Lichen sclerosus and eosinophilic fasciitis as manifestations of chronic graft-versus-host disease: Expanding the sclerodermoid spectrum

Schaffer, Julie V.; McNiff, Jennifer M.; Seropian, Stuart; Cooper, Dennis; Bolognia, Jean L.

doi:10.1016/j.jaad.2005.06.015

Cited by 72 publications

(53 citation statements)

References 56 publications

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“…Finally, sclerotic cGVHD in adults has been associated with the need for prolonged immunosuppression, significant functional impairment and, more variably, decreased survival. 14,15 In our study, patients with sclerotic disease also had worse outcomes, with a significantly increased incidence of steroid-resistant disease and higher death rate. Patients with sclerotic disease were also more likely to have nail dystrophy, preceding peripheral edema and preceding eosinophilia than those with nonsclerotic disease.…”

Section: Demographicsmentioning

confidence: 54%

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Nail dystrophy, edema, and eosinophilia: harbingers of severe chronic GVHD of the skin in children

Huang

Duncan

Boyer

et al. 2014

Bone Marrow Transplant

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The prognostic value of adnexal findings in chronic GVHD (cGVHD) has not been investigated in children. Dermatologic examinations were performed in a severe cohort of 11 children with skin cGVHD seen over a 2-year period. Findings were compared with 25 additional patients with skin cGVHD and 97 control patients. In 36 patients with skin cGVHD, nail dystrophy was present in 45% of patients, and was significantly associated with sclerotic disease and lung cGVHD. Pterygium inversum unguis (PIU) was associated with severe lung disease, with significantly lower % predicted FVC and FEV1 in those with PIU than those without. Forty-four percent of GVHD patients had preceding peripheral edema and 56% had preceding peripheral eosinophilia. Peripheral edema and eosinophilia were significantly associated with sclerotic cGVHD and persisted until the diagnosis of cGVHD in all patients. Comparison of data with control patients showed that incidence of nail dystrophy, incidence of peripheral edema and mean peak peripheral eosinophil count of patients with skin cGVHD was significantly higher than those without cGVHD. This study suggests that nail dystrophy, persistent peripheral edema and persistent peripheral eosinophilia are harbingers of severe cGVHD of the skin in children. The presence of PIU may be a harbinger of severe lung involvement.

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Section: Demographicsmentioning

confidence: 54%

“…[15][16][17][18] Recently, it has been postulated that edema associated with cGVHD may be due to endothelial activation and injury. 18 In our study, peripheral edema was a preceding sign in 13 of 15 patients with sclerotic cGVHD with a mean time of onset of 58.5 days before the development of sclerotic disease.…”

Section: Demographicsmentioning

confidence: 99%

Nail dystrophy, edema, and eosinophilia: harbingers of severe chronic GVHD of the skin in children

Huang

Duncan

Boyer

et al. 2014

Bone Marrow Transplant

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“…Sclerodermoid chronic GVHD often presents with plaques of dermal sclerosis that resembles morphea and eventually progresses to generalized scleroderma or, alternatively, presents with lichen-sclerosus-like features in a genital or extragenital distribution. Since its initial recognition, the spectrum of the fibrosing GVHD variants has been continuously extended, and both lichen-sclerosus-type as well as eosinophilic-fasciitis-type sclerotic lesions are now considered diagnostic features and often result in joint contractures [73]. Poikilodermatous changes can be hallmark signs of chronic GVHD and are predominantly observed on the face, lateral neck and trunk, exceptionally in addition to multiple hemangiomas.…”

Section: Clinical Manifestations Of Cutaneous Gvhdmentioning

confidence: 99%

Cutaneous Graft-versus-Host Disease: A Guide for the Dermatologist

et al. 2008

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Graft-versus-host disease (GVHD) is defined by the aggregation of clinical and pathological manifestations in a recipient of allogeneic stem cells or bone marrow transplantation in which specific immunological as well as nonspecific phenomena lead to characteristic features. GVHD is one of the major complications after hematopoietic stem cell transplantations and responsible for posttherapeutic morbidity, mortality and decrease in quality of life of those patients. GVHD is critically induced and maintained by donor immunocompetent cells that particularly attack epithelia of fast proliferating tissues such as those from the liver, gastrointestinal tract and skin. On the basis of the time of presentation, cutaneous GVHD has been originally divided into an acute and chronic disease. The latter has traditionally been further subclassified into a more epithelial or lichenoid and a predominantly dermal or sclerodermoid form. With respect to the growing importance of this therapeutic procedure and increasing numbers of outpatients presenting with chronic GVHD, this article summarizes the updated knowledge on this disease focused for the dermatologist, and additionally it emphasizes the recent consensus documents on the various aspects of chronic GVHD of the National Institute of Health.

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“…CD3 T-cell dose in the graft, eosinophilia, positive antinuclear antibodies (ANAs), and antecedent nonsclerotic cGVHD skin involvement have been proposed as markers of ScGVHD 4 ; however, previous series included only small samples of patients and retrospective study designs, and registry and cancer center data fail to distinguish between sclerotic and nonsclerotic cutaneous manifestations of cGVHD. [2][3][4][8][9][10][11] The National Institutes of Health (NIH) Consensus Project recently proposed clear definitions of the sclerotic features of cGVHD, 12 but it will take many years to prospectively collect a significant number of patients with this rare cGVHD subset. The NIH cGVHD Natural History cohort comprises a unique referral population of cGVHD patients with significant (63% NIH global score of "severe") refractory disease (median previous therapies ϭ 4) who undergo a comprehensive prospective multisystem evaluation.…”

Section: Introductionmentioning

confidence: 99%

Sclerotic-type chronic GVHD of the skin: clinical risk factors, laboratory markers, and burden of disease

Martires¹,

Baird

Steinberg

et al. 2011

Blood

102

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Chronic GVHD is one of the most severe complications of allogeneic HSCT. The sclerotic skin manifestations of cGVHD (ScGVHD) result from inflammation and fibrosis of the dermis, subcutaneous tissue, or fascia, leading to significant functional disability. Risk factors and clinical markers associated with ScGVHD remain largely unexamined. By using a singlevisit, cross-sectional design, we evaluated 206 patients with cGVHD at the National Institutes of Health. Most patients manifested severe (ie, 63% National Institutes of Health score "severe"), refractory disease (median treatments ‫؍‬ 4). ScGVHD was detected in 109 (52.9%) patients. ScGVHD was associated with greater platelet count (P < .001) and C3 (P < .001), and decreased forced vital capacity (P ‫؍‬ .013). Total body irradiation (TBI) was associated with development of ScGVHD (P ‫؍‬ .002). TBI administered in reduced-intensity conditioning was most strongly associated with ScGVHD (14/15 patients, P < .0001). Patients with ScGVHD had significant impairments of joint range of motion and grip strength (P < .001). Greater body surface area involvement was associated with poorer survival (P ‫؍‬ .015). We conclude that TBI, particularly in reduced-intensity regimens, may be an important risk factor for ScGVHD.

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Lichen sclerosus and eosinophilic fasciitis as manifestations of chronic graft-versus-host disease: Expanding the sclerodermoid spectrum

Cited by 72 publications

References 56 publications

Nail dystrophy, edema, and eosinophilia: harbingers of severe chronic GVHD of the skin in children

Nail dystrophy, edema, and eosinophilia: harbingers of severe chronic GVHD of the skin in children

Cutaneous Graft-versus-Host Disease: A Guide for the Dermatologist

Sclerotic-type chronic GVHD of the skin: clinical risk factors, laboratory markers, and burden of disease

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