DERMATOLOGY REVIEW
Hereditary
Ectodermal
Dysplasia
Symptoms, Inheritance Patterns, Differential
Diagnosis, Management
R. BRIAN
LOWRY, M.B., B.CH., D.C.H.,*
GEOFFREY C. ROBINSON, M.D.,
JAMES
R.
F.R.C.P.(C),t
MILLER, PH.D.†
Patients and pedigrees of both the hidrotic and anhidrotic forms of hereditary
ectodermal dysplasia are presented, together with a discussion of their clinical
manifestations, genetic and management
problems.
ur
.
Jt.
-m-EREDITARY ectodermal dysplasia appears in two forms, the hidrotic and the anhidrotic. The teeth and the hair are involved
similarly in both, but the manifestations in
nails and in sweat glands and the patterns of
inheritance tend to differ.
This paper reviews the clinical signs of
these two forms of the condition, based on
patients and pedigrees which we have studied, and discusses the practical problems of
diagnosis and management.
Historical Considerations
The first
of the anhidrotic form
Thurnam 26 in 1848, although ten years previously Wedderburn
had noted ten cases in a Hindu family in
&dquo;Scinde.&dquo; Wedderburn’s cases are documented in a letter written to Charles Darwin and quoted in The Variations in Plants
were
two cases
reported by
From the Department of Paediatrics, University of
British Columbia, Vancouver, B. C., and Health Centre for Children, Vancouver General Hospital, Vancouver, B. C.
*
Instructor, Department of Paediatrics, University
of British Columbia, 715 West 12th Avenue, Vancouver 9, B. C.
† Associate Professor, Department of Paediatrics,
University of British Columbia, Vancouver, B. C.
and Animals under Domestication.8 An affected female patient was described by Williams when discussing Thurnam’s paper. In
1883, Guilford 13 described a 48-year-old man,
edentulous from birth, who had never perspired and had soft scant downy hair on his
head. Two of his daughters had absence of
many teeth. A maternal uncle was edentulous
and hairless and the maternal grandmother
never had hair or teeth. In the early part of
this century a number of additional case reports appeared. In 1929 %Veech 2? introduced
the term hereditary ectodermal dysplasia and
suggested the name anhidrotic form for those
with inability to perspire.
In the same year, Clouston 5 described 119
cases of the hidrotic form in six generations;
and ten years later he distinguished the
hidrotic and anhidrotic forms.66
Since then, reports of many cases of both
types have appeared in the medical, pediatric, dermatologic and dental literature. The
anhidrotic cases are found in a wide variety
of nationalities and races-English, French,
Norwegian, Danish, German, Spanish, Italian, Russian, Jewish, Japanese, Indians and
Negroes. The hidrotic type, curiously occurs
largely in persons of French descent.
Case Reports
;Vidrot’c Form
Family P. The family is of French-Canadian
origin (pedigree shown in Figure 1).
Case I (R. P.), and Case 2 (L. P.). The propositus (R. P.) and his father (L. P.) are seen in
Figure 2. The boy’s head has sparse hair. The
father has always been bald, shaves once a week,
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Fisc. 1.
Pedigree of the P. famNote autosomal dominant
pattern of inheritance, six generations affected, and no sex
ily.
preference.
and has no body hair. The father and son have
identical abnormalities of the nails of the fingers
and toes; these are dystrophic, small, with cracks
and furrows, and never have to be cut (Fig. 3).
Both individuals have normal teeth and sweat
normally.
Case 3 (A. B.), Case 4 (C. B.), and Case 5
A. B. (V-4), a cousin of the propositus, is
seen with his affected daughter (~lI-3) in Figure 4.
The hair on his scalp and body shows normal
growth, whereas his one-year-old daughter is virtually bald. A. B.’s affected son (VI-4) also has
sparse hair but to a lesser degree than his sister.
The father and his two children show the typical
nail abnormalities (Fig. 5). Their teeth and sweating are normal.
Family SO. The propositus (G. SO.) is the only
(D. B.).
FIG. 2. The propositus R. P. (V-12) and his father.
The latter shows extreme baldness, while his son has
very sparse fair hair. Note lack of eyebrows.
,
known affected person in the pedigree, although
it was not possible to complete the father’s family
history. The national origin is unknown.
The growth of hair on his
normal. His deciduous teeth were
widely separated and the lateral incisors were
peg-shaped (Fig. 6); x-rays revealed only two permanent teeth. His nails, absent at birth, were very
soft, never required cutting, and were spoonshaped. He sweated normally.
