You are on page 1of 9

74 LEPROSY ReVIEW

CLASSIFICATION OF LEPROSY
R. CHAUSSINAND,
Institu t Pasteur, Paris, Xv.

S ince 1 93 1 , that is to say since we have specialised in leprosy,


m uch i n k has flowed on t he problem of t he classification of leprosy
and without m uch success, for leprologists are not yet able or w i l l i n g
to agree on one classification that m ight at l ast b e ado pted b y a l l.

I. Primary Classification
At the present moment there are fo ur primary classifications i n
existence, which are more or less widely accepted. They are : the
classifications of the 1 st W H O Ex pert Com mi ttee on leprosy and of
Madrid which o n l y differ i n a few detai ls ; the I n dian classificat ion;
the classification worked out by t he J a panese leprologists; and
finally Cochrane's classification . N eedless to say, we have n o i n ten­
tion of proposing a fifth.
We are of the o p i n i o n , with Ross I NNES 5 that the W H O and
M adrid classifications are acceptable, i n spite of several i m per­
fections. They seem to us to be markedly clearer and more practical
than the others. What are the criticisms that are most freq uently
levelled at them ?
First of all, the nomenclature used i n the primary classification
is not unanimo usly accepted. Thus although the ex pression "tubercu­
loid leprosy " is used by the vast majority of leprologists, D AVISON
and his co-workers 3 have j ust recently p roposed its suppression, o n
the ground that the histol ogical structure characteristic o f t h i s form
is transient. This objection does not a ppear to us to be val id. The
exact classification of a patient ought to be made on his admission
to anti leprosy treatmen t and it i s n o t perm issible to modify this
classification "a posteriori", -solely. beca use hi stological changes
h ave intervened i n regressive lesions:
I t has been fully established that t he- hist9pathology of ,the
cutaneous lesions of t u berculoid a nd lepromatous patients is
gradually m odified, and before the cure is comp lete it is possible to
detect the pict ure of a n ordinary n on-specific chronic inflammation .
It would be a grave error to try at this stage to classify these patients
as i ndeterm i n ate leprosy (as we saw certai n lepmsy services doing),
makin g the clai m that t he histology of their lesi ons -1S analogous
'
with that of the pathol ogical changes that take place in i n deter­
minate leprosy.
I t is obvious that i t is n o t possible to reclassi fy a tuberculoid or
lepromatous patient as i n determi n ate i f the only reason for doi n g
s o i s that t he histology o f t he regressive skin lesions shows the
pictu re of ordinary chronic inflammati o n .
CLASSIFICAllON or LEPROSY 75

W h i le thc term " Ieproma tou " i s u n i versa l l y accepted, the word
"i ndetermi na te" has been strongly cri t i c i sed . We ca nnot understand
why it should be considered useless i n leprosy classificat i o n . S i nce
the descri pt i on s " t u berculoid leprosy" and "lepromatous leprosy"
are terms based on h i stological data, the ex pression " i ndeterminate
leprosy" seems to us to be correct and i n te l l igi ble, for i t is based
just as much on h i stologica l observations. A case of indeter m i n ate
leprosy is a pat ient present ing the i ndis putable cl i n ical signs of
leprosy, but whose lesi ons show the h i stological pict ure of an
ordi nary chron ic inflammation . T h i s picture may be cal l ed " i ndeter­
min ate" if one takes i nto acco unt the more disti nctive "determ i n ate"
h i sto l ogy of tuberc u l oid lesions and, more markedly, of lepromatou s
lesions . Furthermore the defin ili on " i n determ i n ate" impl ies t h a t w e
are dea l i ng w i t h a freq uently unstable fo rm. O n the other hand we
feel there is 110 profit il1 describing indeterminate leprosy as a "group"
(Ma dr id class(fication).
It is in fact a cl inic ally deined initial ''form''
of the disease which may either remain unchanged or evolve in the end
into one of the other two forms.
1 t w o u ld be u n fort unate to use, as the Indian leprologists w i s h
to do, histological defin itions for the t u berculoid a n d lepromatous
forms and the clinica l defin ition of maculo-anaest het ic leprosy for
the in determinate form. And the mo re so s i nce certa i n s k i n lesions
of t u berculoid leprosy, and somet i mes even lepromatous ones, may
eq ually wel l be descri bed c l i n ically as mac u l o-anaesthet ic.
We feel that the terms " t u berculoid", " i ndeterm i n ate", and
" lepromatous" ought to be retai ned i n the primary classificati o n o f
leprosy. They a r e al ready k n own and accepted by t he maj ority of
lepro logists and it seems u n l i kely to u s that s i m ple and easily u nder­
stood cli n ical defi n i t ions could be fo und to replace s uccessfu l l y the
present h i stologically based terms . One might all the same wonder if
we ought to reserve a plac e for borderline leprosy in the primary
classification. Personal ly we consider· bo rderl i ne leprosy to be an
u nstable variety of the t u berculdid form capable either of regressi n g
to the major t u b erculoid -variet y or of evol v i n g i n to t he lepromatous
form. To u s i t see ms hardly necessary to i ncl ude i t i n the pri mary
classi ficat i o n . RAMOS E S I LVA4 tries to resolve the diffic u l ty by
dividing borderline leprosy i nto two gro u ps, one predo m i nantly
t u berc u l oid and the other predomi nantly lepromatou s . I t seems to
us that i t i s rather �iffic u l t to make this distincti o n i n a pri mary
classificat i o n . We t h i n k it wou l d be preferable to cons ider borderline
'
leprosy, as long as i t remains really " borderline", as an u nstable
variety of t u berculoid leprosy and so bei n g l ogically p laced i n the
secondary classification .
H owever the W H O and M adrid classifications and also those
reco mmended by the I ndian le pro logists and by COCHRANE i ncl ude
borderline leprosy in the primary classification . We feel t hat this i s
76 LEPROSY REVIEW

