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Background: Anti‐laminin‐γ1 (lam‐γ1) pemphigoid, a recently described immunobullous disorder sharing immune serological features of bullous pemphigoid and epidermolysis bullosa acquisita (EBA), is characterized by the detection of serum IgG autoantibodies against the lam‐γ1 chain, a 200 kDa heterotrimeric component of the dermal‐epidermal junction (DEJ). Objective: The aim of the study was to develop an easy‐to‐perform and reliable assay for the serological detection of anti‐lam‐γ1 IgG autoantibodies. The clinical appearance alone is not sufficient to establish diagnosis of anti‐lam‐γ1 pemphigoid and rather requires immune serological evidence of (i) IgG reactivity against the dermal portion of salt‐split human skin; (ii) exclusion of IgG against other components of the DEJ; and (iii) IgG reactivity with a 200 kDa protein of dermal extracts by immunoblot analysis (IB). Methods: The sera of 55 patients with anti‐lam‐γ1 pemphigoid were tested by IB with two recombinant heterotrimers, laminin 111 (lam‐111) and laminin 421 (lam‐421), as well as with a recombinant lam‐γ1 chain monomer. Additionally, a total of 41 control sera from patients with EBA (n = 15), psoriasis vulgaris (PV; n = 14), and healthy controls (HC; n = 12) were tested. Results: Immunoblot analysis revealed a positive reactivity with lam‐111 and/or lam‐421 in 46/55 (84%) of anti‐lam‐γ1 pemphigoid sera. Moreover, 8/9 of the initially non‐reactive sera were positive with the lam‐γ1 monomer, leading to an overall sensitivity of 98.2%. Analyses of 41 control sera with the three lam‐γ1 recombinants led to a specificity of 88%. Specifically, 3/15 EBA sera, 1/14 PV serum and 1/12 HC serum reacted with the lam‐γ1 monomer while only the 3 EBA sera reacted with lam‐421. Conclusions: Here we show a novel two‐step IB assay using the two recombinant laminin trimers and lam‐γ1 chain monomer for the detection of anti‐lam‐γ1 serum IgG with high sensitivity and specificity. This assay will facilitate the diagnosis and further characterization of this disease. Linked article: This article is commented on J. Yamagami et al., p. 632 in this issue. To view this article visit https://doi.org/10.1111/jdv.15555. [ABSTRACT FROM AUTHOR]

Cheilitis granulomatosa (CG) is a rare granulomatous disease of the lips, which has been first described in 1945 by Miescher, and can be associated with peripheral facial nerve palsy, lingua plicata, sarcoidosis, tuberculosis and inflammatory bowel disease.[1] The aetiology of CG is still not well understood, which also hampers treatment. The patient was treated with daily 40 mg oral prednisone that led to improvement but was accompanied by pronounced treatment-induced systemic side effects that occurred shortly after starting therapy. In conclusion, while successful treatment of CG can be very challenging, lenalidomide presents an interesting therapeutic option if other commonly applied drugs show no benefit or cannot be utilized due to unwanted side effects. [Extracted from the article]

Background The treatment of severe dermatological autoimmune diseases and toxic epidermal necrolysis ( TEN) with high-dose intravenous immunoglobulin ( IVIg) is a well-established procedure in dermatology. As treatment with IVIg is usually considered for rare clinical entities or severe clinical cases, the use of immunoglobulin is not generally based on data from randomized controlled trials that are usually required for the practice of evidence-based medicine. Owing to the rarity of the indications for the use of IVIg, it is also unlikely that such studies will be available in the foreseeable future. Because the high costs of IVIg treatment also limit its first-line use, the first clinical guidelines on its use in dermatological conditions were established in 2008 and renewed in 2011. Materials and methods The European guidelines presented here were prepared by a panel of experts nominated by the EDF and the EADV. The guidelines were developed to update the indications for treatment currently considered as effective and to summarize the evidence base for the use of IVIg in dermatological autoimmune diseases and TEN. Results and conclusion The current guidelines represent consensual expert opinions and definitions on the use of IVIg reflecting current published evidence and are intended to serve as a decision-making tool for the use of IVIg in dermatological diseases. [ABSTRACT FROM AUTHOR]