468

DERMATOMYOSlTIS*

By DAVID MITCHELL and JOAN ~'[ACCARTHY.

D ERMATOMYOSITIS is a relatively rare disease which does not appear to have been reported previously in this country. Interest in the condition has increased in recent years, mainly for three

reasons : 1. It is one of the diseases of the collagen group, (Klemperer,

Pollack and Baehr, 1942; Baehr and Pollack, 1947). 2. ACTH and cortisone regularly control the fever and may tide

the patient over a crisis but have little effect on the general course of the disease, (Brunsting, 1951; Ferguson et al., 1952; Kierland and Hines, 1951; Lever, 1951).

3. Malignant disease, often undiagnosed, is significantly but mysteriously associated with many cases, (Brunner and Lobraico, 1951; Curtis, Blaylock and Harrell, 1952; Forman, 1952; Pagcl and Treip, 1955).

Lastly, the pathogenesis of the condition remains obscure in spite of all recent work.

Clinically, the disease may show alt degrees of activity from an acute, rapidly fatal illness to a chronic condition which, with intermissions, may last several years. The mortMity is over 50 per cent. The typical appearance includes an expressionless oedematous face with heliotrope discolouration of the eyelids and surrounding areas. A generaliscd eruption commonly occurs which is most frequently an itchy scaly erythema, although urticaria or lesions resembling erythema multiforme or lupus erythematosus may be seen. There is a diffuse brawny non- pitting oedema. The muscles, of which those of the neck and shoulder- girdle together with the muscles of deglutition, phonation, and respira- tion are most affected, are weak and painful on attempted movement. They are tender on palpation. An irregular fever is present at some stage in most cases, and tachyeardia is the rule in the active phases.

Histologically, there are lesions in the skin and muscles. The predominant changes in the skin are: (i) flattening of the papillae; (ii) vacuolisation of cells in the basal layer; (iii) ocdema of the dermis with swollen collagen fibrils; (iv) mild round cell infiltration about the sub-epidermal capillaries and sweat glands; and finally, (v) there may be desquamation of cornified epithelium with thinning and disappearanee of the papillae with or without an increase in collagen.

In the muscles; there is oedema of the endo- and peri mysium, with a varying degree of round cell infiltration. The muscle fibres show (a) loss of striation, (b) fragmentation of fibrils, (c) granular, waxy or hyaline degeneration, (d) vacuolisation or rupture. There may be marked increase in number with irregularity in size of the sareolemmal nueiei and blebbing of the sheath. Multinucleated cells may be present. Visceral lesions are uncommon. The heart may show changes such as are seen in the skeletal muscles. Ulcerative lesions in the gastro- intestinal tract have been deseribed.

*From the Adelaide Hospital, and the School of Pathology, University of Dublin.

DERMATOMYOSITIS 469

Case Report. Mrs. P. McC., aged 49 years , was seen in consu l t a t i on in J a n u a r y , 1954, while h a v i n g

E .C .T . for invo lu t iona l depress ion , She c o m p l a i n e d of an i t chy r a s h here a n d t he r e on t h e u p p e r h a l f of t h e body, wi th s o m e pa in , w e a k n e s s a n d swell ing in he r a r m s . T h e r a s h was of a n i n d e t e r m i n a t e n a t u r e , a m o d e r a t e e r y t h e m a wi th some scal ing ; it h a d p rev ious ly been d iagnosed as c o n t a c t d e r m a t i t i s . The pa in a n d w e a k n e s s in t h e a r m s were a t t r i b u t e d to in jec t ions wh ich she h a d b e e n h a v i n g . Two weeks l a te r she was seen aga in , when he r condi t ion h a d d e t e r i o r a t e d g r ea t l y ; she a p p e a r e d ser iously ill a n d a d iagnos i s of d e r m a t o m y o s i t i s a t once s u g g e s t e d itself. The r a sh was now conf luen t on t h e face, neck , ches t and- a r m s ; t h e r e was genera l m u s c u l a r weaknes s , p r e v e n t i n g h e r f rom g e t t i n g o u t of bed ; she w a s m o s t conce rned by he r difficulty in swal lowing. She h a d v a g u e pa ins in t h e l imbs . On a d m i s s i o n to hosp i ta l t he phys ica l s igns were c lear ly r e l a t ed to t h e sk in a n d t h e m u s c u l a r s y s t e m . The r a sh had th ree m a i n f e a t u r e s a t t h i s s t a g e : (i) d u s k y pu rp le e r y t h e m a , m a i n l y on eyel ids , cheeks a n d neck ; (ii) sca ly d e s q u a m a t i o n , m a i n l y on ches t , a r m s a n d dor sa of h a n d s ; (iii) pa in fu l c racks in t h e neck folds a n d cubi ta l fossae.

