As a family medicine practitioner, would you immediately refer out a patient with localized scleroderma, such as morphea, or manage them initially?

Management of Localized Scleroderma (Morphea) in Family Medicine

As a family medicine practitioner, you should refer patients with localized scleroderma (morphea) to dermatology, particularly for linear, deep, or generalized subtypes, while superficial plaque morphea may be managed initially with topical therapies before referral if no response occurs. 1

Initial Assessment and Triage

When evaluating a patient with suspected morphea, determine the subtype and severity to guide your referral decision:

Disease Classification That Determines Referral Urgency

Low-severity disease (superficial plaque morphea):

• Limited to superficial dermis 1
• Small, localized plaques without functional impairment 2
• No involvement of face, joints, or underlying structures 2

High-severity disease requiring immediate dermatology referral:

• Linear morphea (especially en coupe de sabre affecting the face) 3, 1
• Deep morphea extending to fascia, muscle, joints, or bone 1
• Generalized morphea with multiple body areas involved 1, 2
• Unilateral generalized morphea in children or adolescents 4
• Pansclerotic morphea 2
• Any morphea affecting the head (risk of neurological and ocular complications) 2

Initial Management Approach for Low-Severity Disease

For superficial plaque morphea only, you may initiate treatment while arranging dermatology consultation:

• Start mid-potent to potent topical corticosteroids 1
• Consider topical tacrolimus or calcipotriol as alternatives 1
• Arrange dermatology referral within 2-4 weeks for phototherapy consideration 1

Critical caveat: Even "low-severity" morphea requires dermatology involvement for phototherapy, which is part of standard treatment and cannot be provided in most family medicine settings 1

When to Refer Immediately to Dermatology

Refer urgently (within 1-2 weeks) for:

• Any linear scleroderma, as these have poor cosmetic and functional prognosis 5
• Pediatric patients with morphea, as childhood-onset disease is more aggressive and requires systemic therapy (methotrexate) 1, 4
• Adult-onset linear morphea, which shows more aggressive course with increased risk of systemic involvement 3
• Lesions causing growth retardation, muscle atrophy, flexion deformities, or poorly healing ulcerations 4
• Facial involvement (en coupe de sabre) due to risk of neurological and ocular complications 2

Why Dermatology Referral Is Essential

Morphea requires specialized assessment and treatment that exceeds typical family medicine scope:

• Disease activity and damage assessment requires validated instruments (LoSCAT) that dermatologists use 1
• First-line systemic therapy is methotrexate for linear, deep, and generalized morphea, which requires dermatology or rheumatology expertise for monitoring 1
• Methotrexate must be continued for at least 12 months after adequate response, requiring specialized long-term management 1
• Phototherapy is standard adjunctive treatment for even "low-severity" disease 1
• Second-line agents (mycophenolate mofetil, biologics, JAK inhibitors, IVIG) are used for refractory cases 1

Multidisciplinary Coordination After Referral

Once dermatology is involved, additional specialists may be needed:

• Physiotherapy for all patients with linear or deep morphea to prevent contractures 1
• Psychiatric counseling for patients with facial involvement or significant cosmetic impact 1
• Orthopedic surgery consultation once disease is inactive for functional reconstruction 1
• Plastic surgery for autologous fat transfer in inactive disease with cosmetic defects 1

Common Pitfalls to Avoid

Do not attempt long-term management of morphea in family medicine without dermatology involvement, as topical therapy alone is insufficient for most subtypes and delays in systemic treatment can lead to permanent damage 1, 2

Do not assume plaque morphea is benign - even superficial forms require dermatology assessment to confirm subtype and rule out deeper involvement 2

Do not miss childhood-onset disease, as pediatric morphea requires aggressive systemic therapy (methotrexate plus corticosteroids) that must be initiated early to prevent permanent deformity 1, 4

Do not confuse generalized morphea with systemic sclerosis - while progression to systemic disease is uncommon, this distinction requires dermatology expertise 5