ARTICLE
Congenital hypertrichoses are classified as generalized or localized
[1, 2]. Primary localized nevoid hypertrichoses are characterized by circumscribed
hypertrichosis of a specific area of the skin [3, 4]. We currently classify
primary localized nevoid hypertrichoses in the following way:
- hairy elbow syndrome,
- hypertrichosis of the auricle ("hairy pinna"),
- eyebrow hypertrichosis,
- hairy polythelia,
- anterior cervical hypertrichosis [1].
We have recently observed a particular type of localized multifocal
hypertrichosis on the trunk and arms, that we think could be included
in this group.
Cases and methods
In 1997, a 41-year-old woman came to our Trichology Unit because she
had long hairs distributed along the upper trunk and arms from birth (Fig.
1). She had also partial diffuse woolly hair: there were short,
fine hairs among the normal hairs. Furthermore, a symmetrical and circumscribed
allotrichia was present in the periauricular areas. In these areas the
hair became coarse, short, curly and whisker-like.
She had a daughter and a son, but only the 12-year-old boy showed the
same type of hypertrichosis on his back, chest and arms (Fig.
2). He also presented a congenital triangular temporal alopecia
and partial diffuse woolly hair.
No other of the mother's relatives showed any trichological abnormality.
Studies were performed on both the mother and the son.
Results
Trichological studies of the woman's scalp hair revealed the presence
of torsions, angular twisting, as well as channeled and flattened hair
segments that alternated with normal areas. We interpreted these findings
as partial diffuse woolly hair. The trichological study of symmetrical
allotrichia showed hairs of the "woolly hair" type and occasionally, pseudomonilethrix
images.
Examination of body hairs revealed that
they were long vellus hairs without medula.
Trichological studies of the boy were very similar to those of his mother.
Biopsy of the back which included a long vellus hair in the center of
the area showed a prevalence of 80% early anagen follicles associated
with numerous vellus follicles. Absence of infiltrate and fibrosis. No
biochemical or hormonal abnormalities were found (free-testosterone, sulfated
dehydroepiandrosterone, androstenedione, 17-hydroxiprogesterone, sexual
hormone binding globulin, prolactin and thyroid hormones). Endocrinological
evaluation carried out considered his gynecomastia as physiological, typical
of adolescence. Other etiologies were excluded due to normal values in
blood test.
Discussion
We propose the term "primary localized multifocal hypertrichosis" for
these cases because the excessive growth of the hair does not occur on
the entire skin surface, only on the upper part of the trunk and arms
in several foci. These hairy findings appear from birth or later. In this
case, there is no association with an underlying defect, so it is considered
to be a primary hypertrichosis.
Furthermore, we have described the association between this special
type of primary multifocal hypertrichosis with non specific dysplasias,
as different forms of coiled and twisted hairs [5-7], and a non-cicatricial
congenital alopecia, as congenital triangular temporal alopecia (CTTA).
The CTTA, as circumscribed alopecia, is due to aplasia of the hair follicle
[8] with an absence of terminal hair follicles, although follicles similar
to those of body hair or indeterminate hair may be seen in the dermis.
The association with CTTA does not seem to be related and is probably
merely fortuitous. Moreover, in the partial diffuse woolly hair, there
is a lower number of terminal follicles, and also, intermediate sized
follicles which probably produce the woolly hair [9]. In this form of
primary localized multifocal hypertrichosis, the biopsy showed that 80%
of the follicles were anagenic, so this might explain why the hairs were
longer than usual, and the majority were vellus follicles.
CONCLUSION
In conclusion, we present an unusual form of hypertrichosis that, to
our knowledge, has not been previously reported. It was associated with
some rare forms of non specific hair dysplasia and non cicatricial alopecia.
This hypertrichosis does not tend to improve with age and a familial form
was recorded. Therefore, it is necessary to explore the scalp hair in
order to find some hairy associations, although systemic abnormalities
have to be excluded.
This paper was presented as a communication in the Second Intercontinental
Meeting of Hair Research Societies held in Washington on November 1998.
REFERENCES
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4. Camacho F. Acquired circumscribed hypertrichosis in the "costaleros"
who bear the "pasos" in the Holy Week of Sevilla. Arch Dermatol
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partial woolly hair. Br J Dermatol 1987; 116: 401-5.
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A, Camacho F. Congenital triangular alopecia (Brauer's nevus). Pediatr
Dermatol 1995; 12: 301-3.
9. Guidetti MS, Fanti PA, Piraccini BM, Barbareschi M, Tosti
A. Diffuse partial woolly hair. Dermatology 1987; 116: 243-7.
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