Family SC. The pedigree has been reported
previously 21 (Fig. 7) and the family is of French
origin. All members sweated normcclly and several
had sensori-neural hearing loss.
Cause 6
head was
(G. SO.).
~‘~~. 3. Dystrophic fingernails of R. P. and his father.
Note that the nail never reaches the terminal end of
the digit, but breaks off at a variable distance.
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Peg-shaped
FIG. 6.
FIG. 4. An affected cousin A. B. (V-4) and his affected
daughter C. B. (VI-3). The father shows normal hair
and eyebrows, while his daughter is virtually bald and
has a small amount of very fine downy hair here.
(M. SC.). The propositus (M. SC.) had
hypodontia, deformity of the fingernails, and normal hair. The toes of the right foot show syndactylism (Fig. 8}, ~vith union of the first with the
high
tone loss
severe
lateral incisors of
in the
right
ear
propositus
and
sensori-neural loss in the left
a
G. SO.
moderately
ear.
Anhidrotic Form
Case 7
second toe, and the third with the fourth. She
also has a severe sensori-neural hearing loss and
attended the provincial school for the deaf for
her education.
Case 8 sic.). The mother (III-5) of the propositus has the characteristic nails and teeth and
a moderate sensori-neural hearing loss. She has
normal hair.
(A. SC.), and Case 10 (L. SC.). A. SC.
the oldest brother of the propositus, has
the characteristic peg-shaped teeth (Fig. 9) and
the nail abnormalities. Polydactyly was present at
birth. He has normal hearing, however, his audiogram shows a sharp dip in the high frequencies.
L. SC. (IV-3) had the same nail and dental defects as other members of the family. He has a
Case ,?
(IV-1),
Dystrophic nails of A. B. and his son. Only
fingers of the father are shown and are holding
his son’s hands in position. Nail growth from digit to
digit is quite variable, but note irregular chipped edge.
Family
C. The
propositus
is the
only affected,
of the family.
member
Case 11 (J. C.). Episodes of fever were frequent
during infancy; the temperature was seldom under 100° F. and occasionally reached 102° or
1030 F. Eczema was present throughout the first
two years, followed by asthma during the preschool period.
months, anhidrosis was
There was a foul nasal discharge. He
was edentulous, but an x-ray indicated six teethtwo centrals, two cuspids and two molars.
At 16 years of age, his general health was excellent. His hair was yellow-blond, eyebrows
sparse, and there was no axillary or pubic hair.
He had a poorly developed nasal bridge and
wrinkling of the skin under the eyes. His incisors
On examination at 14
recognized.
were
peg-shaped (Fig. 10). Fingernails
mal ;
no
sweating
occurred after
severe
were
nor-
exertion.
~’~~. 5.
two
Fic. 7.
of the SC family. Again note the autosomal dominant pattern of inheritance.
Pedigree
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FIG. 9. Note the
widely separated peg-shaped
in A. S.
FIG. 8. Syndactyly of the first and second and also
third and fourth toes on the right foot of propositus
M. S. (it-2).
His mother had similar coloring and her hands
unusually dry, but her teeth and sweating
were normal.
Family Q. The propositus was the only affected
member of the family.
were
Case 12 (G. ~.~. The first of this boy’s many
admissions was at three months of age,
for fever of unknown origin. Bouts of hyperpyrexia continued. He developed asthma at two
years of age. He has never been observed to sweat.
When examined at nine years of age he
weighed 38 lb. and measured 42I~’~&dquo;e His mentality was normal. He had no eyebrows and sparse
pale blond hair (Fig. 11) His voice was hoarse
and he had a foul green nasal discharge. The
nasal bridge was depressed, with lips thick and
protruding. He had only eight teeth and the incisors were peg-shaped. No other members of
the family showed the slightest indication of being similarly affected,
hospital
Clinical Manifestations
Hidrotic Form. This form is characterized
by dystrophic nails, scant or absent hair, and
dental anomalies. Sweating is normal. The
majority of families have been off French or
French-Canadian origin.
Three types of nail defects were described
by Wilkey and Stevenson ~ in their study of
an Ontario family. In this pedigree of 265
persons in six generations 64 were affected.
The first type was seen only in children and
incisors
(IV-1).
consisted of small nails, abnormal transverse
convexity with the distal one-third to onehalf of the nail unattached to the nail bed.
