Nodular Sclerodermatous Chronic Cutaneous Graft-Versus-Host Disease (GvHD): A New Clinicopathological Variant of Cutaneous Sclerodermatous GvHD Resembling Nodular/Keloidal Scleroderma

Abstract

Cutaneous chronic graft-versus-host disease (GvHD) has a broad spectrum of clinicopathological presentations, the most common ones being poikiloderma, lichen planus-like eruptions, lichen sclerosus-like lesions, morphea-like plaques, and deep sclerosis. New forms of chronic cutaneous GvHD with different clinicopathological characteristics have been described, most of them mimicking cutaneous manifestations of autoimmune diseases. We report the case of a 35-year-old man who underwent allogenic stem cell transplantation for a therapy-associated acute myeloid leukemia and developed an acute GvHD with involvement of skin and gastrointestinal tract. He subsequently presented with chronic sclerodermatous cutaneous GvHD, followed by the appearance of indurated erythematous papules and plaques located on his back, resembling the nodular/keloidal form of cutaneous scleroderma on both clinical and histopathological grounds. This peculiar clinicopathologic presentation of chronic cutaneous GvHD was never described previously.

Nonvascularized human skin chronic allograft rejection

“…Therefore, it may be that some pathophysiological mechanisms of injury and disease (eg, the presence of growth factors or cytokines) may be common in such conditions. 23,24 In conclusion, the current report details the features of the very late development of chronic mixed cellular and humoral allograft rejection of the skin, in a unique case after STSG. It also provides new clinical and immunopathological evidence that may be relevant to the current debates on the indications and risks of skin allograft transplants in humans, which can be successful in the early years after the procedure, but which may have dramatic consequences in the long term.…”

Chronic Graft Versus Host Disease: An Update on the Clinical Characterization and Diagnostic Assessment of Cutaneous and Articular Sclerotic Forms

“…Peñas et al add, within this sclerodermiform spectrum, another subtype of localized scleroderma called paniculitis-like (subcutaneous fat tissue), placing it between morpheaform lesions and eosinophilic fasciitis [24].Exceptionally, cases of pansclerotic Morphia type cGVHD have been described. In addition, Italian authors have reported an infrequent localized sclerodermiform picture similar to nodular/cicatricial scleroderma in patients with cGVHD, increasing the clinical sclerotic skin spectrum of the disease [25]. Vascular proliferations within the area of scleroderma that mimic lesions of Kaposi's sarcoma -particularly in the extremities -have also been described recently, proposing the term for this sclerotic variant of cGVHD-associated angiomatosis due to its specificity of cutaneous cGVHD compared to other fibrotic entities [26].…”

Afectación musculoesquelética y del tejido conectivo en pacientes sometidos a trasplante alogénico de progenitores hematopoyéticos con enfermedad injerto contra receptor crónica: caracterización clínica y biológica

“…Peñas y colaboradores (99) agregan dentro de este espectro esclerodermiforme a otro subtipo de esclerodermia localizada denominado paniculitis-like (tejido graso subcutáneo) ubicándola entre las lesiones morfeiformes y la FE-like. Adicionalmente, autores italianos (105) han comunicado un cuadro infrecuente esclerodermiforme localizado similar a la esclerodermia nodular /cicatricial, en pacientes con EICRc, incrementando el espectro clínico cutáneo esclerótico de la enfermedad. Las proliferaciones vasculares dentro del área de esclerodermia que remedan las lesiones del sarcoma de Kaposi, particularmente en las extremidades, también se han descrito recientemente (106) , proponiendo el término para esta variante esclerótica de angiomatosis asociada a EICR debido a su especificidad de EICRc cutáneo en comparación con otras entidades fibrosantes.…”