Case Number : Case 1480-25 February Posted By: Guest

Multiple verrucous desmoplastic trichilemmoma ( Cowden syndrome associated? ).

I think this is a difficult case to interpretate on these images. I see massive hyperkeratosis and acanthosis with focal ulceration and areas of pseudoepitheliomatous hyperplasia. In the dermis there is a lot of stromal mucin, a lymphocytic infiltrate and isolated eosinophils. I would initially perform special stains to discard chronic infections (mycoses particularly) if those are negative I was considering hypertrophic lupus

The history is the same as yesterday's case. Presuming there is a differential clinical with this histology!

I'm really struggling with this one.  Quite like the suggestion of desmoplastic trichilemmoma but I was perhaps more in favour of a BCC. Young age and striking background compact hyperkeratosis does need explaining.  Some of the mucin appears intra-epithelial - can be seen in DTL and of course follicular SCC. IHC will help clarify hopefully and perhaps something we are missing in the history. IHC: CD34, CK20, BerEP4, EMA for starters and perhaps AB/DPAS.

Ah ok they are not scaly patches.... something was not fitting it could be a trichilemmoma as Vincenzo suggested maybe developing in a verruca like we talk some time ago in a case of Dr. Sundram and as Ackerman suggested

Sorry!  The history is incorrect.  The correct history is: 40 year-old female with right hand lesion.  Her mother had a similar lesion on her hand. 

Yes probably what we see is part of an acral verruciform keratosis and a trichilemmoma both part of cowdens syndrome. Dr. Diwan the patient only had acral lesions? or it was also associated with lesions on the face?.

Agree.

I don't know if you will agree with me, but here is what I thought:  This patient had another biopsy from another part of her hand, which showed papillomatosis and acanthosis.  The clinician thought her lesions were clinically epidermal nevi, and her mother had similar lesions as well.  This lesion was crusted, and you can see the crust in some of the upper images.  So I thought there was an epidermal nevus in the background.  I did consider desmoplastic trichilemmoma initially because of the look of it, but I saw ducts, plentifully, which I tried to photograph, and which I think you can see in the bottom six images to varying degrees.  Given the fibrotic milieu, I ended up suggesting that this was a syringofibroadenoma in association with an epidermal nevus.