s school impart a more setiological flavour to their diagnosis of " nodular cutaneous allergides." The evidence for an allergic basis for all of these is unconvincing. At present no very useful purpose seems to be served by associating them with periarteritis nodosa in the sense of the classical disease of Kussmaul and Maier. 14. Civatte, J., Duperrat, B. Ibid, p. 261. 15. Wegener, F. Beitr. path. Anat. 1939, 102, 36. 16. Miescher, G. Dermatologica, Basle, 1946, 92, 225. 17. Bohrod, M. G. Histology of Allergic Lesions in Progress in Allergy, vol. 4. Basic, 1954. 18. Zeek, P. M. New Engl. J. Med. 1953, 248, 764. 19. Schou, M., Juel-Nielsen, N., Strömgren, E., Voldby, H. J. Neurol. Psychiat. 1954, 17, 250. LITHIUM SALTS IN MANIC PSYCHOSIS THE psychiatric treatment of mania may be difficult and unsatisfactory. Sedative drugs often have to be pushed to the point of narcosis or confusion, and even then they may be ineffective. Certainly convulsion therapy is usually effective, but the result may be very short-lived and is much less satisfactory than in depres- sive illness. Any new treatment which may be harmless and effective will therefore be sure of an early trial, and lithium salts appear to hold out a limited promise. Although they have been used since 1949, following some observations in guineapigs, not many people have been interested in them. Schou et al.19 have made a careful clinical assessment of their use in the treatment of 38 cases of mania-30 with the classical " pure " syndrome and 8 with some atypical features, such as delusions and hallucinations. Using placebos as a control, they found evidence of sedation in 14 cases and a less definite action in 18, while in 6 there was no effect. Improvement appeared to be symptomatic, and patients relapsed soon after lithium was withdrawn. The margin between therapeutic and toxic doses was not wide, but with doses of 24-48 m.eq. of lithium a day no serious intoxication was noted. In practice it is unlikely that this treatment will have more than a limited value, either for temporary effective sedation or for repeated treatment of recurrent mania- where the length of the manic phase can reasonably be

Transcript of SKIN LESIONS IN PERIARTERITIS NODOSA

854

single daily dose of 5-10 mg. may rescue a patient froma severe addisonian crisis, and as little as 025 mg. dailyor on alternate days may maintain electrolyte balancethereafter. But it can readily induce excessive sodiumretention, hypertension, and cedema, so it must be givenwith care. Although some patients may remain well onfluorohydrocortisone alone, just as some patients did ondeoxycortone alone, others do not feel as fit as they mightunless cortisone is given too. Thorn et al. found that a

daily supplement of 2-5 mg. of cortisone was usuallyrequired.

Fluorohydrocortisone is also potent as a suppressorof endogenous corticotrophin secretion. Doses of 1-10 mg.daily have reduced the urinary 17-ketosteroids to normalin adrenogenital virilism secondary to bilateral adrenalhyperplasia. A constant watch must be kept for excessivesodium retention, and if there is evidence of this the

patient should have a low-sodium diet and extra potas-sium chloride. The anti-inflammatory action of 1 mg.of fluorohydrocortisone is similar to that of 20 mg. ofcortisone or hydrocortisone. The intense sodium-retain-

ing effect, however, precludes the use of fluorohydro-cortisone in this way, except in the topical treatment ofskin diseases. Here fluorohydrocortisone ointment hasbeen effective; and it is relatively cheap, because asmaller amount of hormone is needed.

10. Nuzum, J. W. jun., Nuzum, J. W. Arch. intern. Med. 1954,94, 6.

11. Ketron, L. W., Bernstein, J. E. Arch. Derm. Syph., Chicago,1939, 40, 929.

12. Bureau, Y., Barrière, H. Ann. Derm. Syph., Paris, 1954,81, 601.

13. Gaté, J., Vayre, J., Saint-Cyr, M. Bull. Soc. franç. Derm.Syph. 1953, 60, 313.

SKIN LESIONS IN PERIARTERITIS NODOSA

SKIN lesions are found in about 25% of cases of

periarteritis nodosa. The best-known manifestation isthe subcutaneous nodule, though this is found in

only 16% of all cases.1o Ketron and Bernstein 11doubted whether the diagnosis could always be madehistologically from the more superficial erythematous orpapular eruptions of periarteritis nodosa, because thevessels involved in these changes were too small to showthe distinctive lesions of the disease. That it may never-theless be useful to make a biopsy examination of suchlesions is shown by the experience of Bureau andBarrièrep who describe a fatal case of periarteritis nodosawith various skin lesions including a

" new " manifesta-tion-an apparently superficial circumscribed red scalyeruption resembling tinea, which showed clearer histo-logical evidence of periarteritis than the grosser skinlesions elsewhere.

