Restrictive dermopathy (RD) is a congenital diso-Collagen bundles are oriented parallel to the epidermis and elastic fibers are absent (A: H & E, x 200, B: Verhoeff-van Gieson, x 200).
rder typified by an abnormal face, rigid skin and flexion contractures. This disorder was first described by Toriello in 1983, and Witt et al used the term ‘Strictive dermopathy 😮 describe two premature neonates in 1986.
Clinically, the disorder results in rigidity or tautn- ess of the skin causing fetal akinesia or hypokinesia deformation sequence (FADS). FADS induces limitation of movement at joints leading to contractures and in the chest results in pulmonary hypokinesia. Insufficient fetal swallowing of fluid induces polyhydramnios.
The natural history of the disease is typical. Pregnancy is complicated by polyhydramnios, premature birth, and fetal distress. The affected infants die soon after birth due to resoiratory insufficiency secondary to thoracic stiffness. canadian antibiotics
RD shows characteristic clinical features. The skin is taut, rigid and cutaneous superficial vessels can be seen through shiny skin. The face shows an absence of eyebrows, a small and pinched nose, micrognathia, a fixed ihaped open mouth, and low-set ears. Flexion contractures of the extremities, and dorsiflexion of the feet are also characteristic. The radiologic findings are variable. These include poorly mineralized skull with large fontanellae, thin dysplastic clavicles, and ribbon like ribs. The humerus and forearm bones are also overtubulated. Histopathologic findings show a relatively -thickened epidermis, a flat dermo-epidermal junction, an overall thinned dermis with hypoplastic appendages, decreased or absent elastic fibers with collagen fibers arranged in parallel to the epidermis, and an abnormally thick layer of subcutaneous fat.
The mechanism of this disease is not yet fully understood, but several hypotheses have been proposed. Witt et al suggested that altered collagen metabolism could represent abnormalities of skin, bone, and the fetal membranes which are seen in RD. In addition, Paige et al found that fibroblasts from the patients of RD showed poor growth in vitro, and did not produce normal collagen. They proposed that altered collagen metabolism produced an abnormal mature and stiff dermis. This abnormal collagen indicates an abnormally mature state of the skin collagen, and implies a profound decrease or complete arrest in collagen turnover. This causes a thin dermis with abnormally-arranged collagen bundles and lack of elastic fiber. buy cialis soft tabs
Holbrook et al suggested that modified dermo-epidermal interaction could be responsible for the development of RD. They found relative lack of differentiation-specific keratins in affected fetal skin, and suggested this abnormal keratin would lead to arrested development of hair follicles and eccrine sweat glands.