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Langerhans cell histiocytosis, characterized by xanthomatous deposits in the absence of hyperlipidaemia. A 57-year-old man presented with a 4-year history of multiple enlarging coalescing brown-yellow papules, plaques and nodules on his face and body. XD is a very unusual non-Langerhans cell histiocytosis within the juvenile xanthogranuloma grouping, characterized by xanthomatous deposits in the absence of hyperlipidaemia. [Extracted from the article]

A retrospective cohort study on risankizumab dose optimization in adult patients with plaque psoriasis found that most patients experienced significant improvement in Psoriasis Area and Severity Index (PASI) scores following dose optimization, with rare adverse events reported. The study, conducted in Canada and Portugal, had a limited sample size and heterogeneous population, necessitating further research to generalize the findings. Risankizumab was shown to be highly effective in patients with moderate-to-severe plaque psoriasis, with a majority of male participants achieving PASI90 or PGA 0/1, and experiencing minimal adverse events. The study received ethical approval from relevant institutions. [Extracted from the article]

Pigmented epithelioid melanocytomas (PEM) are intermediate-grade melanocytic lesions with frequent lymph node involvement and rare metastases that tend to follow an indolent course with a favorable outcome. We report two unique cases of congenital PEM with PRKCA fusion transcripts: a multifocal PEM with an aggressive incompletely resectable scalp tumor and a solitary palmar PEM with newly reported ITGB5-PRKCA fusion. Through these case reports and a summary of previously reported cases, we outline the spectrum of disease of PEM and highlight the key clinical and histopathologic features associated with PEM with PRKCA fusion transcripts. We also discuss the treatment options and suggest that surgical excision without further adjuvant systemic treatment is reasonable first-line therapy given the favorable prognosis.

Background: Alopecia areata (AA) is an autoimmune non-scarring alopecia that affects the scalp or any hair-bearing areas in the body. The pathophysiology of AA is complex, but Th1, Th2, and Th17 cytokines dysregulation, as well as chemokines, immunoglobulins and other biomarkers have been shown to play a role in the pathogenesis of the disease., Objective: To conduct a systematic review and Meta-analysis to identify biomarkers that reflect AA activity and severity that could be used to better assess disease activity and response in both trials and clinical practice., Methods: A literature search was conducted using the PUBMED, EMBASE and the Cumulative Index to Nursing and Allied Health Literature (CINAHL) from inception to December 2021. Articles reporting on associations between AA and serum clinical biomarkers (cytokines, chemokines, antibodies, immunoglobulins, and others) were included. Serum biomarkers were identified in patients with AA and were correlated with disease severity and patient characteristics (ex. age, sex, comorbidities). The quality of the studies was assessed using the National Heart, Lung, and Blood Institute's Quality Assessment Tool for Case-Control Studies. Meta-analysis pooling of the standardized mean differences (SMD) by the method of Cohen using the common-effect inverse-variance model was performed. For the Meta-analysis, data was pulled for all the markers with a minimum of 4 studies with means and standard deviations. Analysis of data reported as Median with range or inter-quartile range (IQR) revealed that the data was too skewed to recommend calculation and use of mean with standard deviation (SD). If the data were not skewed, mean and SD were calculated., Results: One thousand seven hundred fourteen studies were screened, with 91 included, reporting on a total of 52 biomarkers. Meta-analyses revealed pooled SMD that were significant for interleukin 6 (IL6), C-reactive protein (CRP) and vitamin D., Conclusions: Serum IL6 and CRP levels are significantly increased in patients with AA compared to healthy age and sex matched controls. Conversely, serum vitamn D levels are significantly decreased in patients with AA compared to healthy age and sex matched controls. This data has the potential to influence the clinical guidelines for the diagnostic workup of AA to include testing the serum levels of CRP and vitamin D., Competing Interests: Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Cathryn SIbbald reports a relationship with Pfizer that includes: consulting or advisory, funding grants, and speaking and lecture fees. Cathryn SIbbald reports a relationship with AbbVie Inc. that includes: consulting or advisory. Cathryn SIbbald reports a relationship with LEO Pharma Inc. that includes: consulting or advisory. Cathryn SIbbald reports a relationship with Novartis that includes: speaking and lecture fees. Cathryn SIbbald reports a relationship with Arcutis Biotherapeutics, Inc. that includes: speaking and lecture fees., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Pediatric tinea capitis displays a wide range of prevalence, with significant variability among populations. We retrospectively extracted the medical records of 456 pediatric patients diagnosed with tinea capitis during the years 2010-2021, from the dermatology outpatient clinics in two tertiary medical centers. Three species were isolated in 90% of patients: T. tonsurans , M. canis , and T. violaceum. While T. tonsurans presented a six-fold increase in incidence during the years 2019-2021, M. canis maintained stable incidence rates. Furthermore, terbinafine was the most efficient antifungal agent against T. tonsurans , achieving complete clinical clearance in 95% of patients, as compared to fluconazole (68%) and griseofulvin (38%) ( p < 0.001). The mycological cure was recorded in 61/90 (68%) of patients with available data, at an average of 10 weeks. For patients with M. canis , griseofulvin and fluconazole were equally efficient (73% and 66%, respectively) ( p = 0.44). Kerion was described in 36% and 14% of patients with T. tonsurans and M. canis , respectively, ( p < 0.001). In conclusion, since 2019, there has been a significant increase in the prevalence of T. tonsurans , establishing this pathogen as the most common cause for tinea capitis in our population. Our data suggest that terbinafine is effective and presents high cure rates for tinea capitis in the pediatric population.

