Generalised plane xantomas associated with IgG monoclonal gammapathy - case discutions
Cutaneous xanthomas are yellow, brown, pink or orange macules, papules, plaques or tendinous infiltrations of macrophages containing lipid droplets.
A female patient, 49 years of age, presented for yellow-orange, circumscribed, discretely infiltrated, asymptomatic plaques symmetrically distributed periocular, latero-cervical, latero-thoracic and in the submamary folds. The lesions began 5 years ago in the periocular regions and progressively extended in the latero-cervical, latero-thoracic and submamary regions.
Patient’s history is only significant for uterine fibroma (surgically removed 8 years ago), poliarthrosis, osteopenia, chronic urticaria and H. pylori gastritis. The patient denies smoking, alcohol intake or any medical therapy in the past 5 years. On physical examination the patient is obese (BMI 34,5 kg/mp), presents poliadenomegaly, arterial hypertension (180/100 mm Hg), poliarthralgias and acral parestesia. Paraclinical investigations showed a high ESR (54 mm at 1 hour), hyperproteinemia (93 mg/dl), hyperglobulinemia (g - 27%), A/G=0,82 and elevated serum IgG (2069 mg/dl). Lipidic profile was within normal limits.
Renal function, hepatic function and serum glucose were also normal. Imagistic investigations did not show significant changes for the disease. Histopathological exam showed macrophages xantomatous cells coalescents or sparced throughout the dermis. Based on the clinical aspects and histopathological exam the diagnosis of disseminated plane xanthomas was established.
Paraclinical investigations suggested a monoclonal IgG gammopathy that required hematological evaluation.
Cutaneous xantomas, depending on the clinical aspect and location, may signal the presence of an underlying metabolic disorder, or a lymphoproliferative disease. Normolipemic plane xantomas are often associated with multiple myeloma. Cutaneous lesions may precede with several years the onset of a multiple myeloma.