Today I met a 70+ year-old African-American woman with a 3 month history of asymptomatic, moderately firm dusky red papulonodular lesions of the right neck. No lymphadenopathy was appreciated. Her medical history was remarkable for hypertension and diabetes, treated with valsartan, insulin, and metformin. My clinical differential included cutaneous metastases, lymphoma cutis, or doubtfully an infectious process (such as actinomycosis). She said that she was biopsied by another dermatologist – the diagnosis was Rosai-Dorfman Disease (RDD) based on histiocytes staining positively for S-100 and CD68, and negatively for CD1a.
The latest review of cutaneous RDD by Al-Khateeb (1) notes the following:
The literature search yielded 578 published cases of C-RDD affecting any skin area; of these, 65 cases (11.2%) had facial skin involvement. The male-to-female ratio was 1:1.5, and the average age at presentation was 43.5 years (standard deviation, 12.4 yr). The racial distribution of facial C-RDD was, in descending order, 74.5% in Asians, 20% in Caucasians, and 5.5% in blacks. The most commonly affected facial skin regions were the cheeks and periorbital area, and most lesions were multiple in number and bilaterally distributed. The vast majority of facial C-RDD lesions presented as asymptomatic, nonulcerative, red, nodular plaques with durations ranging from 1 month to a few years. Many methods have been attempted for the treatment of facial C-RDD. However, the combined cure rate for all published treatment methods was only 28.6%. Surgical excision was the most effective treatment method, and corticosteroids were the least effective.
The clinical course is unpredictable, ranging from spontaneous regression to fatalities, regardless of therapy. Typically, progression of the disease is slow. Therapeutic modalities have included surgical excision, radiation, steroids (systemic, topical, intralesional), and various combinations of chemotherapy (2), including methotrexate. Other modalities have included dapsone, thalidomide, and isotretinoin (3).
The etiology of RDD is unknown, currently considered as a reactive immunodysregulation, possibly secondary to viral infections such as Epstein-Barr or Human Herpes Virus 6. The prognosis of cutaneous RDD is reasonably good; however, it may be associated with other disorders, including bilateral uveitis, antinuclear antibody positive lupus erythematosus, rheumatoid arthritis, hypothyroidism, lymphoma and HIV infection (4).
I have been reading about cutaneous RDD for decades, and have seen a case or two at regional conferences; now being confronted with a RDD patient, I remain as uncertain as ever about its etiology, appropriate work-up, and treatment. I am approaching this diagnosis with caution. Although she might have cutaneous RDD, I think it would be best consider the cutaneous form of RDD a diagnosis of exclusion. I have begun a rudimentary work-up with a CBC, LFTs, chemistry profile, SPEP, and chest X-ray, and opted to refer her to hematology-oncology to rule out any systemic involvement, possibly by PET-CT. I prescribed topical clobetasol, pending further studies.
Am I being overly cautious?
- Al-Khateeb TH. Cutaneous Rosai-Dorfman disease of the face: A comprehensive literature review and case report. J Oral Maxillofac Surg 2016; 74: 528-40.
- Maia RC, et al. Rosai-Dorfman disease: A report of eight cases in a tertiary care center and a review of the literature. Braz J Med Biol Res 2015; 48: 6-12.
- Sun NZ, et al. Cutaneous Rosai-Dorfman disease successfully treated with low-dose methotrexate. JAMA Dermatol 2014; 150: : 787-8.
- Fang S, Chen AJ. Facial cutaneous Rosai-Dorfman disease: A case report and literature review. Exp Ther Med 2015; 9: 1389-92.