Nodules, as Bureau and Barriere point out, are mostcommonly found along the course of small arteries,particularly on the legs but also on the wrist, forearm,angle of the jaw, forehead, and neck. There may bemore superficial papular elements, sometimes with centralnecrosis, reproducing the appearance of some forms oftuberculides. Erythematous macules are common,

particularly on the limbs ; they may become purpuricor lead to a secondary blister with an appearance resemb-ling erythema multiforme. Purpura, which is said to bethe most common lesion, may be massive. Nearly alwaysit is associated with other skin changes-erythema,nodules, or blisters. Occasionally there is dull erythema-tous or cyanotic reticulation of the skin-so-called livedoracemosa. Telangiectatic plaques have also been seen.Necrosis of the skin may develop either in a way resemb-ling familiar forms of gangrene of the extremities or

secondary to blisters which break, leading to the formationof sharply outlined ulcers. These may be severe andresemble those in the " phagadenic " ulceration 13 whichoccasionally accompanies ulcerative colitis. The mucosseof the mouth, nose, and rectum may show papular ornodular lesions, transient blisters, necrotic ulcers, or

massive infiltration 14 Nasal infiltration with a tuberculoidgranuloma was described by Wegener 15 in patients whodied and in whom necropsy revealed widespread viscerallesions of this type and in addition the vascular changesof periarteritis nodosa.

It is a long clinical jump to Miescher’s 16 benign case inwhich skin infiltration resembling Bazin’s disease showedhistological changes interpreted as periarteritis nodosaleading into a tuberculoid process. Other workers have

reported cases in which skin lesions of the clinical andhistological type described in the systemic disease havebeen found in patients who remained otherwise well.

Perhaps until the significance of the histological lesion ofperiarteritis nodosa is understood, such cases should notbe grouped with those of the major disease. It is not yetclear what types of necrotising arteritis are in fact

setiologically identical, even if their pathogenesis andhistological appearance seem similar. Bohrod 17 sees

enough similarity between them to suggest that they doform a group, whereas Zeek 18 continues to discourage thegrouping together of all forms of necrotising arteritis,among which she recognises as many as five categories:hypersensitivity arteritis, allergic granulomatous arteritis,rheumatic arteritis, temporal arteritis, and classicalperiarteritis nodosa.

Dermatologists are aware of puzzling cases of skinlesions which have in common two main features-first,histological evidence of vascular inflammation and,secondly, a perplexing lack of evidence of any generalphysical disease. Many of the nodular and necrotisinglesions formerly diagnosed as tuberculides are now lessconfidently regarded as manifestations of tuberculosis.Some are called " nodular vasculitis," whereas Gougerot’sschool impart a more setiological flavour to their diagnosisof " nodular cutaneous allergides." The evidence for an

allergic basis for all of these is unconvincing. At presentno very useful purpose seems to be served by associatingthem with periarteritis nodosa in the sense of the classicaldisease of Kussmaul and Maier.

14. Civatte, J., Duperrat, B. Ibid, p. 261.15. Wegener, F. Beitr. path. Anat. 1939, 102, 36.16. Miescher, G. Dermatologica, Basle, 1946, 92, 225.17. Bohrod, M. G. Histology of Allergic Lesions in Progress in

Allergy, vol. 4. Basic, 1954.18. Zeek, P. M. New Engl. J. Med. 1953, 248, 764.19. Schou, M., Juel-Nielsen, N., Strömgren, E., Voldby, H. J.

Neurol. Psychiat. 1954, 17, 250.

LITHIUM SALTS IN MANIC PSYCHOSIS

THE psychiatric treatment of mania may be difficultand unsatisfactory. Sedative drugs often have to bepushed to the point of narcosis or confusion, and eventhen they may be ineffective. Certainly convulsion

therapy is usually effective, but the result may be veryshort-lived and is much less satisfactory than in depres-sive illness. Any new treatment which may be harmlessand effective will therefore be sure of an early trial, andlithium salts appear to hold out a limited promise.Although they have been used since 1949, following someobservations in guineapigs, not many people have beeninterested in them. Schou et al.19 have made a carefulclinical assessment of their use in the treatment of 38cases of mania-30 with the classical " pure " syndromeand 8 with some atypical features, such as delusions andhallucinations. Using placebos as a control, they foundevidence of sedation in 14 cases and a less definite actionin 18, while in 6 there was no effect. Improvementappeared to be symptomatic, and patients relapsed soonafter lithium was withdrawn. The margin betweentherapeutic and toxic doses was not wide, but with dosesof 24-48 m.eq. of lithium a day no serious intoxicationwas noted.

In practice it is unlikely that this treatment will havemore than a limited value, either for temporary effectivesedation or for repeated treatment of recurrent mania-where the length of the manic phase can reasonably be