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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sinus Histiocytosis with massive lymphadenopathy is an idiopathic, histologically distinctive disorder that characteristically produces painless cervical lymphadenopathy. Other node groups are frequently involved and mass lesions in extranodal sites occur in approximately 30% of patients. In most cases the disease is chronic and the course is often characterized by exacerbations and remission. We report the case of a 66 years old white woman affected by sinus histiocytosis with massive cervical and inguinal lymphadenopathy. Subcutaneous nodules appeared in her left arm and low back region with the histological features of sinus histiocytosis. Cellular infiltrate was composed predominantly of histiocytes aggregating in granuloma like clusters. These cells had large round or kidney shaped nuclei; cytoplasm was abundant, vacuolated, often containing phagocytized lymphocytes. Multinucleated cells were also observed; reactive plasmocytosis was present in the nodule periphery. During the follow-up the patient maintained good physical conditions, despite a moderate weight loss. The size of her nodules regressed within one year. Immunologic alterations, consisting of polyclonal hypergammaglobulinemia and persistent inversion of T4/T8 lymphocyte ratio, were observed. Sinus histiocytosis usually offers a good prognosis; in our patient infective and/or autoimmune complications could be favoured by advanced age, extranodal involvement and immunological depression.
G Ital Dermatol Venereol 1989 Apr
PMID:[Skin involvement in Rosai-Dorfman disease. Description of a case]. 280 94

A 72-year-old woman presented with the sudden onset of multiple, large, firm, subcutaneous nodules in her right arm and left thigh. Biopsy of the lesions revealed a septal and lobular panniculitis of unknown cause. The lesions regressed spontaneously without therapy over a period of a few weeks. A follow-up review of the microscopic slides from the biopsy disclosed a lymphohistiocytic and plasma cell proliferation with lymphophagocytosis by histiocytes, the characteristic morphological features of sinus histiocytosis with massive lymphadenopathy. This diagnosis was further supported by the immunocytochemical demonstration of S100 protein reactivity within the histiocytic cells. A seven-year follow-up has failed to reveal any evidence of recurrence or progression of the disease. Sinus histiocytosis with massive lymphadenopathy should be entertained in the differential diagnosis of cutaneous lesions presenting with septal and lobular panniculitis.
Arch Dermatol 1988 Aug
PMID:Histiocytic lymphophagocytic panniculitis. An unusual extranodal presentation of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). 340 Oct 30

Sinus histiocytosis with massive lymphadenopathy is a rare clinicopathologic entity that affects mainly children and young adults. Although the lymph nodes are the most common sites of involvement, extranodal infiltrates may arise in the orbits, skin, upper respiratory tract, and bone, as well as other organs. Approximately 10% of patients have had cutaneous infiltrates, but very few of them have had lesions limited to the skin. We treated a 15-year-old female with cutaneous nodules as the sole manifestation of the disease.
Pediatr Dermatol 1987 Nov
PMID:Sinus histiocytosis clinically limited to the skin. 342 58

Cutaneous lesions of Rosai-Dorfman disease (RDD) are usually associated with nodal or other extranodal localization. We describe a female patient with RDD clinically limited to the skin. The patient presented with asymptomatic red-brown papules and nodules on the legs, arms, back, and nose. Histologically, the lesions consisted of a proliferation of large histiocytes occasionally showing emperipolesis. Histiocytes were also observed within dilated lymphatic vessels. Immunohistochemical study showed that histiocytes expressed S-100 protein and both macrophage and monocyte markers. All lesions resolved completely with Roentgen therapy. No recurrence has been observed over a 3-year follow-up period.
Br J Dermatol 1996 Apr
PMID:Purely cutaneous Rosai--Dorfman disease. 873 85

Several types of cutaneous lesions have previously been described at the sites of herpes zoster scars. We describe 16 patients with cutaneous lesions which had developed on herpes zoster scars. Biopsies were taken from these lesions, and a polymerase chain reaction assay was used to detect the viral genome in paraffin-embedded specimens. Histopathological findings enabled diagnosis of nonspecific granulomatous dermatitis in five patients, granulomatous vasculitis in two patients, lichen sclerosus in two patients, and pseudolymphoma, keloid, sarcoidal granuloma, granuloma annulare, granulomatous folliculitis, lichen planus and cutaneous Rosai-Dorfman disease, each in one patient. Varicella-zoster virus DNA was not identified in any of the patients. Granulomatous folliculitis, lichen sclerosus and cutaneous Rosai-Dorfman disease have not previously been described in herpes zoster scars, but they are three new cutaneous reaction patterns that may have developed within these scars. Our investigations indicate that the cutaneous reactions appearing in herpes zoster scars are not due to the persistence of varicella-zoster virus DNA within the lesions.
Br J Dermatol 1998 Jan
PMID:Cutaneous reactions at sites of herpes zoster scars: an expanded spectrum. 953 41