In the second, the nail was slightly thickened,
and showed several longitudinal striations,
ridges and grooves. The free edges were usually irregularly convex, flat against the nail
bed and often chipped. The third was the
most severe defect, the nails were shorter and
narrower, with greatly increased thickness.
The nail was set at an angle to the finger so
that the free edge was raised from the nail
bed and was blunt, rough and thick.
In texture the hair is softt and downy,
though the color may be darker than in the
anhidrotic form. Typically it is short, scanty
and sparse. Men shave infrequently, eyebrows and eyelashes are frequently absent.
Pubic and axillary hair may be scanty or
absent. The sweat glands are active and there
is no flattening of the nasal bridge.
The dental anomalies include anodontia,
more commonly hypodontia, and peg-shaped
incisors and canines. Both the primary and
secondary dentition are affected. Prior to
eruption of the teeth, x-rays of the mandible
showing dental hypoplasia or aplasia may be
of diagnostic value when other findings are
minimal.
Clouston described patients with poor
teeth and hypodontia. Wilkey and Stevenson 28 did not find any deformities of the
teeth in their series.
The variable expressivity of the hidrotic
form is illustrated in our cases. In the P
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Ftc. 10. Anhidrotic form. Peg-shaped incisors of propositus J. C. (IV-6). Again note wide separation,
though actual shape is not as extreme as in some.
the majority of members had affected hair but one male had normal hair
growth, and the development of baldness in
his offspring came as a great shock to his
wife, who was unaware of the existence of
the ectodermal dysplasia in the family. The
teeth were unaffected in the P pedigree but
were affected in the other two. All the pedi-
pedigree,
grees showed dystrophic nails similar to the
second and third types described above. Additional abnormalities in the SC. pedigree included polydactyly, syndactyly and sensorineural hearing loss.
Diagnosis may be difficult when the clinical manifestations appear as isolated findings.
We have encountered typical peg-shaped
teeth and hypodontia in children without
any other obvious manifestations of this syndrome.
One case report 15 of ectodermal dysplasia
described a ten-month-old boy with almost
complete absence of scalp hair and eyelashes.
He had two lower central incisors which were
normal in appearance, and x-rays of the jaws
did not show a deficiency of tooth buds. The
nails
were
normal. It
seems more
appropriate
look upon this as an instance of baldness
of undetermined cause, since there were no
other evident manifestations of the syndrome.
Onychodystrophy has been described as an
isolated finding. In such situations the importance of inquiring about and preferably examining other members of the family is
obvious.
to
’
Fit. 11. anhidrotic form. Propositus G. Q. (III -7) at
nine years of age- Note the wrinkled skin, thick
everted lips, and peg-shaped lateral incisors. Hair is
thin.
Anhidrotic Form. A patient with the fully
developed syndrome has the triad of inability to sweat, anodontia or hypodontia, and
scanty hair. Biopsies show a complete lack of
eccrine sweat glands, although in one report
the apocrine sweat glands were present in
the axillae .2 These patients suffer extreme
discomfort in hot weather and on exertion
and have frequent bouts of unexplained fever. One child died following a convulsion
with a temperature of 108° F.9 Many adult
clothes during
patients pour water over their
the hot weather. Bowen 3 described a fiveyear-old boy whose &dquo;environmental adjustment resembled that of a turtle.&dquo; He spent
much of his time out of doors in large- tub
of water and submerged himself at intervals
and then resumed his play until his clothes
needed further wetting.
Either anodontia or severe hypodontia may
be seen. Both the deciduous and the permanent teeth are affected. The incisors and
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canines are frequently absent or when present
may be peg-shaped or conical. The lack of
teeth causes failure of development of the
alveolar ridges and results in a senile-appearing face with protruding lips.
The scalp hair is very sparse, fair and short
always, and fine in texture. The axillary and
pubic hair are variably affected. Many patients lack eyebrows and eyelashes.
The protruding lips, the underdeveloped
nasal bridge, the prominent supraorbital
ridges and frontal eminences create a characteristic profile. The nasal mucosa may be
atrophic and be associated with a greenish
fetid mucopurulent nasal discharge.
Other less frequent abnormalities include
absence of mammary glands, absence of tears, eczema, asthma. As a rule, this
form of ectodermal dysplasia is not characterized by dystrophic nails, although there are
a few reports of the anhidrotic form with
affected nails.4 In our experience, the nail
dystrophy is quite characteristic in the hidrotic form, but we are not convinced that
typical nail dystrophy occurs in the anhidrotic form.
