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Summary: These guidelines for the management of congenital ichthyoses have been developed by a multidisciplinary group of European experts following a systematic review of the current literature, an expert conference held in Toulouse in 2016, and a consensus on the discussions. These guidelines summarize evidence and expert‐based recommendations and intend to help clinicians with the management of these rare and often complex diseases. These guidelines comprise two sections. This is part two, covering the management of complications and the particularities of some forms of congenital ichthyosis. What's already known about this topic? Various symptomatic treatment options exist for congenital ichthyoses, but there are no European guidelines. What does this study add? These European guidelines for the management of congenital ichthyosis may help to improve outcomes and quality of life for patients. Linked Comment:Akiyama. Br J Dermatol 2019; 180:449–450. Plain language summary available online [ABSTRACT FROM AUTHOR]

Background IgA vasculitis (Ig AV) is assumed to be uncommon in adults. Objectives To determine the incidence rate of histologically proven Ig AV in the adult Slovenian population. Methods A retrospective chart review of adult patients diagnosed with Ig AV was performed at the departments of rheumatology, nephrology, infectious diseases and dermatovenereology at an integrated secondary/tertiary university teaching hospital. In order to avoid missing miscoded cases, the Institute of Pathology, University of Ljubljana, Slovenia, provided a list of all patients with an Ig AV-compatible histological pattern on biopsy. The annual incidence rate of histologically proven Ig AV was calculated. Results Eighty-one new cases of Ig AV were identified from June 2010 to June 2013. The estimated annual incidence rate of Ig AV was 5·1 per 100 000 adults [95% confidence interval ( CI) 3·4-7·4]; in men it was 6·1 per 100 000 (95% CI 3·9-10·6) and in women it was 3·7 per 100 000 (95% CI 1·8-6·8). Conclusions Although we only included histologically proven cases of Ig AV, the annual incidence rate of 5·1 per 100 000 adults is 3-6-times higher than previously reported. [ABSTRACT FROM AUTHOR]

Summary: Ichthyosis is the term used to describe continual and widespread scaling of the skin. There are several genetic types which are present from birth and persist life‐long – the "congenital ichthyoses". There is no agreed treatment ‐ different doctors use different approaches, so this group of experts from all over Europe worked together to develop guidelines. They reviewed the medical literature and met to discuss the evidence and to make recommendations. A separate paper (Part 1) covered treatments for the skin condition itself. This paper, Part 2, provides guidelines for managing complications of the congenital ichthyoses. Itch, pain and infections are common, particularly fungal infection, and cancers sometimes occur. Tight skin often prevents eyelids from shutting properly so the eye surface must be protected with lubricant; eyelid massage may help and retinoid medicine may soften tight skin but can dry the eyes further. Surgical options include injecting filler or grafting extra skin to the eyelid: if the patient's own ichthyotic skin is used the problem eventually recurs and mucous membrane from inside the mouth is a promising alternative. Hearing is commonly affected: blockage of ears by a build‐up of skin may be prevented by regular oil drops but syringing or suction may be necessary. In children, growth may be affected and vitamin D supplements are usually recommended. There are special considerations for newborn babies and those with complex ichthyotic disorders such as Netherton Syndrome, epidermolytic ichthyosis and Ichthyosis Prematurity Syndrome. These treatment guidelines should help to improve outcomes and quality of life for patients with congenital ichthyoses. Linked Article: Mazereeuw‐Hautier et al. Br J Dermatol 2019; 180:484–495 [ABSTRACT FROM AUTHOR]