D2.314 - Late Presentation of Cutaneous Chronic Graft Versus Host Disease – A Case Report

Background:

Chronic graft versus host disease (GVHD) is the most prevalent complication following hematopoietic stem cell transplantation, occurring in 60-80% of patients. Cutaneous involvement is the most common manifestation of chronic GVHD, accounting for 90% of cases.

Case Presentation:

A 14-year-old girl was referred for worsening rash over her elbows, knees, and dorsum of hands and fingers over the past 6 months. She had a history of myeloproliferative disorder and underwent a matched unrelated donor hematopoietic stem cell transplant 8 years ago. On examination, there were demarcated, pink-to-hypopigmented, scaly plaques with atrophy and surrounding hypopigmentation over bilateral knuckles, proximal and distal interphalangeal joints, elbows, knees, and right buttock cleft. The oral and genital mucosawere not affected, and her nails were normal. A punch biopsy of her right knee rash showed mildly acanthotic epidermis, basal vacuolar alteration with exocytosis of small lymphocytes, along with occasional apoptotic keratinocytes, some with an adjacent lymphocyte ("satellite cell necrosis"). There was superficial and mid-perivascular lymphohistiocyticinflammatory infiltrate in the dermis. The Alcian Blue stain was negative for dermal mucin deposition and basement membrane was not thickened on PAS stain. 

The patient was treated with topical calcineurin inhibitorswith improvement of her rash after 2 months.

Our patient presents with chronic GVHD 8 years post-transplant, which is uncommon. The median onset of chronic GVHD is 4 months after unrelated donor transplantation, with only 5% of cases diagnosed after 1 year.