an illogical proced u re but �t rictly speaki n g i t is adm issi ble, since it


does not cause any confusion when class ify i n g pat ients. On the other
hand we cannot allow borderline leprosy to be included in the differen t
classifications under completely differen t names. T h u s both the Latin­
American leprologists and Coch rane prefer the terms "dimorfo" or
"dimorphous", w illIe the Indian and Japanese leprologists use
"intermediate" and "atypical". This would not matter much i f all
the terms had exactly the same mea n i n g, but u n fortunately this i s
not the case .
Borderl ine leprosy is descri bed by WADE as an unstable inter­
mediate stage between major tuberculoid leprosy and lepromato us
leprosy, and capable of regressing towards major tuberculoid
leprosy, from which it derives, or of evolving towards the lepro­
mato us fo rm . N ow although the M adrid classification adm its this
defin ition and gi ves eaxct ly the same meaning to the word "dimorfo",
Cochrane gro u ps under the heading "dimorphous" not only Wade's
"bo rderli ne" cases, but also patients in the intermed iate p hase
between the clin ical begi n n i n g of leprosy, w h ich is a lways, according
to this author, potentially lepromatous (we certainly do not s hare
t h i s opinion) and tuberculoid leprosy I n the I ndian and Japanese
classifications the borderline cases are put together with cases of
indeterminate leprosy in a gro u p called respecti vely " i ntermediate"
and "atypical".
I t i s evident that a u n i q ue word i s necessary for an international
classification and the most appropriate term , one w illch avoids
confusions d uring the classification is Wade's term "borderli ne"
u n less t he word "dimorphou s " be henceforth used only as a synonym
of the word "border l ine".
Certain authors describe borderl i ne Jeprosy as " bipolar", basing
their description o n R ABELLO who considers t he tuberculoid and
lepromatou s forms as the " polar" types of the disease . B ut in
geography the north pole never changes into the south pole, and
eq ually in electricity the positive and negative poles are not inter­
changeable. Thus the description "polar types" w h ich Rabello gives
to the tuberculoid and lepromato us forms of leprosy seems to us to
be very questionable si nce poles are immutable. Now it is no longer
possible to clai m that tuberculoid leprosy i s an i m mutable form
which never evolves towards lepromatous leprosy. The polar con­
ception of leprosy and, t herefore, the expression "bipolar", o ught
to be abandoned. * It w o u ld be more logical to s ubstitute the word
"extreme" for " polar", the tuberculoid and lepromatous forms of
leprosy being thus defined as the two extreme types of the disease
But we do not appro ve of the inclusion, proposed by Wade and by the
Indian leprologists, of a pure polyneuritic form in the primary classifi-
• Certain BraziHan authors even use the adjective "infrapoJar" to describe
i ndeterminate leprosy.
CLASSIFICATION OF LEPROSY 77

