What is superficial hemosiderosis (superficial iron deposition disease)?

From the Guidelines

Superficial hemosiderosis should be diagnosed and managed with MRI as the primary imaging modality, and treatment should focus on identifying and addressing the underlying source of bleeding, with iron chelation therapy and supportive care as adjuncts. The condition is characterized by iron deposition in the tissues covering the brain and spinal cord, resulting from chronic bleeding into the subarachnoid space, and typically presents with progressive sensorineural hearing loss, cerebellar ataxia, and myelopathy 1.

Key Considerations

• The underlying source of bleeding should be identified and treated, which may include dural defects, tumors, vascular malformations, or cerebral amyloid angiopathy.
• Iron chelation therapy with deferiprone may be used, but its efficacy is limited and it carries risks, including agranulocytosis, requiring regular blood count monitoring.
• Supportive care, including physical therapy, occupational therapy, and hearing aids, is essential to manage symptoms and improve quality of life.
• Early diagnosis is crucial, as the condition is progressive and often irreversible once neurological damage occurs.

Diagnostic Approach

• MRI is the diagnostic modality of choice, showing characteristic hypointensity on T2-weighted images around the brain stem, cerebellum, and spinal cord due to hemosiderin deposition 1.
• Susceptibility-weighted imaging or gradient echo T2-weighted sequences can help detect blood products in superficial siderosis, manifesting as a hypointense coating over the surface of the cerebellum, brainstem, and cord 1.

Treatment Priorities

• Identifying and treating the underlying source of bleeding is the primary goal of treatment, as it can help prevent further neurological damage and improve outcomes.
• Iron chelation therapy and supportive care should be used as adjuncts to address symptoms and improve quality of life, but should not delay efforts to identify and treat the underlying cause of bleeding.

From the Research

Superficial Hemosiderosis

• Superficial hemosiderosis is not directly mentioned in the provided studies, however, hemosiderin deposition is discussed in the context of stasis dermatitis and superficial siderosis.
• In stasis dermatitis, hemosiderin-laden macrophages and extravasated red blood cells are prominent features, as seen in 2.
• Superficial siderosis, a condition characterized by hemosiderin deposition on the pial surface of the brain and/or spinal cord, is discussed in 3.
• The causes of hemosiderin deposition in superficial siderosis include tumor, vascular abnormality, injury, and dural defect, as mentioned in 3.
• In the context of chronic venous insufficiency, cutaneous findings such as pigmentation and dermatitis can be associated with hemosiderin deposition, as seen in 4.

Related Conditions

• Stasis dermatitis is a chronic inflammatory skin disease of the lower extremities, characterized by eczematous changes and associated with venous hypertension, as discussed in 2, 5, and 6.
• Chronic venous insufficiency can lead to various cutaneous findings, including varicosities, telangiectasia, edema, and pigmentation, as mentioned in 4.
• Superficial siderosis is a rare condition characterized by hemosiderin deposition on the pial surface of the brain and/or spinal cord, as discussed in 3.

Pathogenesis and Management

• The pathogenesis of stasis dermatitis involves inflammation and venous hypertension, as discussed in 5 and 6.
• The management of stasis dermatitis includes compression therapy, topical treatments, and interventional treatment options to correct the underlying causes of venous reflux, as mentioned in 6.
• The treatment of superficial siderosis involves identification and surgical correction of the bleeding source, as well as the use of iron chelators such as deferiprone, as discussed in 3.