Literature Review of Hidradenitis Suppurativa

Introduction

Hidradenitis Suppurativa, also known as pyoderma fistula significa, smoker’s boils or Acne inversa, is a chronic inflammatory condition with a major detrimental impact on individuals’ quality of life and has substantial co-morbidities. This debilitating disease usually affects the perineum, axillae, and the inframammary regions (Danby & Margesson, 2010). The causes of this illness have been attributed to apocrine canal constriction that occurs due to keratin plugs, encompassing some trigger aspects, including sudoresis, tight clothes, heat and stress, to name a few, as well as some facilitating factors such as hormonal and genetic factors. The understanding of this skin condition has changed over time. Currently, it is considered as a condition of follicle occlusion as opposed to the earlier inflammatory course of the apocrine glands.

Clinically, the skin condition often occurs with a tender hypodermic nodule, which does not show until one has reached puberty phase. These nodules spontaneously coalesce and rupture to form deep dermal painful sores. The area that the lesion occurs may cause social embarrassment, which is a factor that would make the patient fail to seek medical intervention. In the past, remedies like surgery, long-term antibiotics and anti-androgens have been considered. However, in the recent past, remedies like tumor necrosis factor-alpha inhibitors have presented medical practitioners with more alternatives in treating this incapacitating disease.

Demographics

Hidradenitis Suppurativa usually occurs after adolescence, but has an average onset age during the second and third decades of an individual’s life. However, some cases where the skin condition presents itself before puberty have been reported. Important to understand is that the inception of this illness after menopause is a rare thing and its occurrence may reduce with time. A study conducted by Revus et al, (2008) indicated that the prevalence in individuals above 55 years of age was significantly lower than that of younger age factions. In another study, Hidradenitis Suppurativa was estimated to be at about 1%. Females in their productive age were more likely to develop this skin condition than their male counterparts (Wolkenstein et al, 2007). Despite the fact that most researchers have generally accepted the idea that women are the most at risk population as far as this condition is concerned, many authors have contested this assertion. They have indicated that in some locations, the condition appears to have sexual partiality; for instance, where perianal acne inversa affects more men than women (Mysore, 2012; Wolkenstein et al, 2007).

Clinical Presentation

The occurrence of some tender hypodermic nodules characterizes the initial stages of this skin condition. As time passes, the nodules rupture, resulting in deep, painful dermal sores. Although the lesion may be rounded in shape, it does not typically show pointing as in the case of furuncles (Kurzen et al., 2008). With time, the ruptured area produces a foul-scenting discharge. As the illness continues to move to the next stage, fibrosis and skin contractures, and an induration of the derma occur. The occurrence of double comedones is a characteristic of the skin condition. It gradually develops in post puberty in individual of all genders who were initially healthy. In fact, the acne inversa lesions occur mainly in the intertriginous apocrine gland in the areas of perianal, axillary, buttocks, pubic, and chest as well as the eyelid areas and retro-auricular areas, among other areas. The areas that are infected with this skin condition’s disease causing organisms correspond with both the apocrine glands and the terminal hair follicles, which depend on the concentration of androgen. Perianal Hidradenitis Suppurativa has been linked with much higher rates of recurrence than that affecting other areas of the body. People suffering from perianal Hidradenitis Suppurativa are usually prone to other diseases that affect the same region, such as the anal canal illness, with a rating of 30 out of every 132 patients as indicated in one study (Alikhan et al., 2009). In fact, perianal Hidradenitis Suppurativa has been connected to a wide range of more debilitating outcomes, including a fistulous boil and Crohn disease.

Clinical Staging

A researcher named Hurley suggested one of the initial stages of this condition and categorized patients into three groups. This classification was largely based on the occurrence and magnitude of sinuses and cicatrization. The fact that this classification is simple and relies on the subjective magnitude of the diseased tissue, the method has been used widely as a foundation for clinical trials. It has become an important basis of approaching therapy for people suffering from this condition. Despite its simplicity, various researchers have argued that the classification does not offer a wide range of options and hence is not adequately sophisticated to evaluate the treatment impacts in medical researches. They have proposed a system that integrates the complicated anatomic regions, the number of lesions, and the specific types (Alikhan et al., 2009). The system also integrates the distance between the lesions as well as the presence of normal uninfected skin between the lacerations. Therefore, the points are amassed in each of the categories and summed up to give not only a regional score, but also a total score.

Diagnosis

The diagnosis of this skin condition is eminently clinical. It is based on the signs and symptoms described by the patient as well as the signs that medical practitioners observe. The first sign and symptom include erythema, hyperhidrosis, pruritus, and discomfort in the area affected by the condition. As the disease advances, the symptoms become evidently manifested. Physical tests identify lesions in the form of compound dispersed abscesses, with protracted drainage in the form of compound fistulas, through the openings of varied features (Katz & Goldberg, 2009). The clinical condition of this disease is characterized by chronic signs and symptoms that may take up to 30 years of development with recurrent sores and fistulas, which after rupturing, leave sequelae  like areas of fistulous orifices, fibrosis, as well as scarce pus-filled secretions (Katz & Goldberg, 2009; Hsiao et al, 2010). Should the illness affect the perianal region, it seldom encompasses the anal sphincter.

