MISCELÁNEO Hidradenitis suppurativa, evaluation using MRI

Dres. Nelson Montaña C(1), Andrés Labra W(2), Daniela Panussis F(1).

1. Radiology Resident, Universidad Mayor. Santiago – Chile. 2. Radiologist, Hospital Barros Luco Trudeau. Clínica Alemana Santiago. Santiago – Chile.

Hidradenitis suppurativa (HS), evaluation using MRI

Abstract: This corresponds to an inflammatory disease of the apocrine glands, distributed mainly in the axi- llary, perineal and perianal regions, clinically characterized by pain, swelling, and smelly discharge. Treatment is expensive, long and leaves large scars, generating economic and psychological problems for the patient. Magnetic resonance imaging (MRI) helps to confirm the diagnosis and determine its extension, which allows it to be distinguished from other entities and to choose the best treatment. To our knowledge, there is only one review of literature dedicated to the characteristics of this pathology using MRI, therefore we present an illustrated literature review in different patients treated at our center. Keywords: Acne inversa, Hidradenitis suppurativa, MRI, Verneuil’s disease.

Resumen: Corresponde a una enfermedad inflamatoria de las glándulas apocrinas, distribuidas principalmente a nivel axilar, perineal y perianal, caracterizado clínicamente por dolor, tumefacción y secreción mal oliente. Su tratamiento es costoso, largo y deja grandes cicatrices, generando problemas económicos y psicológicos para el paciente. La resonancia magnética (RM) ayuda a confirmar el diagnóstico y a determinar su extensión, lo que permite diferenciarlo de otras entidades y elegir el mejor tratamiento. A nuestro conocimiento, existe sólo una revisión de la literatura dedicada a las características de esta patología en resonancia magnética, por lo que presentamos una revisión de la literatura ilustrada en diferentes pacientes tratados en nuestro centro de atención. Palabras clave: Acné reverso, Enfermedad de Verneuil, Hidradenitis supurativa, RM.

Montaña N, Labra A, Panussis D. Hidradenitis supurativa, evaluación por resonancia magnética. Rev Chil Radiol 2014; 20(4): 159-163. Correspondence: Andrés Labra W. / [email protected] Article received 06 June 2014. Accepted for publication 22 august 2014.

