Race for the Case answers

Diagnose this new case by Nancy Shen, MD

A 61-year-old female with history of acute myeloid leukemia (AML) status-post unrelated donor stem cell transplant two months prior presented to the hospital with two weeks of an itchy rash that first appeared on her legs. The patient reported spread of the rash to her face, chest, and back over the past week, although denied blistering, burning, or pain at involved areas. On exam, there were erythematous patches and plaques with thin scale present over the chest, back, and upper and lower extremities, affecting approximately 40% of her body surface area. The patient did not have mucosal involvement and no bullae were present. The patient denied all other symptoms including fever, chills, dyspnea, abdominal pain, vomiting, diarrhea, and urinary changes. She also denied any recent changes in her medications preceding the rash.

Laboratory evaluation was remarkable for chronic thrombocytopenia. Urinalysis, bilirubin, and liver enzymes were normal. A 4-millimeter punch biopsy was obtained from the upper back and submitted for hematoxylin and eosin (H&E) staining. The biopsy returned with vacuolar interface dermatitis with mild perivascular infiltrate in the superficial dermis. Interface change with necrotic keratinocytes extended down epithelial adnexal structures.

1. What are the three most common organs affected in graft-versus-host disease (GVHD)?

Answer: Skin, liver, and GI tract

2. What is the underlying immunologic cause of GVHD?

Answer: Donor T-cells attack host cells, most frequently due to mismatch in tissue human leukocyte antigens (HLA) between donor and recipient

3. What is the time cutoff between acute and chronic cutaneous GVHD?

Answer: 100 days post-transplant

4. What stage and chronicity of skin GVHD does this patient meet?

Answer: Acute GVHD, Stage 2 skin, based on 25-50% BSA

5. What is the first-line systemic treatment for this patient?

Answer: Systemic corticosteroids with either oral prednisone or IV methylprednisolone

6. What are four common cutaneous manifestations of chronic GVHD?

Answer: Lichen planus-like, lichen sclerosus-like, morphea-like, scleroderma-like, fasciitis, poikiloderma