A Case of Eczematoid Graft‐Versus‐Host Disease

Abstract

A 13-year-old boy underwent allogeneic hematopoietic stem cell transplantation (HSCT) for underlying acute lymphoblastic leukemia and achieved neutrophil engraftment 28 days after HSCT. He developed ichthyosis 6 weeks after HSCT and then keratotic follicular papules, palmoplantar keratoderma, and a seborrheic dermatitis-like eruption 18 weeks after HSCT. From skin biopsies he was diagnosed with eczematoid graft-versus host disease (GVHD), which showed spongiosis with scattered necrotic keratinocytes. He responded to oral and topical steroids and an increase in cyclosporine dose. Although uncommon, eczematoid GVHD must be considered in children who have undergone HSCT and then develop an atypical eczematous eruption, especially in the absence of a history of atopy.

Eczematous graft‐vs‐host disease: A report of three cases and review of the literature

“…Eczema-like GVHD has been previously described as an atypical form of GVHD, 1 affecting both pediatric and adult patients, ranging in age from 1.5 to 65 years. [10][11][12][13] Creamer et al reported 10 patients with widespread eczematous eruptions in a background of chronic GVHD following HCT, and described this presentation as "eczematoid GVHD," 10 showing an aggressive chronic clinical course and poor prognosis. All patients in this series developed erythroderma and six patients died either from infection (n = 5) or from the relapse of the background hematolymphoid disease (n = 1).…”

“…11 Other isolated case reports have described eczema-like GVHD in patients who have shown a good response to treatment with complete remission of the disease. 12,13 The average time to presentation following HCT in reported cases of "eczematoid"…”

“…10,11 Clinical features included diffuse erythema, xerosis, fine scale, lichenification, perifollicular accentuation, and severe pruritus. [10][11][12] Secondary impetiginization, weeping, and palmoplantar hyperkeratosis were also observed in cases of eczematoid GVHD. 10 Cumulative clinicopathological features of the previously reported and the current cases of eczema-like GVHD are summarized in Table 1.…”

“…Despite the variable clinical presentation and unpredictable prognosis, the histopathological findings appear to be similar in all cases of eczema-like GVHD. [10][11][12][13] Hence, skin biopsy plays an important role in or without lichenoid papules, widespread exfoliative dermatitis, or erythroderma. 15 A grading system for GVHD has been proposed based on the severity of histopathological changes; however, this grading scheme correlates poorly with the clinical extent of cutaneous eruptions and progression of the disease.…”

“…13 There were notable similarities between one of these patients and one of our cases in Various treatments including moderate to potent topical steroids, topical calcineurin inhibitors, narrowband ultraviolet B, psoralen ultraviolet A (PUVA), and systemic immunosuppressants have been used for the management of eczema-like GVHD. [10][11][12][13] Overall a good response to combined topical treatment and systemic immunosuppressants as well as PUVA as an immunosuppression-sparing therapy is reported. [10][11][12][13] Our patients required a combination of topical and systemic immunosuppression, with one patient having significant improvement with the addition of narrowband ultraviolet B.…”