Clinical dermatology X Concise report

Early-onset hidradenitis suppurativa

R. A. Palmer and M. Keefe
Department of Dermatology, Royal South Hants Hospital, Southampton, UK

Summary A 9-year-old girl developed hidradenitis suppurativa 3 months after the first signs of
adrenarche. Such a close temporal relationship is consistent with the hypothesis that
the disease is androgen dependent. Less than 2% of patients have onset of the disease
before the age of 11 years. The exceptionally early age of onset in our patient may be
partly explained by the fact that she had an early puberty.

17-hydroxyprogesterone (for congenital adrenal hyper-

Report
An 11-year-old girl presented to the dermatology
department complaining of boils in the groin. She was
otherwise well.
She started to develop axillary hair and apocrine
odour at the age of 9 years 0 months, boils in the
inguinal areas at 9 years 3 months, and menarche at
10 years 5 months. Her mother and maternal grand-
mother gave a history consistent with hidradenitis
suppurativa.
At the time of presentation she was on the 97th
centile for height and 85th for weight. Her body mass
index was 19.0 kg/m2. Breast and pubic hair develop-
ment were Tanner stage 4. Examination of the inguinal
areas (Fig. 1) showed closed comedones, open come-
dones, red nodules and atrophic scars, consistent with
hidradenitis suppurativa.
Results of endocrinological investigations are shown
in Table 1. The free androgen index (the testosterone
concentration divided by the sex-hormone binding
globulin concentration) was 0.02 (within the normal
range). Fasting glucose, fasting insulin, lymphocyte
subsets, serum immunoglobulins, and a short
Synacthen test with measurement of cortisol and
Correspondence: R. Palmer, Department of Dermatology, Royal South Hants
Hospital, Southampton, SO14 0YG, UK. Tel.: 144 7979 695798.
Fax: 144 2380 825353. E-mail: roypalmer@totalize.co.uk
Accepted for publication 25 April 2001
plasia), were within normal limits. A bacterial swab
grew only coliforms. A small study of hidradenitis
suppurativa suggested that HLA A1 and B8 predispose
to more severe disease;1 our patient does not have these
antigens.
Before menarche, ovarian androgens are produced in
negligible amounts, and it is the zona reticularis of the
adrenal cortex that produces the only significant
quantities of androgens. The principal adrenal andro-
gens are dehydroepiandrosterone and androstenedione.
Levels of these are low in early childhood, but as they
rise they promote growth and axillary and pubic
hair 2 this is called adrenarche. In girls, pubic hair
typically appears at age 11. Menarche is caused by
increasing serum concentrations of gonadotrophins and
usually occurs later, between 11 and 13 years of age.
The growth of apocrine glands and onset of secretion
begin at puberty.
The clinical features and investigations of our patient
are consistent with an early puberty which is physio-
logical not pathological. She had early adrenarche and
menarche. She developed hidradenitis suppurativa
3 months after the first signs of adrenarche and before
menarche.
It is rare for hidradenitis suppurativa to occur so
young. The disease has been reported in an infant with
congenital adrenal hyperplasia,2 in an obese 7 years
and 8 month-old girl after benign premature adre-
narche,3 and in an obese 8-year-old prepubertal girl.4
We reviewed five studies1,5±8 which reported the age of

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Early-onset hidradenitis suppurativa X R. A. Palmer and M. Keefe

Figure 1 Right inguinal region.

