Two young girls present with nearly identical forehead lesions and mild alopecia. Do you recognize these signs? Can you Dx?
Background.Three young girls present with mild alopecia and also a similar mark on the forehead, like a scar from cut/slash, that is causing them distress.
Presentation Patient #1. A 14-year-old female presents complaining of a disfiguring forehead “dermatitis” that had worsened over 6 to 8 months. She has been an otherwise healthy child/adolescent.
History. Alopecia has worsened and forehead lesion, originally not noticeable, has deepened. Family, personal history noncontributory.
Physical Exam.Seen is a 5.0-cm hypopigmented atrophic, linear plaque-like lesion in the frontal-parietal area of the right forehead; lesion is somewhat thickened. Alopecia in frontal scalp.
Physical Exam. Seen is a 16-cm indurated, linear plaque on the right parietal scalp; mostly very pale yellow, with an erythematous advancing edge.
Presentation Patient #2. An 8-year-old female with a 7.0-cm darkened, atrophic, linear plaque-like lesion in midline of the forehead; lesion appears thickened; alopecia is seen in the frontal scalp.
Differential diagnosis. Discoid lupus, linear morphea/en coup de sabre, cicatraix, Parry- Romberg syndrome, systemic sclerosis, sclerederma, morphea profunda, chemical/toxin exposure, lichen sclerosis, melorheostosis
Diagnosis. Linear morphea. Localized disease distinct from scleroderma, the systemic disease. En coup de sabre describes linear morphea that affects the forehead.
Linear morphea. Unknown etiology, favors light skin/females; onset before age 18; associated with increased collagen, matrix in dermis; may involve cytokine release from small vessel damage.
Possible associations. Localized injury, tick bites, pregnancy, viral infections, autoimmune disease, lichen sclerosis/planus, medications.
Subtypes. En coup de sabre, circumscribed (plaque) morphea, generalized morphea, superficial morphea, linear morphea/scleroderma, progressive facial atrophy, pansclerotic disabling morphea, atrophoderma of Pierini and Pasini
Disease course. Highly variable and individual. Linear lesions more persistent than plaque-type lesions, but often improve over time. Monitor for potential progression.
Treatment. No known effective therapy; Rx aimed at reducing inflammatory activity in early disease is more successful than attempts to decrease sclerosis in well-established lesions.
Who should follow patients with morphea or scleroderma? The bottom line: the practitioner with the most experience should be “the captain of the ship,” but a logical approach is this: An experienced primary care doctor will ensure medical issues are dealt with and will provide (as needed) consultations to handle the above.
Several youths, all younger than age 15 years, present to the acute care clinic over a period of about a year with suprisingly similar signs that include alopecia and a disfiguring mark, like a scar from a slash, on their foreheads. Does this combination sound familiar?Â In the slides below, Dr Jonathan Schneider presents 2 of these interesting cases from the annals of his decades of practice in pediatric and adolescent medicine.