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Table of Contents
CASE REPORT
Year : 2018  |  Volume : 22  |  Issue : 2  |  Page : 79-81

Hidradenitis suppurativa: Presenting at atypical sites


1 Department of Dermatology, Venereology and Leprosy, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
2 Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

Date of Web Publication21-Sep-2018

Correspondence Address:
Dr. Mudita Gupta
Department of Dermatology, Venereology and Leprosy, Indira Gandhi Medical College, Shimla, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jdds.jdds_17_18

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  Abstract 


Hidradenitis suppurativa (HS) is a chronic inflammatory and debilitating disease of apocrine gland-rich areas. Characteristic bilateral involvement of the axilla, perineum, or inguinal folds is a hallmark feature of HS. Certain uncommon sites such as face, neck, jaw region, and legs, which are devoid of apocrine glands, are also involved in some cases. Here, we describe a case of HS involving uncommonly reported sites such as wrist, mid-back, and thigh at initial presentation which were misdiagnosed as scrofuloderma. She later had lesions on less common areas, such as buttocks, and the classical sites, such as groin and axilla, whence a final diagnosis of HS was made. The peculiarity of our case is the onset of disease from atypical sites which are devoid of apocrine glands and terminal hair follicles.

Keywords: Apocrine glands, atypical sites, hidradenitis suppurativa


How to cite this article:
Kumari S, Gupta M, Sharma RK, Negi L. Hidradenitis suppurativa: Presenting at atypical sites. J Dermatol Dermatol Surg 2018;22:79-81

How to cite this URL:
Kumari S, Gupta M, Sharma RK, Negi L. Hidradenitis suppurativa: Presenting at atypical sites. J Dermatol Dermatol Surg [serial online] 2018 [cited 2018 Dec 16];22:79-81. Available from: http://www.jddsjournal.org/text.asp?2018/22/2/79/241907




  Introduction Top


Hidradenitis suppurativa (HS) was first described as “Velpeau's disease” by Velpeau in 1839 with abscesses in the axillary, mammary, and perianal region.[1] As the name hidradenitis suggests, it was thought to be a chronic suppurative, cicatricial, and recurrent disease of apocrine glands. This concept was questioned because other areas such as face, neck, retroauricular area, and legs were also involved in some cases.[2],[3] HS, being a disease of variable presentation and multifactorial origin, is frequently misdiagnosed.[4] Classic lesions are nodules, abscess, chronic discharging sinuses with polyporous comedones, and bridged scars.[1]


  Case Report Top


A 30-year-old female presented with discrete multiple pustular lesions which appeared initially over the thigh, volar aspect of the wrist, and mid-back 15 years back. These lesions progressed in size and number, then coalesced, ruptured, and healed with disfiguring scars. There were waxing and waning of these lesions for the past 10 years. She developed similar lesions over the groin, axilla, and perineum with the same progression for the past 5 years. There was no history of smoking, altered bowel habits, weight loss, joint pain, any other cutaneous lesion, or systemic problem. There was no history of similar complaints in the family. On examination, the patient was thinly built with body mass index of <22. On general physical examination, nothing abnormal was detected. Cutaneous examination showed well-defined atrophic plaques with bridged scarring over the volar aspect of the right wrist, mid-back, and anterior aspect of the left thigh [Figure 1], [Figure 2], [Figure 3]. Both groins were disfigured with scarring, abscesses, and chronically discharging sinuses [Figure 3]. Ziehl–Neelsen staining and Gram staining showed no bacilli. Routine hematological and biochemical investigations were noncontributory. Radiological examination of the chest, pelvis, and limb bones and ultrasonography were normal. Mantoux test was also normal. Lymphogranuloma venereum serology was not contributory. Polymerase chain reaction studies for tuberculosis were also negative. Histopathology shows abundant mixed cell infiltrate in the upper dermis without visualization of appendages [Figure 4]. Hence, the final diagnosis of HS was made.
Figure 1: Deep-seated atrophic scar over the upper mid-back

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Figure 2: Deep-seated atrophic scar over the left thigh

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Figure 3: Multiple atrophic and bridged scars over the groin and right wrist (volar aspect)

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Figure 4: Histopathology revealing mixed acute and chronic inflammatory cell infiltrate around the adnexal structures in the dermis

