DOI:10.2214/AJR.04.1233
AJR 2005; 185:1201-1204
© American Roentgen Ray Society
MRI Features of Hidradenitis Suppurativa and Review of the Literature
Aine M. Kelly1 and
Paul Cronin
1 Both authors: Department of Radiology, University of Michigan, University of
Michigan Hospitals, B1 132 H Taubman Center, 1500 E Medical Center Dr., Ann
Arbor, MI 48109-0030.
Received August 3, 2004;
accepted after revision November 10, 2004.
Address correspondence to A. M. Kelly
(ainekell{at}med.umich.edu).
Introduction
We describe a case of hidradenitis suppurativa in a 31-year-old woman with
Crohn's disease diagnosed by MRI and confirmed histologically. This disorder
is rare and is characterized by recurrent abscesses, sinus tract formation,
and scarring. It shares characteristics with acne conglobata, dissecting
cellulitis of the scalp, and pilonidal sinus.
The imaging findings include marked thickening of the skin, induration of
the subcutaneous tissues, and formation of multiple subcutaneous abscesses.
The differential diagnosis for these findings includes carbuncles,
lymphadenitis, and infected Bartholin's or sebaceous cysts.
Two previous cases are described in the radiology literature, with only one
report describing the MRI findings. However, these two reports describe the
imaging features of the complications of this disease rather than the imaging
features of the disease. We describe the MRI features within the skin and
subcutaneous tissues of the typical form of hidradenitis suppurativa that, to
our knowledge, has not been described previously. We also review the
literature. Hidradenitis suppurativa is usually diagnosed clinically. This
disease may be chronic and progressive; there is no single effective
treatment, and surgical débridement may be required. Our patient
eventually required radical surgery for treatment.
Case Report
A 31-year-old woman with a history of Crohn's disease presented on several
occasions to the emergency department at our hospital. She had Crohn's disease
that was diagnosed 20 years earlier. She had been treated previously with
right hemicolectomy and right iliac fossa end stoma. She had vaginal burning
and more recently had multiple pus-filled lesions discharging into her groin.
These lesions had been confined to her groin, but over the previous few weeks
the lesions had spread to involve the thighs, trunk, back, and face.
On examination, the patient had multiple extensive pustular lesions in
various stages of evolution throughout her groin and including her labia
minora. Some of these were ulcerated and raw, with others draining frank pus.
Lesions measured up to 15 mm in diameter. The labia minora were swollen,
erythematous, and extremely tender. Examination with the patient under
anesthesia revealed multiple sinus tracts and abscesses with induration
involving the entire vulva and purulent drainage from more than 20 sites on
the vulva, medial thighs, and mons pubis. She was referred for MRI because of
her history of Crohn's disease to evaluate for perineal abscess formation.
MRI was performed on a 1.5-T scanner (Signa, GE Healthcare) using our
fistula protocol. This protocol includes the following sequences: a
three-plane localizer, coronal single-shot fast spin-echo, axial T1-weighted
spin-echo, axial T2-weighted fast spin-echo with fat saturation, axial STIR,
sagittal STIR, axial 2D spoiled gradient-recalled echo before and after
contrast administration, and coronal 2D spoiled gradient-recalled echo after
contrast administration.
MRI of the pelvis revealed marked thickening of the skin and induration of
the subcutaneous tissues over the medial aspects of the thighs, perineum, and
mons pubis, which were of low signal on T1-weighted images and high signal on
T2-weighted and STIR images (Figs.
1A and
1B). In addition, multiple
small areas of low signal on T1-weighted and of high signal on T2-weighted
(Fig. 1C) and STIR images were
shown in the subcutaneous tissues. After IV contrast administration, many of
these small subcutaneous areas showed rim enhancement compatible with multiple
abscesses (Figs. 1D,
1E,
1F,
1G). Enlarged lymph nodes were
present in both inguinal regions (Figs.
1H and
1I). No communication with the
bladder, urethra, or rectum was detected
(Fig. 1J), and the abscesses
were distinctly remote from the rectum and anus. These findings are not
compatible with the fistulous tracts associated with Crohn's disease, and
given the clinical history and radiologic appearance, a diagnosis of
hidradenitis suppurativa was considered.
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Fig. 1A —31-year-old woman with hidradenitis suppurativa. Axial
T1-weighted spin-echo MR image (TR/TE, 467/9) obtained through upper thigh
without fat saturation shows marked cutaneous thickening of medial thighs that
is of low signal intensity (arrows).
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Fig. 1B —31-year-old woman with hidradenitis suppurativa. Axial
T2-weighted fast spin-echo MR image (3,800/84) obtained through upper thigh
with fat saturation shows edema of subcutaneous tissues with skin thickening
of high signal intensity (arrows).
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Fig. 1C —31-year-old woman with hidradenitis suppurativa. Axial
T2-weighted fast spin-echo MR image (3,800/84) of labia majora obtained
through perineum with fat saturation shows ovoid area of high signal intensity
(long arrow) that is consistent with abscess and reveals skin
induration and thickening. Also, note enlarged lymph nodes (short
arrows).
