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Images in Clinical Medicine

www.cmj.ac.kr

https://doi.org/10.4068/cmj.2020.56.3.212

Ⓒ Chonnam Medical Journal, 2020 Chonnam Med J 2020;56:212-213

Corresponding Author:

Chong Hyuk Chung

Division of Rheumatology, Department of Internal Medicine, Wonkwang University School of Medicine, 895 Muwang-ro, Iksan 54538, KoreaTel: +82-63-859-2661, Fax: +82-63-855-2025, E-mail: taylorchung @hanmail.net

Article History:

Received June 3, 2020Revised June 16, 2020Accepted June 19, 2020

FIG. 2. (A) A radiograph of the humerus

revealed clumped masses and sheet-

like calcification in axilla and upper

limbs. (B) Sagittal T2 MR image shows

nodular calcifications (circle) as dark

signal masses in right axilla, (C) sheet-

like calcification (arrow) with hyper-in-

tensity in muscle fibers suggest edema

and inflammation in right upper limbs.

FIG. 1. (A) Erythematous changes and shallow ulceration of the skin, overlying chin and anterior neck area. (B) Multiple nodular lesion

on right axilla. (C) Limited range of motion on right elbow joint. (D) Extrusion of white, crumbly matter on right posterior elbow.

Calcinosis Universalis in Juvenile Dermatomyositis

Chong Hyuk Chung*

Division of Rheumatology, Department of Internal Medicine, Wonkwang University School of Medicine, Iksan, Korea

A 12-year-old girl presented with a facial erythematous

rash and limited range of motion of the elbow joint. She

complained of swelling and limitation of the motion of both

elbow joints. This patient presented with a history of juve-

nile dermatomyositis since the age of 5. She had main-

tained muscle strength, but had a waxing and waning skin

rash, despite receiving treatment with multiple medi-

cations, including corticosteroids, intravenous immunoglob-

ulin, methotrexate, and mycophenolate mofetil for over 6

years at our hospital.

On physical examination, she presented with diffuse

palpable subcutaneous nodules in both the axilla and the

upper extremities, erythematous changes, and shallow ul-

ceration of the skin, overlying chin, and anterior neck area

(Fig. 1A). There were multiple tender nodular lesions on

the right axillar and a limited range of motion due to pain

213

Chong Hyuk Chung

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(Fig. 1B, C). Extrusion of white, crumbly matters in the vi-

cinity of the posterior elbow were observed (Fig. 1D). A ra-

diograph of the humerus revealed extensive calcification

in both axillary areas, extending downwards (Fig. 2A).

Magnetic resonance imaging revealed huge calcification

and fluid collection in whole teres major, biceps brachii,

brachialis and proximal pronator teres muscles (Fig. 2B,

C).

Given the patient’s calcinosis universalis, she was treat-

ed with infliximab, intravenous bisphosphonate, and alu-

minum hydroxide.

Juvenile Dermatomyositis (JDM) is a rare autoimmune

inflammatory disease of the muscles, affecting children

and adolescents, presenting with soft tissue calcification

and varying systemic involvement. Calcifications in JDM

usually develop in the chronic phase at the sites of necrosis

and involve the skin, subcutaneous tissue, fascial planes

and muscles.1 Calcinosis in JDM patients can cause more

incapacity and complications than the myopathy itself.2

Soft tissue calcification can be anywhere, including super-

ficial skin (calcinosis cutis), around joints (calcinosis cir-

cumscripta) or deep fascial planes (calcinosis universalis).

There are four patterns of calcification described with

dermatomyositis cases namely: superficial nodular-like

calcified masses, deep calcified masses, deep sheet-like cal-

cifications within the fascial planes, and a rare, diffuse, su-

perficial lacy and reticular calcification which involves al-

most the entire body surface. The last pattern has been

linked with a chronic, severe, non-remitting course, in pa-

tients with dermatomyositis, and needs to be differenti-

ated from myositis ossificans progressive.3 The calcinosis

in dermatomyositis is essentially a dystrophic calcification

without formation of ossification, hence no evidence of zon-

ing is seen in these calcified masses unlike in myositis ossi-

ficans progressive.4

To the best of our knowledge, this is the first report of

Calcinosis Universalis complicating JDM in Korea.

ACKNOWLEDGEMENTS

This study was supported by Wonkwang University

Grant in 2020.

CONFLICT OF INTEREST STATEMENT

None declared.

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