Cutaneous Rosai - Dorfman disease in a patient with late syphilis and cervical cancer - case report and a review of literature.

BACKGROUND: Cutaneous Rosai - Dorfman disease (CRDD) is extremely rare variant of idiopathic histiocytic proliferative disorder, which may manifest as a non-specific macules, papules, plaques or nodules ranging in size and colour from yellow - red to red -brown. CASE PRESENTATION: A 52-year-old female presented with three gradually enlarging, reddish - brown nodules on the right upper extremity lasting six months. The patients denied fever, weight loss, malaise. Clinical examination and imaging tests showed no sign of lymphadenopathy. A biopsy specimen of a nodule showed a dense dermal polymorphic infiltrate with numerous histiocytes exhibiting emperipolesis phenomenon. Immunohistochemical staining of the histiocytes showed S-100 protein (+), CD68(+), but CD1a (-). Aforementioned findings were consistent with CRDD characteristics. Additionally, a routine serological screening and confirmatory serological tests for syphilis were positive. Syphilis of unknown duration was diagnosed. The IgG antibodies titre against Chlamydia trachomatis was elevated. An isolated sensory impairment over the right trigeminal nerve was found on neurological consultation. Comprehensive gynaecological assessment was carried out because of patient's complaints of bleeding after sexual intercourse and led to diagnosis of cervical cancer. The initial therapy with methotrexate was discontinued after three months due to neutropenia. Further therapy with dapson was ineffective, therefore complete surgical excision was recommended. CONCLUSIONS: CRDD is a rare, benign condition especially difficult to diagnose due to lack of general symptoms and lymphadenopathy. Histopathologic examination with immunohistochemical staining, exhibiting characteristic and reproducible findings play a key role in establishing an accurate diagnosis. In the presented case activated histiocytes demonstrated in a lesional skin might be a response to immune dysregulation related to chronic, untreated sexually transmitted infections and cancer.

Histiocitosis intralinfática, a propósito de 2 casos / Intralymphatic Histiocytosis: A Report of 2 Cases

La histiocitosis intralinfática es un proceso benigno caracterizado por placas eritematosas mal delimitadas, a veces reticuladas, otras con nódulos o vesículas en su interior. Su etiopatogenia parece estar relacionada con procesos inflamatorios crónicos en el área afectada, cirugía previa o con enfermedades sistémicas, sobre todo con la artritis reumatoide. Presentamos 2 nuevos casos, ambos asociados a cirugía articular del área afecta y enfermedad osteoarticular (osteocondromatosis sinovial primaria y artritis reumatoide). Esta entidad tiene un comportamiento crónico y no existe un tratamiento específico. En los 2 casos presentados se plantearon distintas opciones terapéuticas, mostrando uno de ellos una respuesta espectacular al tratamiento con pentoxifilina oral junto con tacrolimus tópico

Intralymphatic histiocytosis is a benign condition characterized by poorly defined erythematous plaques (sometimes forming a reticular pattern) as well as the presence of nodules and vesicles. Its etiology and pathogenesis appear to be related to chronic inflammation in the affected area, prior surgery, or systemic disease, particularly rheumatoid arthritis. We report on 2 new cases, both associated with joint surgery in the affected area and osteoarticular disease (primary synovial osteochondromatosis and rheumatoid arthritis). This is a chronic disease and there is no specific treatment. Different treatment options were chosen in the 2 cases described. A spectacular response to treatment with oral pentoxifylline and topical tacrolimus was observed in 1 of the patients

A Singular Case of Intracranial Sinus Histiocytosis without Massive Lymphadenopathy: Isolated Rosai-Dorfman Disease of the Hypothalamus.

BACKGROUND: Sinus histiocytosis with massive lymphadenopathy (also known as Rosai-Dorfman disease [RDD]) is a benign but chronic cervical lymphadenopathy associated with systemic inflammation. Although extranodal manifestations of RDD have been described, isolated central nervous system (CNS) involvement is exceedingly rare. CASE DESCRIPTION: We present the case of a 66-year-old woman who presented with 3 weeks of intermittent headaches, diplopia, and increasing confusion who was found on work-up to have isolated hypothalamic RDD, evidenced by a dense admixture of large histiocytic cells admixed with numerous small mature lymphocytes and some scattered plasma cells and neutrophils on stereotactic brain biopsy. Over 19 months of follow-up, neurologic examination continues to reveal stable bilateral partial abducens nerve palsies without diplopia, and a new gradual onset short-term memory loss. Interim treatment for the histiocytic lesion consisted of 10 cycles of external-beam radiation therapy along with high-dose steroids. The patient currently experiences minimal functional loss from treatment of her intracranial sinus histiocytosis, with a Karnofsky performance status of 80, and she remains without any disease involvement outside of the CNS. CONCLUSION: Because misdiagnosis of a hypothalamic contrast-enhancing lesion could potentially lead to therapeutic mismanagement and poor outcomes, it is important to consider RDD in the differential diagnosis.