Case Number : Case 1486- 04 March Posted By: Guest

If you attended the ISDP session in New Dehli, please try to withhold the temptation to give the answer!

1. ???Herpetic changes...?necrosis caused by vascular trombosis

I will give it a go. It is clearly an acute ulcer, rich in neutrophils and lymphocytes

(mainly in the periphery). The dermal infiltrate seems to surpass the edges of the ulcer and looks quite Deep. There is also this particular infiltrate that to my eyes adopts the shape of follicular units with some foamy histiocytes present (necrotizing folliculitis with abscess formation?). I think we should first rule out an infectious cause but this morphology and the clinical presentation makes me think of pyoderma gangrenosum.

Also was thinking of pyoderma gangrenosum. but there are particular nuclear and cellular changes in sebocytes...Very difficult!

I think that CPC is extremely important in this case. Infectious causes of ulcers due to Herpes family of virus (including zoster) have specific clinical settings that help differentiate from pyoderma.

Agree!

Agree that the infiltrate does appear to be centered on folliculosebaceous units (although quite extensive elsewhere too)....like the suggestion of herpes folliculitis

Very difficult case. I also though about herpes infection because of the sebaceous inflammation. There´s a disease called neutrophilic sebaceous adenitis, but the histology is different as is the clinical description.

I am not able to see typical nuclear alterations suggestive of herpetic infections. I liked Raul's suggestion of pyoderma gangrenosum.

The eosinophilic cytoplasm degeneration is reminiscent of some viral change, herpes or pox ( farmyard pox- like ), but also am not able to see typical nuclear changes... 

Largely ulcerated surface, pan-dermal mixed, predominantly acute inflammation with a secondary vasculitis. These are likely to be necrotic sebaceous glands, along with ballooning and acantholysis. The last image shows occasional nuclei with changes suspicious of Herpes. I would certainly do a HSV IHC. Infection high on the list!

I cannot see cytopathic changes.  I think this is pyoderma gangrenosum.

This is a very rare case of Cowpox. Histological features in this case are typical and include intense, pan-dermal, folliculocentric (a clue), inflammatory process with extensive epidermal necrosis of the whole folliculo-sebaceous unit accompanied by lymphocytes, CD30+ enlarged cells, eosinophils and plasma cells. Striking, large, eosinophilic,  intra-cytoplastmic inclusion (Guarnieri) bodies. Sparse eosinophilic homogenous intra-nuclear inclusions and sparse multi-nucleated forms were also noted but lacked the typical slate grey nuclei seen in herpes.  Before the penny dropped (after I reviewed a lecture given to me by Dr Heinz Kutzner that included an identical case) I also considered herpes. Coxpox is an orthopox virus (same group as Smallpox and Vaccinia - the subsequent vaccine developed for Smallpox). Edward Jenner, in 1796, using fluid obtained from scratches on the hand of the dairymaid Sarah Nelmes injected a boy, James Phipps to protect him from smallpox having made the observation that Dairy maids seemed to be protected from the dreaded disease. See Spot Diagnosis 1196 for a reaction post vaccination posted by Dr Hafeez Diwan.

In the current case the typical histological features and PCR for orthopox virus was kindly confirmed by Dr Heinz Kutzner. The disease is usually passed to humans who have been scratched or bitten from Cats or rats (sometimes bought as domestic pets) that are carrying the disease.  The time-line is relatively characteristic as follows: Day 1-6: inflamed macule; Day 7-12 popular and blister; Day 13-20 blood filled blister, pus, ulceration (+/- secondary infection); Weeks 3-6 deep-seated, hard, crusted sore with surrounding erythema and swelling; Weeks 6-12 eschar sloughs leaving a scar.  There may be accompanying systemic symptoms: malaise, fever, vomiting, sore throat, conjunctivitis, lymphadenopathy. The disease may disseminate and be life threatening in the immunocompromised or in patients with other skin conditions. Treatment is symptomatic with antibiotics for secondary infection. The differential diagnosis includes Orf and milker's nodules (pseudocowpox) which is a parapox virus and histologically similar to Orf being characterised by less conspicuous eosinophilic inclusions in epidermal keratinocytes.  I am pleased to report in the current case the lesion healed spontaneously and the pregnancy progressed without incident. There have been rare outbreaks of this disease from time to time in recent decades in Europe usually linked to the sale and importation of infected rats as domestic pets.

Great case. Dr was there any other clinical information when you received the case, like clinical suspicion of infectious entity? did the patient have mentioned being bitten in the region by an animal or was in contact with cats, rats, etc?

No. The biopsy was done by a breast surgeon - hence the very generous biopsy - and the histological diagnosis was completely unexpected. Patient had several pets and visited other homes in their job but there was no clear history we could ascertain (after making the diagnosis) although often lesions are on exposed areas like the arms, face or neck area and this may be a clue too. Hopefully this is a case of once seen never forgotten!

FANTASTIC :-))