Other Variants of Ectodermal Dysplasia.
Cockayne 7 in his famous book included a
number of hereditary ectodermal defects in
the chapter on ectodermal dystrophics. Franceschetti 12 did likewise in his review in 1953.
Our discussion is limited to the two types
of hereditary ectodermal dysplasia and closely
related variants.
The syndrome of Ellis and Van Creveld
comprising ectodermal dysplasia, chondrodystrophy, polydactyly and congenital heart disease is probably due to a single autosomal recessive gene. The ectodermal dysplasia in this
syndrome usually consists of defective teeth
and nails and resembles the hidrotic form.
Several members of one of our families (SC)
had any syndactyly but with no
evidence ~5i chondrodystrophy or congenital
heart disease; some members of this family
also had sensori-neural hearing loss. Deafness
has been observed with other forms of ectodermal dysplasia.
Marshall 21 described a family with anhidrosis, a depressed nasal bridge, congenital
and juvenile cataracts, myopia and defective ’
hearing. The teeth, hair and nails were not
aphonia,
affected. It
appeared to be inherited as an
autosomal dominant but it is also possible
that the ocular manifestations were due to
a
separate
mutant
gene.
Deafness and nail dystrophy have been described in two sister.&dquo; They were the result
of a consanguinous marriage (first cousins on
the maternal and second cousins on the paternal side), and it is possible that the defects are the result of an autosomal recessive
gene. Helweg-Larsen and Ludwigsen 14 described deafness beginning in adulthood, in
association with the anhidrotic form of ectodermal3. dysplasia. Craniofacial dysostosis has
been reported in one case.1
Kline et al.16 noted a family with both ectodermal dysplasia and corneal dysplasia. The corneal dysplasia was the result of an autosomal
dominant gene and there was independent assortment between this gene and the gene for ectodermal dysplasia. Using a measurement of skin resistance as the frame of reference they described
six female patients who had patchy areas of anhidrosis, some of which were minimal. The other
features of ectodermal dysplasia were thin, brittle, dark hair and hypodontia in three of the
six. They do not describe any affected males in
great detail, but do mention that two died under
age two with infection and hyperpyrexia, and
that a third male, age 54, had scant, fine hair,
poor teeth and a marked intolerance to heat.
There is a curious sex ratio in this pedigree with
38 females to 14 males. Although they do not rule
out an X-linked gene being responsible for this
type of ectodermal dysplasia, they suggest, on the
basis of the skin resistance which identifies nonsweating areas, that it is an autosomal dominant
in which expressivity is sex-affected. It is unfortunate that the male affected members are not
described in greater detail. The clinical features
together with the mode of inheritance are not
typical of the usual anhidrotic form. Kline raises
the question of how to explain all the manifestations of ectodermal dysplasia in terms of a single
biochemical reaction, and discusses the interactions of many genes in tissue and organ difierentiation and development. The only work on the
molecular defect that we are aware of is that of
SCriVer,21t whose observations suggest that there
is a deficiency of stabilizing S-S crosslinks in ectodermal dysplasia hair.
the syndrome mesoectodermal dysplasia,20 there are many abnormalities of the
musculoskeletal system in association with
dental anomalies, cataracts, small stature and
sparse hair. If hair is present, it tends to be
woolly not soft and silky as in anhidrotic ecIn
400
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I
largely inactivated, the X chromosome containing the mutant gene will be present in the majority of cells in that individual and will produce
is
todermal dysplasia. The presence of sweating
and normal nails is a further point of differentiation between the mesoectodermal and
the ectodermal dysplasias.
an affected female. The
fore be expressed either
is of considerable imthe
portance
parents and subsequently the
children in families with either the hidrotic
or anhidrotic form. The impact of baldness
and deformed teeth, particularly when unexpected, is very disturbing indeed.
The hidrotic form is transmitted by an
autosomal dominant gene. The gene appears
to be largely confined to families of French
or French-Canadian origin; two of our families are of French descent. There is considerable variation in the expression of the
signs in the hidrotic form in both males and
females. An individual with only a minor
nail deformity may well have offspring with
the dental problem or alopecia or both.
The anhidrotic form is usually described
as being determined by an X-linked recessive
gene. The preponderance of affected males
and the absence of male to male transmission
tends to confirm that the gene is on the X
chromosomes. At first the condition seemed
to be confined almost exclusively to males.