onset in a total of 225 females and 50 males. The data
in these studies were presented in a comparable manner,
and therefore it is valid to combine the data, giving the
frequency distribution for the age of onset of hidrade-
nitis suppurativa shown in Fig. 2. Only 2% of patients
had onset of the condition before the age of 11 years.
These studies were conducted among patients referred
to secondary care and therefore probably select for
patients with relatively severe disease. Disease severity
may be positively associated with early age at onset, and
therefore the true percentage is likely to be lower than
2%. The peak age at onset among females was 11±
20 years and among males was 21±30 years.
One explanation for this age distribution is that the
disease is androgen-dependent. The earlier onset in
females may partly reflect earlier puberty, even
though most patients have a delay between puberty
and the onset of the condition. Pre-menstrual and
menstrual exacerbations are common.5,7 Some reports
describe a tendency to improve in pregnancy, and
subsequently relapse after childbirth.5 Hidradenitis
suppurativa may be exacerbated by those oral contra-
ceptives that contain a relatively androgenic progesto-
gen, or a high progestogen to oestrogen ratio.9 In
some women the disease can be controlled with
ethinyloestradiol plus cyproterone acetate.10 A com-
parison can be made with other diseases of the skin
adnexae; hirsutism and acne vulgaris are both
androgen-dependent. Rarely hidradenitis suppurativa
occurs in neonates and infants, but subsequently
resolves later in infancy. This may reflect the relatively
hyperandrogenaemic state during this period. How-
ever, if the disease is dependent on androgens, then it
is surprising that it is more common among women
than among men, and the relationship has therefore
been questioned.11
The serum androgen levels of our patient are not
abnormally high. This is consistent with most studies,

Table 1 Endocrinological investigations.

Result Pre-puberty NR Adult NR

Androstenedione (nmol/L) 3.9 0.4±3.3 0.8±11.9

Dehydroepiandrosterone
sulphate (mmol/L) 2.6 0.3±1.5 3.6±9.9
Testosterone (nmol/L) 0.9 , 2.0 0.3±2.5
Sex-hormone binding globulin (nmol/L) 53.3 55±120 30±90
Free androgen index 0.02 , 0.06
Luteinizing hormone day 4 (IU/L) 2.3 , 2.0 0.8±12
Follicle stimulating hormone day 4 (IU/L) 5.3 , 2.0 0.8±11.5
Oestradiol day 4 (pmol/L) , 73.4 40±90 110±180

NR, Normal range.

502 q 2001 Blackwell Science Ltd X Clinical and Experimental Dermatology, 26, 501±503

Figure 2 Age of onset of hidradenitis suppurativa in 275 patients.
which have generally found a higher median concen-
tration of androgens among patients compared with
controls, but which have nevertheless found that the
majority of patients have androgen concentrations
within the normal range.5,6 This situation is analogous
to that found in acne vulgaris and hirsutism. Therefore
androgens may be exerting most of their effect via an
exaggerated end-organ response to relatively normal
androgen levels. The mechanism of this is not known,
but could involve, for example, increased activity of 5-
alpha-1 reductase.
Most apocrine glands open into the upper part of
follicles, above the sebaceous gland. The initiating event
in hidradenitis suppurativa is probably keratinous
plugging of the follicular infundibulum in apocrine-
bearing skin.12 This is consistent with the inclusion of
the condition in the follicular occlusion triad, with acne
conglobata and perifolliculitis capitis. Androgen recep-
tors are present in pilosebaceous duct keratinocytes and
may allow androgens to promote hypercornification.
Keratinous plugging causes rupture of follicles, inflam-
mation and infection, and apocrine glands become
secondarily involved.
The family history of our patient suggests a genetic
predisposition. It would be consistent with the familial
form of hidradenitis suppurativa with autosomal domi-
nant inheritance that has been proposed.13
In conclusion, we hypothesize that the rise in serum
androgens at puberty precipitated hidradenitis suppur-
ativa in our patient, who was genetically predisposed to
the condition. Partly because she had an early puberty
she developed the disease at an exceptionally early age.
q 2001 Blackwell Science Ltd X Clinical and Experimental Dermatology, 26, 501±503
Early-onset hidradenitis suppurativa X R. A. Palmer and M. Keefe
Acknowledgements
We are grateful for the contributions of J. H. Barth
(Institute of Pathology, Leeds General Infirmary), P. Betts
(Department of Paediatrics, Southampton University
Hospitals Trust), P. S. Friedmann, C. Morgan and I.
Pearson (all at the Department of Dermatology, South-
ampton University Hospitals Trust).
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