Do patients with linear morphea of the forehead require referral to neurology and ophthalmology?

Neurological and Ophthalmological Referral for Linear Morphea of the Forehead

Patients with linear morphea of the forehead (en coup de sabre) should be referred to both neurology and ophthalmology for evaluation due to the high risk of extracutaneous involvement affecting underlying structures. 1

Understanding Linear Morphea of the Forehead

• Linear morphea of the forehead, also known as en coup de sabre (ECDS), is a rare variant of morphea characterized by progressive sclerosing inflammation of the skin tissue in the frontal region and scalp 1, 2
• The condition typically presents as a unilateral linear depression on the frontal or frontoparietal forehead 1
• While it commonly begins in childhood, adult-onset cases do occur 1
• The sclerosis in ECDS lesions can invade deeply to involve underlying muscle and bone, and may exist on the same clinicopathologic spectrum as Parry-Romberg syndrome 1

Rationale for Neurological Referral

• Extracutaneous involvement is frequent in ECDS, with neurological findings being among the most common manifestations 1
• The lesion can grow toward underlying structures, causing neurological symptoms such as headaches and seizures 2
• Early diagnosis and intervention are crucial to prevent cranial progression and avoid secondary neurological complications 2
• The American Academy of Ophthalmology recognizes that distinguishing between conditions with similar presentations (like skew deviation) is crucial, as they may indicate serious brainstem pathology 3

Rationale for Ophthalmological Referral

• Ophthalmologic findings are among the most common extracutaneous manifestations in ECDS 1
• The American Academy of Ophthalmology recommends complete ophthalmic examination with emphasis on sensorimotor evaluation when there are concerns about potential neurological involvement 4
• Careful assessment for neuro-ophthalmic signs and symptoms is essential, including checking for Horner's syndrome, cranial nerve palsy, and nystagmus 4
• Fundus examination to check for papilledema or optic atrophy is recommended as part of a comprehensive evaluation 4

Management Approach

• First-line treatment for the underlying ECDS typically includes methotrexate and oral glucocorticoids 1, 2
• Methotrexate has shown efficacy in treating ECDS, with improvement typically seen within an average of 2 months 5
• For cosmetic correction of atrophic lesions, options include corrective surgery, autologous fat grafting, hyaluronic acid filler injections, and micronized acellular dermal matrix fillers 6
• Early diagnosis and treatment are essential to minimize morbidity such as joint contracture in cases involving other body areas 7

Important Considerations

• Disease activity assessment through clinical evaluation or histologic evidence should precede any cosmetic correction procedures 6
• The etiology of ECDS remains unclear but may be autoimmune in origin 1
• Despite adequate pharmacological treatment, ECDS can present a recurrent course and cause long-term sequelae 2
• Regular follow-up with both neurology and ophthalmology is recommended to monitor for development of new extracutaneous manifestations 1, 2