Background: Palmoplantar pustulosis (PPP) and palmoplantar pustular psoriasis (PPPP) are chronic inflammatory skin conditions characterized by eruptions of sterile pustules on the palms and/or soles. Biologic use has been associated with PPP and PPPP development in the literature., Objectives: To identify PPP and PPPP associated with biologics and summarize reported treatments and outcomes., Methods: We systematically searched in MEDLINE and Embase for articles that reported PPP or PPPP during biologic treatment. After a full-text review, 53 studies were included for analysis., Results: We identified 155 patients with PPP/PPPP onset during biologic treatment, with a mean age of 44.1 years and a female preponderance (71.6%). The most frequently reported biologics were adalimumab (43.9%) and infliximab (33.3%). IL-17 inhibitors, secukinumab (7.6%) and brodalumab (1.5%), were reported only in association with PPPP. Overall, 58.8% of patients had complete remission (CR) in 3.6 months and 23.5% had partial remission (PR) in 3.7 months. The most common treatments that led to CR were topical corticosteroids (n = 16) and biologic switching (n = 8)., Conclusions: Clinicians should anticipate PPP or PPPP as potential drug reactions to biologics such as adalimumab and infliximab. Large-scale studies are required to confirm our findings and further explore the pathogenesis for biologic-associated PPP and PPPP., (© 2022 the International Society of Dermatology.)

Introduction: Angiosarcoma developing in chronically lymphedematous tissue, or Stewart-Treves syndrome (STS), is a rare and lethal complication of lymphedema. This systematic review summarizes characteristics and outcomes of STS and other cutaneous malignancies arising in chronic lymphedema., Methods: MEDLINE and EMBASE databases were searched on February 19th, 2021, to identify 200 articles included in the analysis., Results: Of 369 included patients, 89.7% (n = 331/369) had STS and 10.3% (n = 38/369) had other associated malignancies. Mean age of onset was 61.2 years, and 85.9% (n = 317/369) of cases were female. Common risk factors were previous cancer history (69.8%, n = 258/369) and radiation history (53.7%, n = 198/369). Lymphedema was most commonly attributed to surgical causes (68.3%, n = 252/369). STS begins on average 14.9 years after lymphedema with mortality of 53.9% (n = 178/331) and remission rate of 16.1% (n = 53/331). Other malignancies begin on average 23.7 years after lymphedema, with mortality of 10.5% (n = 4/38) and remission rate of 31.6% (n = 12/38). STS and other malignancies had 5-year survivals of 22.4% and 65.2%, respectively (P = 0.00145). For all patients, patients initially treated with excision had the best survival (median: 48 months, 5-year survival: 43.3%) and radiotherapy had the worst survival (median: 10 months, 5-year survival: 6.5%) (P = 0.0141)., Conclusion: Malignancy can appear in lymphedematous tissue many years after lymphedema onset. STS has poorer prognosis compared to other malignancies. Further research should be conducted to better understand the causes, risk factors, and management of this phenomenon., (© 2021 the International Society of Dermatology.)

