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Case of annular pustular psoriasis/circinate erythematous psoriasis induced by hydroxychloroquine in a patient with systemic lupus erythematosus: possible association with CARD-14 mutation. De novo annular pustular psoriasis following mRNA COVID-19 vaccine COVID vaccine-induced pustular psoriasis in patients with previous plaque type psoriasis. [Extracted from the article]

Summary: Background: The Bullous Pemphigoid Disease Area Index (BPDAI) score has been proposed to provide an objective measure of bullous pemphigoid (BP) activity. Objectives: The objective of this study was to calculate BPDAI cut‐off values defining mild, moderate and severe BP. We also aimed to assess the interrater reliability and correlation with the number of daily new blisters, and anti‐BP180 and anti‐BP230 antibodies. Methods: Severity scores were recorded by two blinded investigators. Anti‐BP180 and anti‐BP230 antibodies were measured using an enzyme‐linked immunosorbent assay (ELISA). Cut‐off values defining mild, moderate and severe subgroups were calculated based on the 25th and 75th percentiles of the BPDAI score. Results: In total, 285 patients with BP were enrolled from 50 dermatology departments in Europe. Median BPDAI activity was 37·5 points (range 0–164). Cut‐off values corresponding to the first and third quartiles of the BPDAI score were 20 and 57, respectively; thus, these values were used to define mild (≤ 19), moderate (≥ 20 and ≤ 56) and severe (≥ 57) BP. The median BPDAI score for patients with ≤ 10 daily new blisters was 26 [interquartile range (IQR) 17–45], and for patients with > 10 daily new blisters the median score was 55 (IQR 39–82). The BPDAI intraclass correlation coefficient measured at baseline was 0·97 and remained higher than 0·90 up to month 6. The improvement in the BPDAI score was correlated with the absolute decrease in anti‐BP180 ELISA value (Spearman's rank r = 0·34, P < 0·004), but not with anti‐BP230 antibodies (r = 0·17, P = 0·15). Conclusions: This study suggests cut‐off values of 20–57 for BPDAI to distinguish mild, moderate and severe BP, and confirms that it is a robust tool to assess BP severity precisely. What is already known about this topic?The Bullous Pemphigoid Disease Area Index (BPDAI) is a new scoring system to measure bullous pemphigoid (BP) activity.The use of this score in clinical practice is limited by the absence of cut‐off values. What does this study add?Cut‐off values of 20 and 57 were established to distinguish mild, moderate and severe BP using the BPDAI.These disease activity subgroups could help physicians in the management of patients with BP. What are the clinical implications of this work?This study classified patients with BP into three subgroups (mild, moderate and severe) based on the BPDAI score.The BPDAI score is a robust tool to assess BP activity accurately, both at diagnosis and during the course of the disease, and could help physicians tailor treatment to disease activity, thereby providing better management of patients with BP. Linked Comment: Blome and Klein. Br J Dermatol 2021; 184:997–998. [ABSTRACT FROM AUTHOR]

On immunohistochemistry, the vascular growth stained positive for SARS-CoV-2 spike glycoprotein and only slightly positive for SARS-CoV-2 nucleocapsid protein (Fig. Coronavirus disease 2019 (COVID-19)-associated skin manifestations are protean.1 Likewise, the spectrum of anti-Severe Acute Respiratory Syndrome CoronaVirus-2 (SARS-CoV-2) vaccine-induced cutaneous reactions is constantly expanding.2 Although cases of eruptive pseudoangiomatosis (EP) and eruptive cherry angiomas have already been reported, both after COVID-19 and anti-SARS-CoV-2 vaccination,3 current evidence concerning SARS-CoV-2 and cutaneous angioproliferative disorders is limited. Agirgol I et al i .5 described the appearance of diffuse cherry angioma-like lesions on the upper body of a middle-aged man, who had previously developed an erythematous, papulovesicular - and later livedoid - rash during mild COVID-19. [Extracted from the article]

Summary: Background: The anti‐tumour necrosis factor (TNF)‐α adalimumab is the only licenced biologic for moderate‐to‐severe hidradenitis suppurativa (HS). No predictors of response have been identified so far. Objectives: To identify clinical parameters predicting response to adalimumab and confirm its efficacy/safety. Methods: The data of 389 patients with HS treated with adalimumab in 21 Italian centres were reviewed. Sex, age at onset/diagnosis/baseline, body mass index, smoking, phenotype, previous treatments, concomitant antibiotics and 'therapeutic delay', defined as the time from HS onset to adalimumab initiation, were assessed. Response to adalimumab and its impact on quality of life (QoL) were evaluated using the Hidradenitis Suppurativa Clinical Response (HiSCR) and the Dermatology Life Quality Index (DLQI) or the Visual Analogue Scale for pain (VAS pain), respectively. Logistic regression analysis was performed. Results: The therapeutic delay correlated to lack of response to adalimumab at week 16 [odds ratio (OR) 1·92 for therapeutic delay > 10 years; 95% confidence interval (CI) 1·28–2·89; P = 0·0016). HiSCR was achieved in 43·7% and 53·9% patients at week 16 and 52, respectively. Significant reductions in both DLQI and VAS pain were found between week 16 vs. baseline (P < 0·0001 for both) and week 52 vs. baseline (P < 0·0001 for both). Previous immunosuppressants inversely correlated to HiSCR at week 52 (OR = 1·74, 95% CI 1·04–2·91, P = 0·0342). Conclusions: Inverse correlation between therapeutic delay and clinical response was found, supporting early adalimumab use and providing evidence for a 'window of opportunity' in HS treatment. Adalimumab efficacy and safety were confirmed, along with patients' QoL improvement. Immunosuppressants could negatively influence the response to adalimumab inducing a switch to non‐TNF‐α‐driven pathways. What is already known about this topic? Adalimumab is an effective and safe biologic licenced for the treatment of moderate‐to-severe hidradenitis suppurativa (HS) after failure of conventional treatments.There are no reliable parameters that predict the clinical response to adalimumab in this disease. What does this study add? The therapeutic delay, defined as the time from HS onset to adalimumab initiation, significantly correlated to lack of clinical response to this drug, particularly at week 16 of treatment.This study suggests that using adalimumab in the early phases of HS should be highly encouraged. Linked Comment:Zouboulis. Br J Dermatol 2021; 184:10–11. Plain language summary available online [ABSTRACT FROM AUTHOR]

