Scleromyxedema as a manifestation of monoclonal gammapathy of clinical significance

Abstract

The article describes a 57-year-old female patient with lesions of the scalp, trunk and extremities, who was diagnosed with scleromyxedema (myxedematous lichen) by a dermatovenereologist based on clinical data and histopathological examination of a skin biopsy. An immunochemical study of serum proteins revealed an M-gradient in the g3 zone, formed by a Gk clone and amounting to 4.9 g/l. Immunophenotyping of blood cells revealed a population of clonal plasma cells with the CD138+CD38+ CD56-CD10-CD117-kappa immunophenotype (2.26% of all studied events). Upon further examination by a hematologist, 2% of plasma cells were detected in the myelogram. Immunophenotyping of bone marrow cells by flow cytometry revealed 0.144% of plasma cells from all cells with the aberrant immunophenotype CD38dimCD138+CD319+CD19-CD45+/-CD56+/- CD27+/-CD117-/+CD200+CD20-/+. No criteria for symptomatic multiple myeloma or smoldering myeloma were found. The patient was diagnosed with monoclonal gammapathy of clinical significance, with a predominant total skin lesion, scleromyxedema, occurring with monoclonal secretion of Gk. The VRD therapy (bortezomib, lenalidomide, dexamethasone) followed by autologous hematopoietic stem cell transplantation has achieved significant improvement. Gk secretion decreased to a trace, the bone marrow was sanitized, and a significant improvement in the patient's skin condition was noted. The presented case demonstrates the association of scleromyxedema with monoclonal gammapathy and indicates the need to examine patients with scleromyxedema by a hematologist.