cation. We would then have in the same gro u p pa tients with


t u berc u loid o r indeterm inate l e prosy, as wel l as lepromatous cases
who o n l y show polyneuritic lesions since their cutaneous lesions
have disa ppeared . It is i nconcei vable t h is gro u p should be given a
place in the primary classificat ion s ince t h i s classification has the
precise object of defini n g the pri ncipal forms of t he disease with a
v iew to an orderly scientific classification of pat ients. And for the
rest, t h is procedure i s hardly to be recom mended fro m a c l in ica l
point of view si nce the prognosis and the necessary duration of
treatment differ greatly fo'r tuberc u loid, i ndeterminate and lepro­
matous pat ients.
We k n ow t hat it is sometimes difficult to classify correctl y
pat ients who have pure po lyneuritic leprosy, b u t t h is is a rare
occ urrence. A positive M itsuda reaction permits us to exclude the
lepromatous form, and if i t is strongly positive a l l ows us to assert
t hat we are dealing wit h a t u berc uloid leprosy . A wea k l y positive
lepromin reaction , however, indicates rather an i ndeterminate
leprosy, especia l l y in patients with a moderate and even hypert rophy
of nerve t r u n k s . As for subjects insensitive to lepro m i n , i ndeter­
mi nate leprosy is probably w hat exists, unless the cutaneous
stigmata o r alopecia of the eyelashes ind icate that we have a lepro­
matous pat ient w hose cutaneous lesions have disappea red . ]n fact
pure nerve l eprosy i n lepromatous cases probably does not exist, or,
i f i t does, remains purely neu ritic only for a short time si nce the skin
i s rapidly i n vaded by M . /eprae i n this form of the disease.
I n very rare cases w h ich cannot be classified by a res u l t of
c l i n ical methods and the result of the lepro m i n reaction, the classifi­
cat ion is hel ped by the histological exam ination of a small biopsy
taken of a swol len nerve . I n this way we were able to establish a
diagnosis of t u berc u l oid leprosy in three lepromin-positive patients
who showed only a single uni lateral facial paralysis with a m ild
hypertrophy of one or of severa l cervical nerves . These biopsies had
absolutely no harmfu l consequences. I n two of these pat ients treat­
ment w i t h diamino-diphenylsul phone brought only a s l ight improve­
ment, but in the third the facial paralysis had p racti ca l l y disappeared
after 1 1 months of treatment.
We think that patients with polyneuritic leprosy, whether pure
or secondary, ought to be classified u n der one of the three forms of
leprosy-tuberculoid , indeterm inate, or lepromatous . In case of
doubt t he patient co uld be placed provisionally in the group that
seems t he most l i kely one, but with a q uestion m ark until the
classification has been confirmed or rejected by a histological
examinat i o n .
The adoption of a binary classification which covers t h e primary
classification could be achieved by using, in their biological sense,
the terms "benign" and "mal ignant". In o u r opinion this binary
78 LEPROSY REVIEW

classification has above all the adva ntage of avoiding t he con t i n ual
repetition of the words "tuberculoid", "indeterminate", and
"lepromato us" i n the l i teratu re. However, t h i s division might not
fit exactly in the case of borderl ine l eprosy, though in fact t h i s
u n stable variety can n ot be called biologically malignant u n t i l i t has
defi n itely evolved towards the lepromatou s form . The terms ben ign
and malign ant seem to u s to be preferable to lepromatou s and non­
lepromatous, proposed by certain authors.
On the other hand we do not advocate the use of the terms
" open" and "closed!' for the classificat ion of leprosy patients. These
terms would be grammatical ly acceptable i f t hey were used as
follows : "open" to mean that the nasal m ucosa i s positive or that
the cutaneous lesion i s ulcerated, and "closed" to mean t hat the
nasal m ucosa i s negative and the cutaneous lesion is n o n-u lcerated .
H owever, at present we find i n the "open" gro u p patients with only
very few bacilli i n non-ulcerated cutaneous lesions and also patients
with strongly bacilliferous nasal m ucosa and cutaneo us lesions, and
this seems to us u n desirable.
The majority of leprologists consider that patients with few
bacilli and negative nasal mucosa can to all i n tents and purposes be
described as non-contagious. A l I such cases would thus be classified,
u nder t he present system , as "open" and so are subject to t he some­
times irritat i n g adm inistrati ve con seq uences of this designation .
One could perhaps u se for t he A dmin istrati o n in place of the
terms "open" and "closed" the following expressions, which would be
more easily understood by the non-medical : ( i n French, "conta­
gieux") contagious (wi t h positive nasal m u cosa or h ighly bacilliferous
c utaneo us lesions, above all when u lcerated) ; ( French, " presume
n on-contagieu x") presumed non-contagious (with negative nasal
m u cosa, few bacilli in n on-ulcerated cutaneous lesions-); ( French,
" n on-contagieu x") non-contagious (nega�ive to bacterial examina­
tion).