An increasing number of writers are reporting affected females, though in most of these
the expressions are incomplete. A few have
had the complete syndrome. Usually a few
teeth are absent, or some are conical.
counselling
to
Such instances of affected females appeared to
contradict the idea of an X-linked recessive gene.
In 1936 Levity put forward the idea that the
gene was transmitted as an X-linked conditional
dominant to explain its occasional manifestation
in the female. Many writers have argued about
this term and proposed alternative ones, such as
partial or irregular dominance, but these arguments can probably now be put aside in the light
of more recent knowledge.
Lyon 19 postulated inactivation of either .the
maternal or paternal X chromosome early in embryonic life. Once a particular X chromosome is
inactivated, all descendants of that cell have the
same X inactive. This inactivation occurs in a
random fashion, so that the vast majority of females have a mixture of active paternal and maternal X chromosomes in their cells. A few
females, however, will have cells with an overhelming number of paternal X’s, or the reverse.
If the normal X chromosome is the one which
gene may therethe
wholly or partially,
depending upon the proportion of
inactivated normal X chromosomes.
whether this pattern of inheritance is termed
an X-linked recessive or an X-linked dominant
(partial or complete) is a question of semantics.
The words &dquo;recessive&dquo; and &dquo;dominant&dquo; were introduced to distinguish the interaction of a pair
of alleles in the heterozygous state. The minor
signs of the disease in the female makes the use
of the term &dquo;recessive&dquo; inexact, but the conventional usage of the term persists as still having
clinical value.
Available genetic evidence points to the gene
of this disorder being on the X chromosome. The
majority of people with the full syndrome are
male. The majority of affected females can probably be explained on the basis of the inactivated
X hypothesis. However, a most unusual pedigree
of six affected females in one family were described by Bernard et c~l.= in 1963, who also reported a further female case from another family.
The latter seemed to have a possible translocation between numbers one and two chromosomes
with a partial deletion of one arm of pair 1.
The family with six affected females is more complicated, but could be explained in terms of an
X-linked dominant with lethality for males. There
could have been a traz~slocation between an X
chromosome with the mutant gene and one of
the autosomes. However, no obvious morphologic
changes were noted in the karyotypes of three
which were studied. Other instances of affected
females may conceivably be due to nondisjunction
outcome
Genetics
Genetic
mutant
Turner’s syndrome (XO), or to the testicular feminization syndrome where the chromosomal sex is male (XY) but the phenotypic sex is
female.
producing
Variable expressivity is less marked in the
anhidrotic form. :Minor variations of the disease may be expected in a small percentage
of females as explained.
Management
Patients with the hidrotic form do not preas many immediate problems. Dental
and hair problems are common in both formsEarly dental consultation should be obtained whenever there is anodontia or hypodontia, in order that the child may be fitted
with a prosthesis. Serial sets of artificial teeth
may be required as the child matures. The
presence of artificial teeth fills out the maxillary and mandibular arches and prevents the
sent
’
’
undesirable
protruding lips, thereby improv401
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ing the profile. Samat and associates have
published a good report on a 14-year study
of facial growth in a patient with the anhidrotic form .23 They had to make five sets of
dentures for their patient during the period
from six to 16 years. They stress the need for
dentures as the child grows.
The chief concern with patients having the
anhidrotic form is their inability to sweat,
with resultant fever in hot environments.
These children must be kept in a cool environment with a minimum of clothing; and
fans and air conditioning may be required in
warm climates. Bower 3 recommends a special hollow mattress filled with cold water or
new
ice.
Lipschutz ~ reported
chologic problems due
a
to
with psyfacial deformities
patient
which were ameliorated by plastic surgery.
An external lubricant tan be applied regularly if the skin is excessively dry.
These patients, if they are to function normally in saciety, need’a great deal of care and
attention. They should stay out of warm
climates. Clouston suggested that these patients would work best in a non-sweating occupation in a moist temperate climate with a
minimum of present and future worry, &dquo;such
as a maritime Government job.&dquo;
Acknowledgment
7.
Milford, 1933.
8. Darwin, C.: The Variations of Plants and Animals
under Domestication. New York, D. Appleton
and Company, Inc., 1893, vol. 2, p. 319.
9. Drago, R. P. and Ehrenreich, T.: Ectodermal dysplasia of anhidrotic type in prolonged fever in
an infant. New York J. Med. 61: 2473, July 15,
1961.
