Spontaneous periorbital ecchymosis (Non-traumatic raccoon eyes)

Specialty: Hematology.

• Spontaneous periorbital purpura
• amyloid raccoon eyes
• eyelid hematoma due to capillary fragility

Why it occurs

• Systemic light chain amyloidosis / AL Amyloidosis (disease characterized by the extracellular deposition of amyloid fibrils derived from monoclonal immunoglobulin light chains that infiltrate the skin and muscle vascular walls, severely weakening them; low-intensity microtrauma, cough or Valsalva maneuvers cause spontaneous capillary rupture)
• Multiple myeloma with secondary AL amyloidosis
• Severe corticosteroid-induced Cushing's syndrome or ectopic ACTH production (with extreme atrophy of the periocular dermal supporting connective tissue)
• Severe deficiency of coagulation factor X (specifically associated with amyloidosis due to adsorption and selective sequestration of factor
• Scurvy / Severe vitamin C deficiency (alteration of collagen synthesis that weakens capillary vascular support).

Initial workup

Serum and urinary protein electrophoresis with high sensitivity immunofixation and quantification of free kappa and lambda light chains in serum (to identify plasma cell clonality); abdominal subcutaneous fat aspirate or rectal biopsy (initial screening methods with high diagnostic sensitivity for staining with Congo red, showing classic apple-green birefringence under polarized light); bone marrow biopsy with immunohistochemistry to evaluate percentage of clonal plasmacytes; quantification of coagulation Factor X levels in plasma; echocardiogram with Strain technique (will reveal classic pattern of apical preservation or "cherry on top" suggestive of cardiac amyloid infiltration) and levels of cardiac biomarkers (markedly elevated NT-proBNP and Troponin T).

Standard management

• Daratumumab — monoclonal antibody directed against CD38 abundantly expressed on clonal plasma cells; mainstay of AL amyloidosis treatment by rapidly eliminating the source of amyloidogenic light chain production
• Bortezomib — subcutaneous proteasome inhibitor that stops the transcription and production of aberrant monoclonal proteins
• Melphalan — high-dose oral or intravenous alkylating agent used in conditioning regimens prior to autologous hematopoietic stem cell transplantation
• Dexamethasone (high-potency systemic corticosteroid that is part of the therapeutic regimens for the control of the neoplastic plasma cell population).