Summary: Background: The infection caused by the recently identified SARS‐CoV‐2, called coronavirus disease‐19 (COVID‐19), has rapidly spread throughout the world. With the exponential increase of patients worldwide, the clinical spectrum of COVID‐19 is being better defined and new symptoms are emerging. Numerous reports are documenting the occurrence of different cutaneous manifestations in patients with COVID‐19. Objectives: To provide a brief overview of cutaneous lesions associated with COVID‐19. Methods: A literature search was performed in the PubMed, Scopus and Web of Science databases up to 30 April 2020. This narrative review summarizes the available data regarding the clinical and histological features of COVID‐19‐associated skin manifestations. Results: The literature reports showed a great heterogeneity in COVID‐19‐associated cutaneous manifestations, as well as in their latency periods and associated extracutaneous symptoms. Pathogenic mechanisms are unknown, although the roles of a hyperactive immune response, complement activation and microvascular injury have been hypothesized. Based on our experience and the literature data, we subdivided the reported cutaneous lesions into six main clinical patterns: (i) urticarial rash; (ii) confluent erythematous–maculopapular–morbilliform rash; (iii) papulovesicular exanthem; (iv) chilblain‐like acral pattern; (v) livedo reticularis–livedo racemosa‐like pattern; and (vi) purpuric 'vasculitic' pattern. These six patterns can be merged into two main groups: the first – inflammatory and exanthematous – includes the first three groups listed above, and the second includes the vasculopathic and vasculitic lesions of the last three groups. Conclusions: The possible presence of cutaneous findings leading to suspect COVID‐19 puts dermatologists in a relevant position. Further studies are needed to delineate the diagnostic and prognostic values of such cutaneous manifestations. What is already known about this topic? The infection caused by the SARS‐CoV‐2, called coronavirus disease‐19 (COVID‐19), has rapidly spread throughout the world, becoming pandemic.The heterogeneous spectrum of COVID‐19‐associated cutaneous manifestations is based on preliminary reports of different types of skin lesions, leading to a need for clarity. What does this study add? A summary of the clinical and histological features of COVID‐19‐associated skin manifestations is provided.Six main clinical patterns can be identified: (i) urticarial rash; (ii) confluent erythematous–maculopapular–morbilliform rash; (iii) papulovesicular exanthem; (iv) chilblain‐like acral pattern; (v) livedo reticularis–livedo racemosa‐like pattern; and (vi) purpuric 'vasculitic' pattern. Plain language summary available online [ABSTRACT FROM AUTHOR]

The article offers information on a study related to the problems and pitfalls in differential diagnosis of multisystem non-Langerhans cell histiocytoses (MS-NLCH). Topics discussed include categories of MS-NLCH include foamy-cell NLCH, Reticulohistiocytoses, and Rosai-Dorfman disease; investigation of clinical, histopathological, ultrastructural and molecular features of patients in the study; and the patients developed progressive syndrome involving skin, bone and central nervous system.

Hidradenitis suppurativa (HS) is a chronic inflammatory disease of the hair follicle. Albeit, its pathogenesis, is multifactorial; HS is regarded as mainly auto-inflammatory in its origin.1 In particular, the high expression levels of interleukin (IL)-17A in the bloodstream2 and in lesional skin3,4 of patients with HS, allow us to consider secukinumab as a potential drug capable of arresting the inflammatory cascade that characterizes this complicated pathology. The HiSCR was achieved only by 10% of patients (3/31) at week 5, by 26% of patients (8/24) at week 16 and by 41 % of patients (7/17) at week 28. [Extracted from the article]

SAPHO syndrome, namely Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis, is a rare autoinflammatory chronic disease presenting with non‐infectious inflammatory osteitis, sterile joint inflammation and skin manifestations, including palmoplantar pustulosis and severe acne. The case of a 15‐year‐old boy affected by SAPHO syndrome and hidradenitis suppurativa (HS) is presented and discussed. Coexistence of these two diseases may represent a therapeutic challenge and this case confirms literature data reporting the efficacy of the combination of methotrexate and adalimumab in SAPHO complicated by HS. [ABSTRACT FROM AUTHOR]

The article presents a case study of demodex folliculorum longitudinal appearance with reflectance confocal microscopy in a 54-year-old patient with a pigmented lesion on the cheek.