Eosinophilic fasciitis (Shulman syndrome), a rare entity and diagnostic challenge, as a manifestation of severe chronic graft-versus-host disease: a case report

Abstract

Background Shulman’s disease, or eosinophilic fasciitis (EF), is a rare autoimmune disease, characterized by sclerodermic skin lesions with progressive induration and thickening of the soft tissues. Chronic graft-versus-host-disease (GVHD) presenting as EF is a very rare manifestation of cutaneous GVHD. Case presentation We report an unusual case of EF in a 46-year-old Caucasian male patient who had received an allogenic hematopoietic stem cell transplantation in the context of relapsed/refractory multiple myeloma. The diagnosis was challenging, with the patient presenting hepatic dysfunction, normal eosinophils count, and incomplete clinical signs. Magnetic resonance imaging (MRI) and skin biopsy confirmed the diagnosis of EF. Early initiation of specific treatment with corticosteroids and prednisolone achieved complete response. Conclusion In practice, incomplete signs in this rare complication should lead to MRI as it is a major tool to guide decision-making based on the skin biopsy, allowing a rapid diagnosis and the initiation of treatment without delay.

Graft versus host disease-related eosinophilic fasciitis: cohort description and literature review

“…Studies and reviews specifically of cGVHD-related fasciitis are limited. Most of them are descriptions of isolated cases [21,[27][28][29][30][31][32][33][34][35] or case series with small sample sizes [19,20,[36][37][38], or uncontrolled series [19,26]. For this reason, the cohort of our hospital is the second largest series to be reported in the literature after that of the Seattle group [26], whose publication extended the data they published in 2014 [19].…”

“…5). This included 10 individual case reports [21,[27][28][29][30][31][32][33][34][35], five retrospective case series [19,20,[36][37][38] and one extension of a single prospective observational study [26]. EF-like cGVHD was first reported in 1987 by van den Bergh et al [34] and later in 1990by Markusse et al [34].…”

Diagnostic Utility of Magnetic Resonance Imaging in Eosinophilic Fasciitis: A Case Report and Review of Literature

“…EF most commonly presents with cutaneous involvement as reported in the vast majority of patients [ 2 , 9 , 10 ]. Symptoms often evolve temporally, beginning with an edematous phase, followed by skin induration with a "peau d’orange" appearance, which then progresses to tightness and stiffness of the skin and deep fascia [ 2 , 11 , 12 ].…”

“…Other common findings include polyclonal hypergammaglobulinemia and elevated inflammatory markers, both of which are seen in more than 50% of patients [ 2 ]. While peripheral eosinophilia is a characteristic feature of EF, it is not required for diagnosis and is a transient finding that does not correlate with disease severity, with levels fluctuating over time independent of the disease activity [ 4 , 9 , 10 , 17 ]. However, there have been reports hypothesizing a potential correlation between disease activity in EF and other biomarkers such as serum aldolase levels [ 18 ].…”

Eosinophilic Fasciitis and Smoldering Multiple Myeloma: An Exceptional Association in Young Adults

“…These are mainly global bone marrow aplasia, aplastic anemia, autoimmune thrombocytopenic purpura, myelomonocytic leukemia, Hodgkin's disease, and monoclonal gammopathies. The association of EF and multiple myeloma remains rare in the literature [4,10], unlike monoclonal gammopathies, which are frequently described during EF. Our young patient presents smoldering multiple myeloma, which was discovered fortuitously during the systematic workup requested at the time of the diagnosis of EF and does not require specific treatment for the moment.…”