Mechanism
- Complication of allogenic hematopoietic stem cell transplantation
- Caused by cytotoxic action of the donor’s T lymphocytes on the recipient’s tissues
Clinical presentation
- Acute manifestions (first 100 days adter transplantation)
- Skin: erythematous or erythematosquamous, similar to toxic dermatitis
- May be localized or diffuse
- NET like pattern in most severe form
- Treatment: increase immunosuppression
- Chronic
- Skin
- Sclerodermiform forms → similar to morphea
- Localized to pansclerotic
- Difficult to treat, immunosupressants are often disappointing, physical therapy is essential
- Lichenoid form
- Similar to lichen planus
- Other organs
- Ocular: dry syndrome with synechial blepharoconjunctivitis
- Oral mucosa: microstomia, erosions
- Genital mucosa (erosive lichen sclerosus)
- Sclerotic leukomelanoderma
Treatment (often hematology)
- Immunosupression
- JAK and tyrosine kinase inhibitors
- Photopheresis
- topical corticosteroids
- Emollients
- Physical therapy
Ophtalmological and oral follow-up