Sclerodermoid Chronic Graft vs Host Disease

Final Diagnosis: Sclerodermatous Chronic Graft Versus Host Disease.

Diagnosis comment: The biopsy shows evidence of chronic graft versus host disease. There is a fully evolved picture of sclerodermotous chronic graft versus host disease. It is characterized by a hyalinizing pattern of fibrosis occupying much of the dermis with relative sparing of the lower part of the dermis; the classic scleroderma phenotype amidst the fibroblasts is identified. In particular, the fibroblasts no longer express CD34 and show focal acquisition of smooth muscle actin staining.

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Slide 1: The patient is a 60-year-old female with a history of AML, acute myeloid leukemia in remission. The patient is status post a stem cell transplant and presents with a new rash involving the face, oral mucosa, and genital mucosa that began about 2 weeks ago. Clinical examination disposes hyperpigmented papules and thin plaques involving the nose, cheeks, temple and forehead with a few hypopigmented xerotic thin papules scattered on the back.

Slide 2: The epidermis is focally attenuated with loss of the rete ridge pattern. There are many melanophages within the superficial corium. Commencing the mid reticula dermis and extending in this deeper reticular dermis, the collagen bundles are of somewhat wider caliber.

Slide 3: An active lymphocyte-mediated interface dermatitis involving adnexal structures or the epidermis is not observed recognizing that one may have existed given the epidermal thinning and pigment incontinence.

Slide 4: There is an infiltrate of mononuclear cells around blood vessels within the mid and deep reticular dermis.

Slide 5: The CD34 preparation shows an area within the deeper dermis where the CD34 staining pattern is markedly diminished although elsewhere the CD34 staining pattern is normal.

Slide 6: The CD34 preparation shows an area within the deeper dermis where the CD34 staining pattern is markedly diminished although elsewhere the CD34 staining pattern is normal.