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Fig. 1D —31-year-old woman with hidradenitis suppurativa. Axial
T1-weighted spoiled gradient-recalled echo MR image (250/3.1) obtained through
perineum with fat saturation after IV contrast administration at same level as
C also shows ovoid area of high signal intensity (long arrow)
after IV contrast administration consistent with abscess. Also, note enlarged
lymph nodes (short arrows).
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Fig. 1E —31-year-old woman with hidradenitis suppurativa. Axial
T1-weighted spoiled gradient-recalled echo MR image (250/3.1) of labia majora
obtained through perineum with fat saturation after IV contrast administration
shows marked enhancement of indurated and thickened skin and of subcutaneous
tissues (long arrows). Also, note enhancing enlarged lymph nodes
(short arrows).
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Fig. 1F —31-year-old woman with hidradenitis suppurativa. Axial
T2-weighted fast spin-echo MR image (3,800/84) obtained through mons pubis
with fat saturation shows several high-signal-intensity foci (arrows)
consistent with abscesses in cutaneous and subcutaneous tissues anterior to
symphysis pubis.
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Fig. 1G —31-year-old woman with hidradenitis suppurativa. Axial
T1-weighted spoiled gradient-recalled echo MR image (250/3.1) obtained through
mons pubis with fat saturation after IV contrast administration shows marked
enhancement of indurated and thickened skin and inflammatory stranding of
subcutaneous tissues (arrows).
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Fig. 1H —31-year-old woman with hidradenitis suppurativa. Coronal
T1-weighted spoiled gradient-recalled echo MR image (240/2.4) obtained through
mons pubis with fat saturation after IV contrast administration shows enlarged
lymph nodes (thin arrows). Also, note marked enhancement of indurated
and thickened skin and inflammatory stranding of subcutaneous tissues
(thick arrow).
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Fig. 1I —31-year-old woman with hidradenitis suppurativa. Coronal
T1-weighted spoiled gradient-recalled echo MR image (240/2.4) obtained with
fat saturation after IV contrast administration shows small rim-enhancing area
(thick arrow) within medial thigh that is compatible with abscess.
Also, note skin induration and thickening with inflammatory stranding of
subcutaneous tissues (thin arrows).
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Fig. 1J —31-year-old woman with hidradenitis suppurativa. Coronal
T1-weighted spoiled gradient-recalled echo MR image (240/2.4) obtained with
fat saturation after IV contrast administration shows normal appearance of
rectum.
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Before MRI, the patient underwent vulvar biopsy. Several days later the
histology results became available. The results revealed scar with intense
chronic inflammation with ruptured squamous-lined tracts in the dermis,
consistent with hidradenitis suppurativa.
Unfortunately, our patient has required extensive surgical treatment with
abscess incision, drainage, and evacuation. She later underwent radical
resection of the lower abdomen, bilateral thighs, and bilateral buttocks. This
procedure was then followed with radical vulvectomy, further thigh resection
and groin resection, and mons pubis resection.
Discussion
Hidradenitis suppurativa, first described in 1839 by Velpeau, is a chronic
disease manifest by recurrent abscesses, sinus tracts, and scarring
[1]. This disease typically
affects the genitofemoral area in women or axillae in both sexes. It is also
known as Verneuil's disease or acne inversa. It is a member of the follicular
occlusion tetrad along with acne conglobata, dissecting cellulitis of the
scalp, and pilonidal sinus
[1].
Little is known about its cause to date
[2], but it is thought that the
initial event is follicular hyperkeratosis with occlusion of the follicle.
Dilatation of the follicle is followed by rupture and spillage of contents
into the surrounding dermis. This induces a chemotactic response with a
resultant inflammatory cell infiltrate. The apocrine glands are secondarily
involved, and secondary infection may also occur.
Hidradenitis suppurativa has a prevalence of approximately 1 in 300
[3] and is more common in
women, with a ratio of three females to each male affected. Onset is most
common from childhood to middle age with a peak during puberty. Onset is
rarely described in patients before puberty or after menopause.
In approximately a quarter of the cases, there is a family history with an
autosomal-dominant mode of inheritance
[4]. Several disease entities
have been reported as being associated with hidradenitis suppurativa including
Crohn's disease, Dowling Degos disease (acquired reticulate pigmented macules
in the flexures), and arthropathy
[5]. A hormonal influence is
cited [4], and antiandrogen
therapy has been used with benefit
[4]. Bacterial infection is
implicated in the pathogenesis of the disease
[4]. Obesity may aggravate the
disease. Smoking is reported more frequently among patients with hidradenitis
suppurativa [4]. The diagnosis
is clinical, and no specific diagnostic test exists. Hidradenitis suppurativa
has a variable clinical course
[6].
Sinus tracts may develop, and fistula formation has been described.
