Dermatology - Graft Versus Host Disease
The entirety of organ failure brought on by histoincompatible, immunocompetent donor cells attacking immunocompetent host tissues is known as graft-versus-host disease (GVHD). A particular organ's expression of GVHD is known as graft-versus-host reaction (GVHR) (e.g., cutaneous GVHR). Usually happening 10–30 days following bone marrow transplantation (BMT), acute cutaneous GVHR ensues. It is the most common and earliest. After liver and other gastrointestinal transplants, GVHR are also frequently observed. Chronic cutaneous GVHR presents as lichenoid and sclerodermoid alterations and happens more than 60 days following allogeneic BMT. Localized or widespread pruritus, discomfort upon pressure, nausea, vomiting, stomach pain, watery diarrhea, and jaundice are some of the symptoms of acute GVHR. First, the hands, feet, and upper trunk all develop small, distinct macules and/or papules, particularly on the palms and soles. On the face, macules confluence and are frequently painful and erosive. Mild edema with violaceous tint, periungual regions, and pinna may be present. Many times, erythema develops in a perifollicular array. Erythema lessens if it is managed or treated, leading to desquamation and postinflammatory hyperpigmentation. If it worsens, macules and papules develop into erythroderma, which is widespread and confluent. Then, there are subepidermal bullae, particularly at pressure or trauma sites, palms, or soles, and a positive Nikolsky sign (skin that peels off when gently pressed). In patients with chronic GVHD, there may be confluent patches of dermal sclerosis with overlaying scale resembling scleroderma, primarily on the trunk, buttocks, hips, and thighs, and/or flat-topped (lichen planus–like) papules of violaceous color, initially on distal extremities but subsequently widespread. Severe generalized sclerodermoid alterations with necrosis and ulceration on acral and pressure sites also involve the face in cases of more severe disease. The differential comprises erythroderma, toxic epidermal necrosis, viral exanthem, and exanthematous medication response. PUVA, extracorporeal photopheresis, or topical glucocorticoids can be used to treat lesions. Methylprednisolone, tacrolimus, cyclosporine, mycophenolate mofetil, etanercept, and infliximab are used to treat systemic effects and rejection.
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