INTRODUCTION
Hidradenitis suppurativa (HS), also known as acne inversa, is a chronic
inflammatory skin disease characterized by painful deep subcutaneous nodules
with phlogistic signs, which are initially hard and progress to have a soft
consistency. Complications include the formation of fistulas and
suppuration.
HS occurs in 1-4% of the world population, most commonly in the second and third
decades of life, with a higher incidence at 23 years of age, and is more common
in women (3.3F:1M), with an estimated prevalence of 1 in 300 adults. It mostly
affects the inframammary (23%) and inguinal (93%) regions in women and the
gluteal (40%) and perianal regions (51%) in men, all rich in apocrine
glands1.
Etiology of HS is still poorly understood and is suggested to occur due to
occlusion of the apocrine duct of the hair follicles by triggering factors such
as friction of the adipose tissue, poor hygiene, excessive sweating, heat, tight
clothing, depilation, and deodorants. Diagnosis is eminently clinical through
the identification of typical recurrent lesions that include suppurative and/or
painful nodules, abscesses, sinus tracts, or scars1,2.
There are no pathognomonic tests, but laboratory abnormalities in leukocyte
counts and C-reactive protein levels can be detected. The possible differential
diagnoses are abscesses, carbuncles, lymphogranuloma venereum, inflammation of
the Bartholin’s gland, furunculosis, and infection of epidermoid cysts, among
others.
HS is a disease with variable progression and is difficult to manage, which can
be done with topical or systemic therapy or by surgical excision. Imaging
assessment, although not specific enough for a diagnosis, is useful to determine
the extent of the disease, can exclude differential diagnoses, and allow for
planning of the surgical procedure. The objective of this work was to review the
imaging assessment of HS and to depict a case with magnetic resonance
imaging.
METHODS
This article is a literature review. Articles in English, Portuguese, and Spanish
were selected in the SciELO, PubMed, Bireme, and Google databases from January
2017 to February 2018. The following keywords were used: “hidradenitis
suppurativa,” “magnetic resonance,” “ultrasound,” “acne inversa”.
RESULTS
Although the diagnosis of HS is clinical, exams can have an important role in the
grading, characterization, assessment of differential diagnoses, and surgical
planning, especially magnetic resonance imaging, with the potential to reduce
postoperative recurrences.
DISCUSSION
HS is a chronic and slowly-evolving disease. The signs and symptoms of the
disease can emerge years after the first presentation and its clinical diagnosis
is based on three criteria2:
1. Typical lesions (that is, painful deep-seated nodules): abscesses,
bridge scars, draining fistulas, and pseudocomedones
2. Lesions in at least one typical area: armpits, inframammary region in
intermammary folds, groin, perineum, or buttocks
3. Two recurrences of the lesions in a period of 6 months
Hurley’s classification stratifies the disease in three stages. Stage I is
characterized by the presence of an abscess, single or multiple, without
draining fistulas or scars. Stage II is characterized by a recurrent abscess,
single or multiple, with draining fistulas, scars and well-spaced lesions. In
stage III, there is diffuse involvement or with multiple interconnected fistulas
and abscesses throughout the region2.
There is no single effective or curative treatment for HS, but there is a
wide-range of therapies for its management, which include diverse treatments
such as antibiotic therapy, immunosuppression, TNF-alpha inhibitor drugs,
surgery, and radiation2. Most therapies
for HS have low levels of evidence (categories C and D).
The surgical approach of mild to moderate cases of HS (Hurley stages I and II) is
performed through local excisions and primary closure. In the more severe cases
(Hurley stage III), the radical surgical approach is the treatment of choice and
has presented better results, with low recurrence rates. It consists in more
extensive surgeries with closure by secondary intention or grafts2.
Assessment using only clinical parameters generally underestimates the extent and
staging of the lesions3. Based on
ultrasound findings, Zarchi & Jemec4
changed the conduct in 28 of 32 patients (82%), due to under-staging of the
clinical assessment. Moreover, Worstman et al.5 identified collections not clinically diagnosed in 26 of 34
patients (76%) by ultrasonography, in addition to subclinical fistulas in 10
patients (29%).