Competing Interests: Dr. Jensen Yeung has been a speaker, consultant, and investigator for AbbVie, Allergan, Amgen, Astellas, Boehringer Ingelheim, Celgene, Centocor, Coherus, Dermira, Eli Lilly, Forward, Galderma, GSK, Janssen, Leo, Medimmune, Merck, Novartis, Pfizer, Regeneron, Roche, Sanofi Genzyme, Takeda, UCB, Valeant, and Xenon. Dr. Ronald Vender has participated in clinical trials, and has received honoraria for acting as a consultant and/or as a speaker at events sponsored by 10.13039/100006483AbbVie, 10.13039/100002429Amgen, Boehringer Ingelheim, 10.13039/100006436Celgene, 10.13039/501100009754Galderma, GSK, Janssen, LEO Pharma, Eli Lilly, MSD, 10.13039/100004336Novartis, 10.13039/100011110UCB, and 10.13039/100004319Pfizer. Dr. Vimal H. Prajapati has been an advisor, consultant, investigator and/or speaker for AbbVie, Actelion, Amgen, Aralez, Arcutis, Asana, Aspen, Bausch Health, Boehringer Ingelheim, Bristol-Myers Squibb, Celgene, Cipher, Concert, Dermira, Eli Lilly, Galderma, GlaxoSmithKline, Homeocan, Incyte, Janssen, LEO Pharma, L'Oreal, Medexus, Novartis, Pediapharm, Pfizer, Regeneron, Sanofi Genzyme, Sun Pharma, Tribute, UCB, and Valeant.

Abstract: Patch testing, used in the assessment of allergic contact dermatitis, is ideally avoided in patients receiving immunosuppressive therapy because of concerns with reductions in accuracy; however, this is not well characterized in the literature. This systematic review summarizes patch testing results in patients receiving immunosuppressive therapy. We identified 16 studies, comprising 195 patients with dermatitis or psoriasis, who were patch tested while receiving immunosuppressants. Of these, 7 studies, comprising 85 patients with dermatitis, patch tests were performed before and during immunosuppression. Overall, 67.9% (n = 19) of the dermatitis patients receiving dupilumab maintained positive reactions to an allergen that previously graded as a 2+/3+ reaction. Several immunosuppressants were also associated with positive patch test results for various allergens. These include dupilumab, cyclosporine, and low-dose prednisone (≤10 mg/d) for dermatitis, and tumor necrosis factor α inhibitors, ustekinumab, and methotrexate for psoriasis. Ideally, it is preferable to patch test when patients are not receiving oral immunosuppressants or immunomodulators. However, clinicians may choose to assess the risks and benefits of patch testing for each patient given the impact of allergic contact dermatitis on patient quality of life., Competing Interests: J.Y. has been a speaker, consultant, and investigator for AbbVie, Allergan, Amgen, Astellas, Boehringer Ingelheim, Celgene, Centocor, Coherus, Dermira, Eli Lilly, Forward, Galderma, GSK, Janssen, Leo, Medimmune, Merck, Novartis, Pfizer, Regeneron, Roche, Sanofi Genzyme, Takeda, UCB, Valeant, and Xenon. The other authors have no funding or conflicts of interest to declare., (Copyright © 2021 American Contact Dermatitis Society. All Rights Reserved.)

Squamous cell carcinoma (SCC) can present as a rare complication of longstanding hidradenitis suppurativa (HS) lesions. Limited data exist on characteristics and outcomes of SCC development within HS lesions. The purpose of this review is to conduct a literature review of previously reported cases of SCC development in pre-existing HS lesions. EMBASE and MEDLINE searches were conducted in OVID on June 25, 2020, to yield 59 studies. Of the 95 patients (mean age: 52.9 years) who developed a total of 122 SCCs within HS lesions, the majority were males (77.9%, n = 74/95). The most common sites of SCC were the gluteal region (47.5%, n = 58/122), the perianal region (18.9%, n = 23/122), and the genitals (13.9%, n = 17/122). The mean duration between HS onset and SCC development was 25.5 years. Of the patients that had outcomes reported, 54.0% (n = 34/63) experienced SCC metastasis, 43.1% (n = 28/65) experienced SCC recurrences, and 58.7% (n = 44/75) experienced a mortality outcome. The most common documented reasons for death included metastasis (34.1%, n = 15/44) and sepsis (13.6%, n = 6/44). Patients with longstanding HS lesions can develop SCCs with a high rate of metastasis and mortality. Thus, chronic HS lesions, especially in the gluteal, perianal, genital, and perineal regions, should be monitored regularly for timely SCC diagnosis and management., (© 2021 the International Society of Dermatology.)