IT. Binary Classification


I n order t hat it m ight be u niversally accepted the binary classifi­
cation should be simple and based principally on clinical observa­
tion . The most elementary classification would thus be to s ubdivide
each of the three forms of leprosy into "cutaneous", "neuritic", and
"cutaneous-neuritic". But t he usefu l n ess of a more detailed classifi­
cation is u ndeniable. A n d thus it is necessary to attempt to defin e
the different varieties of t he forms of leprosy.
B ut it should always be borne in mind that t here are certa i n
intermediate a n d transitory stages t hat exist between different forms
and even between certain varieties of leprosy, and which can some­
times be detected only by h i stological examjnatio n . In our opi nion
'
these intermediate stages can not be considered as varieties as we
C LASSIFICATION OF LE PROSY 79

describe t hem, and they o ught not, except fo r borderline leprosy,


to be taken into acco unt i n t he bi nary classificatio n . S i m i larly the
react ional states, whether of l o n g or short duration, which alter, for
good or for i l l , the normal course of t he disease. ca nnot be classified
as different varieties. The use of t he terms " pretu berculoid", "tuber­
culoid reaction", "tuberculoid reactional transformation", "pre­
lepromatous", "lepromatous reaction" and "nodular erythema" will
perm it us to describe t hese transitory stages of the disease.
The disti nction between the different varieties of leprosy i s
essential l y grou nded o n t h e clin ical aspect o f t h e cutaneous lesions,
except of course for the purely neuritic cases.

Tuberculoid Leprosy
According to the M adrid classification this form of leprosy i s
divided, from t he cutaneous aspect, i n t o the three varieties "macu­
lar" , " m i nor" and "major" . We would add to this l ist borderli n e
leprosy.
One may wonder whether t here is any profit in considering pure
macular t u berculoid leprosy as a true variety . (We would mention
t hat in this article we are using t he terms "macule" and "macular"
in their strict dermatological sense.) In fact it is rare for an u ndoubted
case of tuberculoid leprosy to show only typical macular changes.
A carefu l clin ical exa m i n ation generally allows us either to detect a
very mild infiltration or to recognise p reviously-i n fi l trated tubercu­
loid lesions t hat are now regressing. Besides, the most of the strictly
macular erythematous lesions which are included in this variety
exceptionally prove to be purely tuberculoid. They are, more often
than not, pretuberculoid or even prelepromatous indeterminate
lesions, whose exact nature can often only be determined by bacterio­
l ogical or-histological methods.
As for t he term inology, " m acular" is a descriptive word, whereas
"mi n or", "major", and " borderline" i ndicate different degrees of
the infect i o n . So if we wish to i nclude this v ariety in the binary
classificati o n it wo uld be preferable to replace the word "macular"
by a more appropriate term. The adjective "atypical" m ight be suit­
able, since the infiltration, absent from the macule, is one of the
princi pal c l i ni cal characteristics of the tuberculoid cutaneous lesions.
Finally we prefer the term " major tuberculoid" to "reactional
t uberculoid", for the l atter is often confused with the expression
"tuberculoid reaction" by doctors unfamiliar with leprology.
We thus have the following list of varieties of t uberculoid
leprosy :
Tuberculoid leprosy
atypical (macular, well-defined) (?)
minor (micropapular, well-defined)
major (in filtrated, in a plaque or a ring, well-defined)
80 LEPROSY REVIEW

borderline (more or less infiltrated, i n a plaque or a ring, ill-defined) .


One may however object to this method of classification which is
based principally on the degree of t he i n fection while t he terminology
at present used to describe the varieties o � the lepromato us form is
certainly cli n ically descriptive.

