Cutaneous mucinosis includes a heterogeneous group of skin diseases characterized by the deposition of mucin in the interval of the dermis. Mucin is a protein ordinarily found as part of the dermal connective tissues. Mucin is a mucopolysaccharide produced by mast cells and fibroblasts and includes hyaluronic acid and sulfated glycosaminoglycans. Because hyaluronic acid holds water, in disease states where mucin production is increased, the dermal connective tissue swells, which is defined as myxedematous. Systemic mucin deposition may include systemic involvement; monoclonal gammopathy or paraproteinemia have been detected in the large majority (83.2%) of scleromyxedema cases.
A 50-year-old female presented with pruritic, flesh-colored papules spread throughout the body. She also exhibited a characteristic lion face appearance. These lesions had first appeared 18 months before in the neck. The protein electrophoresis and bone marrow biopsy of the patient were normal. Histopathological examination revealed widespread mucin accumulation with alcian blue and colloidal iron stains in the papillary and reticular dermis. Granulomas were not observed with CD68 staining, and no accumulation was observed with amyloid staining. The final diagnosis was cutaneous mucinosis.
In this paper we present the case of a patient with generalized primary cutaneous mucinosis without any systemic disease.
Keywords: Cutaneous mucinosis, lion face, scleromyxedema, hydroxychloroquine