[Diffuse normolipemic xanthomatosis and IgG monoclonal dysglobulinemia]

Area of Science:

• Dermatology
• Hematology
• Pathology

Background:

• Diffuse cutaneous xanthomatosis is a rare condition.
• Normolipaemic xanthomatosis presents challenges in diagnosis and understanding its pathogenesis.
• Monoclonal gammopathies can be associated with various cutaneous manifestations.

Observation:

• A 56-year-old man presented with diffuse cutaneous xanthomatosis without mucosal lesions or diabetes insipidus.
• Histopathological examination revealed dermal histiocytic infiltration with Touton's cells and iron deposits, but no X bodies.
• The patient had normal plasma lipid levels but exhibited lambda G monoclonal dysglobulinemia with increased medullary plasmacytic cells.

Findings:

• The diagnosis of disseminated xanthomatosis was established.
• Differential diagnosis from other histiocytic proliferations, like diffuse plane xanthoma, was considered difficult.
• A link between normolipaemic xanthomatosis and dysglobulinemia was noted, but the exact pathogenic mechanism remained unclear due to the absence of specific factors.

Implications:

• This case highlights the complexity of normolipaemic xanthomatosis and its association with monoclonal gammopathies.
• Further research is needed to elucidate the pathogenic mechanisms linking lipid metabolism, histiocytic infiltration, and dysglobulinemia.
• Accurate diagnosis requires careful histopathological evaluation and consideration of underlying hematological disorders.