Abstract
Introduction
Hidradenitis suppurativa (HS) is a potentially disabling chronic inflammatory disease. The intermammary location, not clearly specified in the usual phenotypic classifications, entails significant functional and cosmetic compromise.
Case Presentation
Eleven cases of predominantly intermammary disease with multiple fistulization and disfiguring scarring were observed at our Department between January 2016 and March 2020. They were young (mean age = 22 years) obese or overweight women, in which the medical-surgical approach has led to variable results.
Discussion/Conclusion
Intermammary HS possibly represents a unique phenotypic presentation, likely to be fistulizing and severe. We believe that anatomo-functional considerations, which can be paralleled to pilonidal sinus disease, contribute to its pathophysiology and striking appearance, and further emphasize the importance of multidisciplinary endeavor to reduce the morbidity associated with this seemingly overlooked location.
Keywords: Hidradenitis suppurativa, Intermammary, Sinus pilonidal
Established Facts
Hidradenitis suppurativa appears to be a disease of phenotypic heterogeneity, and subtype classification is still embryonic.
Intermammary location is not usually specified within present classifications but otherwise contemplated under the broader designation of “breasts”.
Novel Insights
Intermammary hidradenitis suppurativa is a less common presentation and possibly represents a distinct phenotype, prone to the development of severe fistulae.
We propose that some anatomical and functional specificities contribute to its clinical course and suggest its comparison to the pilonidal sinus.
Introduction
Hidradenitis suppurativa (HS) is a complex chronic inflammatory skin disorder that can result in irreversible tissue damage and scarring, with a significant impairment of quality of life. As such, early diagnosis and treatment are mandatory. HS appears to be a disease of phenotypic heterogeneity, and subtype classification is still embryonic. Intermammary location is not usually specified within present classifications, but otherwise contemplated under the more broad location of “breasts” [1, 2, 3].
Case Presentation
A total of 198 patients with HS were observed between January 2016 and March 2020 at the “Hidradenitis Suppurativa Consultation” of our Department. We noticed the striking significance of intermammary lesions in some cases. Namely, this was the prevailing location of HS inflammatory activity in 11 patients (n = 11), all presenting with mutilating fistulae with scar formation. The diagnosis was made clinically and, in some cases, confirmed by ultrasound (Fig. 1).
Fig. 1.
Young women presenting with severe intermammary HS. Note the big breasts. Fourteen-year-old with severe, disfiguring and mutilating intermammary fistulas (a); Fifteen-year-old patient with isolated intermammary affection, with fistulizing disease on presentation (b); Forty-year-old patient with untreated disease for 2 decades, with an abscess visible on ultrasound (c); Seventeen-year-old patient with intermammary fistulas who underwent surgery, with a favorable functional and cosmetic result (d). HS, hidradenitis suppurativa.
Demographic and epidemiologic features of the patients are presented in Table 1. All patients were female and mostly young (mean = 22 years, [14–56]). The mean age of HS onset was 14.1 years, with a median diagnostic delay of 3.8 years. Eighty-two percent of patients were overweight (n = 5, 45.5%) or obese (n = 4, 36.4%). The smoking rate was 9.1%, and family history for HS was absent. Comorbidities included acne vulgaris (91%, n = 10), acne conglobata (1%, n = 9.1), pilonidal sinus (PS) disease (27.3%, n = 3), hyperandrogenism (45.5%, n = 5, including polycystic ovarian syndrome [PCOS], n = 2; median FG hirsutism scoring of 15), dyslipidemia (27.3%, n = 3), arterial hypertension (18.2%, n = 2), diabetes mellitus type 2 (9.1%, n = 1), and psoriasis (9.1%, n = 1). Two patients presented with isolated intermammary lesions, with additional HS lesions located elsewhere in all other cases. More precisely, the most commonly affected body sites were the axillary (n = 7, 63.6%) and inguinal (n = 7, 63.6%) areas, with both being present in 86% of those cases (6/7); for the remainder “breast region,” concomitant inframammary lesions were seen in 6 patients (54.5%) and in the breasts itself in 2 patients (18.2%); vulva (n = 2, 18.2%) and thighs (n = 2, 18.2%) were also affected. Seventy-three percent (n = 8) of patients had 3 or more afflicted body areas. The distribution of Hurley stages I, II, and III patients were 9.1% (n = 1), 82% (n = 9), and 9.1% (n = 1), respectively. Severity was accessed as mild, moderate, and severe according to ISH4 score in 9.1% (n = 1), 54.5% (n = 6) and 36.4% (n = 4), respectively. According to DLQI index, the disease had a moderate (45.6%, n = 5) or a very large effect (36.4%, n = 4) on most patients' life. Treatment consisted of medical therapy in all cases, combined with surgery in 18.2% (n = 2), with variable success (Table 1).
Table 1.