Perianal hidradenitis suppurativa mimics the presentation of Crohn's disease,
anal fistula, pilonidal sinus, or perianal abscess
[7,
8]. Crohn's disease and
hidradenitis suppurativa may coexist, as in our patient, making diagnosis
difficult. Because our patient did not have sinus tract formation extending to
the bowel, distinguishing the cutaneous and subcutaneous manifestations of
Crohn's disease from those of hidradenitis suppurativa was easier. Nadgir et
al. [8] described perirectal
sinus tract and fistula formation caused by hidradenitis suppurativa on
double-contrast barium enema examination simulating inflammatory bowel disease
in a patient without inflammatory bowel disease.
In our patient, MRI revealed marked thickening of the skin, induration of
the subcutaneous tissues, and formation of multiple subcutaneous abscesses.
The thickened skin and indurated subcutaneous tissues were of low signal on
T1-weighted images and high signal on T2-weighted and STIR images, probably
reflecting tissue edema. The areas of abscess formation were of low signal on
T1-weighted images and high signal on T2-weighted and STIR images and showed
peripheral rim enhancement after IV contrast administration. The disease was
confined to the skin and subcutaneous tissues of the perineum and medial
thighs. No communication with the pelvic organs, such as the bladder, urethra,
rectum, or anus, was present. In addition, the lymph nodes in both inguinal
regions were enlarged.
The differential diagnosis for these appearances includes carbuncles,
lymphadenitis, and infected Bartholin's or sebaceous cysts. Complications may
be local or systemic. Infection may develop, leading to septicemia, and MRI
features of a lumbosacral epidural abscess have been described
[9]. Anemia or leukocytosis may
occur. Scarring and fibrosis lead to contractures and decreased mobility. With
chronic disease, strictures may develop in the anus, urethra, or rectum. In
addition, disfiguring genital edema may develop. Arthropathy associated with
hidradenitis suppurativa has variable clinical features, and its activity is
associated with hidradenitis suppurativa disease activity
[5]. Squamous cell carcinoma
may rarely develop in chronically inflamed areas and scars in long-standing
cases [4].
There is no single effective treatment, and therapies include systemic
antibiotics, topical antiseptics, and compresses. Intralesional
corticosteroids have been used, as have systemic retinoids. Antimetabolites
and antitumor necrosis factor antibody have shown efficacy in one patient with
Crohn's disease and hidradenitis suppurativa. Antiandrogens and
5
reductase inhibitors also may provide some benefit. Surgical removal
of all involved tissue is the definitive treatment
[4], as in our patient.
Postoperative recurrence is common after incision and drainage and limited
surgical excision. Laser treatment with carbon dioxide is an alternative in
mild to moderate cases. Several authors have reported the benefit of radiation
therapy [4].
In conclusion, hidradenitis suppurativa is a rare disease that typically
affects the genitofemoral area, axillae, or both. MRI findings are relatively
nonspecific with skin thickening and induration of the subcutaneous tissues
that are of low signal on T1-weighted images and high signal on T2-weighted
and STIR images. There also may be subcutaneous abscess formation; abscesses
are of low signal on T1-weighted images and high signal on T2-weighted and
STIR images and show peripheral rim enhancement after IV contrast
administration. Although these are the typical features of hidradenitis
suppurativa, these are not distinguishable from those of erysipelas
(inflammation of the epidermis, usually due to a bacterial infection) or
cellulitis (inflammation of the dermis). If sinus tract formation and fistula
formation are noted and scarring is present, then the diagnosis of
hidradenitis suppurativa should be considered. Hidradenitis suppurativa is
still a possible diagnosis in patients with Crohn's disease because these
entities can coexist, as in our patient.
References
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–54[CrossRef][Medline]
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of hidradenitis suppurativa. Ann R Coll Surg Engl1997; 79:83
–89[Medline]
- Fitzsimmons JS, Guilbert PR, Fitzsimmons EM. A family study of
hidradenitis suppurativa. J Med Genet1985; 22:367
–373[Abstract/Free Full Text]
- Slade DE, Powell BW, Mortimer PS. Hidradenitis suppurativa:
pathogenesis and management. Br J Plast Surg2003; 56:451
–461[Medline]
- Hamoir XL, Francois RJ, Van den Haute V, Van Campenhoudt M.
Arthritis and hidradenitis suppurativa diagnosed in a 48-year-old man.
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- Jemec GB. Hidradenitis suppurativa. J Cutan Med
Surg 2003; 7:47
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- Church JM, Fazio VW, Lavery IC, Oakley JR, Milsom JW. The
differential diagnosis and comorbidity of hidradenitis suppurativa and
perianal Crohn's disease. Int J Colorectal Dis1993; 8:117
–119[Medline]
- Nadgir R, Rubesin SE, Levine MS. Perirectal sinus tracks and
fistulas caused by hidradenitis suppurativa. AJR2001; 177:476
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- Russ E, Castillo M. Lumbosacral epidural abscess due to
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Introduction
Case Report
