JDV131.
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EDITOR IAL
What’s new in acne inversa (alias hidradenitis suppurativa)?
Blackwell Science, Ltd
Thomas Jansen,* Gerd Plewig†
Departments of Dermatology and Allergology, Ruhr-University Bochum and †Ludwig-Maximilians-University of Munich, Germany.
*Corresponding author, Department of Dermatology and Allergology, Ruhr-University Bochum, Gudrunstrasse 56, D-44791 Bochum, Germany,
tel. +49 0234 509 3411; fax +49 0234 509 3409
von der Werth JM, Williams HC. The natural history of
hidradenitis suppurativa. JEADV 2000; 14: 389–392.
Acne inversa (alias acne triad, acne tetrad, pyodermia fistulans
sinifica, and hidradenitis suppurativa) is a chronic, suppurative,
and cicatricial disorder that affects skin areas rich in apocrine
glands, such as the axillae, groin, and perineum.1,2 The diagnosis
is primarily clinical, based on the presence of both sinus tracts
and abscesses with a characteristic distribution. These lesions
usually respond poorly to antibiotics and tend to recur.
Eventually chronic abscess formation with progressive scar-
ring may become constant. Recurrent, foul-smelling discharge
from draining sinuses may cause extensive soiling of clothes,
forcing the individual to limit social contact and forfeit
employment. Attendant physical discomfort may preclude
sexual contacts or even walking. Malignant degeneration in
chronic sinus tracts is a rare long-term result. Squamous cell
carcinomas that arise in chronic scarred and inflamed skin
tend to be more aggressive than those resulting from chronic
actinic damage and are associated with local invasion or
recurrence after excision, distant metastasis, and high mortality.3
Articular manifestations occur sporadically in patients with
acne inversa. The findings are similar to those seen in other
seronegative spondyloarthropathies, except for lack of association
with HLA-B27.
It has been speculated that acne inversa appears to be another
cutaneous manifestation of Crohn’s disease.4 However, this
remains to be verified. Perianal lesions are the initial presenta-
tion in 5% of all patients with Crohn’s disease and, at times,
these two diseases may be clinically indistinguishable. The
comorbidity of acne inversa and Crohn’s disease has been
reported, the latter typically preceding the development of
acne inversa.
The classic view of acne inversa is that it is an occlusive and
pyogenic disorder of the apocrine glands, an hypothesis that
seemed confirmed with its experimental reproduction by Shelley
and Cahn5 in 1955. However, apocrine gland involvement is
incidental but not essential to the pathogenesis. Inflammation
of apocrine glands is a secondary phenomenon. More recent
studies have shown that acne inversa is actually a defect of
terminal follicular epithelium.6 The earliest change seen in
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JEADV (2000) 14, 342– 343
acne inversa is plugging of follicular hyperkeratosis. The
association of acne inversa with several disorders in which
poral occlusion is prominent, such as Fox–Fordyce disease,
pityriasis rubra pilaris, acanthosis nigricans, steatocystoma
multiplex, and Dowling–Degos disease,7 adds support to the
follicular theory of origin of acne inversa. Thus the term
hidradenitis suppurativa is a misnomer and should be
abandoned.
The inciting influences for the follicular occlusion have not
been fully elucidated but may result from a folliculitis induced
by local frictional trauma. Chemical irritants such as deodor-
ants, mechanical irritation, depilation, and shaving have been
considered as aetiological factors. However, no significant
difference in the use of these items compared with age-matched
controls was found. Obesity may not be an aetiological factor,
although the increased skin-to-skin contact may promote
follicular hyperkeratosis. An individual predisposition to
follicular occlusion and sinus tract formation is probably
important. Genetic factors may play a role, as a familial form
of the disease has been described.8 Several reports in the
literature link acne inversa to a single gene transmission.
The pattern of transmission and the number of affected indi-
viduals are consistent with autosomal dominant inheritance.
HLA associations are not consistent. Androgenic influences
may contribute to the predisposition, although hormonal
abnormalities are usually not demonstrable in these patients.9
The absence of acne inversa in children and its onset post-
pubertally suggest the importance of hormonal factors in its
pathogenesis. Cigarette smoking has been suggested as an
aetiological factor in the development of the disease, but
as yet, this has not be confirmed. Most patients with acne
inversa are otherwise healthy, and no predictable association
with systemic diseases such as diabetes mellitus is to be anti-
cipated. Immune defence mechanisms are normal in affected
patients. Bacterial involvement of acne inversa is not a
primary pathogenic event, but is secondary to the disease
process. The microbiological flora is not constant and may
change unpredictably. Various bacteria can be isolated
from the lesions, particularly staphylococci, streptococci, and
gram-negative rods.10 Several authors have implicated Strepto-
coccus milleri and Chlamydia trachomatis as major pathogens
© 2000 European Academy of Dermatology and Venereology
JDV131.fm Page 343 Saturday, February 10, 2001 3:42 PM
in acne inversa, but this could not be confirmed by others.
It is likely that bacteria may contribute to the inflammatory
process.
Acne inversa is curable, despite the pessimism of many who
have been frustrated in their efforts to obliterate the disease.
Although surgery can often be avoided in early stages of acne
inversa, it is an essential part of the treatment of advanced
forms and should be performed as early as possible.11 Wide
excision, well beyond the clinical borders of activity, is man-
datory, regardless of the localization of the disease. Best
functional results appear to be obtained with healing by
granulation (secondary intention). With rare exceptions, surgical
intervention is sufficient to stop the disease. Improper surgery
is doomed to fail and delays definitive treatment, although
exceptional cases resolve spontaneously. Patients should be
cautioned that new lesions may develop at a site not affected
at the time of their initial surgery. In some cases, carbon
dioxide laser treatment may provide an alternative efficient
method for the management of acne inversa. The advantage
of laser treatment has been considered to be improved hae-
mostasis, which affords better visualization and therefore more
complete removal of the affected tissue. Systemic isotretinoin
(13-cis-retinoic acid) is not as beneficial in acne inversa as it
has been shown to be in acne vulgaris. In general, drugs such
as retinoids, corticosteroids, antibiotics, and antiandrogens
are not curative, and relapse is almost inevitable when such
treatment is withdrawn. However, these drugs may be useful
as an adjunctive treatment to reduce the inflammation before
and after surgery.
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© 2000 European Academy of Dermatology and Venereology JEADV (2000) 14, 342 –343
Editorial 343
References
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2 Plewig G, Steger M. Acne inversa (alias acne triad, acne tetrad or
hidradenitis suppurativa). In: Marks R, Plewig G, editors. Acne
and Related Disorders. Dunitz, London 1989: 345– 357.
3 Dufresne RG Jr, Ratz JL, Bergfeld WF. Squamous cell carcinoma
arising from the follicular occlusion triad. J Am Acad Dermatol
1996; 35: 475 –477.
4 Tsianos EV, Dalekos GN, Tzermias C, Merkouropoulos M, Hatzis
J. Hidradenitis suppurativa in Crohn’s disease. A further support
to this association. J Clin Gastroenterol 1995; 20: 151–153.
5 Shelley WB, Cahn MM. The pathogenesis of hidradenitis
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6 Yu CCW, Cook MG. Hidradenitis suppurativa: a disease of
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8 Von der Werth JM, Williams HC, Raeburn JA. The clinical genetics
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9 Barth JH, Layton AM, Cunliffe WJ. Endocrine factors in pre- and
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10 Jemec GBE, Faber M, Gutschik E, Wendelboe P. The bacteriology
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11 Banerjee AK. Surgical treatment of hidradenitis suppurativa. Br J
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