The re was , in addi t ion , a h a r d b r a w n y o e d e m a , w h i c h d id no t p i t on p res su re , found m a i n l y on t h e neck , a r m s a n d th ighs . T h e musc les , w h i c h h a d all a long been the s i te of v a g u e (bu t n e v e r severe) pa ins , could no t be e x a m i n e d in de ta i l on accoun t of t he o e d e m a . The re was, as would be expec ted , t e n d e r n e s s on h a n d l i n g t h e l imbs , b u t i t could n o t be localised. T h e m u s c u l a r w e a k n e s s a n d loss of v o l u n t a r y power were r e m a r k a b l e ; a t t h e t i m e of admis s ion she could n o t ra ise h e r h e a d f rom the pil low nor m o v e a b o u t in t h e bed. She could m o v e the jo in ts o f t h e l imbs s l ight ly , b u t cou ld n o t raise t h e a r m s or legs f rom the bed.

A p a r t f rom th i s , e x a m i n a t i o n w a s essen t ia l ly n e g a t i v e a n d s u c h inves t i ga t i ons as were possible were n o t he lpfu l . Owing to the pecul ia r o e d e m a , v e n e - p u n c t u r e was a l m o s t imposs ib le , b u t t h e blood itself , i ts to ta l p ro te in c o n t e n t a n d i ts m a i n e l ec t ro ly tes were all n o r m a l in va lues . Crea t ine-crea t in ine s tud ies were n o t m a d e . The re was art i r regular f eve r (average t e m p e r a t u r e 100°F.) a n d a t a c hyea rd i a a b o u t 100 pe r m i n u t e .

On t h e fou r th i n -pa t i en t d a y cor t i sone t h e r a p y was b e g u n in t he u s u a l dosage by m o u t h . B e y o n d cont ro l l ing t h e fever , t h e d r u g h a d l i t t le effect . The re was some f ad ing of tile r ash , wh ich b e c a m e less i tchy. The o e d e m a m a y h a v e dec reased a l i t t le , b u t t h e genera l condi t ion and , in pa r t i cu la r , t h e m u s c u l a r w e a k n e s s were n o t inf luenced . W h e n 1.5 g m . h a d b e e n g i v e n in e ight d a y s it was decided to s top cor t i sone b y g r a d u a l w i thd rawa l and to s u b s t i t u t e A C T H ; before th i s h a d been done, however , t h e p a t i e n t d ied s u d d e n l y . She h a d been s leep ing qu ie t ly a t 9 a . m . a n d th ree q u a r t e r s of a n h o u r l a te r s u d d e n l y b e c a m e d y s p n o e i c a n d c y a n o s e d ; she was unab l e to t a lk a n d h e r b r e a t h i n g was e m b a r r a s s e d b y secre t ions . A f t e r t h e in jec t ion of a t rop ine a n d t h e a d m i n i s t r a t i o n of o x y g e n she a p p e a r e d m o r e comfor tab le a n d r e m a i n e d semi -consc ious all t h e m o r n i n g . A t 3.25 p . m . she s e e m e d to choke a n d resp i ra t ions sudden ly ceased .

