Destombes–Rosai–Dorfman disease of the anterior abdominal wall: Extranodal sinus histiocytosis with massive lymphadenopathy

estombes–Rosai–Dorfman disease—often simply referred to, in the literature, as Rosai–Dorfman disease (RDD)—is a rare, nonmalignant disorder of histiocyte proliferation typically involving the cervical lymph nodes. A subset of patients with RDD, however, display extranodal manifestations highly variable in presentation, more challenging to diagnose, and less likely to spontaneously regress when compared to the nodal manifestations. This study describes the case of a young African male presenting himself with multiple nodules involving the anterior abdominal wall, who was found to have extranodal RDD. The current mode of diagnosis and the clinical management of RDD are reviewed.

Kikuchi-Fujimoto disease with multiple extra-nodal features - A clinical mimic

Extranodal involvement in Kikuchi’s disease is uncommon. A 31-year-old previously healthy Indian woman was admitted with high grade fever, multiple joint pain and skin rash for 3 weeks. She had negative anti-nuclear antibodies and had features of Kikuchi’s disease on lymph node biopsy. She also had multiple extranodal manifestations including erythematous maculopapular rash, symmetric polyarthritis and hepatosplenomegaly. Kikuchi’s disease with extranodal involvement can clinically mimic diseases like hematological malignancies, connective tissue disorders and certain infections. A lymph node biopsy plays a crucial role in making an accurate diagnosis by excluding other diseases. A discussion on the importance of differentiating Kikuchi’s disease from systemic lupus erythematosus is included.

Purely Cutaneous Rosai-Dorfman Disease: a True Clinical Diagnosis Challenge

Rosai-Dorfman disease (RDD) is a rare idiopathic benign disease, self-limited non-Langerhans cell histiocytosis, most frequently presented as a massive bilateral and painless lymphadenopathy, associated with fever and weight loss. Extranodal manifestations of RDD represents a true diagnosis challenge for clinicians and surgical pathologists. A 64-year-old female, known with arterial hypertension and type 2 diabetes, presented to our Surgery Clinic for a left arm painless cutaneous tumour, having its onset one year before, and rapidly enlarged in the last two months. Physical examination revealed a firm and elastic cutaneous nodular lesion of 4 x 2 cm in size, prominent to the skin, with central ulceration area of 10 x 5 mm, located on the posterior side of the left arm. Surgery was performed, with lymphoma as a differential diagnosis at intraoperative extemporaneous examination. The RDD disease diagnosis was considered at histopathological analysis and confirmed by immunohistochemistry. Herein, we describe a rare case of purely cutaneous RDD presenting as unique cutaneous ulcerative nodular lesion, surgical treated only, and without local relapse after one-year follow-up.

Extranodal Rosai–Dorfman Disease Presenting as a Mediastinal Mass with Pulmonary Artery Invasion

Rosai–Dorfman disease (RDD) is a rare, nonmalignant disorder of histiocyte proliferation typically involving the cervical lymph nodes. However, a subset of patients with RDD will display extranodal manifestations that are highly variable in presentation, more challenging to diagnose, and less likely to spontaneously regress compared to nodal disease. While case reports of extranodal involvement in nearly every organ system exist, documented instances of mediastinal and pulmonary artery involvement are particularly rare. This study describes the case of a middle-aged woman presenting with new onset right heart failure who was found to have extranodal RDD in the form of a large mediastinal mass with invasion and occlusion of the main pulmonary arteries.

Rosai-Dorfman Disease: A Rare Cause of Cervical Lymphadenopathy

ABSTRACT Rosai-Dorfman disease is also known as sinus histiocytosis with massive lymphadenopathy (SHML) is a rare clinico-pathological condition. It is a benign condition which causes significant cervical lymphadenopathy in children and young adults. The clinical presentation varies from isolated nodal involvement to significant extranodal manifestations. The clinical features are usually mild, but rarely life-threatening complications can occur in some individuals depending on the site of involvement. Here, we present two cases of Rosai-Dorfman disease, both diagnosed on fine needle aspiration cytology (FNAC) and histopathology and responded well to steroids. One of the patients had extranodal site involvement in the form of bilateral nasal mass which is very rare. How to cite this article Ekhar VR, Shelkar RN, Rane S, Anand A, Lanjewar K, Jain SKT. Rosai-Dorfman Disease: A Rare Cause of Cervical Lymphadenopathy. Int J Head Neck Surg 2014;5(3): 152-154.

Rosai-Dorfman disease presenting with extensive cutaneous manifestation - Case report

Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia. The skin is the most common site affected. Extranodal manifestations have been reported in 43% of cases. In this study, we report an atypical case of Rosai-Dorfman disease in a female with massive cutaneous manifestation on the thigh, associated with a minimal lymphadenopathy limited to the regional inguinal lymph nodes.

Rosai-Dorfman Disease Presenting as an Isolated Extranodal Mass of the Carotid Sheath: A Case Report

Rosai-Dorfman disease is a rare, benign granulomatous disease that typically presents with massive cervical lymphadenopathy. In less than 50% of cases, other soft-tissue manifestations may also be found in the head and neck. Rosai-Dorfman disease can be difficult to diagnose because of its rarity and its ability to mimic, both clinically and radiologically, more common diseases such as lymphoma. The histopathologic diagnosis can also be difficult to make, particularly when the disease exhibits extranodal manifestations. We present a case of isolated extranodal Rosai-Dorfman disease involving the carotid sheath, without the typical massive adenopathy.