Cardwell, Leah A.

AbstractSolid organ and hematopoietic stem cell transplantation may be complicated by the development of post‐transplant lymphoproliferative disorders (PTLDs). The World Health Organization categorizes PTLDs into four entities including non‐destructive, monomorphic, polymorphic, and classical Hodgkin lymphoma types. The most common PTLDs are B‐cell lymphomas, with T‐cell lymphomas accounting for only a few cases. Cutaneous T‐cell lymphomas are rarer still in post‐transplant patients with primary cutaneous peripheral T‐cell lymphoma being an extraordinarily rare subtype in this population. PTLDs may be aggressive and are often associated with high morbidity and mortality. Advances in medicine have led to increased awareness of PTLDs and improved diagnostic tools which assist in the diagnosis of these conditions. However, the clinical and histopathologic heterogeneity of PTLDs may make diagnosis a challenge. In the transplant patient population, the cutaneous manifestations of the lymphoproliferative disease may mimic other conditions, such as eczematous dermatitis and graft‐vs‐host disease. Herein, we report a case of post‐transplant primary cutaneous peripheral T‐cell lymphoma not otherwise specified (PTCL‐NOS) in a pediatric heart transplant patient and describe the clinical presentation and diagnostic histopathologic features.

Abstract: Common variable immunodeficiency disorder is a primary immunodeficiency disorder characterized by reduced levels of serum immunoglobulins and impaired antibody response. This condition may be associated with development of noninfectious granulomatous dermatitis of the skin which may be disfiguring and destructive. There are no published guidelines for the treatment of cutaneous granulomas in this patient population. In recent studies, rubella virus–positive cells in granulomas were localized to M2 macrophages which have an important role in wound healing and the secretion of immunoregulatory cytokines. We present a case of treatment-refractory, disfiguring common variable immunodeficiency disorder–associated granulomatous dermatitis. Immunofluorescence microscopy of the biopsy specimen confirmed the presence of rubella vaccine capsid proteins in M2 macrophages within the granuloma, a newly recognized phenomenon in this patient population. This knowledge may serve to identify future therapeutic targets or preventative strategies for granulomatous dermatitis in patients with primary immunodeficiency disorder.