- Indian Rheumatology association
Abhishek Patil MD Medicine, DNB Rheumatology , PG Diploma MSK USG (UCAM, Spain)
Consultant and Head, Dept of Clinical Immunology and Rheumatology , Manipal Hospitals, Bengaluru
CLINICAL DETAILS :
A 63 year female presented with progressive skin thickening on the face and whole body as shown in figure. There was no history of Raynaud’s.
Other tests showed ANA positive 1:100, 2+, speckled pattern, ANA profile negative
Skin biopsy: thickened dermis and thickened collagen fibres with increased dermal mucin
She was treated as scleroderma with steroids and hydroxychloroquine with no response.
Describe the cutaneous findings and diagnosis?
Answer: The clinical images show generalized waxy papules with induration. This clinical picture is consistent with Scleromyxedema. Patients Serum protein electrophoresis showed an M band of 0.8 g/dL and Immunofixation electrophoresis showed IgGk monoclonal gammopathy
Scleromyxedema is a chronic cutaneous disease generally associated with monoclonal IgG protein and presents with prominent skin induration and can mimic scleroderma (SSc). Absence of Raynaud’s, waxy papules, widespread involvement of head and neck, trunk, upper and lower limbs help to clinically differentiate from SSc. Treatment involves Immunoglobulin G/ high dose steroids/ Melphalan/ bortezomib / stem cell transplantation.
