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Cases report
Dermatology Department of Xinhua Hospital
Shanghai Jiaotong University, Medical school
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

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
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:
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；






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

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 groundglass appearing cytoplasm.
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


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.
What
is the diagnosis?
Langerhans cell histiocytosis(LCH)
accompany with or not
multicentric reticulohistiocytosis(MH)
/
reticulohistiocytoma(RH)
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.


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.

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

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
+
+
HAM56 +
+
S100
-
-
FxⅢ
-
+
HHF35 -
+
CD68
Large multinucleated giant cells with distinctive eosinophilic granular cytoplasm.

The preferred diagnosis
LCH with RH
Cases 2
 yellow papules with multi-organs involved


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.

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

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
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.
What is the diagnosis:
Langerhans cell histiocytosis
or
Juvenile xanthogranuloma
or
both mixed / overlapped

Prefer to think:
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.

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 RosaiDorfman disease belongs to classⅡb
subtypes of non-LCH.

However, there has been a report about
coexistence of LCH and cutaneous RosaiDorfman disease in a Chinese child.

To me, many quandaries are yet to be
resolved in regard to this seeming overlap
of histiocytic disorders.
Refernces

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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.
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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.

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.
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10. Barnhill RL, Neil Crowson A, Busam KJ, Granter SR
textbook of dermatopathology, McGraw-hill health
professions division, 1997:104-5
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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