A Unique Case of Trichorrhexis Nodosa\p=m-\"Bamboo
Hairs"
EARL W. NETHERTON, M.D., Cleveland
In 1949 I examined a little girl who had
a congenital ectodermal defect manifested
by a unique type of fragilitas crinium which,
so far as I have been able to determine,
has not been discussed in the literature. I
have been diffident about reporting my find-
ings because the patient lived in a distant
city and, therefore, was unable to remain
under prolonged observation. However, a
r\l=e'\sum\l=e'\of her history, together with
mounted hairs or photographs of them, has
evinced such interest among several eminent
dermatologists that I am encouraged to be-
Submitted for publication May 24, 1958.
Emeritus Member of the Staff, Department of
Dermatology, The Cleveland Clinc Foundation, and
The Frank E. Bunts Educational Institute.
Fig. 1.—Photograph of patient when 11 years
old, showing lack of hair on sides and back of
scalp. Hair failed to grow to normal length.
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lieve that such a rare type of fragilitas
crinium warrants this report.
Report of a Case
A girl, aged 4 years, was admitted to The Cleve-
land Clinic on Nov. 22, 1949, because the hair on
the scalp had failed to grow to normal length
(there had been no area of baldness), and a
pruritic eruption had been present since birth. The
mother stated that at birth the patient's entire
cutaneous surface was extremely red and that for
several weeks thereafter there was a generalized
exfoliative dermatitis emitting a foul odor. The
erythroderma gradually disappeared, leaving a per¬
sistent papular squamous eruption disseminated
over the trunk and the extremities. Partial remis¬
sion of this eruption occurred during each summer
season, although it had never disappeared entirely.
Fig. 2.—Photograph of patient's back, showing
chronic dermatitis that resembles chapping (age
11 years).
 At the age of 2 years, the patient had had vac¬
cinia. Since infancy she had had frequent attacks
of upper respiratory infection. The mother stated
that the child was "frequently restless, irritable,
and difficult to feed because of her preference for
liquid foods."
There was no familial history of hay-fever,
asthma, or eczema. Two siblings, aged 8 and 2
years, were normal.
Physical examination disclosed that the patient
was thin and poorly nourished. The skin was
abnormally dry', and there were poorly demarcated
areas of pink scaly dermatitis on the forehead and
neck. There was excessive seborrheic scaling on
the scalp, but there were no follicular keratosis
pilaris-like papules on the scalp. Patches of suba-
cute erythematous scaly dermatitis which resembled
chapping were scattered over the trunk and ex¬
tremities, as well as groups of small discrete
acuminate pink follicular papules (Fig. 2). Some
of these papules had apical keratotic spines. Pru¬
ritus was paroxysmal and was said to be usually
severer during the night. The teeth and nails were
normal. The lymph nodes of the neck, axillae, and
inguinal regions were palpable. The spleen and
liver were not enlarged.
There were no significant abnormal physical
findings other than the eruption and changes in¬
volving the eyelashes, eyebrows, and hairs of the
scalp. The hair on the scalp was dry' and fragile
and lacked the luster and beauty commonly seen
in the hair of young girls. Hairs on the occiput
and sides of the scalp were no longer than 3 or
4 cm. (Fig. 1) and developed transverse fractures
so easily that it was difficult to remove them from
the follicles by traction. Casual inspection did not
reveal nodes involving the hairs of the scalp.
Microscopic examination of hairs from the scalp
and the eyebrows revealed a unique type of nodosity
or jointed swelling of the hair shafts.
Appar¬
ently- the jointedness accounted for the easy frac¬
turing of the hair shaft and its failure to grow to
a normal length. Some eyelashes and downy hairs
from the arms showed small nodes, but they were
not the well-developed jointed swellings of the
type seen in the hairs from the scalp.
A biopsy specimen from the scalp unfortunately
was not obtained. However, tissue from an erythe¬
matous plaque on the patient's back was removed.
Sections from this biopsy specimen revealed sur¬
prisingly few significant histopathologic changes.
The hair follicles and accessory glands were nor¬
mal. The epidermis was normal except for small
areas of parakeratosis. There was mild edema and
inflammatory perivascular infiltrate in the upper
corium.
The most fascinating findings were the poly¬
morphic abnormalities of the hairs from the head.