We report a patient with purely cutaneous Rosai-Dorfman disease (RDD) who presented with a solitary, asymptomatic plaque on the back of her left thigh, with characteristic, large histiocytoid cells exhibiting emperipolesis histologically. Cutaneous lesions occur in 27% of patients with lymph node involvement in RDD however purely cutaneous disease has only been reported on 18 previous occasions. The aetiology is unknown, although it is though to be a reactive disorder rather than neoplastic, possibly an immunological response to an infectious agent.
Clin Exp Dermatol 1998 Jan
PMID:Cutaneous Rosai-Dorfman disease. 966 10

We report 2 cases of cutaneous Rosai-Dorfman disease (CRD) presenting with pustular and acneiform lesions. To our knowledge, these are the first reports of CRD presenting in this pattern. One patient had spontaneous resolution of the lesions, whereas the other was treated with cryotherapy with clinical improvement. We present a review of the literature regarding CRD and highlight the benign nature of this condition.
J Am Acad Dermatol 1999 Aug
PMID:Cutaneous Rosai-Dorfman disease presenting as pustular and acneiform lesions. 1042 25

Sinus histiocytosis with massive lymphadenitis or Rosai-Dorfman disease (RDD) is a rare, benign, proliferative histiocytic disease of unknown origin. It predominately affects the lymph nodes The skin is the extranodal organ most frequently involved, although a few patients present with only lesions of the skin and soft tissues, without involvement of the lymph nodes (purely cutaneous RDD). Ocular involvement is rare, and generally localized to the eyelid. We report an unusual case of cutaneous sinus histiocytosis without nodal involvement and with bilateral uveitis in a girl. As far as we know, this is the first case reported in which cutaneous and ocular RDD occurred without nodal involvement.
Pediatr Dermatol
PMID:Cutaneous sinus histiocytosis and chronic uveitis. 1108 66

We report a patient with sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) who presented with widespread nodal and extranodal involvement affecting the skin, orbits and nasal sinuses, complicated by the development of autoimmune haemolytic anaemia. The aetiology and pathogenesis of this multisystem disorder are unknown but are thought to represent a reactive histiocytic process to an infective agent rather than a neoplastic or other primary condition. Prognosis is generally good but clinical or laboratory evidence of immune dysfunction tends to predict a poorer outcome. We describe the clinical course of the patient and review the literature on this disease.
Br J Dermatol 2001 Aug
PMID:Rosai-Dorfman disease complicated by autoimmune haemolytic anaemia: case report and review of a multisystem disease with cutaneous infiltrates. 1153 2

The cutaneous variant of Rosai-Dorfman disease is a rare benign lymphoproliferative disorder that occurs without lymphadenopathy or internal organ involvement. We present a 55-year-old woman with a diffuse exanthematous eruption that evolved into pruritic papulopetechial and purpuric lesions on the back and lower extremities, diagnosed clinically as a vasculitis. The patient's lesions cleared spontaneously with no recurrence. Histopathologic examination showed a dense dermal nodular lymphohistiocytic infiltrate with occasional eosinophils, plasma cells, and neutrophils and with numerous extravasated red blood cells. The large, foamy histiocytes exhibited abundant pale cytoplasm, feathery cytoplasmic borders, and emperipolesis. A few histiocytes had longitudinal nuclear grooves resembling Langerhans cells; however, these cells were positive for S-100 protein and negative for CD1a. The clinical presentation of the cutaneous variant of Rosai-Dorfman disease is generally nonspecific. We present this case because of the unusual clinical presentation mimicking a vasculitis.
J Am Acad Dermatol 2002 May
PMID:Cutaneous sinus histiocytosis (Rosai-Dorfman disease) presenting clinically as vasculitis. 1200 23


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