Indeterminate leprosy.
I n this form of leprosy t here are, from the cutaneous point of
view, no varieties, since all the lesions are strict ly macular. At most
one might make distinctions on the gro unds of colour. But these
lesions are al most always hypo pigmented.
As for erythematous macules, bacteriological and above a l l
histol ogical methods reveal t hat w e a r e most often dealing with
indeterminate pretubercu loid, or even prelepromatous lesions.
Finally hyperpigmented macules are extremely rare.

Lepromatous leprosy.
In lepromato us leprosy there are i n rea lity o nly two cutaneous
varieties : lepromatous l eprosy with figurate lesions and diffuse
lepromatous leprosy.
H owever, we may find cases, of ordinary recent lepromatous
leprosy, with nothing but figurate lesions of the same type. It w i l l
therefore b e of u s e for the prognosis and fo r the assessment of
therapeutic res ults to classify such patients i n a more precise way.
To do this we m ight subdivide the variety "figurate" i n to "papular",
"macular", " nodular", and " i nfiltrated".
But s uch patients (that i s showing skin lesion s a l l of the same
type), are relatively rare. M ost lepromato us patients have, i n varying
proportions, skin lesions of widely differing types. And this sub­
division could only be appl ied to them with difficulty. But one could
then specify that a certa i n type of lesion is "predo m i nati ng" .
We may thus l i st the fol lowing binary classification for lepro-
matous leprosy:

}
Binary Classification
Lepromatous Leprosy
figurate diffuse
papular
macular "pure" or
nodular "predominati ng"
.
infiltrated
The essence of this study of leprosy classification is summarised
in Table I which is appended. So as not to overload the scheme we
have not mentioned the bacteriological, immunological, and histo­
logical featu res of the different varieties and forms of leprosy.
Besides, these features are not n ow in question .. Those varieties
81 LEPROSY R EVIEW

which do not seem to be absol utely i ndispensable h ave been mar ked
with a q uestion mark.

Conclusion
An acceptable classification of leprosy co uld be ra pidly decided
on if leprologists would agree to remove from cons ideration certain
regional or personal preferences, to which i t is hard to attach any
real i m portance . And this res ult could be achieved easily s i nce n o
doctrinal differences exist in clin ical, i m m u n ological, or histological
as pects . T t is high time that we attained s uch a res ult for it is hard
to bel ieve that only a few years from the 90t h A n n i versary of the
di scovery of the baci l l u s by A R M A U E R H A NSEN, leprologi sts are st i l l
searchi ng for an acce ptable classification o f leprosy.

Acknowledgement
We are very gratefu l to Dr. J . Ross Innes for arranging for the
translation i nto- English of this article, which was s u b m i tted i n
French.

References
1. CHAUSSINAND, R . , DESTOMBES, P. et BOURCART, N. , "Transformation en lepre
tu beICuloide de deux cas de lepre i ndetermi nee prelepromateuse au cou rs
d'un etat de react ion". (En impression, 1111. J. of Leprosy.)
2. COCH RANE, R. G., " Leprosy in t heory and practice". John Wright ( Bristo l,
1 959.)
3. DAVISON, A. R., KOOIJ, R. and WAINWRIGHT, J . , "Classification of Leprosy .
. I. Application of t he M adrid Classification of Various Forms of Leprosy".
Int. J. Leprosy, 28, 2 ( 1 960).
4. R AMOS E SILVA, J . , "Ainda sobre os criterios basicos da classificacao da
lepra". 0 Hospilal, 58, 2 ( 1 960 ).
5. Ross INNES, J., "Personal communication" ( 1 960).
TABLE I

CLASSIFICAnON OF LEPROSY

TUBERCULOID INDETERMINATE LEPROMATOUS

CUTANEOUS
I ----
CUTANEOUS CUTANEOUS

atypical (macular) ( ?) macular ( ?) figurate


minor (micropapular) (hypopigmented, and rarely papular "I "pure"
erythematous or hyper- macular or
major (in a plaque or a
ring, infiltrated,
well-defined)
pigmented) nodular
infiltrated J "predominating"

diffuse
borderline (in a plaque or a
ring, m ore or less
infiltrated, ill-
defined)

NEURITIC NEURITIC NEURITIC

pure · pure pure ( ?)

secondary secondary secondary

CUTANEOUS-NEURITIC CUTANEOUS-NEURITIC CUTANEOUS- NEURITIC


- ----

You might also like