Title:An Unusual Presentation of a Rare Scleroderma Mimic: What is Behind the Scenes?
Volume: 15
Issue: 2
Author(s): Rehab Ali Ibrahim*, Nour El-Hoda Hussien Abdalla, Engy Amr Husssein Shabaan and Noha Bassiouny Hassan Mostafa
Affiliation:
- Physical Medicine, Rheumatology and Rehabilitation Department, Faculty of Medicine, Ain Shams University, Cairo,Egypt
Keywords:
Scleroderma, scleredema, scleroderma mimics, multiple myeloma, monoclonal gammopathy, fibrosis.
Abstract:
Background: Scleroderma or systemic sclerosis (SSc) is a rare systemic autoimmune
disease. Many conditions mimic the presentation of SSc, especially skin thickening and fibrosis.
One of these conditions is scleredema, a rare collagen and mucin deposition disorder which was
found to be associated with diabetes mellitus, streptococcal infection or monoclonal gammopathy.
Case Presentation: A 55 years old female presented with insidious onset and progressive course of
diffuse skin thickening of face, neck, arms, forearms, thighs, chest, back, and excluding both hands
and feet of 6 years’ duration associated with arthralgia, dysphagia and dyspnea on exertion of 1-
year duration. There was no history of Raynaud’s phenomenon. Erythrocyte sedimentation rate was
100 mm/1st h, autoantibodies for SSc were negative, nail fold capillaroscopy normal, pulmonary
function tests showed restrictive pattern and high-resolution computed tomography showed interstitial
lung fibrosis. Patient was not fulfilling the American collage of rheumatology/European League
Against Rheumatism classification criteria for SSc. Skin biopsy was done and revealed histological
appearance of scleredema. Investigations were done for disease association with scleredema. The
patient was not diabetic, antistreptolysin O titer was normal, serum protein electrophoresis, immunofixation
and bone marrow biopsy were done, and the patient was diagnosed as scleredema associated
with immunoglobulin A kappa multiple myeloma. Treatment by combination of bortezomib,
cyclophosphamide, and dexamethasone was started with marked clinical and hematological
improvement.
Conclusion: Many conditions mimic SSc including scleredema, which may be the initial presentation
of multiple myeloma. Rheumatologists and dermatologists should be able to recognize these
conditions to provide the suitable management and follow-up for these patients.
