ARTICLE
Diffuse partial woolly hair (DPWH) is a rare condition of scalp hair
growth, first described by Ormerod et al. in 1987 [1]. It is clinically
characterized by the presence of two distinct hair populations: straight,
blond or brown, long hairs intermingled with short, fine, hypopigmented
and curly hairs. The thin and wavy hair population is composed of easily
pluckable hair [1-3] leading only occasionally to a diffuse chronic alopecia.
Scanning electron microscopy studies show the typical pattern of woolly
hair, with thin, curled and oval shaped hairs, in the clinically affected
population of hair. Familial and sporadic cases have been reported.
We report here 6 patients initially diagnosed as DPWH [4]. A careful
study of these patients lead us to believe that they may have a previously
undescribed variant of curly hair.
Case report
Case 1. A 15-year-old girl on sequential treatment with Diane®
(ethinyl-estradiol (0.035 mg) and cyproterone acetate (2 mg)) associated
with a spironolactone regimen for hirsutism and alteration of the menstrual
cycle.
From the age of 12, she presented with thin, curly hairs between normal
ones, in a proportion of 20% (Fig.
1). She also complained of seborrhea. She had no history of other
drug use or hair cosmetic procedures.
Case 2. A 11-year-old girl with a non-relevant past medical history.
Three months ago her mother noticed the presence of some (25%) thin and
curly hairs between normal hair. They denied any hair cosmetic procedures.
Case 3. A 14-year-old girl with no relevant medical background.
From the age of 9 she observed a progressive curling of some hair between
normal hair in a proportion of 30%. She referred to an "electrified" aspect
of the hair and her condition improved when staying in the country-side.
The proximal areas of the curly hair were not affected. No drugs or physical
hair manipulation were reported. After three years of follow-up the condition
spontaneously improved. Her mother had a similar hair alteration that
also resolved at adolescence.
Case 4. A 15-year-old girl with no relevant medical history.
Two years ago she noticed the presence of some wavy hair intermingled
between normal long blond hair, affecting aproximately 25% of the hair.
She denied seasonal variations or use of hair-drier. The condition improved
after 1 year of follow-up.
Case 5. A 13-year-old girl with no significant medical background.
From the age of 11 she noticed some curled hair in between her normal
hair. Observation disclosed 30% of the hair to be curled over otherwise
normal looking hair. The proximal areas of the curled hair were not affected.
At 15 years of age the condition spontaneously resolved. Her sister had
similar findings to a less intense degree.
Case 6. A 12-year-old girl with no relevant medical or family
hair history. From the age of 10 her mother noted a progressive curling
of some hair intermingled in a normal hair population in a proportion
of 20%. The patient denied any hair cosmetic procedures. The proximal
areas of the curly hair were unaffected (Fig.
2).
Evolution
In cases 3, 4 and 5, the patients were followed-up and in all three
the abnormal curling spontaneously improved in a period varying from 1
to 3 years, as had occurred with the mother of patient 3.
Histopathology
Vertical and transverse sections of scalp biopsy in cases 3 and 4 were
performed. Some small anagenic follicles with normal hair shaft sections
were observed between normal sized hair follicles, in a proportion of
1 every 7-8 (approximately 15%).
Scanning electron microscopy (SEM)
SEM of normal and curly hair of cases 3, 4, 5 and 6 was performed.
Curly hairs had a diameter between 30-60 µm with flattened aspects,
oval shaped sections, canalicular formations and single torsions. All
these alterations are considered characteristic of woolly hair. The curled
pattern was more prominent in the distal part of the hair, where there
was marked cuticular weathering (Fig.
3).
Clinically normal hair had a diameter between 60-80 µm, a normal
shape and low intensity structural alterations similar to those found
in the curled hair population, but also with significant cuticular damage
(Fig. 4).
SEM studies were performed after spontaneous improvement in case 3 but
substantial alterations were not found.
Comment
Woolly hair (WH) is a rare congenital condition that has been classically
classified by Hutchison et al. [5] into three distinct types: Hereditary
woolly hair is an autosomal dominant trait in which there is a variable
degree of tight curling in all hairs throughout the scalp. An autosomal
recessive form, termed familial woolly hair, is characterized by abnormal,
tightly-curled, fine, white or blond hair that tends to be short and is
present from birth. The third type is the woolly hair naevus in which
the hair within a well demarcated area is curly and lighter than the normal
hair and has a reduced diameter. Acquired progressive kinking of hair,
whisker hair and diffuse partial woolly hair can be considered nowadays
as acquired types of woolly hair or more precisely acquired types of curly
hair [1-3, 6].
From the original article by Ormerod et al. [1], the following
criteria are of value for the diagnosis of diffuse partial woolly hair
(DPWH).
1 The anomaly is limited to the scalp hair and comprises apparently
normal straight hairs and abnormal curly hairs, diffusely intermingled.
2 The straight hairs are predominant (70-80%), obscuring the
shorter and thinner curly hairs, which makes the clinical picture unremarkable
at first glance and require careful examination.
3 The straight hairs are long, blond or brown, 70-80 µm
thick, and are firmly anchored in the follicles.
4 The curly hairs are thin (30-40 µm), hypopigmented, easily
pluckable and shorter (2-7 cm).
5 The chief complaint of the patients is the loss of hair, predominantly
composed of curly hairs.
6 The usual age of onset of DPWH is at puberty, but the earliest
report registered is in a 4-year-old girl and the latest in a 19 year
old. A father and an uncle are reported [1] but their ages are not given.
7 Most patients are female in a proportion of 12 to 4.
8 The course of the disease is chronic and progressive, leading
occasionally to a diffuse thinning of the hair in adulthood.
