SCC Amputation and Prosthesis

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The Hand

DOI: 10.1016/0072-968X(75)90016-91975; 7; 179J Hand Surg Eur VolMICHAEL GIPSON

Squamous Cell Carcinoma in Epidermolysis Bullosa Dystrophica

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Squamous Cell Carcinoma in Epidermolysis Bullosa Dysrrophica Michael Gipson

S Q U A M O U S C E L L C A R C I N O M A I NE P I D E R M O L Y S I S B U L L O S D Y S T R O P H I C

M I C H A E L G I P S O N , S h e f f i e l dThe po lydysp las t i c fo rm o f Ep ide rmolys i s bu l losa dys t roph ica i s a r a re sk ind i sease cha rac te r i sed by mul t ip le bu l l ae o f the sk in deve lop ing spon taneous lyand in r e sponse to t r auma , wi th a h igh t endency to neop las t i c change usua l lyd u r i n g t h e f o u r t h d e c a d e . Th e c a s e d e s c r i b e d r e q u i r e d b i l a t e r a l u p p e r l i mb a mp u -ta t ions fo r squamous ce l l ca rc inoma .

C SE REPORTA th i r ty -e igh t yea r o ld ma le has su f fe red f rom a l if e - long t end ency to seve reb l i s t e r ing o f the sk in fo l lowing minor t r auma . He has been t r ea ted wi th s t e ro idsin the fo rm o f P redn i so lone 15 mg . pe r day) fo r the l a s t fou r teen yea r s andnow has s t e ro id induced ca ta rac t s wi th ve ry poor v i s ion in h i s l e f t eye . He a l sosu f fe r s f rom seve re dyspeps ia caus ing anaemia . Ba r ium swa l low shows a l a rgein t ra tho rac ic s tomach pouch wi th r e f lux . He needs r egu la r an tac ids and pe r iod icb lood t r ans fuss ions fo r anaemia .He has been t r ea ted in th i s un i t s ince 1952 . Un t i l the age o f twe lve yea r she had some f inge r s f r ee and was ab le to g r ip a pen bu t s ince then a l l h i s f inge r shave been cove re d by a g love o f sk in and sca r ti s sue F ig . 1 ). A ra d iogr aph showsa l l the d ig i ts p re sen t wi th in the m i t t en han d F ig . 2) . He a lso has s imi lar changesin h is fee t .In May 1952 h i s r igh t thumb was sepa ra ted and g ra f t ed and in Ju ly 1952the f ingers of h is r ight han d were s im ilar ly f ree d Fig . 3) . In Se pte mb er 1952func t ion in th i s hand was sa t i s f ac to ry and t r ea tmen t to the l e f t hand was p lanned .

Fig. 1.Fig. 2.Fig. 3.Right arm showing fingers covered by glove of skin and scar tissue.X-ray show ing all digits present w ithin mitten hand.Result af ter separation and grafting of the right thumb and fingers 1952).

The Hand Vol. 7 No. 2 1975 179

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Fig. 6.Fig. 7. Rece nt photograph showing no evidence of recurrent carcinoma.Re cent photograph show ing right powered prosthesis and butterfly lever activated byleft stump, visible on left chest wall.to c iga re t t e s and a lcoho l , na il -b it ing , exam ina t ion ne rves , some phob ias e .g.agoraph ob ia ) , some fo rm s o f depress ion and l ack o f conf idence in ac to r s . Fo rtyp ing manu sc r ip t s and theses ma in ly ) he uses h i s pow ered r igh t a r t if i c i al l imband a shor t no n-po wer ed l e f t p rosthes i s and can type a t fo r ty - f ive words pe rmi n u t e .Mos t o f the t ime he wears on ly h i s powered r igh t p ros thes i s . Th i s be ingthe mos t use fu l a s he s t i l l has h i s own e lbow f l ex ion . The compressed a i r poweri s con t ro l l ed by a bu t t e r f ly l eve r on h i s l e f t ches t ac t iva ted by h i s l e f t a rm s tumpto open an d c lose a spli t r ing Fig . 7) .He ra re ly uses the fu l l l e f t a rm p ros thes i s a s i t i s ve ry cumbersome . Threepress bu t ton l eve r s on the r igh t h ip a re ac t iva ted by the r igh t e lbow to f l ex thee lbow, ro ta te the wr i s t and open a nd c lose the sp l i t r ing.He wears spec ia l boo t s fo r h i s de fo rmed fee t . The boo t s a re l ea the r wi thz ip -up f ron t s and p la s tazo te in so le s .The pa t i en t i s qu i t e aware o f the poss ib i l i t i e s o f fu r the r ma l ignanc ie s andthe chances o f deve lop ing l e s ions on h i s f ee t wh ich may necess i t a t e fu r the ra mp u t a t i o n s .

