Showing posts with label hidradenitis supprativa. Show all posts
Showing posts with label hidradenitis supprativa. Show all posts

Monday, November 16, 2020

The Buried Penis

November 2020

In June of 2019 we presented a 26 yo man whose main problem was scrotal lymphedema related to hidradenitis supprativa (HS). In 2018, he had surgery at a major academic center to excise active HS lesions in both crural folds.  The operative report states that 20x8 cm were excised from the right groin and 20x6 cm from the left groin. This seems to have been antecedent to the development of scrotal lymphedema. His HS been recently managed with adalimumab, 40 mg per week. Gradually, the active inflammatory lesions have improved, although he still has some fistulous drainage. 

His main problem now is a buried penis. It’s interesting how he has adapted to this. The patient is reclusive. He lives with his parents and spends his days playing video games. When discussing these he displays a vast, enthusiastic knowledge. 

He is very anxious about spending time in hospital and prefers to stay home where his attentive parents care for him. 

We are presenting him to discuss "the buried penis," an unusual disorder, and to see if anyone has helpful suggestions. There were some recent in-depth reviews of “the buried penis” and we will see if the authors are interested in commenting.

Note:  It appears that there are surgical approaches that can help patients like this, but only a very small number of urologic or plastic surgeons have the interest and expertise in this topic.  Dr. Joel Gelman (see articles referenced below) kindly discussed the management of massive scrotal lymphedema  with me   He related the case of Wesley Warren whose 132 pound scrotal lymphedema he removed in 2013.   
 
Reference: 
1. Evaluation and management of adult acquired buried penis. Ho TS, Gelman J.
Transl Androl Urol. 2018 Aug;7(4):618-627. Free Full Text
 
2. Reconstruction of Massive Localized Lymphedema of the Scrotum: Results, Complications, and Quality of Life Improvements. Wisenbaugh E, Moskowitz D, Gelman J. Urology. 2018 Feb;112:176-180. Conclusions: Surgical treatment of MLL of the scrotum can be performed successfully for masses even up to 61 kg (134 lbs). Short-term wound complications are common, but subjective QOL scores improve dramatically. Despite expectations, most patients gained weight after mass removal, which indicates that they would benefit from a comprehensive weight loss plan that includes, but is not limited to, scrotal surgery. Free Full Text.
3. Various YouTubes of Wesley Warren's surgery can be found online.

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
 

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.
 

Friday, January 08, 2016

Atypical Granular Cell Tumor

This 55 yo woman was seen for three painful lesions (left abdomen of two months duration and right axilla of one week duration).  She is in good general health and takes no medications by mouth.



Pathology of lesion abdomen:

Granular cell tumor with atypical features consistent with at least "atypical granular cell tumor."
NOTE: The specimen exhibits a diffuse dermal interstitial proliferation of S100-protein positive and CD68 positive epithelioid cells with abundant finely granular, eosinophilic cytoplasm that is negative for high and low molecular weight keratins and Mart-1/Melan-A. The granular cells show nuclei that are variably pleomorphic as well as that are intermittently vesicular with large nucleoli. These changes are diffuse throughout the tumor and are consistent with the atypical variant of granular cell tumor.  The photomicrographs are courtesy of Dr. Hyejin Leah Chung , a dermatopathology fellow at Boston University School of Medicine.  (They are 10x, 20x, 40x and 40x)





Axillary Lesion: Inflammatory nodule consisten with hidradenitis.

Diagnosis: Atypical granular cell tumor is a problematic diagnosis.  A small percentage of these may be malignant granular cell tumors and they have a worrisome behavior.  The fact that the axillary lesions are presumably not related is curious.  This patient needs a full work-up and follow-up.


Referencea:
1. Case for diagnosis.
Leyva AM et.al.  An Bras Dermatol. 2014 May-Jun;89(3):523-4.
Abstract: Granular cell tumour is a rare tumour of neural origin usually located on the face and the neck. The biological behaviour is usually benign. However, certain clinical and histopathological features should alert physicians to a malignant behaviour. This case report describes the occurrence of a granular cell tumour in the inguinal area that resembled a malignant tumour. The histopathological study revealed typical features of granular cell tumour and an extension study confirmed the absence of metastasis. This case highlights the importance of considering this disorder in the differential diagnosis of ulcerated nodules and of managing atypical granular cell tumor appropriately.  Free Full Text.