http://jhs.sagepub.

com
The Hand
DOI: 10.1016/0072-968X(75)90016-9
1975; 7; 179 J Hand Surg Eur Vol
MICHAEL GIPSON
Squamous Cell Carcinoma in Epidermolysis Bullosa Dystrophica
http://jhs.sagepub.com
The online version of this article can be found at:
Published by:
http://www.sagepublications.com
On behalf of:
British Society for Surgery of the Hand
Federation of the European Societies for Surgery of the Hand
can be found at: The Hand Additional services and information for
http://jhs.sagepub.com/cgi/alerts Email Alerts:
http://jhs.sagepub.com/subscriptions Subscriptions:
http://www.sagepub.com/journalsReprints.nav Reprints:
http://www.sagepub.com/journalsPermissions.nav Permissions:
1975 The British Society for Surgery of the Hand. All rights reserved. Not for commercial use or unauthorized distribution.
by on July 16, 2008 http://jhs.sagepub.com Downloaded from
Squamous Cell Carcinoma in Epidermolysis Bullosa Dysrrophica--Michael Gipson
SQUAMOUS CELL CARCI NOMA I N
EPI DERMOLYSI S BULLOSA DYSTROPHI CA
MI CHAEL GIPSON, Sheffield
The polydysplastic f or m of Epidermolysis bullosa dystrophica is a rare skin
disease characterised by multiple bullae of the skin developing spontaneously
and in response to t rauma, with a high t endency to neoplastic change usually
during the fourt h decade. The case described required bilateral upper limb ampu-
tations for squamous cell carcinoma.
CASE REPORT
A thirty-eight year old male has suffered from a life-long t endency to severe
blistering of the skin following mi nor trauma. He has been treated with steroids
(in the form of Prednisolone 15 mg. per day) for the last fourt een years and
now has steroid induced cataracts with very poor vision in his left eye. He also
suffers from severe dyspepsia causing anaemia. Bari um swallow shows a large
i nt rat horaci c stomach pouch with reflux. He needs regular antacids and periodic
blood transfussions for anaemia.
He has been treated in this unit since 1952. Until the age of twelve years
he had some fingers free and was able to grip a pen but since t hen all his fingers
have been covered by a glove of skin and scar tissue (Fig. 1). A radiograph shows
all the digits present within the mitten hand (Fig. 2). He also has similar changes
in his feet.
In May 1952 his right t humb was separated and grafted and in July 1952
the fingers of his right hand were similarly freed (Fig. 3). In September 1952
function in this hand was satisfactory and t reat ment to the left hand was planned.
Fig. 1.
Fig. 2.
Fig. 3.
Right arm showing fingers covered by glove of skin and scar tissue.
X-ray showing all digits present within mitten hand.
Result after separation and grafting of the right thumb and fingers (1952).
The Hand---Vol. 7 No. 2 1975 179
1975 The British Society for Surgery of the Hand. All rights reserved. Not for commercial use or unauthorized distribution.
by on July 16, 2008 http://jhs.sagepub.com Downloaded from
Squamous Cell Carcinoma in Epidermolysis Bullosa Dysrrophica--Michael Gipson
The patient, however, did not ret urn for furt her t reat ment , as the condition of
the right hand ret urned to its pre-operative state in about a year. The patient
and his parents both felt surgery was pointless.
In 1967 he developed a squamous cell carci noma of the left hand. Initially
he refused operation but eventually agreed when the t umour had extended almost
to the elbow (Fig. 4). In April 1968 amput at i on of the left arm was performed
t hrough the mid-humerus. At this stage he was sent to a limb fitting centre where
he was given a prosthesis for his left humeral stump and in addition a powered
prosthesis fitting over his right mi t t en hand.
In January 1973 he presented with a suspicious area on his right forearm
and hand, biopsy of which confirmed that it was a squamous cell carci noma
(Fig. 5). In March 1973 the right hand was amput at ed t hrough the wrist joint.
The histological report showed that the margin of the specimen contained carci-
noma and in April 1973 re-amput at i on was performed 1 inches above the wrist
joint. His amput at i on stumps are now well healed and he shows no evidence to
date either of recurrence of squamous cell carcinoma or of any new lesions
(Fig. 6).
FAMI LY HISTORY
There is no previous history of this condition in this patient' s family. This
is consistent with the diagnosis of Epdermolysis bullosa dystrophica (Polydysplastic
form) which is of recessive inheritance whereas the hyperplastic form is dominant.
PERSONAL HISTORY
The patient lives at home with his parents. He is completely independent
for all his personal needs in dressing, washing, feeding and for toilet purposes.
He drives a small automatic, left hand drive car using his right prosthetic hand
for steering and to control the gear lever and hand brake.
He works at home as a hypnotherapist and a typist. He started as a hypno-
therapist at the suggestion of the Professor of Psychiatry who met him at a
clinical demonst rat i on in Sheffield eighteen years ago and who then trained him
in his depart ment for two years. He now treats patients for a variety of condi-
tions including phant om limb pains in amputees (some being provided for his
t reat ment when he himself attends the limb fitting centre in Oxford), addiction
Fig, 4.
Fig. 5.
Left hand with squamous cell carcinoma extending to the left elbow (1967).
Showing squamous cell carcinoma of right forearm and hand (1973).
180 The Hand--Vol. 7 No. 2 1975
1975 The British Society for Surgery of the Hand. All rights reserved. Not for commercial use or unauthorized distribution.
by on July 16, 2008 http://jhs.sagepub.com Downloaded from
Squamous Cell Carcinoma in Epidermolysis Bullosa Dysrrophica--Michael Gipson
Fig. 6.
