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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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dermatology department of xinhua hospital shanghai jiaotong university medical school

Dermatology Department of Xinhua HospitalShanghai Jiaotong University, Medical school

Cases report

Hong Yu

slide2
Case 1
  • Red hemorrhagic papules with multiple organs involved
slide3
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

history
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;

laboratory examination
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;
slide6
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;
histiopathology examination
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.

immunochemical stain
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

slide28
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.

slide31
Langerhans cell histiocytosis(LCH)

accompany with or not

multicentric reticulohistiocytosis(MH)

/

reticulohistiocytoma(RH)

  • What is the diagnosis?
slide32
Diagnosis of LCHis 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.
slide33
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.

slide34
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

slide35
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.

Large multinucleated giant cells with distinctive eosinophilic granular cytoplasm.

slide38
Cases 2
  • yellow papules with multi-organs involved
slide39
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.

slide41
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

slide43
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

slide46

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.

slide47
What is the diagnosis:

Langerhans cell histiocytosis

or

Juvenile xanthogranuloma

or

bothmixed /overlapped

slide48
Prefer to think:

LCH overlapped JXG

slide50

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.

slide51
Some authors thought there is a cell lineage relationship between RH and adult xanthogranuloma, both of them are class Ⅱa subtypes of non-LCH, and Rosai-Dorfman disease belongs to classⅡb subtypes of non-LCH.
slide52
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.
refernces
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.

slide54
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.

slide55
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