Paediatric onset lymphomatoid papulosis: results of a multicentre retrospective cohort study, on behalf of the EORTC Cutaneous Lymphoma Tumours Group (CLTG).

BACKGROUND: Lymphomatoid Papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population is scarce. OBJECTIVES: To characterize epidemiological, clinical, histopathological, and prognostic features of paediatric LyP. METHODS: This was a retrospective, multicentre international cohort study including 87 cases of children and adolescents with LyP diagnosed between 1998 and 2022. Patients aged ≤ 18 years old at disease onset were included. Diagnosis was made in each centre based on clinical-pathological correlation. RESULTS: Eighty-seven patients from 12 centres were included. The mean age at onset was 7.0 years (range 3 months-18 years) with a male to female ratio of 2:1. The mean time between onset of first cutaneous lesions and diagnosis was 1.3 years (range 0-14 years). Initial misdiagnosis concerned 26.4% of patients. Initially, LyP was most often misdiagnosed as Pityriasis lichenoides et varioliformis acuta (PLEVA), insect bites, or mollusca contagiosa. Erythematous papules or papulonodules were the most frequent clinical presentation. Pruritus was specifically mentioned for 20.7% of patients. The main histological subtype was type A in 55.1% of the cases. If analysed, monoclonal TCR rearrangement was found in 76.5% of the skin biopsies. The overall survival rate was 100% with follow up at 5 years available for 33 patients and at 15 years for 8 patients. A development of associated haematological malignancy (HM) occurred in 9.6% of the cases (7/73), including four mycosis fungoides (MF) cases, one primary cutaneous anaplastic large cell lymphoma (pc-ALCL), one systemic ALCL and one case of acute myeloid leukaemia. If we compare incidence rates of cancer with the world 0-19 years old population from 2001-2010, we estimate a significantly higher risk of associated malignancy in general, occurring before the age of 19 years old with incidence rate ratio of 87.49 (CI 86.01-88.99). CONCLUSIONS: We report epidemiological data from a large international cohort of children and adolescents with LyP. Overall the prognosis of the disease is good, with excellent survival rates for all patients. Due to increased risk of associated HM, a long-term follow-up should be recommended for LyP patients.

Topical cyclosporine hydrogel preparation: A new therapeutic option in the treatment of nail psoriasis.

Nail psoriasis is a chronic nail disorder that commonly affects psoriatic patients causing severe distress despite the limited body surface area. Treatments for nail psoriasis are limited, as nails are often difficult to treat with topical therapies, and among different systemic agents responses are unpredictable. We carried out a prospective study in order to analyze the effectiveness and tolerability of topical cyclosporine hydrogel ointment in nail psoriasis. Three patients, for a total of 44 nails, were treated with topical cyclosporine hydrogel ointment. All nails were evaluated, before starting the treatment, every 28 days and after 12 weeks of therapy, by the same dermatologists, through clinical and onychoscopic evaluations. The patients were also asked to assess on the compliance with product use. Complete response (CR) was observed in 2 of 3 patients; a partial response (PR) was observed in the other patient. Overall, 24 of 44 nails had CR and 20 had a PR. Cyclosporine hydrogel ointment has shown efficacy and safety in the treatment of nail psoriasis. The product has also been shown to be stable in composition, easy to apply and not discomfortable for the patient.

Update on the Management of Pediatric Psoriasis: An Italian Consensus.

INTRODUCTION: Psoriasis affects children with a considerable burden in early life. Treating pediatric psoriasis is challenging also because of the lack of updated specific guidelines. With the recent approval of several biologics for pediatric psoriasis and the ongoing COVID-19 pandemic, the management of young psoriatic patients is facing major changes. A revision of treatment recommendations is therefore needed. METHODS: In September 2021, a board of six Italian dermatologists convened to update treatment recommendations. The board issued evidence- and consensus-based statements covering relevant areas of pediatric psoriasis, namely: assessment of psoriasis severity, management of children with psoriasis, and treatment of pediatric psoriasis. To reach consensus, the statements were submitted to a panel of 24 experts in a Delphi process performed entirely via videoconference. A treatment algorithm was produced. RESULTS: There was full consensus that psoriasis severity is determined by the extension/severity of skin lesions, site of lesions, and impact on patient quality of life. Agreement was reached on the need for a multidisciplinary approach to pediatric psoriasis and the importance of patient/parents education. The relevance of vaccinations, including COVID-19 vaccination, for psoriatic children was acknowledged by all participants. Management issues that initially failed to reach consensus included the screening for psoriasis comorbidities and early treatment with biologics to prevent them and the use of telemedicine to facilitate patient follow-up. There was full consensus that topical corticosteroids are the first choice for the treatment of mild pediatric psoriasis, while phototherapy and systemic therapy are used in children with moderate-severe psoriasis. According to the proposed treatment algorithm, biologics are the first line of systemic therapy. CONCLUSIONS: Targeted systemic therapies are changing the treatment of moderate-severe pediatric psoriasis, while topical corticosteroids continue to be the first choice for mild disease. Children-centered research is needed to further improve the treatment of pediatric psoriasis.

