Dermatology MCQ - Inflammatory Dermatoses - Treatment for Adamantiades–Behçet disease

A 28-year-old man with a confirmed diagnosis of Adamantiades–Behçet disease presents for management. His primary manifestations are recurrent, painful oral and genital aphthous ulcers. He has no history of ocular, vascular, or neurological involvement. After a trial of topical corticosteroids has provided inadequate relief, what is the most appropriate first-line systemic agent for this patient?

A. Colchicine
B. Azathioprine
C. Cyclosporine
D. Infliximab
E. Interferon-alpha

Correct Answer: A. Colchicine

Answer & Explanation

Explanation:

The management of Behçet disease follows a stepwise approach based on the severity and site of involvement. For mucocutaneous disease without major organ involvement (e.g., eyes, CNS, major vessels), the treatment ladder begins with less aggressive agents.

• First-line for Mucocutaneous Disease: Colchicine is widely regarded as the first-line systemic agent for treating oral and genital ulcers in Behçet disease, particularly in males. It is effective, relatively safe, and has a well-established role in controlling the frequency and pain of aphthous lesions.

The other options are reserved for more severe or refractory disease:

• B. Azathioprine: This is a first-line steroid-sparing agent for major organ involvement, such as uveitis. It is typically used when mucocutaneous disease is severe and refractory to colchicine or when systemic corticosteroids are required.

• C. Cyclosporine: This is a potent immunosuppressant used primarily for refractory ocular disease, especially acute posterior uveitis. Due to its side-effect profile (nephrotoxicity, hypertension), it is not a first-line choice for isolated mucocutaneous disease.

• D. Infliximab (Anti-TNFα): Biologic agents like infliximab and adalimumab are highly effective but are reserved for severe, refractory, or sight-threatening manifestations that have failed conventional immunosuppressants (e.g., refractory uveitis, major vessel involvement).

• E. Interferon-alpha: This is an effective treatment for mucocutaneous and articular disease, and particularly for refractory ocular Behçet's. However, due to its significant side-effect profile (flu-like symptoms, depression), it is generally considered a second- or third-line agent, not a first-line choice.

Key Associations: Treatment Ladder for Adamantiades–Behçet Disease

The treatment is tailored to the specific organ systems affected. The following represents a general stepwise approach:

1. Mucocutaneous Disease (Oral/Genital Ulcers, Papulopustular Lesions, Erythema Nodosum):

• First-line: Topical corticosteroids (gels, ointments), topical anesthetics.

• Second-line (Systemic): Colchicine.

• Refractory Cases: Azathioprine, interferon-alpha, anti-TNFα agents (e.g., adalimumab, infliximab). Dapsone or thalidomide may be considered in select, severe cases.

2. Articular Disease (Arthritis/Arthralgia):

• First-line: Colchicine, NSAIDs.

• Refractory: Azathioprine, interferon-alpha, anti-TNFα agents.

3. Ocular Disease (Uveitis, Retinal Vasculitis):

• This is a medical emergency requiring aggressive treatment to prevent blindness.

• First-line: Systemic corticosteroids (high-dose IV pulse for acute sight-threatening inflammation, then oral) PLUS a steroid-sparing agent.

• Steroid-Sparing Agents: Azathioprine, cyclosporine, or interferon-alpha.

• Refractory/Severe: Anti-TNFα agents (especially infliximab and adalimumab) are now often used early in severe cases.

4. Major Vascular Disease (Thrombosis, Aneurysms):

• First-line: High-dose corticosteroids combined with cyclophosphamide or azathioprine. Anticoagulation is controversial and must be weighed against the risk of hemorrhage from pulmonary artery aneurysms.

• Refractory: Anti-TNFα agents.

5. Central Nervous System (CNS) Involvement:

• First-line: High-dose corticosteroids.

• For parenchymal involvement: Add azathioprine, cyclophosphamide, or methotrexate.

• Refractory: Anti-TNFα agents.

Prognosis: The disease course is characterized by remissions and exacerbations. The prognosis is primarily determined by major organ involvement (ocular, neurological, major vascular), which can lead to significant morbidity and mortality if not treated aggressively.