A RARE CASE OF ROSAI DORFMAN DESTOMBES DISEASE PRESENTING WITH LUPUS NEPHRITIS AND CNS SMALL VESSEL VASCULITIS

Introduction:

Rosai-Dorfman-Destombes (RDD) disease is a rare non-Langerhans cell histiocytosis characterized by tissue accumulation of histiocytes commonly seen in children and young adults. RDD can occur as an isolated disorder or in association with autoimmune and malignant diseases. Steroids, immunomodulators and surgical excision can be used in management of RDD.

Methods:

15-year-old male developed left submandibular swelling in November, 2022. USG Neck (outside) showed enlarged lymph nodes in left submandibular region. He later developed similar swellings in right submandibular area and bilateral inguinal area. In January, 2024, he developed facial puffiness followed by bilateral lower limb swelling. He had altered behaviour and irrelevant talks from February, 2024. He presented to Nephrology OPD AIIMS, Rishikesh on 26/2/2024. Urine dipstick showed protein 4+ and blood 2+. 24-hour urine protein was 6.51 gm. He was diagnosed as a case of Nephrito-Nephrotic syndrome. Contrast MRI brain showed patchy areas of multiple hyperintensities in bilateral frontal and parietal lobes with diffusion restriction suggestive of small vessel vasculitis. He had CNS vasculitis due to lupus. USG neck showed bilateral enlarged cervical, submandibular, and inguinal lymph nodes. FNAC of cervical lymph node showed polymorphous population of lymphoid cells showing emperipolesis with predominant histiocytes suggestive of Rosai-Dorfman destombes syndrome. ANA IFA was 3+ positive with end titers of 1:1000 with low c3 and c4. He was started on pulse steroids followed by oral steroids for possible lupus nephritis & CNS lupus vasculitis. Sensorium improved. Renal biopsy report was suggestive of Diffuse proliferative lupus nephritis (Class IV Lupus Nephritis). Lymph nodes size decreased within 2 weeks of start of steroids. 

Results:

Histopathology of Lymph node in our case did not show evidence of RS cells found in Hodgkin’s lymphoma. CB NAAT for AFB was negative. There were no granulomas with caseating necrosis in FNAC. FNAC showed emperipolesis with predominant histiocytes suggestive of RDD. In our case, patient presented as Nephrito-Nephrotic syndrome and had CNS small vessel vasculitis due to lupus without any dermatological, musculoskeletal, or other systemic manifestations which is rare. There was resolution of lymphadenopathy with steroids within 2 weeks of start of therapy. Hence, steroids can play an important role in management of RDD presenting with lymphadenopathy.

Conclusions:

SLE can present as Nephrito-Nephrotic syndrome and CNS small vessel vasculitis only without any other systemic involvement. Steroids can be helpful in management of lymphadenopathy in RDD. We should keep possibility of RDD when there is generalized lymphadenopathy in patients of SLE although the association is rare. 

I have no potential conflict of interest to disclose.

I did not use generative AI and AI-assisted technologies in the writing process.