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E-IJD® - CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 5  |  Page : 626
A rare presentation of acute on chronic graft versus host disease


1 Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Dermatology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Internal Medicine, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
4 Department of Histopathology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication29-Dec-2022

Correspondence Address:
Jagat Ram
Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_140_22

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How to cite this article:
Khurana S, Gupta PC, Kulshrestha A, Vinay K, Khadwal A, Nada R, Bashyal B, Ram J. A rare presentation of acute on chronic graft versus host disease. Indian J Dermatol 2022;67:626

How to cite this URL:
Khurana S, Gupta PC, Kulshrestha A, Vinay K, Khadwal A, Nada R, Bashyal B, Ram J. A rare presentation of acute on chronic graft versus host disease. Indian J Dermatol [serial online] 2022 [cited 2023 Feb 8];67:626. Available from: https://www.e-ijd.org/text.asp?2022/67/5/626/366103




Sir,

Graft versus host disease (GVHD) consists of a myriad range of symptoms caused by an exaggerated donor response to host antigens after allogeneic hematopoietic stem cell transplantation (aSCT). It is a leading cause of morbidity and mortality among these patients and often involves multiple organs.[1],[2] Ocular manifestation can be found in 60–90% of patients with chronic GVHD (cGVHD).[3] We describe a case of a young male who presented with acute on cGVHD with ocular GVHD (oGVHD).

A 21-year-old male, with cGVHD and a history of aSCT, presented to our ophthalmology department with redness and irritation in both eyes. Ocular examination revealed bilateral diffuse conjunctival congestion. He was advised of topical antibiotics and lubricants. Three days later, his ocular symptoms worsened with increased congestion, chemosis, and subconjunctival hemorrhage [Figure 1]. The same day evening, he presented to the emergency with a puffy face, throat pain, and difficulty swallowing. Systemic examination revealed high-grade fever, edematous and ulcerated mucosa of the palate and sublingual mucosa, and erythematous scaly plaques over the trunk [Figure 2]. Suspecting it to be acute GVHD (aGVHD), he was admitted and started on intravenous hydrocortisone, antibiotics, antivirals, and supportive care. Panviral polymerase chain reaction in conjunctival and oral swabs was negative. During the stay, he developed bilateral corneal epithelial defects and dense forniceal pseudomembranes. A bandage contact lens was applied along with the prescription of topical loteprednol 0.5% thrice a day, cyclosporine 0.05% twice a day, vitamin C 10% four times a day, and carboxymethylcellulose 0.5% hourly for his ocular disease.
Figure 1: Anterior segment photograph of the right eye (a) and the left eye (b), respectively, showing corneal epithelial defects and chemosis

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Figure 2: (a) Superficial erosions and hemorrhagic crusting over lips. (b) Erythematous scaly plaques over the upper chest

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Conjunctival biopsy revealed loss of mucus cells, extensive submucosal congestion, and epithelial eosinophilic apoptotic bodies, and was negative for viruses. Thoracic skin biopsy showed thinned epidermis with multiple apoptotic cells. The findings were diagnostic of conjunctival and cutaneous GVHD [Figure 3]. Our patient already had cGVHD, and hence, was now diagnosed as acute on cGVHD, which occurred possibly due to early tapering of corticosteroids or cyclosporine. The ocular symptoms and signs resolved completely in 4 weeks with the absence of any recurrence in 6 months follow-up.
Figure 3: (a) Photomicrograph of skin biopsy from the thoracic region using hematoxylin and eosin showing thinned out epidermis with plenty of apoptotic cells in epidermis accompanied by moderate lymphomononuclear cells in the upper dermis. (×200) (b) Photomicrograph of conjunctival biopsy using hematoxylin and eosin showing many epithelial eosinophilic apoptotic bodies (×400)

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The National Institutes for Health (NIH) has classified GVHD into aGVHD, cGVHD, and overlap syndrome in 2005. Ocular symptoms are most commonly seen in cGVHD and extremely rare in aGVHD and overlap syndrome. According to NIH, oGVHD can be classified based on the severity of symptoms, frequency of lubricants required, and daily activity impairment with scores increasing as the severity increases.[4] Systemic treatment of cGVHD includes immunosuppression with steroids and steroid-sparing agents, which are often unable to control ocular disease, hence necessitating the need for topical treatment. Topical steroids are first-line therapy in GVHD, as they exert non-specific inhibitory effects on the inflammatory response. Topical calcineurin inhibitors like cyclosporine and tacrolimus inhibit T-cell activation and downregulate pro-inflammatory cytokines. Topical interleukin-1 receptor antagonist and sub-anticoagulant dose heparin are shown to have some effect on oGVHD. Surgical interventions like amniotic membrane transplantation are usually kept as the last resort. Cytokine storm in aGVHD can cause pseudomembranes and corneal epithelial sloughing, as seen in our patient, and is indicative of severe systemic involvement.[5] Early diagnosis and management of our patient prevented cicatricial changes [Figure 4]. Long-term follow-up will be required for cGVHD.
Figure 4: Anterior segment photograph of the right eye (a) and the left eye (b), respectively, showing clear cornea and resolution of ocular features at the last follow-up visit

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Tabbara KF, Al-Ghamdi A, Al-Mohareb F, Ayas M, Chaudhri N, Al-Sharif F, et al. Ocular findings after allogeneic hematopoietic stem cell transplantation. Ophthalmology 2009;116:1624-9.  Back to cited text no. 1
    
2.
Westeneng AC, Hettinga Y, Lokhorst H, Verdonck L, van Dorp S, Rothova A. Ocular graft-versus-host disease after allogeneic stem cell transplantation. Cornea 2010;29:758-63.  Back to cited text no. 2
    
3.
Dietrich-Ntoukas T, Cursiefen C, Westekemper H, Eberwein P, Reinhard T, Bertz H, et al. Diagnosis and treatment of ocular chronic graft-versus-host disease: Report from the German-Austrian-Swiss consensus conference on clinical practice in chronic GVHD. Cornea 2012;31:299-310.  Back to cited text no. 3
    
4.
Jagasia MH, Greinix HT, Arora M, Williams KM, Wolff D, Cowen EW, et al. National Institutes of Health Consensus Development Project on criteria for clinical trials in chronic graft-versus-host disease: I. The 2014 Diagnosis and Staging Working Group report. Biol Blood Marrow Transplant 2015;21:389-401.e1  Back to cited text no. 4
    
5.
Lin X, Cavanagh HD. Ocular manifestations of graft-versus-host disease: 10 years' experience. Clin Ophthalmol 2015;9:1209-13.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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