Page 38 - Livre électronique du congrès AFMAPATH 2024

P15. PULMONARY INVOLVEMENT REVEALING WEBER
CHRISTIAN DISEASE

H. LAATOUB, F.AIT HMED ,M. SOUALHI, R. ZAHRAOUI, J.E BOURKADI.

SERVICE DE PNEUMOLOGIE, HOPITAL MOULAY YOUSSEF ,CHU IBN SINA

Introduction :

Weber Christian disease(WCD) or idiopathic lobular panniculitis is a rare condition of unknown
etiology, characterized by recurrent inflammation of subcutaneous adipose tissue, it mainly
affects adults with female predominance. WCD is a nonspecific clinical entity that remains a
subject of debate and its evolution is unpredictable, it is a diagnosis of exclusion. It manifests
clinically with recurrent fever outbreaks and painful subcutaneous nodules, systemic
manifestations of WCD frequently affects the liver, bone, kidneys, sporadically serous, spleen
and lungs.

It could be life-threatening and it is considered a therapeutic challenge for clinicians.

We report a case of respiratory disease revealing the disease of Weber Christian seen at the
pulmonology department of the Hospital My Youssef CHU Ibn Sinaa of Rabat

Case report :

A 50-year-old male, former smoker weaned, Type II diabetic on insulin, who presented 4
months ago stage E acute pancreatis and superficial venous thrombosis 4 months ago, he also
presented a week before his admission a productive cough bringing back muco-purulent
sputum associated with chest pain, exertional dyspnea stage II mMRC and intermittent fever up
to 39°C and general malaise.

During his hospitalization, the patient presented erythematous swelling on the back of both
hands (Figure 1), and painful subcutaneous nodules present in the extremities, a week after, an
acute scrotal edema appeared motivating the realization of scrotal ultrasound, returning in favor
of a bilateral epididimo-orchitis, 4 days after, he presented a painful eyelid swelling and
erythema in the left eye at first then the right one, associated to a ptosis. Orbital CT scan with
contrast revealed diffuse thickening of the preseptal soft parts at the level of the inner canthus

and infiltration of intra and extra conical grease in both eyes.

The biological assessment found an inflammatory anemia at 8g/dL, the Erythrocyte
sedimentation rate was at 100mm with a C reactive protein (CPR) at 400mg/L. The immunological
assessment and viral serology were negative. All hepatic, renal, lipid assessment, 24-hour
proteinuria and lipasemia were normal. Tuberculosis screening was negative. Both
echocardiography and abdominal ultrasound were normal.

The thoracic CT scan showed sub pleural nodules of the ventral segment of the right upper
lobar and atelectasis in the middle lobe and dorsal segment of the left lower lobe associated

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