Competing Interests: Dr Yeung has been a speaker, consultant, and investigator for AbbVie, Allergan, Amgen, Astellas, Boehringer Ingelheim, Celgene, Centocor, Coherus, Dermira, Eli Lilly, Forward, Galderma, GSK, Janssen, Leo, Medimmune, Merck, Novartis, Pfizer, Regeneron, Roche, Sanofi Genzyme, Takeda, UCB, Valeant, and Xenon. Drs Lytvyn, Zaaroura, Mufti, and AlAbdulrazzaq have no conflicts of interest to declare.

Background: We have encountered three cases of follicular eruptions with folliculotropic infiltrates of non-atypical lymphocytes associated with anti-tumor necrosis factor alpha (TNF-α) therapy., Methods: Three patients aged 15 to 56 years treated with anti-TNF-α therapy (one with adalimumab, and two with infliximab) developed follicular eruptions characterized histopathologically by folliculotropic lymphocytic infiltrates. These were studied clinically, histopathologically, immunophenotypically, and molecularly., Results: All three cases were characterized histopathologically by folliculotropic cell infiltrates of non-atypical T (CD3+) lymphocytes with variable follicular exocytosis. Marked reduction in CD7 staining and marked predominance of CD4+ cells over CD8+ cells were observed in 1 and 2 cases, respectively. T-cell receptor (TCR) gene rearrangement studies were monoclonal in 1 case. Discontinuation of anti-TNF-α therapy in all three cases, with corticosteroid creams in 1 case, led to complete resolution. Rechallenge with adalimumab in 1 case resulted in exacerbation. Replacement of therapy with non-anti-TNF-α biologic agents in 2 cases was not associated with recurrence., Conclusion: Follicular eruptions with folliculotropic lymphocytic infiltrates associated with anti-TNF-α therapy may show some immunophenotypical variations and/or monoclonal TCR gene rearrangements but lack sufficient cytomorphological features of folliculotropic MF. They may resolve with discontinuation of anti-TNF-α therapy., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Pigmented contact dermatitis (PCD) is a noneczematous variant of allergic contact dermatitis, and benzyl salicylate is one of its causes. This type of PCD shows nonlichenoid interface dermatitis with pigment incontinence. We aimed to characterize the earliest histopathological changes of this reaction. A 51-year-old man presented with persistent facial eruption composed of hyperpigmented and hypopigmented macules due to exposure to benzyl salicylate present in his aftershave. The biopsies obtained from hyperpigmented and hypopigmented macules, and from the positive patch test site to benzyl salicylate, showed a nonlichenoid focal vacuolar interface dermatitis with mononuclear cells in the papillary dermis and around the pilosebaceous units, along with melanophages. A MART-1 immunostain showed intact melanocytes in all 3 biopsies. A Fontana-Masson stain demonstrated intact melanin in the basal cell layer of a facial hyperpigmented macule and the patch test site, but melanin was reduced in the biopsy taken from a hypopigmented facial macule. There were more epidermal and dermal CD1a+ Langerhans cells in the patch test biopsy than in the other 2 biopsies. Most of the mononuclear cells were CD3+. The CD4+ to CD8+ ratio was approximately 1:1 in the facial macules; yet, CD4+ cells outnumbered CD8+ cells in the patch test biopsy. There were a few TIA-1+ cells in all 3 biopsies. In conclusion, the earliest histopathological and immunophenotypical events in PCD due to benzyl salicylate are similar to those of longer-standing lesions, i.e., a nonlichenoid focal interface dermatitis involving the epidermis and pilosebaceous unit, along with dermal melanophages.