Fig. 7.
Recent photograph showing no evidence of recurrent carcinoma.
Recent photograph showing right powered prosthesis and butterfly lever activated by
left stump, visible on left chest wall.
to cigarettes and alcohol, nail-biting, examination nerves, some phobias (e.g.
agoraphobia), some forms of depression and lack of confidence in actors. For
typing (manuscripts and theses mainly) he uses his powered right artificial limb
and a short non-powered left prosthesis and can type at forty-five words per
minute.
Most of the time he wears only his powered right prosthesis. This being
the most useful as he still has his own elbow flexion. The compressed air power
is controlled by a butterfly lever on his left chest activated by his left arm stump
to open and close a split ring (Fig. 7).
He rarely uses the full left arm prosthesis as it is very cumbersome. Thr ee
press but t on levers on the right hip are activated by the right elbow to flex the
elbow, rot at e the wrist and open and close the split ring.
He wears special boots for his deformed feet. The boots are l eat her with
zip-up fronts and plastazote insoles.
The patient is quite aware of the possibilities of furt her malignancies and
the chances of developing lesions on his feet which may necessitate furt her
amputations.
DI SCUSSI ON
Epidermolysis bullosa was first described by Von Hebra in 1870. It is a
rare condition classified into four main types: simplex, dystrophica (hyperplastic
and polydysplastic types) and lethalis.
Kitlowski and Banfield (1948) described the completely ensheathed digits
and the rapid recurrence of their original position aft er surgery as occurred in
the case described here. Rees and Swinyard (1967) also described t reat ment of
the pseudosyndactyly and comment ed on the t endency for the skin lesions to
occur in the graft ed skin subsequent to surgery. Pers (1965) described a fourt een
year old boy with both hands ensheathed in a mitten of epidermis whose fingers
The Hand--Vol. 7 No. 2 1975 181
1975 The British Society for Surgery of the Hand. All rights reserved. Not for commercial use or unauthorized distribution.
by on July 16, 2008 http://jhs.sagepub.com Downloaded from
Squamous Cell Carcinoma in Epidermolysis Bullosa Dysrrophica--Michael Gipson
were f r eed surgi cal l y al l owi ng spont aneous epi t hel i al r egener at i on wi t h gr af t i ng
to some r aw ar eas on t he vol ar aspect s of t he fingers. I t is obvi ous t her ef or e
t hat t he pseudosyndact yl y is amenabl e to t r eat ment .
The pr obl em of t r eat i ng t he squamous cell c a r c i noma t a whi ch devel op
c ommonl y in t he f our t h decade is mor e difficult. I n t he case descri bed her e t hese
lesions wer e all di f f er ent i at ed and al t hough t hey r equi r ed mut i l at i ng amput at i ons
this par t i cul ar pat i ent , whilst r el uct ant to agr ee t o sur ger y at first, has obvi ousl y
adapt ed well to his disability, ma ki ng t r e a t me nt ver y much wor t hwhi l e.
Cri kel ai r, Hoehn, Domonkos and Bi nker t (1970) descri bed a pat i ent aged
f or t y- si x year s who had amput at i on at t he mi d- humer al l evel f or s quamous carci -
noma of t he l ef t el bow, who also had r adi ot her apy over a t en year peri od f or a
basal cell lesion of t he l eft t hi gh and who had a not he r squamous l esi on of t he
ri ght leg exci sed and gr af t ed. Six mont hs l at er t he same pat i ent had devel oped
t wo f ur t her s quamous lesions on t he ri ght leg and r i ght a r m and l at er f ur t her
new pr i ma r y skin mal i gnanci es appear ed. Despi t e t hese ext ensi ve mal i gnant
lesions t he pat i ent ' s deat h aged f or t y- ei ght year s appear ed to be due to pr obl ems
concer ned wi t h t he ext ent of t he ski n i nvol ved ( r equi r i ng ski n homogr af t s f r om
his mot he r due to i nabi l i t y to find sui t abl e donor ar eas on t he pat i ent hi msel f)
r at her t han t he mal i gnanci es t hemsel ves.
S U M M A R Y
A case of Epi der mol ysi s bul l osa dyst r ophi ca (pol ydyspl ast i c t ype) is descri bed.
Bi l at er al upper l i mb amput at i ons wer e pe r f or me d f or s quamous cell car ci nomat a
and t r e a t me nt of t he pseudosyndact yl y and mal i gnant lesions is discussed.
A C K N O W L E D G E M E N T S
I shoul d l i ke t o t ha nk Mr . Dougl as Campbel l Rei d f or al l owi ng me t o t r eat
this pat i ent , and t he phot ogr aphi c depar t ment of t he Uni t ed Sheffield Hospi t al s
f or t he i l l ust rat i ons.
R E F E R E N C E S
CRIKELAIR, G. F., HOEHN, R. J., DOMONKOS, A. N. and BINKERT, B. (1970). Skin
Homografts In Epidermolysis Bullosa Dystrophica. Case Report. Plastic and Reconstruc-
tive Surgery. 46" 89-92.
KITLOWSKI, E. A. and BANFIELD, E. E. (1948). Epidermolysis Bullosa Hereditaria. Report
of 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 SWINYARD, C. A. (1967). Rehabilitative Digital Surgery in Epidermolysis
Bullosa. Plastic and Reconstructive Surgery. 40: 169-174.
182 The Hand--Vol. 7 No. 2 1975
1975 The British Society for Surgery of the Hand. All rights reserved. Not for commercial use or unauthorized distribution.
by on July 16, 2008 http://jhs.sagepub.com Downloaded from