Our patients did not fulfill all of these criteria. For example cases
3, 4 and 5 did not have hair loss or easily pluckable hair and their conditions
appeared to resolve in 1 to 3 years, violating criteria 4 (easily pluckable),
5 (hair fall) and 8 (chronic and progressive) (Table
II).
The patient studied by Guidetti et al. [3] as well as the case
1 and the mother of patient 3 of Lalevic-Vasic et al. [2] seem
to fit exactly into the criteria of Ormerod for DPWH, nevertheless in
cases 2-4 of Lalevic-Vasic the lack of information cannot lead us to consider
them as true DPWH. They could well be the same acquired type of this entity
here described.
Ormerod et al. in his original article
[1] differentiates DPWH from woolly hair naevus. In DPWH the affected
hair shows frequent kinks which changed the direction of the hair, thus
giving rise to the wavy macroscopic appearance. In contrast, the woolly
hair naevus produces a helical hair from follicles which are curved [7,
8]. In our experience [9-11] we differentiate congenital curly hair (true
woolly hair) and acquired types of curling of hair (Table
I).
In the family studied by Ormerod et al. [1], the anomaly seems
to be transmitted as an autosomal dominant trait. Two out of 4 patients
reported by Lalevic-Vasic [2] appear to be sporadic and may be interpreted
as new mutations. Also the patient reported by Guidetti [3] appears to
be sporadic as well as 4 of our 6 cases.
As early as in 1987, Ormerod et al. [1] pointed out that environmental
factors could contribute to the irregular kinking of the hairs. In our
patients we observed a decrease of this phenomenon over time, and spontaneous
clinical improvement. Clinically it is important to point out that the
worst alterations were found in the distal part of the hair shafts, where
weathering was also present. These findings confirm that our patients
have an acquired type of curly hair that may be induced by environmental
factors or hair cosmetic procedures, specially when a familial predisposition
is present. In fact, in our patients, as well as already reported by Lalevic-Vasic
et al. [2], we could observe mild woolly hair alterations detectable
only by scanning electron microscopy in the normal, non curly population
of hair. Two out of the 6 cases were familial.
In our experience, it is not uncommon to observe a few curled hairs
arising in the superficial part of a hair style, after hair cosmetic procedures.
By closely studying the "curled" hair we confirmed that in fact they are
wavy and not curled nor woolly (Fig.
1). This alteration affects only the distal portion of the hair
shaft (Fig. 2). The wavy
configuration of these hairs, makes them look shorter. The easy pluckability
of the wavy hair population observed by Ormerod et al. [1] was
not present in our patients.
It still remains unclear how these factors could give rise to shaft
alterations as specific and extreme as the characteristic of woolly hair.
It is also true that some small anagen follicles intermingled with regular
sized hair follicles can be found in normal scalp in a proportion around
15%. Whether these follicles correspond to the ones affected is not clear.
CONCLUSION
DPWH should be differentiated from an acquired type of curly hair (acquired
partial curling of hair) (APCH) presenting as "multiple isolated kinking
of hair" as we initially called this phenomenon [4]. APCH is not a rare
condition, though it is infrequently recognized. It involves mostly prepuberal
girls, and it is not a persistent condition (Table
II). It is not related to drugs and it may be induced physically,
cosmetically or by weather conditions because the cuticular damage can
be observed in both type of hair populations. From our point of view,
it constitutes a new type of acquired curly hair. DPWH affects the whole
hair shaft while APCH affects only the distal and thinner part of the
hair shaft, more susceptible to the effects of external agents (weathering)
[12].
Acknowledgments
Our special thanks to Dr. Whitney Hannon for her support in revising
and editing this manuscript.
REFERENCES
1. Ormerod AD, Main RA, Ryder ML, Gregory DW. A family with diffuse partial
woolly hair. Br J Dermatol 1987; 116: 401-5.
2. Lalevic-Vasic BM, Nikolic MM, Polic DJ, Radosavljevic B. Diffuse
partial woolly hair. Dermatology 1993; 187: 243-7.
3. Guidetti MS, Fanti PA, Piraccini BM, Barbareschi M, Tosti A. Diffuse
partial woolly hair. Acta Derm Venereol (Stockh) 1995; 75: 141-2.
4. Ferrando J, Navarra E, Haussman G. Multiple isolated kinking of hair.
European Hair Research Society, Stochkholm, 1993.
5. Hutchison PE, Cairns RJ, Wells RS. Woolly hair. Trans St John's
Hosp Dermatol Soc 1974; 60: 160-7.
6. Cullen ST, Fulghum DD. Acquired progressive kinking of the hair.
Arch Dermatol 1989; 125: 252-5.
7. Bovenmyer DA. Woolly hair naevus. Cutis 1979; 24: 322-5.
8. Lantis SDH, Pepper MC. Woolly hair nevus. Two case reports and a
discussion of woolly hair forms. Arch Dermatol 1978; 114: 233-8.
9. Ferrando J, Gratacós MR, Fontarnau R. Woolly hair. Estudio
histológico y ultraestructural en cuatro casos. Actas Dermosifilogr
1979; 70: 203-14.
10. Rodriguez Pichardo A, Moreno JC, Camacho F, Ferrando J. Woolly hair.
Estudio de cinco nuevos casos. Med Cutan Iber Lat Am 1983; 11:
393-8.
11. Soler-Carrillo J, Azon-Masoliver A, Malvehy J, Ferrando J. Nevo
de cabello lanoso asociado a alopecia triangular congénita. Actas
Dermosifiliogr 1995; 86: 89-95.
12. Dawber RPR. Weathering of hair in monilethrix and pili torti. Clin
Exp Dermatol 1977; 2: 271-7.
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