Dermatology MCQ - Inflammatory Dermatoses - Morphoea management

A 25-year-old woman presents with a 6-month history of an active, expanding, linear band of induration and hyperpigmentation extending from her right wrist to her antecubital fossa, causing mild flexion contracture of her elbow. Which of the following is the most appropriate first-line systemic treatment to halt disease progression and improve her functional outcome?

A. Oral prednisone 1 mg/kg/day as monotherapy
B. Oral methotrexate
C. Oral D-penicillamine
D. Topical calcipotriol
E. Oral hydroxychloroquine

Correct Answer: B. Oral methotrexate

Explanation

This patient has linear morphoea with evidence of active disease (expansion) and functional impairment (contracture). This is a clear indication for systemic immunosuppressive therapy to prevent permanent disability.

• First-Line Systemic Therapy: Methotrexate is the established first-line systemic treatment for severe or progressive forms of morphoea, including linear, generalized, and deep variants. Robust evidence, including randomized controlled trials, supports its efficacy in halting disease activity, softening skin, and improving functional outcomes. It is often used in combination with a short initial course of systemic corticosteroids for rapid control of inflammation.

Why other options are incorrect

• A. Oral prednisone 1 mg/kg/day as monotherapy: While systemic corticosteroids are highly effective for rapidly suppressing inflammation in the active phase, they are not recommended as long-term monotherapy. Their use is associated with significant toxicity, and the disease often flares upon tapering. They are best used as a bridge therapy for a few months, initiated concurrently with a steroid-sparing agent like methotrexate.

• C. Oral D-penicillamine: This was historically used for its antifibrotic effect but has fallen out of favor due to a lack of robust evidence for efficacy and a significant risk of serious adverse effects (e.g., nephrotoxicity, myasthenia gravis, pemphigus).

• D. Topical calcipotriol: Topical vitamin D analogs like calcipotriol have modest anti-proliferative and immunomodulatory effects and may be used for superficial, limited plaque morphoea. They are completely inadequate for deep, linear disease with functional impairment.

• E. Oral hydroxychloroquine: Hydroxychloroquine has mild immunomodulatory effects and is used for conditions like lupus and rheumatoid arthritis. However, it has no proven efficacy for halting the progression of active, deep morphoea and is not a standard treatment for this condition.

Key Associations for Morphoea Management

• Treatment is Indication-Based: Management is tailored to the subtype, location, depth, and activity of the disease. The goal is to control inflammation during the active phase to prevent irreversible damage and disability.

• First-Line for Active, Severe Disease: Methotrexate (0.3-0.5 mg/kg/week, max 25 mg/week) is the cornerstone. A typical regimen includes concurrent oral prednisone (e.g., 0.5-1 mg/kg/day) for 1-3 months, followed by a taper while the methotrexate takes full effect.

• Phototherapy: UVA1 phototherapy is a first-line option for adults and children with active plaque-type or generalized morphoea without deep involvement. It works by inducing collagenase and modulating T-cell function. It is less effective for very deep or linear disease affecting joints.

• Second-Line & Other Therapies:

  • Mycophenolate Mofetil: An effective alternative for patients who cannot tolerate or fail methotrexate.

  • Topical Therapies: High-potency corticosteroids or calcineurin inhibitors can be tried for limited, superficial plaques.

• Monitoring & Adjunctive Care:

  • Physical Therapy: Crucial for patients with linear disease across joints to maintain range of motion and prevent contractures.

  • Monitoring Disease Activity: This is done clinically by tracking lesion expansion, new lesions, and erythema. Tools like the LoSCAT (Localized Scleroderma Cutaneous Assessment Tool) can be used.

  • Surgical/Procedural Intervention: Surgery is reserved for stable, burned-out disease to correct contractures or cosmetic defects. Autologous fat transfer can be beneficial for facial atrophy.

• Prognosis: Plaque morphoea often resolves spontaneously over 3-5 years, leaving residual pigmentation. Linear and deep variants have a more chronic, progressive course and can cause permanent functional and cosmetic sequelae if not treated aggressively and early.