dermatology department of xinhua hospital shanghai jiaotong university, medical school hong yu cases...
TRANSCRIPT
![Page 1: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/1.jpg)
Dermatology Department of Xinhua HospitalShanghai Jiaotong University, Medical school
Hong Yu
Cases report
![Page 2: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/2.jpg)
Case 1
Red hemorrhagic papules with multiple organs involved
![Page 3: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/3.jpg)
A 2-year-old boy hospitalized in Pediatric Department in our hospital was consulted because of skin rash
The lesions of the skin: 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 millet
![Page 4: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/4.jpg)
History
The patient was admitted for recurrent fever in 4 months on 09/09/17
Physical examination
hepatosplenomegaly, enlargement of lymph nodes of neck and groin areas;
![Page 5: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/5.jpg)
Laboratory examination: Blood routing test:
WBC 2.45*109/L↓ , HB 49 g/L↓ , PLT 69*109/L↓ ;
Albumin ↓ No abnormal detection for ANA , HBsAg ,
HCV, HIV ;
![Page 6: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/6.jpg)
Bone marrow smear suspected the diagnosis of Langerhans cell histiocytosis
No microorganism evidence in blood and cerebrospinal fluid
B Ultrasound :hepatosplenomegaly , enlargement of lymph nodes of mesentery
No abnormal findings of skull by CT and MRI , No abnormal detection of chest by X-ray;
![Page 7: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/7.jpg)
Histiopathology examination
Skin biopsy revealed : 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.
![Page 8: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/8.jpg)
![Page 9: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/9.jpg)
![Page 10: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/10.jpg)
![Page 11: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/11.jpg)
![Page 12: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/12.jpg)
![Page 13: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/13.jpg)
![Page 14: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/14.jpg)
![Page 15: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/15.jpg)
![Page 16: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/16.jpg)
Immunochemical stain:
All the histiocytes: VIM (+)
some mononuclear histiocytes: both CD1a and S100 were positive Some mono and all the multinucleated cells: Both CD68 and PAS were positive
![Page 17: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/17.jpg)
![Page 18: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/18.jpg)
![Page 19: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/19.jpg)
![Page 20: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/20.jpg)
![Page 21: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/21.jpg)
![Page 22: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/22.jpg)
![Page 23: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/23.jpg)
![Page 24: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/24.jpg)
![Page 25: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/25.jpg)
![Page 26: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/26.jpg)
![Page 27: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/27.jpg)
![Page 28: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/28.jpg)
Prognosis
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.
![Page 29: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/29.jpg)
![Page 30: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/30.jpg)
![Page 31: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/31.jpg)
Langerhans cell histiocytosis(LCH) accompany with or not
multicentric reticulohistiocytosis(MH)/
reticulohistiocytoma(RH)
What is the diagnosis?
![Page 32: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/32.jpg)
Diagnosis of LCH is based on classical clinical findings and histologic/immunohistochemical
criteria. Previously absolute criteria for diagnosis
depended on finding CD1a or Birbeck granules. Currently, the presence of Birbeck granules is
assumed by immunohistochemical demonstration of langerin (CD207) .
Positivity of one or both of these markers now defines the langerhans cell phenotype.
![Page 33: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/33.jpg)
LCH is a granulomatous lesion containing Langerhans cells as well as normal inflammatory cells and multinucleated giant cells (MGC). The MGC were recently reported to be osteoclast-like and able to produce cytokines that can cause osteolysis; and in bone lesions, the osteoclast-like MGCs were only CD68(+), in the nonostotic sites, they coexpressed CD1a.
Then, the multinucleated cells in our case Can not be explained by LCH.
![Page 34: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/34.jpg)
Multicentric reticulohistiocytosis(MH) multiple cutaneous nodules associated with a destructive polyarthritis, which can be seen in approximately 20% to 50% patients.
Reticulohistiocytoma(RH) flesh or yellow-brown colored nodule
![Page 35: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/35.jpg)
Both of them have similar histological features, but the immunochemical profiles are different as the table; and the
phenotype of RH is identical to
xanthogranuloma.
MH RH
CD68 + +
HAM56 + +
S100 - -
FxⅢ - +
HHF35 - +
Large multinucleated giant cells with distinctive eosinophilic granular cytoplasm.
![Page 36: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/36.jpg)
The preferred diagnosis
LCH with RH
![Page 37: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/37.jpg)
![Page 38: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/38.jpg)
Cases 2 yellow papules with multi-organs involved
![Page 39: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/39.jpg)
A 15-month-old boy was hospitalized in our department with a working diagnosis of LCH .
