Cutaneous Rosai-Dorfman disease (CRDD) is a rare non-Langerhans cell histiocytosis that is limited to the skin. Unlike its separate systemic counterpart, Rosai-Dorfman disease (RDD; sinus histiocytosis with massive lymphadenopathy), CRDD occurs without lymph node or internal involvement. RDD may display cutaneous lesions identical to those of CRDD in 10% of cases.

The median age of onset for CRDD is 40 years, and it displays a 2:1 predilection for females. Individuals of Asian and Northern European descent are also more commonly affected.

Patients with CRDD generally appear well and lack constitutional symptoms. They may have minor cutaneous symptoms (pain, ulceration, tenderness) associated with localized growth but are generally asymptomatic. The etiology is thought to be an exaggerated immunologic reaction triggered by infectious or autoimmune stimuli. CRDD has been described in the presence of human herpesvirus 6, Epstein-Barr virus, parvovirus B19, and human immunodeficiency virus (HIV), as well as post-vaccination. Polyclonal infiltration further supports the notion of a reactive versus a neoplastic process.

CRDD is a benign and self-limited disease. In case series and reviews, the disease does not progress to systemic involvement. The time to resolution is variable. In a review of 65 cases occurring on the face, 37.5% resolved spontaneously after an average 10-month observation period.