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Childhood scleroderma
Published in Aparna Palit, Arun C. Inamadar, Systemic Sclerosis, 2019
T. Muhammed Razmi, Rahul Mahajan
Linear scleroderma involving the face and scalp is also termed as en coup de sabre (Figure 8.10). It is unilateral and may also affect the underlying bone (Figure 8.11) and other organs causing seizures, uveitis, and other ocular and dental abnormalities. Cicatricial alopecia of the eyelashes, eyebrows, or scalp may occur if the lesion overlies these areas. Parry-Romberg syndrome is the progressive hemifacial atrophy (Figure 8.12) involving mainly the lower face with minimal or no skin surface changes. The absence of inflammation and sclerosis prior to the severe atrophy in Parry-Romberg syndrome prompt some researchers to consider it as a separate entity, and it is a close differential diagnosis of en coup de sabre,
Cicatricial alopecias: Pathogenesis, classification, clinical features, diagnosis, and management
Published in Jerry Shapiro, Nina Otberg, Hair Loss and Restoration, 2015
Morphea is a localized form of scleroderma. A linear form of morphea affecting the frontal scalp has been termed linear scleroderma en coup de sabre (Figure 6.30). Ocular and neurological abnormalities can be associated. Linear scleroderma represents the most common cause of cicatricial alopecia in early childhood. It may also be a milder presentation of the rare hemifacial atrophy (Parry–Romberg syndrome), and overlaps have been observed. In this condition, subcutaneous fat, muscle, and bone can be affected and the skin is only secondarily affected without prominent sclerosis.
Acquired monocular elevation deficit in a case of Parry–Romberg syndrome
Published in Strabismus, 2019
Xuhong Zuo, Lusheng Ma, Xvying Zhuo, Yuanbin Li
Parry–Romberg syndrome is a rare progressive hemiatrophy of the face that typically occurs in children and young adults and has a peculiar progression that ceases without apparent cause after a highly variable period.4 A wide variety of ophthalmologic findings have been described in up to one-third of cases.2 The most common periocular disorders include enophthalmos, eyelid, and orbit alterations. The most frequent ocular disorders include corneal and retinal changes, and the most common neuro-ophthalmological disorders involve optic nerve, pupillary dysfunction and incomitant strabismus.5–7
The development of multiple ocular complications in a patient with Parry‐Romberg syndrome
Published in Clinical and Experimental Optometry, 2019
Parry‐Romberg syndrome (PRS) is a rare condition that causes progressive but self‐limited atrophy of facial tissues, muscles, and osteocartilaginous and cerebral structures. PRS can present with a variety of periocular, ocular, neurological and systemic manifestations.2016 While the exact cause of PRS is unknown, it is thought to be due to an inflammatory process or dysfunction of the cerebral sympathetic nervous system.1997
Autologous fat transfer with SEFFI (superficial enhanced fluid fat injection) technique in periocular reconstruction
Published in Orbit, 2018
Benjamin Riesco, Cristina Abascal, Ana Duarte, Rocio Mariel Flores, Guillermina Rouaux, Raul Sampayo, Francesco Bernardini, Martin Devoto
C1: A 29-year-old female patient with Parry Romberg Syndrome (Figure 1a). A total of 24 mL of 0.8 SEFFI was used in the left superior, temporal, and inferior periorbital areas. The eyelids were treated with 6 mL of 0.5 SEFFI (Figure 1b). A satisfactory improvement was obtained at 1 year of follow-up (Figure 1c).