Plaque morphea with neurological involvement—an extraordinary uncommon presentation

Abstract

Localized scleroderma is traditionally considered to be limited to the skin, subcutaneous tissue, underlying bone, and in the craniofacial subtype, also nervous system involvement. However, recent studies have also described other systemic manifestations in these patients. Despite many reports of neurological involvement in patients with the craniofacial linear localized scleroderma, it is extremely rare in patients with the other subtypes of localized scleroderma. Here, we report an extraordinary case of localized scleroderma en plaque (classic morphea), located to the upper trunk and neck, associated with neurological manifestations presented as seizures. Magnetic resonance imaging of the brain showed focal lesions on the contralateral side to the skin involvement. This case is extremely relevant not only due to its rarity, but also because it supports the idea that the pathogenesis of the localized scleroderma is related to a systemic autoimmune process.

Neurological Manifestations in Parry–Romberg Syndrome

“… 85 While they are characteristically ipsilateral to the skin lesions, they may also be contralateral. 4 , 53 , 100 The above-mentioned abnormalities can be present among asymptomatic patients and new lesions may be found on follow-up brain MRI demonstrating subclinical progression in some cases. 1 MRA or cerebral angiograms may demonstrate vascular changes suggestive of vasculitis in some patients (11.5%), 1 , 28 , 73 hypoplastic arteries, 28 , 42 cerebral aneurysms, 2 and other vascular malformations, which could constitute the late sequelae of a vasculitic process.…”

Systemic involvement in localized scleroderma/morphea

“…The extraordinary case of superficial morphea of the trunk and neck with epilepsy and a MRI showing focal lesions on the contralateral hemisphere to the skin involvement has been reported. 39 Morphea can also cause psychologic disabilities. 32 Both children and adults may have higher levels of depression and anxiety compared to age-matched controls.…”

Revisão das Manifestações Extracutâneas da Esclerose Localizada

“…20,21 Os sintomas e achados imagiológicos são semelhantes na MGS e SPR, embora mais frequentes no último, o que é expectável dada a sua maior gravidade. 22 Nas formas lineares com envolvimento da cabeça o sistema nervoso central (SNC) é afetado em 18% -47% dos doentes. 21,23 As lesões neurológicas tipicamente ocorrem vários anos após as lesões cutâneas, embora possam ocorrer de forma síncrona ou até mesmo precedê-las.…”