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We report here 4 patients with restrictive dermopathy (RD) who presented in the paediatric and dermatology department of the University of Heidelberg from 1996 until 2017. All of the patients were premature infants displaying the pathognomonic clinical picture of RD: tense, vulnerable and translucent skin, superficial erosions, joint contractures, reduced motoricity and a typical facies with a small pinched nose, mouth fixed in an o-position, low-set ears and micrognathia (Fig. 1). Skin biopsies for light and electron microscopy showed collagen bundles parallel to the skin surface and a hypoplasia of appendages as common pathological findings. In 2 biopsies, the collagen fibres were small and in 3 of the 4 patients the rete ridges were flattened and elastic fibres were reduced or arranged in tiny clumps (Fig. 2b). All 4 babies developed respiratory insufficiency and died between days 6 and 32 after birth. (For more detailed information on each case see Table SI1). RD has a pathognomonic phenotype; however, the underlying structural skin changes remain to be defined. One possible explanatory approach may be a developmental arrest of extracellular matrix components, e.g. elastic fibres. As this hypothesis has not yet been addressed, immunohistochemical analysis of elastic fibres was performed in healthy newborns and those with RD.

Restrictive Dermopathy: Four Case Reports and Structural Skin Changes