Multicentric Reticulo histiocytosis – A Cas t: Report Porkodi R., Subramaniam Rama krishnan. , Krishnamurthy V.

Chandrasekaran A.N.

Introduction

Multicentric reticulo histiocytosis is a rare systemic disease characterised

by profusion of lipid laden histiocytic nodules in the skin and

mucous membrane and accompanied by a  mutilating polyarthritis.¹ Till

date less than  100 cases of multiccntric rcticulo histiocytosis only

have been reported in literature ²  A case with multicentric reticulo

histiocytosis  is  reported  here for the  rarity of  its  occurrence ,   since  it  is

  the, first case to he encountered among the 20,000 patients who have

attended our outpatient department.

Case Report:

Mrs. P. a 46 year old housewife presented to us for complaints of pain in the interphalangeal and metacarpophalangeal joints of the fingers. knees and wrists of one month duration, associated with definite morning stiffness. She had also noticed multiple small painless, non pruritic nodules on the scalp, pinna of the ears and dorsum of hands since 6 months.

Clinical examination revealed a well nourished individual with stable vital sings. Rheumatologica1 examination  revealed  a symmetrical polyarthrits involving the distal and proximal interphalangeal joints, metacarpophalan-

Dr. Porkodi R.

Asst. Prof. of Rheumatology,

Dr. Subramaniam Ramakrishnan

Asst. Prof. of Rheumatology,

Dr. Krishnamurthy V.

As.st. Prof. of Rheumatology,

Chandrasekaran A.N..

Prof. & Head of the Dept. of the Rheumatology,

Madras Medical College.

Madras-3.

Specially contributed to

The Antiseptic” Vol. 86 ( to) 545-8

geal joints of the hands, wrists and knees

(Fig. 1). Dertmatological examination showed

Fig . l

Polyarthritis in the patient.

multiple, salmon coloured nodules resemb­ ling coral beads in the pcriungual region (Fig. 2) dorsum of the fingers. pinna of the car (Fig. 3) and front of the chest. Other systems exami­ nation was normal. Since this patient had

polyarthritis with multiple skin nodules she

was    referred to the dermatologv department

for opinion and biopsy of the skin nodule

Fig.2

Nodules resembling coral beads in the

periungual region.

Fig. 4

Nodules over the pinna of the ear.

Relevant haematological, biochemical, immunological and radiological investigations

were  performed, but they were not contri-

butoty. Cardiac ultrasound, UGI Endoscopy

and indireci laryngoscopy were normal.

Skin biopsy showed plenty of histio­

cytes of various sizes, some with ground glass

appearance with many nuclei. Number of          multinulceated  giant  cells were seen  (Fig. 4)

This picture was characteristic of multicentric

reticulo histiocytosis.

Fig. 4

Skin biopsy showing histiocytes and

multi-nucleated giant cells.

Magnification: 40

Arthroscopic ,synovial biopsy from the

left knee  showed synovial tissue with proli

feration of synoviocytcs 3-5 layers . Sabaynon

viocytic tissue showed numerous histiocytes

giant cells and diffuse lymphocytes and plasma ,

cells; numerous congested and dilated blood

vessels and adipose tissue extending upto

subsynoviocyte tissue. (Fig. 5).

Discussion:

The differential diagnosis of the condition 

where the arthritis is associated with multiple

nodules are sarcoid, xanthomatosis,myelo

blastomas, lepromatous leprosy, generalised

eruptive histiocytoma, histiocytosis X,Rkeu

Matric fever , rheumatoid arthritis and multicentric retriculo

histiocytosis.  Skin biopsy of the nodules allows identification

of most of these disorders,Especially multicentric reticulo

histocytosis , as is evident in our patient

Cutaneous lesions are light copper nodules resembling

coral beads occuring more often  over the dorsum of

fingers over the nailfolds face ears and chest 5. Nodules

can  occur on mucosal surfaces like lips nasal septum on

organs  like endocardium, none marrowand lymph nodes

multicentric Reticulohistiocytosis. a sys temic disorder

has also manifested with other organ involvement

like myositis, pleural effusion, pericarditis, gastric ulcer.

T. he histop athology of the nodule and the synovium are specific for multicentric reticulo­ h!stiocytosis7  as was  een in our patient‘s skin biopsy and synovial biopsy. Characteristic aggregates of multinucleated giant cells and h1s t10cyte with a ground glass appearance is seen. Electron microscopy of the histiocyte shows large, membrane bound vacuoles with lipid accumulation in most cases.

Fig. 5

Synovial biopsy showing histiocytes and

giant cells.

Magnification 100

First description of multicentric reticuo­ hisriocytosis was made in 1937 by Weber and Frcudental⁴ Other names for multicentric reticulo histiocytosis are lipoid dermatoarthri­ tis, reticulo histiocytoma. Multicentric reti­ culo histiocytosis, occurs predominantly in females than males in a ratio of 3:1 with a mean age of onset being 40 yrs. In 60% of patients joint   symptoms  precede  the   skin symptoms. in 20%

it is the reverse and in the remaining both the articular and dermatological feature s occur simultaneously³• The articular presentation is usually a symmetrical

polyarthritis commonly involving the distal interphalangeal and proximal interphalangeal joints of the fingers, followed  in frequency by knee s, shoulders, ankles, feet and elbows as was seen in our patient .

20-30% of patients with multicentric histio- cytosis have been reported to have malignant neoplasms⁸ of the bronchus, stomach, breast, cervix of lymphomas. We had ruled out the coexistence of any of these malignancies in our patient by investigations.

Treatment

No adequate treatment for multicentric reticulo histiocytosis has been described. The treatment has included trials of low fat diet clofibrate, prednisolone, azathioprine . cyclo phosphamide and isoniazid ³ & 9 . Our patient was administered both systemic steroids and cyclophosphamide.

Our patient had come for follow up for over a year and had shown good response to therapy. But unfortunately she sucumbed to a road traffic accident and died on the spot.

Acknowledgement

Our thanks are due to Mr. M.S. Subra­ manian . Stenotypist for his kind secretarial assistance.

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