Post Mortem. Respiratory : t r a c h e a con ta ined p u s ; basa l p u r u l e n t b ronchi t i s ; lungs showed wide-

sp read conges t ion a n d o e d e m a ; lef t p leura l c a v i t y c o n t a ined 15 ce. clear yel low fluid. Cardiovascular : s l ight h y p e r t r o p h y of lef t vent r ic le ; m i n i m a l a t h e r o m a of a o r t a ;

n u t m e g a p p e a r a n c e of l iver ; conges t ion of spleen. (;astro-inte,stinal: o e s o p h a g u s s h o w e d th i cken ing a t lower end a n d f r a g m e n t a t i o n

of m u c o s a ; s t o m a c h showed m u s c o s a l erosions. Genito-urinary : k i d n e y s conges ted . ]3rain : n o t e x a m i n e d . No o the r a b n o r m a l i t i e s were found , excep t : para -aor t ic l y m p h nodes (L. 1-2) en l a rged

to a b o u t 2 cm. , t h e c u t sur face showing necrosis sugges t i ng neop la sm. No p r i m a r y g r o w t h was f o u n d in spi te of careful searct~.

His to log ica l e x a m i n a t i o n of all o rgans showed t h e expec ted non-specific c h a n g e s conf i rming t h e gross a p p e a r a n c e s . I n add i t ion t h e following f indings were n o t e d :

Para.aortic lymph nodes : e x t e n s i v e inf i l t ra t ion b y anap las t i e carc inoma, sugges t i ve of b ronchogen ie origin. (Fig. 1).

Skin (from shoulder) L (~ig. 2) : D e r m a l col lagen fibres increased in n u m b e r , swollen a n d f r a g m e n t e d ; s u b - e p i d e r m a l capi l lar ies s u r r o u n d e d by l y m p h o c y t e s a n d p l a s m a cells. ~ p i d e r m a l c h a n g e s inc luded s o m e hype rke ra to t i c p lugging, f l a t t en ing o f t h e papi l lae , wi th s e m e l ique iac t ion and vacuo l i s a t ion of t h e basa l layer.

Muscle (from deltoid) (Fig. 3) : Muscle fibres showed loss of s t r ia t ion, oedema , a n d inc reased ce l lu lar i ty of the endo- a n d p e r i m y s i u m . Scat tered m y o c y t e s were v a c u o l a t e d a n d h a d u n d e r g o n e m a n y degene ra t i ve c h a n g e s .

P s e u d o " g i an t ceils " (Fig. 4), wh ich m a y h a v e been loci of r egenera t ing musc l e , were sca t t e red t l~roughout tile section. B o t h t h e d e r mi s and t im s u b c u t a n e o u s t i s sue showed o e d e m a wi th local collections of l y m p h o c y t e s a n d ~ l a s ~ cells.

470 I R I S H JOURNAl , OF M E D I C A L S C I E N C E

FIr~'. 1. Para-aortic lymph node showing anaplastic carcinoma. (X 200)

Fie. 2. Skin : showing flattening of the papillae, peri- capillary small round cells, para-keratotie plugging,

fragmentation of collagen. (X 45)

References. 1. Baehr, G. and Pollack, A. D. (1947). J . A . M . A . , 134, 1169. 2. Brunner, lg. G. and Lobraico, R. V., Jn. (1951). Ann. Int. Med., 34, 1269. 3. Brunsting, A. L. (1953). Proc. Xth. Int. Con2ress of D3rmcttology, 1952. Bri t .

Med. J . , ii, 127. 4. Curtis, A. C , Blaylock, H C., I-Iarrell, E. R., Jn. (1952). J . A . M . A . , 150, 844.

D E R M A T O M Y O S I T I S 471

Fic.. 3. Muscle (from deltoid) showiug disordered pat tern. (X 200)

Voluntary muscle showing pseudo-giant cells. (X 520)

FIG. 4.

5. Ferguson, B. C., Rosenbaum, J, D., and Tolman, M. M. (1952). Arch. Derm. Syph., Chicago, 65, 535.

6. Forman, L. (1952). Brit. Med. J . , ii, 911. 7. Kie~land, R. R. and Hines, E. A. (1951). Arch. Derm. Syph., Chicago, 64, 549. 8. Klemperer, P., Pollack, A, D., and Daehr, G. (1942). J . A . M . A . , 119, 331. 9. Lever, W. F. (1951). 1Vew Enff. J . Med., 245, 359.

10. Pagel, W., and Treip, G. S. (1955). J. Clin. Path., 8, I.