Introduction Epidemiology First described by Velpeau in 1839(1) and then Its prevalence is estimated to be up to 1% in the by Verneuil in 1864(2) suppurative adenitis is a general population(7,8), currently there are controversies recurring inflammatory disease of the apocrine as to which gender it generally affects more, although glands which are located in the subcutaneous there are locations that favor more one gender: the cellular plane and are distributed mainly in the inguinal form is more frequent in women, the perianal armpits, and perianal region(3,4). It greatly form more in men and the axillary has no preference(5,7,8). compromises the quality of life of patients given This condition is rarely seen in patients prior to puberty the great pain it causes, associated bad odor, or after menopause, varying its presentation in ages high recurrence of the symptoms, costly treatment between 11 and 50 years old, with an average of 23 and fibrotic sequelae that lead to disfigurement years(6-8). In up to 25% of cases there is family history and large surgical resections of the patient(4,5). Its with an autosomal dominant inheritance pattern(6,9). mechanism is unclear and its management is very Some risk factors are trauma, use of androgens, broad, using topical treatments, oral medications using antiperspirants, obesity, smoking(7,10,11). Also or surgical resections and should be personalized described have been some autoimmune defect(12,13) for each patient based on their clinical and imaging and hormonal influences(4). There also exists a link to characteristics(3,4,6). other diseases such as Crohn’s disease (with which 159 Dr. Nelson Montaña C, et al. Revista Chilena de Radiología. Vol. 20 Nº 4, año 2014; 159-163. it constitutes a differential diagnosis), Dowling Degos Features in MRI disease and inflammatory arthritis(14). The reasons for requesting a magnetic resonance imaging (MRI) of the in this disease are three: Physiopathology confirm the diagnosis, determine its extent and make The apocrine glands are found in the thickness of the differential diagnosis with Crohn’s disease and subcutaneous tissue, distributed in the axillary area, Crypto-glandular inflammatory disease. On examination, perineum, inguinal crease and to a lesser extent in a thickening of the dermis and subcutaneous plane the interscapular region, periareolar region, pubis, is observed, which is of low signal on T1 sequences, retroauricular, scalp, eyelids and malar regions. high signal on T2 sequences, with well defined edges They open into a hair follicle and are responsible (Figure 1). Presents enhancement with the use of for the odor in perspiration. Although the cause why paramagnetic contrast agent (Figure 2) and restricts this disease is triggered is not entirely clear, it is diffusion (Figure 3). These alterations are associated postulated that an obstruction occurs in the hair duct with rounded collections in the subcutaneous plane and its apocrine glands, followed by an infection of corresponding to abscesses, which are of low signal the follicle and after that an eventual rupture in the on T1, high signal on T2 sequences, sometimes diffi- subcutaneous space generating abscesses in its cult to identify because of the adjacent inflammatory thickness. These, like other abscesses, can evolve changes, becoming evident with the use of Gadoli- to resolution or fistulization with important fibrotic nium, presenting annular enhancement with the use scarring phenomena(3,4,6,9). of contrast and restricted diffusion (Figure 4). You can also observe sinus tracts distributed in the sub- Clinical condition cutaneous layer of the affected areas that flow to the It is a condition of insidious onset, beginning with skin (Figure 5), which in the case of perianal location, itching associated with erythema and then hyperhi- these can lead to the in its distal third and the drosis. Subsequently, the lesions become painful, , constituting a differential diagnosis with nodular and coalesce forming abscesses, draining Crohn’s disease and Crypto-glandular disease, both into the skin(3,6). In the perianal affected area, they being able to coexist(9,16,19). can also drain into the rectum and anus(3,9) and can The patient’s age, its classic distribution, fistulas coexist with two other inflammatory diseases, Crohn’s draining into the skin from small subcutaneous abs- disease and Crypto-glandular inflammatory disease, cesses and high recurrence of the condition orient us also constituting a differential diagnosis(9,15,16,19 20). to the diagnosis of HS. The Crypto-glandular disease Once the acute condition has passed, there is a high usually has no chronicity and recurrence of the disease rate of recurrence, which eventually leads to scarring and is limited to the perianal region. Crohn’s disease is and local deformity associated with the presence usually accompanied by other manifestations such as of fistulas. The diagnosis is purely clinical, creating ulcerations, colonic strictures, fistulas of the digestive some classification systems to guide treatment(17,18). tract and skin lesions(9,20).

1a 1b

Figure 1. Pelvis MRI. Axial T1-weighted sequence (a) and T2 (b) in a patient suffering from Crohn’s disease and hidradenitis suppurativa as a complication. Thickening of the dermal plane and subcutaneous tissue of the inner thighs is observed, extending to the perineum. This thickening has well defined borders representing the inflammatory process secondary to the compromising of the apocrine glands. 160 Revista Chilena de Radiología. Vol. 20 Nº 4, año 2014; 159-163. MISCELÁNEO

Figure 2. Pelvis MRI. Sagittal T1-weighted sequence with fat saturation in portal-venous phase after injection of paramagnetic contrast medium in a patient with hidradenitis suppurativa. A substantial enhancement of the dermal plane and subcutaneous tissue of the intergluteal cleft, perineum region and inner thighs is observed.

3a 3b

Figure 3. Pelvis MRI. Axial diffusion sequence (a) b800 and (b) ADC map at the same level as displayed in Figure 1. An important restricted diffusion with correlation in ADC map of the thickening of the dermal plane and subcutaneous tissue previously described can be seen.

4a 4b

Figure 4. Pelvis MRI. Axial T1-weighted sequence with fat saturation in portal-venous phase after injection of paramagnetic contrast in patients illustrated in Figures 1 (a) and 2 (b), showing significant uptake of the paramagnetic contrast agent by the inflammatory process in both patients and in the thickness of this nodular images can be identified that present annular enhancement with the gadolinium, compatible with abscesses (arrows).

161 Dr. Nelson Montaña C, et al. Revista Chilena de Radiología. Vol. 20 Nº 4, año 2014; 159-163.

5a 5b

Figure 5. Pelvis MRI. Axial T2-weighted sequence in the patient of Figure 1, distal slice at the external anal sphincter (a) and another immediatelydistal to the latter (b) 2 fistulas originating at 12 and 2 o’clock (hourly positions) are evident, of descending trajectory and merging to drain together in the skin of the inner side of the left thigh.

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