Graft-versus-host disease (GvHD)
Definition & Epidemiology
- GvHD = Donor immune cells recognize recipient antigens as foreign → systemic immune reaction after allogeneic stem cell or organ transplantation due to HLA incompatibility.
- Incidence: GvHD occurs in 4–60% of allogeneic Tx, depending on HLA match and prophylaxis.
- Skin almost always involved (>90% of all GvHD manifestations with cutaneous symptoms).
Pathogenesis — 3 Phases (Ferrara-Model)
- Phase 1 — Toxic-conditioning: Conditioning damage triggers: Cytokine storm + donor T-cell activation with clonal expansion of alloreactive T-cells.
- Phase 2 — Activation & Effector: Donor T-cells attack via FasL + perforin/granzyme + TNF-α/IFN-γ.
- Phase 3 — Late Fibrosis / Sclerosis: Th17 / Th2 shift + fibroblast activation → Scleroderma-like skin changes and fibrosis of internal organs.
Acute GvHD (aGvHD, < 100 days post-Tx)
- Clinical presentation: Initially maculopapular rash on acral areas/ear helix/cheeks/neck (DD: AME!), later confluent to erythrodermic.
- Severity stages (Glucksberg / Mount Sinai):
- Stage 1: Rash < 25% BSA
- Stage 2: 25–50%
- Stage 3: > 50%, generalized
- Stage 4: Erythroderma + bullae / epidermolysis (TEN-like!) + mucositis — prognostically unfavorable
- Extra-cutaneous: Hepatitis (transaminases / bilirubin ↑), GvHD enteritis (secretory diarrhea, abdominal pain, bloody stools), conjunctivitis.
Chronic GvHD (cGvHD, > 100 days post-Tx)
- NIH criteria 2014 with diagnostic and distinctive manifestations.
- Skin manifestations — 3 course types:
- Lichenoid cGvHD: Shows lichen planus-like papules + Wickham striae plus reticular oral mucosa — DD: Lichen planus.
- Scleroderma-like cGvHD: diffuse skin sclerosis, possibly fascial involvement, nail changes, pterygium unguium.
- Poikilodermatous presentation: Hyper-/hypopigmentation + atrophy + telangiectasias — DD: Mycosis fungoides poikilodermatica.
- Mucositis: xerostomic / lichenoid oral GvHD, Sicca syndrome (eye, mouth, vagina), reticular mucosal changes.
- Other organs: Lungs (BOS, bronchiolitis obliterans), liver (cholestatic), GI, eyes (Sicca).
Diagnosis
- Clinical presentation + timing (acute < 100 d, chronic > 100 d).
- Histology (punch biopsy, perilesional): Apoptotic keratinocytes in basal layer, vacuolization, lymphocytic infiltrate ("satellite cell necrosis"), in scleroderma-like cGvHD thickened dermis + fibrosis.
- Differential diagnoses: Drug reaction (DRESS, maculopapular AME), viral exanthem, toxic shock, engraftment syndrome.
Differential diagnoses
- Lichen sclerosus
- Limited systemic sclerosis (CREST syndrome)
- Lupus erythematosus gesicht
- Dermatomyositis
- Mycosis fungoides
- Atopic dermatitis (atopic eczema)
- Lichen planus
- Psoriasis vulgaris (plaque psoriasis)
Practise Graft-versus-host disease (GvHD) in the app
Flashcards with spaced repetition, exam questions and spot-the-diagnosis on this topic – in DermaFuchs, free of charge.
In the DermaFuchs app: 2 flashcards · 2 clinical images
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Note: This page is intended for medical education and does not replace diagnosis or treatment decisions in individual cases. Treatment and follow-up content is available in the app.