Abstract
The article describes a 57-year-old female patient with lesions on 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 specimen. 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 gammopathy of clinical significance, with predominant generalized skin involvement, associated with a monoclonal IgG secretion. VRD-based therapy (bortezomib, lenalidomide, dexamethasone) followed by autologous hematopoietic stem cell transplantation led to 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 gammopathy and indicates the need for patients with scleromyxedema to be examined by a hematologist.