There is evidence that 64% of patients with HS in the anogenital region present
anal fistulas and its incomplete resection is one of the major causes of HS
recurrence (up to 25%). Therefore, clinical assessment using imaging methods
becomes fundamental and Doppler ultrasonography and magnetic resonance imaging
(MRI) stand out among the existing imaging techniques. Ultrasonography is a more
accessible and available method and is a powerful tool for the initial imaging
exam in the assessment of HS. On the other hand, MRI stands out for observations
of fistulous tracts, especially in anogenital lesions3, providing high-definition images of the hypodermis and
deep tissues, and is useful when there is an association with other diseases,
such as Crohn’s disease3.
The imaging results include fistulas, granulomas, abscesses, scar bridges, and
dermal and subcutaneous thickening of well-delimited borders6. Abscesses are characterized by
circumferential morphology in the subcutaneous tissue, with a low signal in T1
and high in T2, with evident contrast enhancement due to the contrast medium and
restricted diffusion (Figures 1 to 3). Fistulas in the subcutaneous and dermal
tissues of the anal canal and the distal part of the rectum can mimic Crohn’s
disease or cryptoglandular disease. These diseases can also coexist with HS,
which impairs the ability to determine a diagnosis6,7.
Figure 1 - Fat-suppressed gadolinium-enhanced axial T1-weighted MR images
(left) and axial T2-weighted (right) showing intense contrast
enhancement in the affected region (delimited by the red line) and
subcutaneous abscesses forming fistulous tracts (delimited by the
orange line).
Figure 1 - Fat-suppressed gadolinium-enhanced axial T1-weighted MR images
(left) and axial T2-weighted (right) showing intense contrast
enhancement in the affected region (delimited by the red line) and
subcutaneous abscesses forming fistulous tracts (delimited by the
orange line).
Figure 2 - Axial T2 fat-saturated images of the pelvis (upper left corner),
diffusion-weighted image (upper right corner), T1 fat-supressed
(lower left corner) and T1 fat-supressed post-contrast (lower right
corner) showing hyperintensity of the subcutaneous tissue and
abscesses forming fistulous tracts (delimited by the solid orange
line), restricted diffusion in the region affected by edema and
abscesses (delimited by the dashed orange line) and intense contrast
enhancement in the subcutaneous tissue in the affected area
(delimited by the solid red line).
Figure 2 - Axial T2 fat-saturated images of the pelvis (upper left corner),
diffusion-weighted image (upper right corner), T1 fat-supressed
(lower left corner) and T1 fat-supressed post-contrast (lower right
corner) showing hyperintensity of the subcutaneous tissue and
abscesses forming fistulous tracts (delimited by the solid orange
line), restricted diffusion in the region affected by edema and
abscesses (delimited by the dashed orange line) and intense contrast
enhancement in the subcutaneous tissue in the affected area
(delimited by the solid red line).
Figure 3 - Fat-suppressed gadolinium-enhanced anal canal coronal T1-weighted
(left) and anal canal coronal T2-weighted (right) showing an intense
contrast enhancement of the subcutaneous tissue in the affected
region (delimited by the red line) and hyperintensity (representing
oedema) of the subcutaneous tissue, associated with abscesses
forming fistulous tracts (delimited by the orange line).
Figure 3 - Fat-suppressed gadolinium-enhanced anal canal coronal T1-weighted
(left) and anal canal coronal T2-weighted (right) showing an intense
contrast enhancement of the subcutaneous tissue in the affected
region (delimited by the red line) and hyperintensity (representing
oedema) of the subcutaneous tissue, associated with abscesses
forming fistulous tracts (delimited by the orange line).
To differentiate HS from cryptoglandular disease, the time to disease progression
should be considered, which tends to be shorter for the latter. Moreover,
cryptoglandular disease is limited to the perianal region. In relation to
Crohn’s disease, HS presents more inflammatory findings (subcutaneous edema and
granulomas), greater bilaterality, less occurrence of fistulas and less
sphincter involvement8. Biopsies are
required in cases of doubt with other diagnoses such as atypical perianal
Crohn’s disease, carcinoma, and tuberculous ulcer.