History hemorrhagic macules and papules on his chest and
abdomen since 8-month-old accompanied by fever At 12 months of age, he developed yellow papules,
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.
![Page 40: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/40.jpg)
![Page 41: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/41.jpg)
Skin biopsy revealed:
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
![Page 42: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/42.jpg)
![Page 43: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/43.jpg)
Immunohistochemical staining :
Histiocytes near the center of the
specimen was positive for CD68
Whereas the infiltrate at the periphery was
reactive for CD1a and S100
![Page 44: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/44.jpg)
![Page 45: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/45.jpg)
![Page 46: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/46.jpg)
The patient’s general condition progressively deteriorated as he developed anemia, hepatosplenomegaly with hepaticdysfunction, xanthochromia, and generalized lymphadenopathy.
The patient died from disseminated intravascular coagulopathy on day 12 of his hospital admission, having received no systemic therapy.
![Page 47: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/47.jpg)
What is the diagnosis:
Langerhans cell histiocytosis
or Juvenile xanthogranuloma or
both mixed / overlapped
![Page 48: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/48.jpg)
Prefer to think:
LCH overlapped JXG
![Page 49: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/49.jpg)
![Page 50: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/50.jpg)
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.
![Page 51: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/51.jpg)
Some authors thought there is a cell lineage relationship between RH and adult xanthogranuloma, both of them are class a subtypes of non-LCH, and Rosai-Ⅱ
Dorfman disease belongs to class b Ⅱsubtypes of non-LCH.
![Page 52: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/52.jpg)
However, there has been a report about coexistence of LCH and cutaneous Rosai-Dorfman disease in a Chinese child.
To me, many quandaries are yet to be resolved in regard to this seeming overlap of histiocytic disorders.
![Page 53: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/53.jpg)
Refernces 1. Weitzman S, Egeler RM.Langerhans cell histiocytosis: update for the pediatrician. Curr Opin Pediatr. 2008 Feb;20(1):23-9. 2. Dermnet NZ Non-Langerhans cell histiocytosis http://www.dermnetnz.org/dermal-infiltrative/non-langerhans.html 2007 3.da Costa CE; Annels NE; Faaij CM; Forsyth RG ; Hogendoorn PC ; Egeler RM Presence of osteoclast-like multinucleated giant cells in the bone and nonostotic lesions of Langerhans cell histiocytosis. Journal of Experimental Medicine 2005:201(no.5) 4.Hoeger PH, Diaz C, Malone M, et al. Juvenile xanthogranuloma as a sequel to Langerhans cell histiocytosis: a report of three cases. Clin Exp Dermatol 2001;26:391-4.
![Page 54: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/54.jpg)
5. Patrizi A, Neri I, Bianchi F, et al. Langerhans cell histiocytosis and juvenile xanthogranuloma: two case reports. Dermatology 2004;209:57-61 6.Pe´rez-Gala S, Torrelo A, Colmenero I, et al. Juvenile multiple xanthogranuloma in a patient with Langerhans cell histiocytosis. Actas Dermo-Sif 2006;97:594-8. . 7. Shani-Adir A, Chou P, Morgan E, et al. A child with both Langerhans and non-Langerhans cell histiocytosis. Pediatr Dermatol 2002;19:419-22. 8.Kong YY, Kong JC, Shi DR. Cutaneous Rosai-Dorfman disease: a clinical and histopathologic study of 25 cases in China. Am J Surg Pathol 2007;31:341-50.
![Page 55: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/55.jpg)
9.Zelger B, Cerio R, Soyer HP, Misch K, Orchard G,
Wilson-Jones E Reticulohistiocytoma and multicentric
reticulohistiocytosis. Histopathologic and immunophenotypic distinct entities. Am J Dermatopathol. 1994 Dec;16(6):577-84.
10. Barnhill RL, Neil Crowson A, Busam KJ, Granter SR
textbook of dermatopathology, McGraw-hill health
professions division, 1997:104-5
11. Hong Yu, Jinchen Kong, Yan Gu , Bo Ling, Zhengjun Xi, Zhirong Yao.A child with coexistent juvenile xanthogranuloma and Langerhans’ cell histiocytosis. Journal of American Academy of Dermatology vol.62 No.2,2010:329-332
![Page 56: Dermatology Department of Xinhua Hospital Shanghai Jiaotong University, Medical school Hong Yu Cases report](https://reader030.vdocuments.net/reader030/viewer/2022033015/56649ec15503460f94bcd3d3/html5/thumbnails/56.jpg)