The analysis and diagnosis of this skin condition when in the chronic form are easily performed clinically, depending on the coloproctologistic experiences of the clinician. In some cases, the application of biopsy is needed to confirm the diagnosis; for instance, in the case of tuberculosis ulcerations, perianal Crohn’s disease or even in carcinoma cases (Kurokawa et al, 2007). The acute Hidradenitis Suppurativa is characterized by inflammation that later progresses to an infectious process affecting the superficial and deep tissue with sores, cellulitis and suppuration. However, the chronic conditions are because of fistulas and the association of various important structures, including the coccyx and sacrum, the great caliber vessels and urethra, and the anal sphincter.

Differential Diagnosis

Various illnesses should be considered and ruled out as a differential diagnosis. These include anorectal fistulas, Crohn’s disease, and cutaneous tuberculosis, to name a few (Canoui-Poitrine et al, 2009). In Hidradenitis Suppurativa, the differential diagnosis is extensive due to the wide range of diseases with similar symptoms. However, it is usually simple and easy to differentiate the disease from the others. One way of differentiating this skin condition is by observing the appearance of the lesion. Other ways include characteristic locations, recovery of compound species of bacteria on performing culture and sensitivity test as opposed to one, lack of laboratory results, resistance to antibiotics, and a post-pubertal age of onset.

Treatment

There are wide ranges of available treatments for Hidradenitis Suppurativa. These include hormonal therapies, surgical treatment, radiotherapy, immunosuppresses, cryotherapy, systemic retinoids, intralesional corticosteroids, antibioticotherapy, and the local care of the infected area or the lesion. However, not all these treatment options isolated have shown to be effective for patients suffering from this skin condition (Yazdanyar & Jemec, 2011). Therefore, surgical treatments seem to be the ideal approach to treating Hidradenitis Suppurativa. In essence, the treatment varies from a simple incision and an acute sore drainage to a radical excision of the entire tissue with the apocrine glands.

Conclusion

Despite the fact that the prevalence of this debilitating skin condition is high, it receives little attention and scholarly influences. Overwhelming treatment options have been described, and novel remedies are still evolving. Conservative curative measures play a significant role in treating this condition, but surgical procedure is the only treatment option for a cure, especially if the condition is in advanced stages. As the understanding of the Hidradenitis Suppurativa condition continues to grow, so will be the therapeutic options.

 

References

Alikhan, A., Lynch, P. J., & Eisen, D. B. (2009). Hidradenitis suppurativa: A comprehensive review. Journal of the American Academy of Dermatology.  

Canoui-Poitrine F, Revuz JE, Wolkenstein P, Viallette C, Gabison G, Pouget F, et al. 2009. Clinical characteristics of a series of 302 French patients with hidradenitis suppurativa, with an analysis of factors associated with disease severity. J Am Acad Dermatol; 61:51–7.

Danby FW, Margesson LJ. 2010. Hidradenitis suppurativa. Dermatol Clin. ; 28:779–93. 

Hsiao, J. L., Antaya, R. J., Berger, T., Maurer, T., Shinkai, K., & Leslie, K. S. (2010). Hidradenitis suppurativa and concomitant pyoderma gangrenosum: a case series and literature review. Archives of dermatology146(11), 1265-1270.

Katz, R. D., & Goldberg, N. H. (2009). Marjolin ulcer arising within hidradenitis: a case report and literature review. Annals of plastic surgery62(2), 173-4. 

Kurokawa I, Nishimura K, Yamanaka K, Mizutani H, Tsubura A, Revuz J. 2007. Cytokeratin expression in squamous cell carcinoma arising from hidradenitis suppurativa (acne inversa). J Cutan Pathol; 34:675-8. 

Kurzen H, Kurokawa I, Jemec GB, Emtestam L, Sellheyer K, Giamarellos-Bourboulis EJ, et al. 2008. What causes hidradenitis suppurativa?. Exp Dermatol; 17:455-6.

Mysore, V. (2012). ACS(I) Textbook on Cutaneous and Aesthetic Surgery. New Delhi: Jaypee Brothers Pvt. Ltd.

Revuz JE, Canoui-Poitrine F, Wolkenstein P, Viallette C, Gabison G, Pouget F, et al. 2008. Prevalence and factors associated with hidradenitis suppurativa: results from two case-control studies. J Am Acad Dermatol; 59:596-601.

Wolkenstein P, Loundou A, Barrau K, Auquier P, Revuz J. 2007. Quality of life impairment in hidradenitis suppurativa: a study of 61 cases. J Am Acad Dermatol; 56:621-3.

Yazdanyar, S., & Jemec, G. B. E. (2011). Hidradenitis suppurativa: a review of cause and treatment. Current opinion in infectious diseases24(2), 118-23. 

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