Sunday, June 23, 2019

No Worst, There Is None


The patient is a 26 yo man with an eight-year history of Hidradenitis supprativa (HS).  This began in his axillae but has progressed to groin, perineum and scrotum.  He has been treated doxycycline, minocycline, resection for groin and buttocks sinus tracts three years ago, and lastly Humira for over the past year, utilizing the standard doses recommended in the literature.He has constant pain for which he takes oxycodone 20 mg six times a day.  He was seen this week because of continuing pain, drainage and low grade fever (38 C).   He is essentially home bound and is cared for by a devoted mother.

The most affected area at present is his genitalia.   He has massive involvement of his scrotum with inflammatory draining lesions. 

Clinical Image:

Impression:  Ongoing hidradenitis stage III mostly affecting the scrotum at this point.  The picture is similar to what some label as “Scrotal Elephantiasis.”

Discussion: I do not think the Humira is impacting on the local disease at this time and he may need a surgical approach.  This could be ongoing inflammatory disease; but may eventuate into chronic lymphedema.  Another possibility is that the resection of the groin lesions may have caused impaired drainage.  Against this is the lack of leg edema.

We are looking for therapeutic suggestions and whether any of our members have successfully treated similar patients.  There are a few articles on surgical approaches in the urology literature.

References:
1.  Scholl L1, et. al. [Surgical treatment options for hidradenitis suppurativa/acne inversa].
[Article in German] Hautarzt. 2018 Feb;69(2):149-161.
Abstract:  Hidradenitis suppurativa/acne inversa (HS/AI) is a chronic inflammatory skin disease. Therapy consists of conservative and surgical treatment options. In Hurley stages II and III, surgical intervention is regarded as the method of choice for areas with irreversible tissue destruction. Resection techniques with different grades of invasiveness are described in the literature. Nevertheless, there is no generally accepted concept regarding resection and reconstruction techniques or specific postoperative care. Due to lack of definitions of recurrence after surgery and poor study quality, recurrence rates are difficult to determine.

2. Kimball AB, et. al. Two Phase 3 Trials of Adalimumab for Hidradenitis Suppurativa.
N Engl J Med. 2016 Aug 4;375(5):422-34. Full Free Text.

3. Hormonal therapies for hidradenitis suppurativa: Review.
Clark AK, Quinonez RL, Saric S, Sivamani RK. Dermatol Online J. 2017 Nov 12;23(10)..
Abstract: Hidradenitis suppurativa is a recurrent inflammatory skin condition characterized by abscesses and boils, predominantly in the groin, armpit, and buttocks areas. HS is not a life-threatening condition, but severely impairs quality of life in those affected. Finding a successful treatment approach for HS has been challenging, in part because of the lack of a gold-standard treatment method, limited research-based information, and the nature of clinical variation in the disease. Treatment commonly consists of antibiotics, anti-inflammatory therapy, hormonal therapy, and more invasive clinical procedures. Treatment is chosen by the degree of severity by which the condition presents and is modified accordingly. This review describes the roles of hormones in the pathogenesis of hidradenitis suppurativa and describes the use of hormonal therapy such as, finasteride, dutasteride, spironolactone, and oral contraceptives. The outcomes of the use of these modalities in various clinical studies are summarized.


*  The discounted retail cost of Humira for HS (40 mg weekly) is $10,000 USD a month or $120,000 year year.
 

6 comments:

  1. Having also seen this patient, I am very interested in knowing others thoughts. My impression with the Humira is that once you've formed these sinus tracts, it does virtually n othing for them but may help prevent formation? Or at least that's the hope. Would love to see others input...

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  2. Richard Sontheimer wrote: There are recent articles in the surgical literature (one is attached) suggesting that radical excision with closure by scrotal flaps can both physically and psychologically reduce the burden of this challenging hidradenitis complication.

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  3. Robert Shapiro wrote: I had a patient in the past with the same clinical appearance. Massive isolated idiopathic scrotal edema. He didn't have pain or fever. Humira wasn't available at the time, but I seriously doubt it would have been helpful as well.

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  4. Cheng Leng Ong wrote: This is the worst case scenario of this destructive disease. The only way out is to get an experienced general surgeon, who knows the anatomy and pathology and typology around the public and scrotal regions to dissect and free the penis.

    If the hidradenitis suppurativa is still active, we have to resort to the most effective biologics available after screening for contraindications. More than twenty years ago, I was treating a secondary school boy with problematic hidradenitis suppurativa of his axillae. It improved slightly before I sent him to a local general surgeon. He is doing well since then!

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  5. Addendum from Richard Sontheimer:

    We have aggressive academic plastic and urologic surgeons here at the University of Utah. I've had one similar young adult Indian male hidradenitis patient here undergo such surgery with a good outcome.

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  6. Prof. Bhushan KumarJune 26, 2019

    Present condition relates more to obesity and aftereffects of surgery leading on to lymph stasis resulting in Elephantiasis.
    More surgery will lead to more complications. It is not exactly due to failure of Humira. The disease does burn itself out in many cases. Any further and naturally more heroic surgery will lead to further worsening. Weight reduction is the best option.

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