A clinical case of B-cell lymphoma in a patient with overlap, primary Sjogren's syndrome and scleredema adultorum of Buschke

Abstract

This is a presentation of case report on B-cell lymphoma in a patient with overlapping primary Sjogren's syndrome (PSS) and scleredema adultorum of Buschke. Scleredema adultorum of Buschke (SB) is a rare disorder characterized by a diffuse indurative edema and skin tightening. A putative classification of this disease is based on the etiological factors and encompasses three types: Type 1 is associated with an infectious disease; Type 2 with paraproteinemia; Type 3 – with diabetes mellitus. There are very rare cases involving malignant neoplasms or autoimmune diseases. This clinical case describes a rare combination of SB with an autoimmune disease – PSS. It highlights the importance of studying other SB associations, in particular with autoimmune diseases, to ensure proper management and treatment of these patients. For a 51-year-old patient, the disease onset featured a dense facial edema, dry skin, dry eye syndrome and a feeling of sand in the eyes, subfebrile fever and dense skin formations in the low neckline, mammary glands and interscapular region, which was pathohistologically regarded as scleredema Buschke. Lymphadenopathy, xerophthalmia, sialodenitis, Raynaud's syndrome were revealed, lung damage (alveolitis and bronchiolitis), positive ANA, anti-SSa/La, antiSSb/Ro were diagnosed. Based on this symptom complex, the patient was diagnosed with an overlap syndrome of SB with PSS. The use of combined therapy with methotrexate and methylprednisolone allowed to reduce the disease activity, clinical and laboratory appearance of the disease. However, 9 months after the onset of the first skin symptoms, there was a bulging of the left eye, widespread lymphadenopathy. Histopathological examination revealed the diagnosis of non-Hodgkin B-cell lymphoma. The use of rituximab therapy resulted in a significant regression of the orbital tumor. The relevance of this clinical observation is not only due to the rarity of combined pathology, but also to the need for an early diagnostics of lymphoma developing against the background of PSS, based on the risk factors, which include low levels of C4 and/or C3, enlargement of the parotid salivary glands, cryoglobulinemia, persistent splenomegaly’s systemic activity and lymphadenopathy, since the frequency of this disease is quite high in this patient population.

Keywords

• scleredema adultorum of buschke
• sjogren’s syndrome
• lymphoma
• overlap syndrome
• clinical case