Background Although diphenylcyclopropenone (DCP) is frequently used for the treatment of alopecia areata (AA), large studies with more than 100 patients are still scarce.
Objective To determine the ...efficacy of DCP immunotherapy in a large cohort of patients with AA who had been treated in our institute from January 2000 to December 2006.
Methods A total of 142 patients with AA undergoing topical DCP therapy in a self‐controlled design were evaluated retrospectively.
Results Seven patients (4.9%) were anergic to DCP. Two of 135 patients (1.5%) discontinued DCP therapy because of adverse effects. Fifty‐one patients (37.8%) had a complete response (CR: >90% re‐growth of hair), 20 patients (14.8%) exhibited a partial response (PR: >50–90% re‐growth), 26 patients (19.3%) experienced a minimal response (MR: 10–50% re‐growth) and 38 patients (28.1%) had no response after DCP therapy (NR: <10% re‐growth). Bivariate logistic analysis revealed that severity of hair loss at the beginning of DCP (P = 0.001) is the only significant prognostic factor for therapeutic outcome. Twenty‐three patients (45.1%) with CR had relapses upon discontinuation of the treatment or even during prolonged DCP therapy.
Conclusion Topical immunotherapy with DCP of patients with AA is rather effective and mostly well tolerated. The extent of hair loss before therapy is the main predictor for the therapeutic success of DCP. However, DCP therapy is associated with a high degree of relapse of which patients should be well informed.
The broad differential diagnosis of neonatal erythroderma often poses a diagnostic challenge. Mortality of neonatal erythroderma is high due to complications of the erythroderma itself and the ...occasionally severe and life‐threatening underlying disease. Early correct recognition of the underlying cause leads to better treatment and prognosis. Currently, neonatal erythroderma is approached on a case‐by‐case basis. The purpose of this scoping review was to develop a diagnostic approach in neonatal erythroderma. After a systematic literature search in Embase (January 1990 – May 2020, 74 cases of neonatal erythroderma were identified, and 50+ diagnoses could be extracted. Main causes were the ichthyoses (40%) and primary immunodeficiencies (35%). Congenital erythroderma was present in 64% (47/74) of the cases, predominantly with congenital ichthyosis (11/11; 100%), Netherton syndrome (12/14, 86%) and Omenn syndrome (11/23, 48%). Time until diagnosis ranged from 102 days to 116 days for cases of non‐congenital erythroderma and congenital erythroderma respectively. Among the 74 identified cases a total of 17 patients (23%) died within a mean of 158 days and were related to Omenn syndrome (35%), graft‐versus‐host disease (67%) and Netherton syndrome (18%). Disease history and physical examination are summarized in this paper. Age of onset and a collodion membrane can help to narrow the differential diagnoses. Investigations of blood, histology, hair analysis, genetic analysis and clinical imaging are summarized and discussed. A standard blood investigation is proposed, and the need for skin biopsies with lympho‐epithelial Kazal‐type related Inhibitor staining is highlighted. Overall, this review shows that diagnostic procedures narrow the differential diagnosis in neonatal erythroderma. A 6‐step flowchart for the diagnostic approach for neonatal erythroderma during the first month of life is proposed. The approach was made with the support of expert leaders from international multidisciplinary collaborations in the European Reference Network Skin‐subthematic group Ichthyosis.
Summary
Background
Oral liarozole, a retinoic acid metabolism‐blocking agent, may be an alternative to systemic retinoid therapy in patients with lamellar ichthyosis.
Objective
To demonstrate the ...efficacy and safety of once‐daily oral liarozole in the treatment of moderate/severe lamellar ichthyosis.
Methods
This was a double‐blind, multinational, parallel phase II/III trial (NCT00282724). Patients aged ≥ 14 years with moderate/severe lamellar ichthyosis Investigator's Global Assessment (IGA) score ≥ 3 were randomized 3 : 3 : 1 to receive oral liarozole (75 or 150 mg) or placebo once daily for 12 weeks. Assessments included: IGA; a five‐point scale for erythema, scaling and pruritus severity; Short Form‐36 health survey; Dermatology Life Quality Index (DLQI); and safety parameters. The primary efficacy variable was response rate at week 12 (responder: ≥ 2‐point decrease in IGA from baseline).
Results
Sixty‐four patients were enrolled. At week 12, 11/27 (41%; liarozole 75 mg), 14/28 (50%; liarozole 150 mg) and one out of nine (11%; placebo) patients were responders; the difference between groups (liarozole 150 mg vs. placebo) was not significant (P = 0·056). Mean IGA and scaling scores decreased from baseline in both liarozole groups at weeks 8 and 12 vs. placebo; erythema and pruritus scores were similar between treatment groups. Improvement in DLQI score was observed in both liarozole groups. Treatment with liarozole for 12 weeks was well tolerated.
Conclusions
The primary efficacy variable did not reach statistical significance, possibly owing to the small sample size following premature termination. However, once‐daily oral liarozole, 75 and 150 mg, improved scaling and DLQI and was well tolerated in patients with moderate/severe lamellar ichthyosis.
What's already known about this topic?
Oral liarozole, a retinoic acid metabolism‐blocking agent, may be an alternative to systemic retinoid therapy for patients with lamellar ichthyosis.
What does this study add?
While the primary endpoint was not met, compared with placebo, once‐daily oral liarozole, 75 or 150 mg, decreased overall severity and scaling, but not erythema and pruritus, and improved Dermatology Life Quality Index in patients with lamellar ichthyosis.
Oral liarozole was well tolerated.
Summary
Background Epidermolytic ichthyosis (EI) is a hereditary keratinization disorder caused by mutations in the keratin 1 (KRT1) or keratin 10 (KRT10) genes. In most cases of severe EI, ...heterozygous single point mutations are found at the highly conserved helix boundary motifs of KRT1 and KRT10 that play a critical role in filament formation. The presence of palmoplantar keratoderma suggests KRT1 mutations, whereas KRT10 mutations in most instances give rise to the nonpalmoplantar variants.
Objectives To identify the underlying mutations in patients with EI and to correlate genotype and phenotype.
Methods Mutation analysis was performed in 28 patients with EI by direct sequencing of KRT1 and KRT10 genes.
Results We identified 14 different mutations, of which four have not been published previously.
Conclusions Identification of novel mutations and genotype–phenotype correlations in EI allows improved understanding of disease pathogenesis as well as better patient management.
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
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