Elastosis perforans serpiginosa

This code describes a rare, chronic skin disorder characterized by transepidermal elimination of abnormal elastic tissue. It presents as serpiginous (snake-like) or annular (ring-shaped) papules and plaques, often with central atrophy, typically on the neck, face, and flexural areas. The condition is often associated with connective tissue disorders, such as Ehlers-Danlos syndrome or Marfan syndrome, or certain systemic conditions.

Use this code when documentation clearly identifies a diagnosis of elastosis perforans serpiginosa. This diagnosis is typically made based on characteristic clinical presentation and confirmed by skin biopsy showing transepidermal elimination of elastic fibers. It is appropriate for both initial diagnosis and subsequent encounters for management of the condition.

• Ensure the physician's documentation explicitly states "elastosis perforans serpiginosa" or provides a clear clinical description consistent with the condition.
• Investigate for any underlying associated systemic or connective tissue disorders, as these may require additional coding.
• Do not confuse with other perforating dermatoses unless the specific diagnosis of elastosis perforans serpiginosa is confirmed.