Cytophagic histiocytic panniculitis: is it a macrophage activation syndrome in situ?
نویسندگان
چکیده
منابع مشابه
Cytophagic histiocytic panniculitis: is it a macrophage activation syndrome in situ?
Patients A 10-month-old year boy and a 4-month-old-year girl presented with multiple erythematous and indurated noduless on the limbs, and developed high-grade fever and biological features suggestive of MAS several months after the onset of cutaneous disease. Serologic test for EBV was negative and bone marrow examination revealed hemophagocytosis. A homozygous splicing mutation for the UNC13D...
متن کاملHistiocytic cytophagic panniculitis.
A 33 year old man developed fever, malaise, jaundice, pancytopenia, coagulation abnormalities, hepatomegaly, pleural effusions and a subcutaneous lump. Biopsies revealed a lobular panniculitis with the presence of cytophagic histiocytes; erythrophagocytosis was also demonstrated in the liver and bone marrow. Despite the use of chemotherapy (CHOP) his clinical condition deteriorated and he died ...
متن کامل[Histiocytic cytophagic panniculitis].
UNLABELLED A HISTOPATHOLOGICAL DIAGNOSIS: Histiocytic cytophagic panniculitis is the specific skin finding in the reactive hemophagocytic syndrome. It is a feverish nodular eruption which accompanies the other clinical and biological features of the reactive hemophagocytic syndrome. Histopathologic changes are diagnostic and consist in a lobular panniculitis characterized by the presence of a h...
متن کاملCytophagic histiocytic panniculitis in systemic lupus erythematosus.
This paper presents a case of cytophagic histiocytic panniculitis in a Japanese woman, who had systemic lupus erythematosus complicated with Hashimoto's thyroiditis and lupus nephritis from the age of 12. The patient had painful multiple purplish subcutaneous nodules on the face, trunk and extremities, high fever and liver dysfunction without coagulopathy. The histological features of the skin ...
متن کاملCytophagic histiocytic panniculitis: A diagnostic challenge
Cytophagic histiocytic panniculitis (CHP) was first described in 1980. It classically presents with intermittent recurrent episodes of pyrexia subcutaneous nodules, pancytopenia and liver dysfunction, but most patients have in addition a multitude of other manifestations which confuse the clinical picture. Despite the variable clinical course, the disease is often fatal. Diagnosis is based on h...
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ژورنال
عنوان ژورنال: Pediatric Rheumatology
سال: 2011
ISSN: 1546-0096
DOI: 10.1186/1546-0096-9-s1-p308