Case Number : Case 1719 - 29 December - Dr Arti Bakshi Posted By: Guest

Dr Arti Bakshi has got a nice case with plenty of clinical history to get you heading in the right direction.

Cheers, Geoff Cross - DermpathPRO Projects

Thinking of demodicosis. Can present with rosacea like clinical picture. This might explain the conjunctivitis prior to the flare. 

I see a granulomatous dermatitis with marked edema of papillary dermis. Some areas of the epidermis also show some particular features like intracorneal neutrophils, parakeratosis and lack of granular layer. The differential is large but I thought that hystiocytoid sweet syndrome could be a possibility as it can present with conjunctivitis and lesions on the face. IHC can help

Sorry, forgot to mention that demodicosis is a good suggestion of Mona as clinically (due to topography) we are almost obliged to consider it (even without seeing the slide!)

I go with rosacea fulminans.

I don’t think demodicosis is a real disease, but rather a complication of hair follicle rupture.

Due to the history of psoriasis, maybe the conjunctivitis is a component of the triad of Reiter’s syndrome in this patient.

What about drug(?steroids)-induced rosacea like reaction?

4 hours ago, Raul Perret said:

I see a granulomatous dermatitis with marked edema of papillary dermis. Some areas of the epidermis also show some particular features like intracorneal neutrophils, parakeratosis and lack of granular layer.

very good thinking, really agree with you Raul

My first impression was histiocytoid sweet.

I know an histiocytoid variant of Sweet Syndrome, but with myeloid morphological and immunophenotypical features ( MPO+ ), as a Hafeez Diwan's case showed sometime ago, if I remember correctly. This is instead a granulomatous lesion. 

However I agree that a first impression could be SS on low magnification, especially because of the prominent papillary dermal edema. In any case, my knowledge of SS is very low...

Good to see a range of differential diagnoses as this was a difficult case! Following clinico-pathological correlation, we went for rosacea fulminans, so well done to Robledo for getting the diagnosis right! 

Demodicosis is a good thought, but the granulomas were not centered around demodex mites. The immunos did not support histicytoid Sweets and as Vincenzo rightly pointed out, the histology is more suggestive of a granulomatous process. For those unfamiliar with this entity, Rosacea Fuminans (also called pyoderma faciale)is a rare subtype of rosacea which presents acutely with painful nodules, pustules and sinuses, typically in young women. It differs from usual rosacea in its abrupt onset,  absence of a prior telangiectatic phase and absence of ocular symptoms. Acne fulminans comes in the d/d clinically, but there is no association with comedones and there are no systemic symptoms in rosacea fulminans. There is limited histological description of rosacea fulminans, but follicular based acute inflammation, granulomatous inflammation and dermal oedema are features of this entity.

We found it difficult to explain the conjunctivitis in this case, but the clinicians felt that it was probably an unrelated and separate process.

Thanks for all your comments and wish you all a very happy New year!

Thanks Arti and Happy New Year to all of you. Wish you all the best in 2017. 

Happy New Year to all of you.