Acta Dermato-Venereologica 99-13CompleteContent | Page 31
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SHORT COMMUNICATION
Rosai-Dorfman Disease with Systemic Multiple Involvement: A Case Report
Jiangfan YU 1 , Bingsi TANG 1 , Yaqian SHI 1 , Puyu ZOU 1 , Guiying ZHANG 1 , Yan DING 2 * and Rong XIAO 1 *
Department of Dermatology, Second Xiangya Hospital of Central South University, Changsha, 410011, and 2 Department of Dermatology,
Hainan Provincial Dermatology Disease Hospital, Haikou 570206, China. *E-mail: [email protected]; [email protected]
1
Accepted Oct 14, 2019; E-published Oct 14, 2019
Rosai-Dorfman disease (RDD) is an uncommon, benign
non-Langerhans’ cell histiocytic disorder, typically invol-
ving bilateral painless cervical lymphadenopathy. RDD
only rarely affects extranodal sites, such as the skin, orbit,
respiratory tract, central nervous system and soft tissues
(1). RDD occurs most commonly in children or young
adults, with no obvious difference according to sex. With
no specific laboratory data, diagnosis of RDD is usually
based on pathology, characterized by proliferation of
histiocytes (S-100 and CD68-positive, CD1a-negative)
evidently displaying phagocytosis, an inflammatory set-
ting of lymphocytes, and a mass of plasma cells.
We report here a rare case of a patient with RDD with
combined bilateral macular damage and systemic exten-
sive skin lesions, accompanied by elevated interleukin
(IL)-6 in the blood.
CASE REPORT
A 54-year-old man initially presented with a 1.5-year history of
painless red nodules and masses on the left cheek and both legs.
Six months later, he developed congestion, oedema, photophobia
and itching in both eyes, which ophthalmologists considered to
be conjunctivitis or scleritis (Fig. 1a, c). In order to confirm the
diagnosis, he presented to a clinic at a local hospital, where he
underwent a skin biopsy and immunohistochemistry. The results
suggested primary skin CD4-positive T-cell proliferative disease.
However, the lesions were still in progress after intramuscular
injection of interferon-2α (3 million IU, every other day) and
local corticosteroid injection (compound betamethasone injection
1 ml once a month).
The patient was admitted to our hospital for diagnosis and
treatment. Laboratory data revealed increased erythrocyte sedi-
mentation rate (ESR), C-reactive protein (CRP), and IL-6 levels.
The following tests were normal: body temperature, liver and
renal function test, microorganisms, autoimmune antibody (e.g.
antinuclear antibody and rheumatoid related antibody), bone mar-
row cytology, albumin, serum IgG4, IgA, IgM, IgG, IgE, alpha-
globulin, beta-globulin and gamma-globulin. Enhanced computed
tomography (CT) revealed enlarged mediastinal lymph nodes.
Fig. 1. Clinical images. (a) Mild conjunctival edema and eye congestion
before treatment; (b) Eye symptoms disappeared after treatment; (c)Red
nodules and masses on the legs before treatment; (d) Skin lesions resolved
after treatment, leaving some pigmentation.
Magnetic resonance imaging (MRI) indicated right popliteal no-
dules, which may be the enlarged lymph nodes. Colour Doppler
ultrasound showed vitreous opacity of the eyes and enlargement
of lymph nodes in the neck, axilla, and groin. Corneal confocal
microscopy revealed a normal epithelial and stromal layer of the
right eye, and a large number of hypo-reflective keratic precipita-
tes (KPs), hyper-reflective KPs and inflammatory cells deposited
on the endothelium as well as founded in the anterior chamber.
Optical coherence tomography of both eyes showed the partial
thickness of retinal pigment epithelium became thinner and hyper-
reflective bulges were observed. Anterior chamber puncture found
that both IL-6 and IL-10 were increased in the aqueous humour.
Another skin biopsy was performed of the same lesion on the right
knee. Haematoxylin and eosin (HE) stains showed perivascular
infiltration of many small lymphocytes mixed with histiocyte
and plasma cells in the superficial and deep dermis. Hemosiderin
was also seen in the dermis (Fig. S1 1 ). IgG4 (+)/IgG (+)>40%.
This indicated that the skin lesions had probably been caused by
IgG4-related disease. The rearrangement of T cell receptor was
negative. Furthermore, immunohistochemical test revealed that
the large histiocytes were positive for S100, and the histiocytic
marker CD68. The cells were negative for Langerhans’ histiocytic
marker CD1a. The histiocytes were also focally positive for IgG,
IgG4 (+) /IgG (+) >40% (Fig. S2 1 ). A diagnosis of RDD was made
based on the clinical presentation and pathology results.
Oral prednisone (20 mg daily, reduced by 5 mg each month)
was added to the treatment. The patient then remained on a main-
tenance dose of 5 mg prednisone. The follow-up period was 18
months. During this time, the patient achieved clinical remission
of the skin and eye lesions (Fig. 1 b, d), with no recurrence of the
symptoms described above. ESR, CRP, IL-6, and colour Doppler
ultrasound of the eyes were all normal. The enlarged lymph no-
des in other parts of the body returned to normal, and enhanced
CT scan revealed that the enlarged mediastinal lymph nodes had
reduced in size.
DISCUSSION
RDD can be a general disorder, often affecting the lymph
nodes. Unlike nodal disease, extranodal disease could
involve any site on the patient’s anatomy. Head and
neck lesions are the most common extranodal lesions,
ophthalmic lesions have been reported in only 10–11%
of patients (usually manifesting as lymphoproliferation
in the soft tissues of the orbit and eyelid) (2, 3), and
multiple system involvement is less common. The patient
described here had macular involvement, bilateral ocular
vasculitis, extensive body lesions, and mediastinal lymph
node enlargement, which are rarely reported.
Diagnosis of RDD is usually based on its pathological
manifestations (4). The usual microscopic features are
https://www.medicaljournals.se/acta/content/abstract/10.2340/00015555-3336
1
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2019 Acta Dermato-Venereologica.
doi: 10.2340/00015555-3336
Acta Derm Venereol 2019; 99: 1305–1306