Article Text

PDF
THU0384 Spontaneous panniculitis (weber-christian disease): Clinical course variants
  1. B.S. Belov,
  2. O.N. Egorova,
  3. O.V. Pushkova,
  4. Y.A. Karpova,
  5. S.G. Radenska-Lopovok
  1. Institute of Rheumatology of Russian Academy of Medical Sciences, Moscow, Russian Federation

Abstract

Background Spontaneous panniculitis (SP) is a rare, little studied chronic inflammatory disease, which belongs to a group of connective tissue diseases. The variety of clinical symptoms of SP causes difficulties in diagnostics and adequate treatment.

Objectives To study distinctive features of SP treatment as of today.

Methods We studied 28 SP patients (26 women, 2 men), age 30-74, duration of disease 8.1±2.4 years. In addition to general clinical examination we determined the level of α1-antitrypsin, amylase, lipase, ferritin, creatine phosphokinase, we also carried out chest CT scan, ultrasound scan of the node and pathomorphology study of skin biopsy specimen taken from the area of the node.

Results 29% of patients had a subacute disease and 64% of patients had a chronical disease. SP featured multiple (17 patients) painful (VAS intensity of 69±24.2 mm) nodes, mostly concentrated on the lower (26) and upper (14) extremities, less frequently the nodes were found on the chest, abdomen and face (5). 36% of cases were identified as nodule panniculitis characterized with isolated well-defined nodes, i.e. bright pink indurations of up to 5 cm in diameter. Plaque panniculitis, developed due to conglomeration of individual nodes into a red-purple nodular lesion, was identified in 46% of patients. In case of infiltration panniculitis (18%) individual bright crimson nodes or conglomerates discharged yellow oily material simulating an abscess. In 69% of cases the occurrence of nodes was accompanied with fever, polyarthralgias, myalgias and accelerated ESR, which was the same as in case of plaque and infiltrating panniculitis. Histological examination confirmed the subcutaneous fat (SCF) damage such as infiltration of lymphocytes contaminated with a few Tutton cells and lipophages. Ultrasonic scanning of skin made it possible to identify well-defined lobulation characterized with higher echodensity with an anechogenic circle. Treatment depended on the type and the state of the disease. It included nonsteroidal anti-inflammatory (13), antioxidant (28), aminoquinoline (10), glucocorticoid (18) and cytostatic (18) drugs. Due to the treatment there was a positive dynamics in 64% of cases for patients with nodule (10) and plaque (8) panniculitis.

Conclusions SP is a rare recurrent polisyndrom disease which is basically caused by SCF damage. The variety of forms of the disease requires well-defined approaches to verification of the diagnosis and differentiated treatment.

Disclosure of Interest None Declared

Statistics from Altmetric.com

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.