Key words chronic cutaneous graft-versus-host disease (GVHD), immune modulation therapy, pimecrolimus.Chronic cutaneous graft-versus-host disease (GVHD) is a severe clinical and pathological manifestation that occurs in patients after allogenic peripheral blood stem cell or bone marrow transplantation. 1 Acute and chronic forms of cutaneous GVHD show various manifestations including exanthema, erythrodermia and toxic epidermal necrolysis as acute reactions, and lichenoid or sclerodermatous changes as chronic reactions. 2 The beginning of the treatment of chronic GVHD is systemic immunosuppression and the efficacy of extracorporeal photopheresis, UVA1 phototherapy, and narrowband UVB phototherapy is currently under investigation for treatment of GVHD. 3 Nevertheless, cutaneous chronic GVHD may also respond to topical therapy such as corticosteroids, azathioprine, tacrolimus and pimecrolimus. 4,5 Pimecrolimus (SDZ ASM 981) is part of the new class of novel ascomycin immunomodulating macrolactams, and was developed for the treatment of inflammatory skin disease. 6 We report a case of a generalized cutaneous GVHD in a 9-year-old Iranian boy that occurred after bone-marrow transplantation for b-thalassemia major. The patient showed a significant improvement with topical pimecrolimus 1% cream twice daily for 9 weeks.
Case reportA 9-year-old Iranian boy was admitted to our department in April 2005 from the Mediterranean Institute of Hematology due to disfiguring lesions on the face. His medical history included b-thalassemia major for which he underwent bone marrow transplantation six years earlier followed by chemotherapy and radiation. He successively developed multiple endocrinal disorders such as growth hormone deficiency, autoimmune thyroiditis, osteoporosis and hypothyroidism. Two years after bone marrow transplantation the patient presented with GVHD with minimal cutaneous and mucosal involvement. Afterwards he started having severe GVHD with recurrent attacks of chest infections from Pseudomonas Aeruginosa, mycoplasma, Streptococcus Pneumoniae and chronic bronchiolitis; also, cutaneous involvement was ingravescent. He was treated with systemic antibiotics, cyclosporine A and a low dose of cortisone, but he experienced many steroid-related toxicities, such as glaucoma and diabetes, obesity and mild hypertension.When the patient came to our institution he presented cutaneous chronic GVHD with erythematous, hyperpigmented and hypopigmented lesions. Poikilodermic lesions with scaling, reddish, pruritic plaque with severe atrophy were prevalent in the seborrheic areas of his face (Fig. 1). Diffuse multiple scleroatrophic lesions were visible on the trunk. Furthermore he presented mild dystrophy of the oral mucosa, palate and tongue and onicodystrophy. A punch biopsy of the affected skin was compatible with the sclerotic late stage of chronic GVHD. It showed atrophy of epidermidis, with orthokeratotic maturation, dilation of bloodstream small vessels of superficial derma and the presence of macrophages containing ...