978

DISEASES OF THE APPENDAGES

MONILETHRIX

Synonyms.—Moniliform or beaded hair; Nodose hair (Smith); Aplasia pilorum
intermittens (Virchow); Aplasia pilorum moniliformis (Behrend); Fr., Aplasie monili-
forme des cheveux et des poils (Hallopeau and Leredde); Nodosité des poils (Brocq);
Ger., Spindelhaare.

This rare affection of the hair was first described by Walter Smith,
and later by Bulkley, McCall Anderson, Payne, Thin, Lesser, Hallo-
peau, Beatty,¹ Gilchrist,² Morrow,³ Ruggles,⁴ and others. The affection
is usually confined to the scalp, and sometimes to a limited portion of
it; but exceptionally it has been noted on other regions as well; in the
cases described by Gilchrist, Morrow, and Ruggles it was limited to the
legs. The hairs are made up of elongated, fusiform-looking nodes and
connecting, narrowed atrophic portions, so that the hair has a beaded
appearance. In fact, it may appear as if made up of a series of thin,
connected spindles. The nodular parts are much darker than the nar­
rowed portions, the latter being almost colorless, so that the hair has a
ringed aspect. The whole shaft is involved from root to the free ex­
tremity. The hairs readily break, not at the nodes, as in trichorrhexis
nodosa, but at the thin portion, between the nodes, and the broken end
of which is frayed or brush-like. So fragile are they that most of the hair
of the scalp or affected part of it is broken off near or at the surface,

Fig. 245.—Monilethrix—hair showing the breaks at the internodes.

and a variable degree of alopecia is noticeable, slightly similar to the con­
ditions observed in some patches of ringworm. This resemblance is
somewhat added to by a keratosis pilaris of the parts, which gives an
exaggerated appearance of the stuffed follicular openings or goose-flesh
surface sometimes observed in recent cases of this latter disease. The
baldness may be finally quite extensive. Cases vary somewhat in degree,
some being slight, and recognizable only on close inspection, others being
quite conspicuous. In some of the hairs there may be continuous thin­
ning, the whole hair appearing simply atrophic

Etiology and Pathology.—The affection occurs in both sexes.
It begins in early infancy,—in the first year or two,—and is looked upon
as congenital. There are some exceptions to this on record: among which
one of Smith’s cases and Unna’s case, and those of Gilchrist, Morrow,

¹ Beatty and Scott, “Moniliform Hairs (Monilethrix),” Brit. Jour. Derm., 1892,
p. 171—an excellent review and résumé of all recorded cases to date, 24 in number
(Walter Smith (2), Liveing (1), Thin (1), Kaposi (2), Vidal (3), Unna (1), Bulkley (1),
Bury (1), McCall Anderson (5), Luce (1), Lesser (1), Payne (2), Hallopeau and Lefévre
(1), Arnozan (1), Abraham (1)); and description of an additional new case by Beatty.
Literature references are given.

² Gilchrist, “A Case of Monilethrix, with an Unusual Distribution,” Jour. Cutan.
Dis., 1898, p. 157 (with illustrations and an analytic table of all previously published
cases (60), with bibliography, which includes cases recently exhibited before Dermato-
logical Society of London by Colcott Fox (1896), Galloway (1896), and Anderson (1897).

³ Morrow, ibid., 1899, p. 41 (case demonstration).

⁴ Ruggles, ibid., 1900, p. 500 (with illustrations).

MONILETHRIX

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and Ruggles, in which the disease appeared much later—in the last
3 cases—all physicians—in adolescent or adult life. McCall Anderson’s
patients were of a family in which, in six generations, there were 14 cases
among 27 persons; and Sabouraud¹ records, in five generations of a family,
17 cases. Payne’s 2 cases were brothers, and Hallopeau saw 3 cases in
the same family, 3 of whose relatives also were affected. Beatty’s case
was a brother of one of Smith’s patients; and 2 other children, deceased,
were affected similarly, and there was a suspicious history of baldness in
the maternal uncle and maternal grandmother² While these family
tendencies point to a hereditary affection, the possibility of contagion
is one that cannot be arbitrarily set aside, although it is true that a con­
tagious disease would ordinarily not be such a rare one. The common
view, however, is that the disease is congenital, and that the thinned
portions of the hair-shaft result from some defective development, the
thicker or node-like portion being scarcely, if at all, beyond the normal
thickness. There is much less pigment in the thinner portion—almost
nil, in fact; the cases of Lesser, Gilchrist, and Ruggles were exceptional
in this respect, the thinner part being the darker portion. From Scott’s
microscopic investigations it would appear that at one time the papillae
over the entire affected region are forming nodes, at another time inter-
nodes. There is usually an associated follicular hyperkeratosis—
keratosis pilaris. In fact, Brocq, Ledermann,³ and others think that the
malady is closely related to keratosis pilaris. Both Gilchrist and Scott
examined for organisms, but without result. Bonnet believes the nar­
rowing due to an intermittent muscular contraction around the follicle
just below the point where the sebaceous gland empties into it, and
where the young hair-cells are still soft and readily compressed. Vir-
chow, Kaposi, and a few others believe it due to periodic aplasia of the
hair. Nervous shock is said to have given the start to the disease in
Sabouraud’s series and also in Unna’s patient.

Treatment.—Very little, if anything, is to be hoped from treat­
ment. In one area in Gilchrist’s case the disease, after existing for a
number of years, disappeared spontaneously. Possibly in the later ac­
quired cases, remedies, both constitutional and local, the former of an
invigorating and the latter of a stimulating character, such as prescribed
in alopecia areata, might be of service. In all instances in which the
hypërkeratosis element is marked, weak sulphur and salicylic acid oint­
ments should be used to lessen or remove this.

¹ Sabouraud, Annales, 1892, pp. 781 and 830 (good review of the subject).

² At a meeting several years ago of the Philadelphia Dermatological Society, I. M.
Koch exhibited 2 cases—brothers; and Dore, Brit. Jour. Derm., 1911,p. 111, presented
before the Dermatological Section of Royal Soc’y of Medicine, London, 2 cases, brothers.
In the discussion Galloway referred to 2 cases, (2 brothers); as the boys grew older the
affection became less obvious, being covered up by the healthy hairs. McMurray and
L. Johnston, Australasian Med. Gaz., Jan. 25, 1913, p. 74, also report a series—mother
and 2 children; observed in all in the first month of life.

³ Ledermann (3 cases), Berlin, klin. Wochenschr., 1903, p. 332.

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