Kindler syndrome is an autosomal recessive disorder characterized by neonatal blistering, sun sensitivity, atrophy, abnormal pigmentation, and fragility of the skin. Linkage and homozygosity analysis in an isolated Panamanian cohort and in additional inbred families mapped the gene to 20p12.3. Loss-of-function mutations were identified in the FLJ20116 gene (renamed "KIND1" [encoding kindlin-1]). Kindlin-1 is a human homolog of the Caenorhabditis elegans protein UNC-112, a membrane-associated structural/signaling protein that has been implicated in linking the actin cytoskeleton to the extracellular matrix (ECM). Thus, Kindler syndrome is, to our knowledge, the first skin fragility disorder caused by a defect in actin-ECM linkage, rather than keratin-ECM linkage.

Onychomadesis is characterized by separation of the nail plate from the matrix with persistent attachment to the nail bed and often, but not always, eventual shedding. Onychomadesis has been associated with infection, autoimmune disease, critical illness and medications. To our knowledge a literature review of all associations with onychomadesis has not been completed previously. Most commonly, onychomadesis has been reported in association with pemphigus vulgaris and hand-foot-mouth disease, and following chemotherapy or antiepileptic medications. This article summarizes these key culprit associations, postulates the pathogenesis of nail matrix arrest and summarizes the clinical outcomes. We conducted a retrospective review of cases of onychomadesis reported from January 1960 to March 2013. Using the PubMed database, the literature was searched using the following terms: 'onychomadesis' and 'proximal nail shedding'. Also, an Ovid search was carried out using the same terms. In total 56 articles have been published, including our previously reported series of idiopathic onychomadesis. Articles pertaining only to Beau's lines and not true onychomadesis were excluded. Onychomadesis has been associated with autoimmune disease, other major medical illness, neonatal illness, medication and infection.

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Pyoderma gangrenosum (PG) is a sterile neutrophilic disorder that rarely affects children. Clinical, epidemiological, and therapeutic data on pediatric PG is poor as there are many newly reported associated diseases and drugs. This paper aims to review all recent available data on pediatric PG. A systematic review of the literature was conducted using Embase, Medline, and Cochrane databases. A total of 132 articles were included in the review. The most commonly reported underlying diseases in pediatric PG are inflammatory bowel diseases followed by hematologic disorders, vasculitis, immune deficiencies and Pyogenic Arthritis, Pyoderma gangrenosum and Acne (PAPA) syndrome. More than half of the cases occur with no underlying disease. The most frequently reported clinical presentation is multiple disseminated ulcers. Treatment should be tailored according to the underlying etiology. It includes systemic steroids, corticosteroid sparing agents such as dapsone and cyclosporine, and TNF-alpha inhibitors such as adalimumab and infliximab. Response to treatment is high with cure rates reaching 90%. A high index of suspicion and a thorough workup are mandatory in the management of pediatric PG.