Demographic, epidemiologic, and clinical characteristics of patients with intermammary lesions of HS
| Mean age of onset, years | 14.1±3.1 |
| Mean duration of disease, years | 3.8±5.8 |
| Mean BMI | 29.0±3.8 |
| Smoking habit, % (n) | 9.1 (1) |
| Comorbidities, % (n) | |
| Acne vulgaris | 91.0 (10) |
| Acne conglobata | 9.1 (1) |
| Sinus pilonidal | 27.3 (3) |
| Hyperandrogenism, Polycistic ovary syndrome/hyperandrogenism (mean FG = 15) | 45.5 (5), 18.2 (2) |
| Dyslipidemia | 27.3 (3) |
| Arterial hypertension | 18.2 (2) |
| Diabetes mellitus type 2 | 9.1 (1) |
| Psoriasis | 9.1 (1) |
| Hurley stage, % (n) | |
| I | 9.1 (1) |
| II | 82 (9) |
| III | 9.1 (1) |
| ISH4, % (n) | |
| Mild | 9.1 (1) |
| Moderate | 54.5 (6) |
| Severe | 36.4 (4) |
| DLQI, % (n) | |
| Small effect (2–5) | 18.2 (2) |
| Moderate effect (6–10) | 45.5 (5) |
| Very large effect (11–20) | 36.4 (4) |
| HS locations, % (n) | |
| 1 (isolated intermammary) | 18.2 (2) |
| 2 | 9.1 (1) |
| 3 | 27.3 (3) |
| 4 | 9.1 (1) |
| 6 | 27.3 (3) |
| 9 | 9.1 (1) |
| HS locations (other than intermammary), % (n) | |
| Axilla | 63.6 (7) |
| Groin | 63.6 (7) |
| Inframammary | 54.5 (6) |
| Breast | 18.2 (2) |
| Vulva | 18.2 (2) |
| Thighs | 18.2 (2) |
| Nape | 18.2 (2) |
| Retroauricular | 9.1 (1) |
| Pubis | 9.1 (1) |
| Buttocks | 9.1 (1) |
| Abdomen | 9.1 (1) |
| Upper trunk | 9.1 (1) |
| Past or current treatments | |
| Medical, % (n) | |
| Topical antibiotics | 81.8 (9) |
| Intralesional corticosteroids | 27.3 (3) |
| Tetracyclines | 63.6 (7) |
| Clyndamicn + rifampicin | 54.5 (6) |
| Retinoids | 18.2 (2) |
| Adalimumab | 18.2 (2) |
| Surgical (excision), % (n) | 18.2 (2) (and 3 more awaiting surgery) |
HS, hidradenitis suppurativa.
Discussion/Conclusion
Although HS predilection for intertriginous skin is well-established [2], intermammary location is very rarely mentioned in medical literature. However, this particular location may conceal anatomic specificities, which favor a rapidly progressing disease and mutilating scarring.
Young, overweight women (and with large breast volume) with sinus tracts and a rapidly progressing fistulizing and scarring phenotype characterize our sample. In addition, in hirsute women, terminalization of the hair in the intermammary area may further predispose to follicular occlusion. These singularities lead us to postulate that intermammary HS could be akin to pilonidal sinus. Both are complex and multifactorial disorders which pathophysiology remains to be fully elucidated, yet sharing the initiation phenomena of follicular occlusion in a pilosebaceous-apocrine unit of a genetically prone individual, being part of the so-called follicular occlusion tetrad [3, 4]. Moreover, SP has also been considered as a localized variant of HS [5]. Young overweight adults are the most affected population [2, 4, 6]. As in HS, in chronic PS disease (PSD) infected abscesses as well as draining fistulae are found, with intermittent drainage and pain. Shrinkage and skin scarring may also occur secondarily [6]. Furthermore, the phylogenetic homology between the upper and lower limbs, and in particular chest wall and pelvis, has long been contemplated in anatomical bibliography [7, 8].
We further emphasize and propose an anatomo-functional parallel in which intermammary HS could mirror PSD outside the sacrococcygeal region. Namely, both regions are areas of insertion of muscle bundles (pectoralis and glutes), being subjected to great tension and traction forces. Covering muscular plane, there is adipose tissue with a median groove in both locations (intermammary and intergluteal clefts), where these mechanical stretching factors are supposedly intensified. Like PSD [6], and according to our series, intermammary HS also seems to be mostly seen in young reproductive populations, in which overweight is almost always present. Besides, big breasts aggravate the occlusion and friction forces already promoted in this area, thus favoring progression to fistulae and explaining the predominant intermammary disease in women.
HS in this cosmetically sensitive location is particularly bothersome, causing important physical and psychological distress. In addition to the morbidity of recurrent drainage, it is also acknowledged that the presternal region has an increased incidence of scar hypertrophy, namely in women (including fair-skinned). This can be partially due to different stressing forces caused not only by body movements but also by female breasts [9, 10]. Moreover, the frequent development of extensive cutaneous sinuses in these area favors a thoughtful surgical approach [11] alongside medical management, thus confronting these young patients with a permanent scar. Hence, early diagnosis, prompt medical treatment, and lifestyle corrective measures should not be overlooked in order to prevent such scenario whenever possible.
Paucity of data concerning intermammary HS is conspicuous and this paper underlines features of this specific location. We highlight the need for further studies on this subtype, underlining the demand for a suitable and timely intervention with multidisciplinary endeavor, including surgical evaluation.
Statement of Ethics
Written informed consent was obtained from the patient and/or legal guardian for this case report and any accompanying images. This study complied with the Declaration of Helsinki.
Conflict of Interest Statement
The authors have no conflicts of interest to declare.
Funding Sources
None of the authors received any financial support for the present study.
Author Contributions
Dr. Ana Luísa João: contributed to data collection and analysis and interpretation, and drafting of the manuscript. Dr. Nélia Cunha: contributed to data collection and revision of the article. Dr. Joana Cabete: contributed to data collection, data analysis and interpretation, and revision of the article.
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