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.

Follow-up June 16, 2020:  Since we last reported on this patient he has had scrotal surgery and has been back on Humira for three months at 40 mg a week.  He cannot urinate easily and is home bound.  He feels his scrotum is getting worse, but comparison with the photo of June 2019, today's photo is less impressive.  We'll see if anyone has helpful suggestions:
June 16, 2020

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.

Wednesday, June 19, 2019

Congenital Nail Dystrophy

The patient is a nine month old fraternal twin with mild developmental delays. No other pertinent history

She has opaque toenails that grow at an upward angle from the nail bed since birth. The great toenails continue to have an increasingly severe upward slant so that they are almost at a 90 degree angle to the nail bed. The nail plates show no thickening. Her fingernails are all normal. The skin of the feet and the rest of the body appear normal. No peeling or scale. No rash. The foot anatomy is grossly otherwise normal. The twin does not have the same condition. The parents are unable to put shoes on her because she seems to be in pain from her toenails. She does not seem bothered by socks or soft slippers. She is not walking yet, but should be within 2-3 months. they cut the nails short, but it doesn't offer enough relief to use shoes.  

Clinical Image:

Has anyone seen a similar patient?  What are your thoughts?

Note:  The infant will be seen by a dermatologist in a few days and a KOH prep will hopefully be done.