CONCLUSION
Although the diagnosis of hidradenitis suppurativa is eminently clinical, the
complementation of the clinical assessment with imaging exams has the potential
to reduce postoperative recurrences due to better staging and characterization
of the lesions.
The most important imaging methods for the assessment of HS include Doppler
ultrasonography and magnetic resonance imaging. MRI is particularly relevant in
more severe cases of the disease and is a useful technique to establish a
diagnosis for HS, determine the extent of the lesion, and plan the optimal
therapeutic management. MRI results are relatively nonspecific, with thickening
of dermal and subcutaneous tissues, with low intensity signal in T1 and high in
T2 and STIR. However, the presence of these findings together with fistulas,
sinus tracts, and scars, especially in the anal canal, are highly suggestive of
HS.
COLLABORATIONS
AAL
|
Analysis and/or data interpretation, conception and design study,
final manuscript approval, methodology, writing - original draft
preparation, writing - review & editing.
|
GNM
|
Analysis and/or data interpretation, conception and design study,
data curation, writing - original draft preparation.
|
BMMLD
|
Conception and design study, data curation, writing - original draft
preparation.
|
GDS
|
Conception and design study, conceptualization, project
administration, supervision, writing - review & editing.
|
LQS
|
Conceptualization, methodology, project administration, resources,
supervision.
|
REFERENCES
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hidradenitis suppurativa. Actas Dermosifiliogr. 2015;106 Suppl 1:49-59. DOI:
https://doi.org/10.1016/S0001-7310(16)30007-2
2. Revuz J. Hidradenitis suppurativa. Presse Med. 2010;39(12):1254-64.
PMID: 20965688 DOI: https://doi.org/10.1016/j.lpm.2010.08.003
3. Wortsman X. Imaging of Hidradenitis Suppurativa. Dermatol Clin.
2016;34(1):59-68. DOI:
https://doi.org/10.1016/j.det.2015.08.003
4. Zarchi K, Jemec GB. The role of ultrasound in severity assessment in
hidradenitis suppurativa. Dermatol Surg. 2014;40(5):592. DOI: 10.1111/dsu.12437
DOI: https://doi.org/10.1111/dsu.12437
5. Wortsman X, Moreno C, Soto R, Arellano J, Pezo C, Wortsman J.
Ultrasound in-depth characterization and staging of hidradenitis suppurativa.
Dermatol Surg. 2013;39(12):1835-42. DOI:
https://doi.org/10.1111/dsu.12329
6. Montaña CN, Labra WA, Panussis FD. Hidradenitis supurativa:
evaluación por resonancia magnética. 2014. Rev Chil Radiol.
2014;20(4):159-63.
7. Kelly AM, Cronin P. MRI features of hidradenitis suppurativa and
review of the literature. AJR Am J Roentgenol. 2005;185(5):1201-4. PMID:
16247134 DOI: https://doi.org/10.2214/AJR.04.1233
8. Monnier L, Dohan A, Amara N, Zagdanski AM, Drame M, Soyer P, et al.
Anoperineal disease in Hidradenitis Suppurativa : MR imaging distinction from
perianal Crohn’s disease. Eur Radiol. 2017;27(10):4100-9. DOI:
https://doi.org/10.1007/s00330-017-4776-1
1 . Hospital de Base do Distrito Federal, Brasília,
DF, Brazil.
2 . Universidade Católica de Brasília, Brasília,
DF, Brazil.
Corresponding author: Arthur Ataíde Lopes, SEPS
710/910, Ed. Via Brasil, Galeria, Loja 40, Asa Sul, Brasília, DF, Brazil, Zip
Code 70390-108. E-mail: arthurbsb@gmail.com
Article received: April 22, 2018.
Article accepted: April 16, 2019.
Conflicts of interest: none.