Diagnostic Challenge in Recurrent Skin Rash After Autologous Bone Marrow Transplantation

Abstract

Engraftment syndrome, autologous graft-versus-host disease (GVHD), and infection after autologous hematopoietic cell transplantation can have similar clinical presentations. Here, we describe a patient with refractory Ewing sarcoma who had recurrent skin rash after autologous hematopoietic cell transplantation. Although the rash was diagnosed as GVHD histologically, this case illustrates the diagnostic dilemma of distinguishing engraftment syndrome, autologous GVHD, or concomitant viral infection. Because therapy for these entities is different, distinguishing them is important. Establishment of diagnostic criteria and understanding of the pathophysiology of these entities may lead to better management and to improved therapy of refractory cancer.

Acute Renal Endothelial Injury During Marrow Recovery in a Cohort of Combined Kidney and Bone Marrow Allografts

“…CLS or engraftment syndrome (ES) has been reported in patients receiving autologous stem cell or allogeneic bone marrow transplants (BMTs), a condition occurring during neutrophil recovery, as described by Spitzer and colleagues (10,11). Varied manifestations include fever, rash, pulmonary infiltrates, fluid retention, diarrhea and hepatic injury and AKI (10)(11)(12)(13)(14)(15)(16). ES may be independent of graft versus host disease and a host versus graft reaction (10,11,17,18), but its pathogenesis is unknown.…”

“…Varied manifestations include fever, rash, pulmonary infiltrates, fluid retention, diarrhea, and hepatic injury and AKI (10–16). ES may be independent of graft versus host disease and a host-versus-graft reaction (10, 11, 17, 18), but its pathogenesis is unknown.…”

Varicella‐zoster virus encephalomyelitis with a prominent demyelinating component

“…Our patient did have a history of a generalized, pruritic rash 2 months before onset of neurologic presentation. Although it can be difficult to distinguish between GVHD‐associated rashes and VZV rashes, both the skin biopsy and the improvement of the rash with steroid treatment are consistent with GVHD. Although the possibility that the patient did have an unreported VZV rash prior to presentation at our hospital cannot be excluded, this likely represents one of the rare cases in which VZV‐induced neurological disease occurs sine zoster rash .…”

Skin Signs of Graft Versus Host Disease

“…Differential diagnosis of skin symptoms includes engraftment syndrome, toxic epidermal necrolysis, irritant or allergic contact dermatitis, lichen planus, morphea, scleroderma, erythroderma, toxic shock syndrome, stevens-johnson syndrome, staphylococcal scalded skin syndrome and most commonly and importantly drug eruptions and viral exanthemas [34,38]. Drug-induced rashes are very common in post-transplant patients due to the amount of medications that these patients have to take.…”

“…The diagnosis of GVHD is complicated by the complex immune status of the patient and by the fact that other eruptions that are common in immunosupressed patients can be easily confused with GVHD [34]. An accurate diagnosis can be achieved by using specific histological and immunohistochemical criteria [35][36][37].…”