The variations ranged from slight rotation along
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the axis of the hair, with a wave-like irregularity
of the cortex of the hair shaft, to well-developed
jointed nodes that resembled the joints of a bamboo
pole. The nodes varied in size and were irregularly
distributed along the hair shaft.
Almost seven years after the initial examination,
in September, 1956, I had the privilege of examin¬
ing the patient again in her home city. The hair
on the scalp was dry, without normal luster, and
was fragile. The hair on the sides and posterior
surfaces of the scalp had failed to grow to normal
length. Hairs removed from the scalp showed the
same unique type of joint-like nodose swelling of
the shaft which was observed at the first examina¬
tion in 1949. Likewise, additional biopsy specimens
of skin taken by her family physician showed
changes identical with those observed in 1949.
The skin of all areas was abnormally dry, and
there were numerous areas of squamous dermatitis
which resembled chapping. There were groups of
small acuminate skin-colored follicular papules
scattered over the trunk and extremities. The skin
in the cubital and popliteal fossae was thickened.
Pruritus was present but said to be not trouble¬
some. Seborrheic scaling of the scalp was not
profuse.
Laboratory Studies
Hairs from the scalp were mounted in balsam,
for study of the morphologic details of the hair
shafts. The nodose swellings showed great varia¬
tion in size (Fig. 3). The earliest indication of
abnormality comprised a narrow indentation of the
cortex of the hair, forming a shallow sulcus, which
appeared to surround the shaft. This change was
sparse or frequent, occurring at irregular intervals
and throughout the length of the hair. One hair
showed early indentation of the atrophie portion
of the shaft, immediately- distal to the orifice of its
follicle. As the abnormality had progressed,
swellings of the shaft had developed, the sulcus had
enlarged, and, on the proximal portion of the hair
shaft, a concavity had formed into which fitted the
adjacent enlargement of the distal portion of the
hair. These combined changes had resulted in
the formation of a pseudojointed nodose swelling
that, as previously stated, closely resembled the
appearance of the joints of a bamboo pole.
Changes observed in the large nodes suggest that
there had been an invagination or intussusception
of portions of the hair shaft. An additional com¬
ponent of the abnormality of growth of the hair
shaft is a granular degeneration that is manifested
by the presence of clumps both of fine and of
larger black granules in areas of the hair shaft
and more particularly within the medullary portion
of some hairs. A dense concentration of these
granules may be seen in the concave portion of the
jointed swellings. Complete fracture of the hair
 Fig. 3.—Photomicrograph showing nodose swelling of hair shaft which resembles joints of
bamboo, (a) Early indentation of cortex of hair shaft; ((') polymorphic joint-like nodes; (c)
concavity of hair shaft at the point of fracture.

occurs at the site of the nodosity, and much of Comment on Case

the dark granular substance remains within the
cupped end of the proximal portion of the hair.
It is believed that the granules are not an in¬
fectious organism. Cultures of hairs planted on
Sabouraud's medium did not show growth of a
pathogenic fungus.
The dermatosis that this patient showed
is considered to originate from a congenital
ectodermal defect. The dry ichthyotic skin
and the erythematous scaly dermatitis that
persisted from birth are manifestations

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 compatible with the diagnosis of a dry type
of erythroderma ichthyosiforme congeni-
tum. It is significant that there was no
change in the characteristics of the eruption
or abnormalities of the hair during the
seven-year interval between the first and
the second examination of this patient.
It is regrettable that a biopsy specimen
of the scalp was not obtained. A stud)- of
serial sections of the hair papillae might
have added much valuable information, as
the mechanism of formation of the nodes
is obscure.
Comment
The most familiar diseases in which
fragility and nodose swellings of the hair
shaft are prominent characteristics are
trichorrhexis nodosa and monilethrix. No¬
dose swellings that resemble those seen in
trichorrhexis nodosa have been observed in
pili torti.1 Nodular concretions of infectious
origin surrounding the hair shafts in piedra
nostros and lepothrix differ greatly in ap¬
pearance from those seen in trichorrhexis
nodosa and monilethrix. Formerly, the con¬
cept was widely accepted that abnormal
changes involving the hair shaft in trichor¬
rhexis nodosa are of infectious origin. This
concept now has been largely discarded, and
the present consensus is that the longi¬
tudinal splintering of the shaft and the
formation of nodes, which resemble the in¬
terlocking of two small brushes at sites of
fracture of the hair shaft, probably are the
sequelae of too-frequent shampooing and
excessive dryness of the hair. Some chemi¬
cals commonly applied to the hair may be
important etiologic factors.