10. Ellis, R. W. B. and Van Creveld, S.: A syndrome
characterized by ectodermal dysplasia, polydactyly, chondro-dysplasia and congenital morbus
cordis. Report of three cases. Arch. Dis. Child.
15: 65, 1940.
11. Feinmesser, M. and Zelig, S.: Congenital deafness
associated with onychodystrophy. Arch. Otolaryng. 74: 507, Nov. 1961.
12. Franceschetti, A.: Les dysplasies ectodermiques et
les syndromes hereditaires apparanges. Dermatologie 106: 129, 1953.
13. Guilford, S. H.: A dental anomaly. Dental Cosmos 25: 113, March 1883.
14. Helweg-Larsen, H. J. and Ludwigsen, K.: Congenital familial anhidrosis and neurolabrinthitis. Acta Dermatovener. 26: 489, 1946.
15. James, T.: Ectodermal dysplasia. Acta paediat. 41:
16.
September 1959.
17. Levit, S. G.: The problem of dominance in man.
J. Genetics 33: 411, 1936.
18. Lipschutz, H.: Anhidrotic ectodermal dysplasia. J.
Albert Einstein Med. Center 11: 33, January
1963.
19. Lyon, M. F.: Sex chromatin and gene action in
21.
patients.
22.
References
1. Andersen, T. H. and Pindborg, J. J.: Ectodermal
dysplasia and craniofacial dysostosis. Odontol.
T. 55: 484, 1947.
2. Bernard, R., Giraud, F., Rouby, M. and Hartung,
M.: Seven cases of ectodermal dysplasia in females, six in the same family: genetic discussion. Arch. Franc. Pediat. 20: 1051, November
1963. Yr. Bk. 1964-1965, p. 158.
3. Bowen, R.: Hereditary ectodermal dysplasia of
the anhidrotic type. Southern Med. J. 25: 481,
May 1932.
4. Brain, R. T.: Familial ectodermal defect. Proc.
Roy. Soc. Med. 31: 69, 1937.
5. Clouston, H. R.: A hereditary ectodermal dystrophy. Canad. Med. Assoc. J. 21: 18, 1929.
6. Clouston, H. R.: The major forms of hereditary
ectodermal dysplasia. Canad. Med. Assoc. J.
40: 1, 1939.
229, 1952.
Kline, A. H., Sidbury, J. B., Jr. and Richter, C. P.:
The occurrence of ectodermal dysplasia and
corneal dysplasia in one family—an inquiry
into the mode of inheritance. J. Pediat. 55: 355,
20.
The authors wish to thank Dr. Joseph Bensimon
for assistance with some of the pedigrees; Dr. Margaret Corey for help with the French translations; Dr.
Hilda Walker for permission to study one of the
E. A.: Inherited Abnormalities of the
Skin and Its Appendages. London, Humphrey
Cockayne,
23.
the mammalian X-chromosome. Amer. J. Hum.
Genet. 14: 135, 1962.
Malagon, V. and Taveras, J. E.: Congenital anhidrotic ectodermal and mesodermal dysplasia.
Report of two cases with atrichia and amastia.
Arch. Dermat. and Syph. 74: 253, 1956.
Marshall, D.: Ectodermal dysplasia. Report of a
kindred with ocular anomalies and hearing defect. Amer. J. Ophth. 45: 143, 1958.
Robinson, G. C., Miller, J. R. and Bensimon, J.
R.: Familial ectodermal dysplasia with sensorineural deafness and other anomalies. Pediatrics
30: 797, 1962.
Samat, B. G., Brodie, A. G. and Kubacki, W. H.:
Fourteen-year report of facial growth in case
of complete anodontia with ectodermal dysplasia. Amer. J. Dis. Child. 86: 162, August
1953.
24. Scriver, C. R. et al.: A molecular abnormality of
keratin in ectodermal dysplasia. J. Pediat.
67:
946, 1965.
25. Sunderman, F. O.: Persons lacking sweat glands.
Arch. Intern. Med. 67: 846, 1941.
26. Thurnam, J.: Proc. Roy. Med. and Chir. Soc. 31:
71, 1848.
27. Weech, A. A.: Hereditary ectodermal dysplasia.
Amer. J. Dis. Child. 37: 766, 1929.
28. Wilkey, W. D. and Stevenson, G. H.: A family
with inherited ectodermal dystrophy. Canad.
Med. Assoc. J. 53: 226, 1945.
402
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