Reactions physicians after studying Melda’s files

When I searched the literature I came across cases of severe eye allergy and vernal conjunctivitis associated with this condition. Both allergic and autoimmune/rheumatic conditions can be of influence. Birch sensitivity appeared in the ISAC test for allergy. Blood results for autoimmune and rheumatic tests also look normal. TOM1 gene mutation can cause immune deficiency. There is a predisposition for immunodeficiency in TOM1 gene

My suggestion is: 
- Repeat studies made - Additional immune system tests: eg looking at vaccine responses and lymphocyte subsets. 
- If she does not have frequent infections and her development is good for her age, these tests (with what we will do in addition) are sufficient.

This is an inflammatory process. There is no neoplasm. At first it looks like a Juvenile Xanthagranuloma, but has become smaller. Splenedore-Hoeppli is not a disease, but a consequence/outcome. It is clear that your daughter's immune system is disturbed. There is an underlying cause that triggers the immune system. I would advise (if this has not already been done) to investigate parasites (including in the intestines eg intestinal worm) and tuberculosis.

My colleagues and I reviewed the medical documents of Miss. Melda Yildiz.

We agree with your clinical impression; Allergic Conjunctival Granuloma although pathology report of lesion biopsy does not match completely with this clinical entity and the presence or absence of the characteristic histopathologic features of an allergic conjunctival granuloma/ Splendore-Hoeppli phenomenon such as epithelioid histiocytes, eosinophils, Giant cells, and amorphous eosinophilic material is not mentioned.

The possibility of another similar situation; “Teddy Bear Granuloma”, a foreign body granuloma mainly induced by synthetic fibers of toys, pullover sweaters, blankets, and so on should also be excluded,but your case is more compatible with Splendore-Hoeppli phenomenon .

The recommended treatment plan is: 
a combination of corticosteroids mainly topical forms, (short curse) Tacrolimus eye drop, Olantin (Olopatadine) eye drop, lubricants, and surgical excision of large ocular surface lesions non-responsive to medical treatment.

Many thanks for your email. It seems an interesting case.
Could you provide to me some histlogical and/or cytlogical images.
Waiting for your answer.

In the article Splendore-Hoeppli phenomenon, by Divya Gopinath: you can read the importance to investigate the cause of the infection and that the reaction in the eye is mainly caused by immune cells (type III or Type IV reaction) and isn't caused by IgE antibodies (type I reaction = classic allergy with hives, shortness of breath, low blood pressure).

Has the patient been evaluated for Steven Johnson syndrome? I didn't see it in the file. It can be viewed from this angle. There seems to be an ocular cicatrizing condition. In the differential diagnosis, ocular pemphigus, severe allergic conjunctivitis, trachoma can be considered.

First of all, get well soon, Unfortunately, I do not have any studies on this phenomenon. As far as I can see, it has little to do with primary immunodeficiencies, and more allergic diseases are being researched. I hope a definitive diagnosis can be made and your daughter will recover.

Thank you for history and pictures about Melda.  Have you got any slit lamp photos of the lesion taken by Ophthalmologist?  The photos you kindly sent do not show enough detail to make a clinical diagnosis. The biopsy taken can be non-specific inflammatory changes, so the diagnosis is made on the clinical appearance. Has topical cyclosporine been trialled.  The dose may need to be higher eg 1%bd, but limited by tolerance (tends to sting). You could trial a lower dose if not tolerated.  Wishing her the very best.

I would like also to see images of her histology slides not only the report. I would like to see them myself and review them. If that’s possible would be great so I can satin it here and examine it throughly.
If you can provide 5 unstained slides to me and one H&E stain.