Pili torti and monilethrix are examples
of ectodermal defects.2 Monilethrix usually
is hereditary3 and is characterized by
numerous small discrete papules, similar to
those of keratosis pilaris, on the scalp, and
sparse growth of short fragile beaded hair.
The nodose swellings are uniform in size
and shape and are located at regular inter¬
vals throughout the entire length of the
hair. There is notable constriction of the
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hair shaft between the nodes. Abnormal
dentition and other manifestations of the
ectodermal defect may be present. Twisting
of the hair around its long axis occurs in
some of the beaded hairs. However, this
change is minor and does not approach the
degree of rotation seen in pili torti. Dan-
forth4 has pointed out that normal hair
tends to be ribbon-like and frequently is
rotated to some degree around the long axis.
He believed that this feature of normal hair
had been neglected by previous observers.
It is illogical to surmise that the varied
abnormal changes involving the hair shaft
in the case just reported could occur in
progressive stages in the lifeless shaft of
an adult hair. As in monilethrix, the pri¬
mary disturbance probably occurs within
the hair papillae. In this case, the unique
features are the abnormalities of the hair
shaft, which were responsible for its failure
to grow to a normal length. In regard to
this case, Drs. M. F. Engman, Lee McCar¬
thy, Herman Pinkus, Richard Weiss, Har¬
old Cole, Fred Weidman, and others, were
unanimous in stating that never had they
seen similar nodosa swellings of the hair
shaft.
After observing mounted hairs, Engman 5
stated: "It does not look like trichorrhexis
nodosa or any of the hair conditions that I
have ever seen or know of." He also com¬
mented that the nodes "looked exactly like
the joints in a fishing pole" and suggested
the name of "bamboo hairs." McCarthye
stated: "I have come to the conclusion that
you have in all probability a new type of
hair atrophy, a possible variant of trichor¬
rhexis nodosa. .
Certainly, the photographs
.
show the bamboo-like disturbances of the
.
hair beautifully." He was of the opinion
that the nodes resulted from "a disturbance
in the hair papillae which later resulted in
an abnormality of the adult hair." Pinkus7
wrote: "The hairs of your case are most
remarkable, looking, as you say, like the
knots of bamboo or in some instances al¬
most like the joints of equisetum (horsetail)
with one end inserted into the cuff-shaped
 other half. ...I do not remember that my
father mentioned anything similar. I also
consulted his book on diseases of the hair.
rummaged in the old reprint collection of
my father, but could find nothing compara¬
ble."
Summary
A case is reported of unique type of
atrophy of the hair with fragility and no¬
dose swellings of the hair shaft, which
grossly resemble the joints of bamboo. Hair
of the scalp was most particularly involved ;
however, eyelashes, eyebrows, and hair on
the arms were similarly abnormal.
The fragilitas crinium and concomitant
eruption are considered to be essential com¬
ponents of a congenital ectodermal defect.
The eruption resembled the cutaneous fea-
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tures of the dry type of erythroderma
ichthyosiforme congenitum.
. .
The Cleveland Clinic, 2020 E. 93d St., (6).
REFERENCES
1. Ronchese, F.: Twisted Hairs (Pili Torti),
Arch. Dermat. & Syph. 26:98-109 (July) 1932.
2. MacKee, G. M., and Rosen, I.: Monilethrix:
A Clinical and Histological Study, with Report of
6 Cases and a Review of the Literature, J. Cutan.
Dis. incl. Syph. 34:506-525 (July) 1916.
3. Tobias, N.: XXXV. Monilethrix: Report of
5 Cases of the Familial and Hereditary Type,
Arch. Dermat. & Syph. 8:655-664 (Nov.) 1923.
4. Danforth, C. H.: Studies on Hair, with Spe-
cial Reference to Hypertrichosis, Arch. Dermat.
& Syph. 12:76-94, 1925.
5. Engman, M. F.: Personal communication to
the author, March 30, 1950.
6. McCarthy, L.: Personal communication to the
author, March 22, 1950.
7. Pinkus, H.: Personal communication to the
author, April 1, 1950.