Background: Periodic acid-Shiff (PAS) stain may help to diagnose fungal infection in biopsies from dermatoses of the palms and soles. It is questionable whether PAS stain should be used routinely or only when tinea is suspected clinically., Methods: A total of 195 consecutive punch biopsies of dermatoses from the palms (90) or soles (105) were stained with PAS, regardless of the clinical differential diagnosis., Results: PAS stain showed fungi in the corneal layer of 6 (3%) of the 195 biopsies. Tinea was included in the differential diagnosis in 48 cases, of which 3 (6%) were PAS positive. PAS stain was also positive in 3 (2%) of 147 cases in which tinea was not suspected clinically. All 6 PAS-positive cases were detected in reaction patterns not readily classified as particular diagnostic entities: non-inflammatory keratoderma (2, 11%), chronic lichenified dermatitis (2, 6%), spongiotic psoriasiform dermatitis (1, 2%), and spongiotic dermatitis (1, 4%)., Conclusions: There is a low concordance rate between the clinical suspicion and the actual demonstration of fungi by PAS stain in dermatoses of the palms and soles. Routine PAS stains in non-suspected cases have a relatively low yield, which may be improved by limiting the PAS stain to reaction patterns not readily classified as particular diagnostic entities., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Staphylococcus lugdunensis is an emerging pathogen in skin and soft tissue infections that was previously considered a commensal. The aim of this study was to elucidate the characteristics of skin infections by S. lugdunensis and its appropriate management, in a tertiary referral medical center. The clinical files, bacterial cultures and histopathology reports of all S. lugdunensis isolates from skin infections over a period of 8 years (September 2009-September 2017) were reviewed. S. lugdunensis was isolated from 29 patients with skin infections, aged 7-89 years (mean 33.3 years). A state of immune suppression (drug-induced, malignancy or diabetes) was present in five patients (17%). Folliculitis and cutaneous pustulosis were the most common presentations (16 cases, 55%), followed by secondary infection of hidradenitis suppurativa (five cases, 17%). Other sources of isolation were infected molluscum contagiosum (two cases), folliculitis decalvans (one case), dissecting cellulitis (one case), abscess (one case), cyst (one case), impetigo (one case) and granuloma after trauma (one case). The in vitro antibiotic sensitivity tests showed susceptibility to most tested antibiotics, although a few isolates were resistant to gentamycin, penicillin and oxacillin. In 19 of 20 patients for whom follow ups were available, cutaneous manifestations improved or resolved with proper local and/or oral antibiotic therapy. S. lugdunensis may play a role as a primary or secondary pathogen in various skin infections, most commonly folliculitis and pustulosis. Proper antibiotic therapy may lead to improvement or resolution., (© 2018 Japanese Dermatological Association.)

Background: Several cases of pityriasis lichenoides (PL) have been reported to evolve into mycosis fungoides (MF)., Objective: To elucidate clues to this progression., Methods: Fifty-eight patients with PL between 2000 and 2013 (follow-up: 3-16 years, average: 8.3)., Results: A total of 3 (5.2%) of the 58 patients with PL developed MF after 3-11 years of prolonged clinical course. Papules and small plaques characterized PLs, and patches and larger plaques subsequent MFs. A total of 35 of 41 (85%) followed up non-MF associated patients with PL reported lasting complete remissions. Histopathologically, apoptotic keratinocytes disappeared mostly or completely in subsequent MFs. The presence of epidermotropism, folliculotropism, and epidermal lymphocytic nuclear atypia in PLs was not predictive of MF. CD8 cells were the dominant intraepidermal lymphocytes in the 3 PLs but remained so in only 1 subsequent MF. CD7 lymphocytes decreased substantially in 2 MFs, and lymphocytic nuclear atypia increased markedly in 1. T-cell receptor gene rearrangement studies demonstrated clonal populations in 1 of 2 studied PLs and in all 3 subsequent MFs., Conclusions: A few PLs may evolve into MF. Prolonged clinical course, appearance of patches and larger plaques, markedly increased lymphocytic nuclear atypia, marked diminution of apoptotic keratinocytes and CD7 and CD8 lymphocytes, and clonal T-cell receptor gene rearrangement may serve as clues.

Pseudolymphomatous reactions have been described to occur in tattoos. Most cases have exhibited T-cell predominance and polyclonal T-cell receptor gene rearrangements. One case with monoclonal IgH gene rearrangements progressed into B-cell lymphoma. Lichenoid infiltrates are commonly described but lymphoid follicles much less frequently. We report a case with mixed lichenoid and follicular T- and B-cell reaction to red tattoos. The histopathology and the immunohistochemical studies were constant with a mixed T- and B-cell pseudolymphoma, the IgH gene rearrangement study was polyclonal, but the T-cell receptor gene rearrangement study was monoclonal. The patient who responded to intralesional corticosteroid injections remains under close scrutiny.