title: Lichen sclerosus and eosinophilic fasciitis as manifestations of chronic graft-versus-host disease: expanding the sclerodermoid spectrum.
abstract: Chronic cutaneous graft-versus-host disease (GVHD) is classically divided into two major clinical categories--lichenoid and sclerodermoid. Although diffuse areas of sclerosis as in scleroderma characterize the more advanced stages of the sclerodermoid form, the initial circumscribed plaques would be more correctly described as morpheaform. Eosinophilic fasciitis (EF) (a fibrosing disorder related to deep morphea) and lichen sclerosus (LS) have also been reported as manifestations of sclerodermoid GVHD. However, these two presentations of GVHD have not been emphasized in the dermatologic literature. We describe 6 patients, all of whom developed LS and two of whom also developed EF in the context of chronic GVHD. Each patient presented clinically with hypopigmented plaques that exhibited wrinkling, scaling, and follicular plugging. These lesions demonstrated the classic histologic features of LS including epidermal atrophy; a subepidermal zone of pale-staining, homogenized collagen; and a bandlike lymphocytic infiltrate. Although all patients eventually developed morpheaform and/or sclerodermoid GVHD, LS was a prominent part of the initial presentation of chronic cutaneous GVHD in every case. The LS lesions tended to occur on the neck and upper to mid aspect of the trunk, whereas morpheaform lesions favored the lower aspect of the trunk. EF involved the extremities (sparing the hands and feet), and was characterized clinically by an acute onset of pain and edema followed by induration with a rippled appearance. This case series serves to expand the spectrum of sclerodermoid GVHD, with LS as the most superficial and EF as its deepest manifestation.
authors.affiliation: Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut, USA. schafj04@med.nyu.edu
authors.forename: Julie V
authors.initials: JV
authors.lastname: Schaffer
authors.forename: Jennifer M
authors.initials: JM
authors.lastname: McNiff
authors.forename: Stuart
authors.initials: S
authors.lastname: Seropian
authors.forename: Dennis L
authors.initials: DL
authors.lastname: Cooper
authors.forename: Jean L
authors.initials: JL
authors.lastname: Bolognia
mesh_terms: D000328:Adult; D002908:Chronic Disease; D004802:Eosinophilia / Q000209:etiology*; D005208:Fasciitis / Q000209:etiology*; D005260:Female; D006086:Graft vs Host Disease / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000601:surgery; D018459:Lichen Sclerosus et Atrophicus / Q000209:etiology*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012074:Remission Induction; D033581:Stem Cell Transplantation