Possible Association of Cutaneous Rosai-Dorfman Disease and Chronic Crohn Disease

Abstract

IMPORTANCE Cutaneous Rosai-Dorfman disease (CRDD), a variant of Rosai-Dorfman disease limited to the skin, has a wide range of clinical presentations. Rosai-Dorfman disease is believed to result from an aberrant response to antigens, caused by immunosuppressive macrophages. Macrophage-mediated immunosuppression is also implicated in the pathogenesis of Crohn disease, linking these otherwise unrelated entities. To our knowledge, the coexistence of these disorders has been described in only 2 cases, 1 of them confined to the skin and soft tissue. OBSERVATIONS We present a series of 3 patients who developed purely CRDD in the context of long-standing Crohn disease. Statistical estimates suggested that the association of these 2 disorders is not due to chance (P < .001). CONCLUSIONS AND RELEVANCE Our case series provides the clinical correlate to the pathogenetic parallels between CRDD and Crohn disease. Crohn disease is frequently complicated by various skin manifestations, which may be mimicked by CRDD. Therefore, it may be prudent for clinicians to include CRDD in the list of differential diagnoses when examining skin lesions in patients with Crohn disease.

A case of cutaneous Rosai-Dorfman disease (CRDD) with underlying calvarial involvement and absence of BRAFV600E mutation

“…RDD limited only to the skin, referred to as “cutaneous” RDD, is a rare variant and involves a distinct demographic distribution. 1 , 3 Although systemic RDD is most common in Caucasian and African American men younger than 20 years, CRDD is usually found in Caucasian and Asian women in the fifth decade of life. 1 CRDD is especially rare in African Americans, as seen in our patient.…”

“…The lack of BRAF V600E mutation in our patient also further supports that RDD may represent a reactive, rather than neoplastic, condition, and is clinically significant as it illustrates no role for targeted therapy with BRAF/mitogen-activated protein kinase pathway inhibitors. Although the exact origin of RDD is unknown, it is postulated to reflect a hyperstimulation of humoral immunity, 2 , 3 , 14 which is evidenced by coexistence of RDD with autoimmune disease, hematologic malignancies, and postinfectious conditions. Histology plays an essential role in obtaining a definitive diagnosis.…”

“…Cutaneous lesions can occur in about 10% of patients, however, RDD limited only to cutaneous involvement is particularly rare. 2 , 3 Moreover, concomitant cutaneous RDD (CRDD) and bone RDD has rarely been reported in the English-language literature. 4 , 5 Here, we presented a case of CRDD on the scalp with underlying bony involvement.…”

An unusual case of multiple cutaneous Rosai‐Dorfman disease involving two separate parts of the body

“…3 It is called CRDD when only involving the skin. [5][6][7] The clinical manifestations of CRDD range from nodules, pustules, and papules to plaques and patches. [5][6][7] The clinical manifestations of CRDD range from nodules, pustules, and papules to plaques and patches.…”

“…4 The etiology and pathogenesis of RDD are unclear, maybe associated with virus infection or autoimmune disorders. [5][6][7] The clinical manifestations of CRDD range from nodules, pustules, and papules to plaques and patches. 8 Kong et al summarized three main types: papulonodular type (79.5%), indurated plaque type (12.8%), and tumor type (7.7%).…”