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Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school. Cases report. Hong Yu. Case 1 Red hemorrhagic papules with multiple organs involved. A 2-year-old boy hospitalized in Pediatric Department in our hospital was consulted because of skin rash
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Needle size of fresh colored papules over the patient’s head and face, with a few greasy scaly brown patches on the scalp
A few needle size red hemorrhagic papules on the trunk,
among them on the back there is one papule as large as a
hepatosplenomegaly, enlargement of lymph nodes of neck and groin areas;
WBC 2.45*109/L↓，HB 49 g/L↓，PLT 69*109/L↓；
Mononuclear and multinucleated histiocytes infiltrate densely in the dermis, with some inflammatory cells, such as lymphocytes, neutrophils and eosinophils. A few of the mononulcear cells were with reni-form nuclei, and multinucleated cells had ground-glass appearing cytoplasm.
both CD1a and S100 were positive
Both CD68 and PAS were positive
After chemotherapy, the skin lesions have been
improved, most of the papules over the head and
face were disappeared, however, the patient is
still in severe condition, with a high fever, anemia,
hypoproteinemia and hepatosplenomegaly, and he obviously has a bulging abdomen.
accompany with or not
Currently, the presence of Birbeck granules is assumed by immunohistochemical demonstration of langerin (CD207) .
Can not be explained by LCH.
multiple cutaneous nodules associated
with a destructive polyarthritis, which can be
seen in approximately 20% to 50% patients.
flesh or yellow-brown colored nodule
phenotype of RH is
identical to xanthogranuloma.
Large multinucleated giant cells with distinctive eosinophilic granular cytoplasm.
LCH with RH
initially on the forehead and spreading over the
rest of his face and scalp. At the same time, the
lesions of the trunk became more numerous.
A dense dermal infiltrate of foamy
histiocytes, among them Touton giant cells At the periphery of the biopsy specimen were
mononuclear histiocytes with
kidney-shaped nuclei in the papillary dermis
Histiocytes near the center of the
specimen was positive for CD68
Whereas the infiltrate at the periphery was
reactive for CD1a and S100
The patient’s general condition progressively deteriorated as he developed anemia, hepatosplenomegaly with hepatic
dysfunction, xanthochromia, and generalized lymphadenopathy.
The patient died from disseminated intravascular coagulopathy on day 12 of his hospital admission, having received no systemic therapy.
Langerhans cell histiocytosis
LCH overlapped JXG
In the last decade, there have been several reports of JXG developing from LCH. Six children with LCH developed JXG within several months or years after having received systemic or topical therapy.
In addition, there is a 2-year-old boy with Juvenile multiple xanthogranuloma on his skin and Langerhans cell histiocytosis on his tibia.
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