The syndrome of Stevens-Johnson (SJS) and toxic epidermal necrolysis (TEN) are serious, mostly medicated, cutaneous adverse reactions, normally linked to a high degree of morbidity and mortality. Recently, the SJS/TEN management guidance, the Indian guidelines and the United Kingdom directives have been written in details. However, there is no agreement on SJS/TEN management. In this paper, we would like to conceptualise SJS/TEN system in order of Indian schedule. It has been found useful to discontinue all drugs early, take help steps (hydration, electrolytes, and skin care denuded), corticosteroids, and cyclosporine. Oral provocation tests are only available to those receiving full remission that are strictly attentive during hospitalisation. As there is no majority, care on an individual basis should be personalised.

Nephronophthisis (NPHP) is an autosomal cystic kidney disease which is the most common hereditary disease. It is caused by mutations in 11 genes, known as nephrocystins (NPHP1-11, NPHP1L). With the identification of an increasing number of these genes, our knowledge of nephronophthisis changes and improves our comprehension of the pathomechanisms of NPHP. Ciliary expression of nephrocystins with other cystoproteins, such as polycystins 1 and 2, and fibrocystins have been documented in recent studies. These findings have directed our emphasis towards a pathomechanism with ciliary (ciliopathy) and planar cell polarity abnormalities (PCP). Furthermore, novel nephrocystin genes have been found to cause considerably larger than predicted illness spectrum of NPHP. In the same NPHP gene, various mutations might cause varied severity of the illness. In this study, we discuss about NPHP pathomechanisms and highlight the clinical heterogeneity of the illness. With the potential of oligogenicity in NPHP, the clinical range has grown even more complicated.