D I S U S S I O NEpidermolysis bul losa was f i rs t descr ibed by Von Hebra in 1870. I t i s ara re c ond i t ion c la ss i fi ed in to fou r ma in types : simplex , dys t roph ic a hype rp la s t i cand po lydysp las t i c types ) and l e tha l is .Ki t lowsk i and Banf ie ld 1948) desc ribed the com ple te ly enshea thed d ig i t sand the r ap id r ecu r rence o f the i r o r ig ina l pos i t ion a f t e r su rge ry a s occur red inthe case desc r ibed he re . Rees an d Swinya rd 1967) a l so desc r ibed t r ea tm en t o ft h e p s e u d o s y n d a c t y l y a n d c o mm e n t e d o n t h e t e n d e n c y f o r t h e s k i n le s io n s t oocc ur in the g ra f t ed sk in subsequen t to su rge ry . Pe r s 1965) desc r ibed a fou r tee nyea r o ld boy wi th bo th hands enshea thed in a mi t t en o f ep ide rmis whose f inge r sThe Hand Vol . 7 No. 2 1975 181



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w e r e f r e e d s u r g i c a l ly a l lo w i n g s p o n t a n e o u s e p i th e l ia l r e g e n e r a t i o n w i t h g r a f t i n gt o s o m e r a w a r e a s o n t h e v o l a r a s p e c t s o f t h e f i n g e r s . I t i s o b v i o u s t h e r e f o r et h a t t h e p s e u d o s y n d a c t y l y is a m e n a b l e t o t r e a t m e n t .T h e p r o b l e m o f tr e a t in g t h e s q u a m o u s c e ll c a r c i n o m a t a w h i c h d e v e l o pc o m m o n l y i n t h e f o u r t h d e c a d e i s m o r e d i f f i c u l t . I n t h e c a s e d e s c r i b e d h e r e t h e s el e si o n s w e r e a ll d i f f e re n t i a t e d a n d a l t h o u g h t h e y r e q u i r e d m u t i l a ti n g a m p u t a t i o n st h i s p a r t i c u l a r p a t i e n t , w h i l s t r e l u c t a n t t o a g r e e t o s u r g e r y a t f i r s t , h a s o b v i o u s l ya d a p t e d w e l l t o h is d is a b il it y , m a k i n g t r e a t m e n t v e r y m u c h w o r t h w h i l e .C r i k e l ai r , H o e h n , D o m o n k o s a n d B i n k e r t ( 19 7 0 ) d e s c r ib e d a p a t i e n t a g e df o r t y -s i x y e a r s w h o h a d a m p u t a t i o n a t t h e m i d - h u m e r a l l ev e l f o r s q u a m o u s c a r c i -n o m a o f t h e le f t e lb o w , w h o a l so h a d r a d i o t h e r a p y o v e r a te n y e a r p e r i o d f o r ab a s a l c el l l e si o n o f t h e l e f t t h i g h a n d w h o h a d a n o t h e r s q u a m o u s l e s i o n o f th er i g h t le g e x c is e d a n d g r a f t e d . S ix m o n t h s l a t e r t h e s a m e p a t i e n t h a d d e v e l o p e dt w o f u r t h e r s q u a m o u s l e s i o n s o n t h e r i g h t l e g a n d r i g h t a r m a n d l a t e r f u r t h e rn e w p r i m a r y s k i n m a l i g n a n c i e s a p p e a r e d . D e s p i t e t h e s e e x t e n s i v e m a l i g n a n tl e si o n s t h e p a t i e n t ' s d e a t h a g e d f o r t y - e i g h t y e a r s a p p e a r e d t o b e d u e t o p r o b l e m sc o n c e r n e d w i t h t h e e x t e n t o f t h e s k i n i n v o l v e d ( r e q u i r in g s k i n h o m o g r a f t s f r o mh i s m o t h e r d u e t o i n a b i l i t y t o f i n d s u i t a b l e d o n o r a r e a s o n t h e p a t i e n t h i m s e l f )r a t h e r t h a n t h e m a l i g n a n c i e s t h e m s e lv e s .

S U M M R YA c a s e o f E p i d e r m o l y s i s b u l l o s a d y s t r o p h i c a ( p o l y d y s p l a s t i c t y p e ) is d e s c r i b e d .B i l a te r a l u p p e r l im b a m p u t a t i o n s w e r e p e r f o r m e d f o r s q u a m o u s c el l c a r c i n o m a t aa n d t r e a t m e n t o f t h e p s e u d o s y n d a c t y l y a n d m a l i g n a n t l e si o n s is d i sc u s se d .

C K N O W L E D G E M E N T SI s h o u ld l i k e to t h a n k M r . D o u g l a s C a m p b e l l R e i d f o r a l lo w i n g m e t o t re a tt h is p a t i e n t , a n d t h e p h o t o g r a p h i c d e p a r t m e n t o f t h e U n i t e d S h e ff ie ld H o s p i t a l sf o r t h e i l l u s t r a t i o n s .

R E F E R E N C E SCR I KE L A I R, G . F . , HOE HN , R . J . , DOM O NKO S, A . N . a nd BI N KE RT , B . (1970) . Sk inHomografts In Epidermolysis Bullosa Dystrophica. Case Report . Plastic and Reconstruc-tive Surgery. 46 89-92.KI TL OW SK I, E. A. and BA NF IEL D, E. E. (1948). Epidermolysis Bullosa Hereditar ia . Re portof a Case with Unusual Associated Deformities. Plastic and Reconstructive Surgery. 3:481-488.PERS, M. (1%5). Skin Grafting in a Case of Epidermolysis Bullosa. Acta Chirurgica Scandi-navica. 129: 333-339.REES, T. D . and S W INY AR D, C. A. (1967). Rehabil itative Digital Surgery in EpidermolysisBullosa. Plastic and Reconstructive Surgery. 40: 169-174.

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SCC Amputation and Prosthesis