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  Discussion Top


HS is a devastating disease of apocrine gland-rich areas characterized by classic morphology, anatomic location, and recurrent, chronic clinical course.[2] Subcutaneous abscesses, sinus tracts, and scarring in apocrine gland-bearing skin are hallmark features of HS. Symmetrical involvement of the axilla, groins, perianal and perineal region, mammary and submammary skin, buttocks, and pubic region is seen in decreasing order of frequency.[5]

Exact pathogenesis of HS is unknown and it is thought to be multifactorial in origin [Table 1]. Conventionally, inflammation and occlusion of apocrine ducts were thought be a prime mechanism in pathogenesis. Follicular unit occlusion due to distortion or hyperkeratinization is now thought to be a major contributory factor to the development of HS. Chronic friction from adipose tissue in obese, sweat, heat, stress, and tight clothing further increases the risk of occlusion. Increased end-organ sensitivity to androgens leads to hyperkeratinization of pilosebaceous unit.[6] Association of disease in families suggests a genetic predisposition, but classical site involvement is more commonly seen in such cases. Altered immune responses and subclinical inflammation to colonized bacteria are thought to contribute to the development of HS. It is the diffuse inflammation and secondary infection which destroys apocrine and sebaceous glands.[5],[7] Smoking promotes follicular plugging, impairs host innate immunity, activates Toll-like receptors hence creating a pro-inflammatory milieu, and aggravates HS morbidity. Inflammatory bowel disease, spondyloarthropathies, and pyoderma gangrenosum are seen in association with HS.
Table 1: Pathogenetic factors for hidradenitis suppurativa

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Our case was nonsmoker, nonobese, no family history, or any other triggering factor. HS of atypical sites, such as scalp, eyelids, retroauricular and preauricular skin, chest, thighs, abdomen, posterior neck, and legs, have been described in literature.[1],[3],[8] Most of these sites involved were either occlusive, androgenic sensitive or having terminal hair. Some of these cases were associated with triggering factors such as obesity and smoking. There are few case reports only describing HS at nonocclusive, nonandrogenic vellus hair sites with no triggering factor.

Naasan and Affleck divided HS into three groups depending on site involved, triggering factor, and prognosis into “axillary–mammary” (previously “typical” HS), “follicular,” and “gluteal.”[5] Follicular form involves ear, chest, neck, and leg. Follicular HS has higher disease severity, earlier onset, and longer duration and is more common in men, especially in smokers and obese people.

Our case was a patient of HS with not only atypical presentation at atypical sites but also an atypical follicular variant. The disease being multifactorial in origin any lesion; any lesion with chronic sinuses and bridged scarring with remissions and relapses should be suspected to be HS.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Patil S, Apurwa A, Nadkarni N, Agarwal S, Chaudhari P, Gautam M, et al. Hidradenitis suppurativa: Inside and out. Indian J Dermatol 2018;63:91-8.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Micheletti RG. Natural history, presentation, and diagnosis of hidradenitis suppurativa. Semin Cutan Med Surg 2014;33:S51-3.  Back to cited text no. 2
    
3.
Gharami RC, Mandal MK, Patra AC. Hidradenitis suppurativa in unusual sites. Indian J Dermatol Venereol Leprol 2003;69:7-8.  Back to cited text no. 3
  [Full text]  
4.
Alsharif AA. Recurrent acute hidradenitis suppurativa: Case report with 12 month follow up. Int J Adv Res 2016;4:1008-11.  Back to cited text no. 4
    
5.
Naasan H, Affleck A. Atypical hidradenitis suppurativa. Clin Exp Dermatol 2015;40:891-3.  Back to cited text no. 5
    
6.
Andrade MM, Constantino JR, Gomes DM, Faria FF, Oliveira RG, Ferreira RM, et al. Hidradenitis suppurativa: Literature review and case report. J Coloproctol 2012;32:196-201.  Back to cited text no. 6
    
7.
Smith MK, Nicholson CL, Parks-Miller A, Hamzavi IH. Hidradenitis suppurativa: An update on connecting the tracts. F1000Res 2017;6:1272.  Back to cited text no. 7
    
8.
Syed ZU, Hamzavi IH. Atypical hidradenitis suppurativa involving the posterior neck and occiput. Arch Dermatol 2011;147:1343-4.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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