A case report of tinea capitis in infant in first year of life.

BACKGROUND: Tinea capitis is a cutaneous fungal infection common among 3 to 7 year old children but it is rare in the first year of life. CASE PRESENTATION: We present a case of a 12-month-old infant with erythematous scalp lesions combined with hair loss. He was suspected of dermatophytosis and mycological analysis of all suspected lesions was performed. Clinical features and culture results confirmed tinea capitis caused by Microsporum canis. The infant patient was treated with griseofulvin for 2 months. However, 15 days later at the end of treatment he presented with a single vesicle positive for M. canis. Griseofulvin therapy continued for another month. After 3 months of follow-up, no recurrence was observed. CONCLUSIONS: In infant, sometimes tinea capitis is misdiagnosed and underreported because it is similar to other scalp pathologies. Therefore, if erythematous scalp lesions are present, they must be examined from a mycological point of view to inform the differential diagnosis. Once diagnosed, treatment of tinea capitis can pose a dilemma because different factors may influence the choice between equally effective therapies (i.e. safety, age, formulation, cost). This case report suggests that it is important to establish an accurate diagnosis and  treatment for this dermatophytosis to avoid recurrences or therapeutic failures, especially in infants.

Autoimmune connective tissue diseases and pregnancy.

Autoimmune connective tissue-diseases are more frequent in women and deserve a multidisciplinary approach in which the dermatologist play a major role together with other physicians. Pregnancy in these patients has to be considered a high-risk situation, because of possible worsening of the mother's disease and increased morbility and mortality for the fetus; also, therapies have to be chosen carefully because some drugs cannot be used during pregnancy. For all these reasons, the decision to become pregnant needs to consider the type of disease, stage of disease, age and clinical condition, and requires a multidisciplinary approach. A correct counselling, a close monitoring, a specific approach based on the risks involved and the use of appropriate therapies are the keys to obtain optimal pregnancy outcomes.

Juvenile psoriasis: an epidemiological study of 69 cases.

BACKGROUND: Psoriasis is a common inflammatory skin disease that affects people of every age; prevalence in pediatric population is unknown because of the lack of studies. METHODS: We collected data about patients 0-18 years old coming to our center in the period from November 2010 to September 2012. RESULTS: We found 69 children (38 males, 31 females) affected by psoriasis, with a male-to-female ratio of 1.2 and a prevalence of 0.75%. Plaque psoriasis was the most common clinical manifestation (72.54%), followed by guttate psoriasis (13%). The most frequent comorbidities were overweight and obesity (26%). Most patients had a mild disease with PASI<10 (72.5%). the majority had no or limited effects on quality of life derived from psoriasis. The most frequent involved areas were the extremities (58%), followed by the scalp (45%). Eighty-four percent of children were treated with emollient agents, 50.7% with topical keratolytics, 24.6% with local steroids, vitamin D analogues or combination. CONCLUSIONS: The number of young patients affected by psoriasis confirmed rare literature reports on the topic. Most children showed mild form of disease. Nearly every child with mild psoriasis treated with topical therapy obtained good results.

Treatment patterns with systemic antipsoriatic agents in childhood psoriasis: an Italian database analysis.

BACKGROUND: The majority of available systemic therapies have never been systematically investigated in moderate to severe childhood plaque psoriasis. For this reason, treatment preferences for moderate to severe psoriasis in childhood are still unknown. The aim of this study was to investigate the systemic treatment patterns of moderate to severe psoriasis in children and adolescents aged 18 or older in Italy. Additional secondary outcomes were duration of treatment and reasons for discontinuation. METHODS: In order to define differences in treatment patterns, we performed a chart review of all consecutive patients treated with systemic drugs during an index period of 5 years. Consecutive sampling of all patients with psoriasis aged ≤18 years, who had been treated with at least one systemic drug over a 5-year period, was made. RESULTS: The records of 58 consecutive patients, 27 males, 31 females. with moderate to severe psoriasis treated with at least one systemic therapy were reviewed. The median age (standard deviation) at the start of the first systemic treatment was 11.7±3.7 years. The most preferred first-line systemic treatment was cyclosporine, which was administered as first systemic treatment in 53.4% of patients, followed by acitretin in 22.4% of patients, etanercept and PUVA respectively in 8.6%, methotrexate in 6.8%. 48.2% of patients received a second systemic treatment due to inefficacy or side effects of the first-line therapy during the index period. Because of the small sample, and voluntary contribution, selection bias may have occurred. CONCLUSIONS: A considerable variation in the management of the first-line systemic therapy in children with moderate to severe psoriasis was observed. Cyclosporine was most commonly preferred as a first-line treatment. The availability of new therapeutic agents could change the scenario of treatment patterns in childhood psoriasis.

Effectiveness and Safety of Acitretin in Children with Plaque Psoriasis: A Multicenter Retrospective Analysis.

BACKGROUND: Acitretin is licensed for and is most commonly used to treat psoriasis. Little information exists about its efficacy and safety in childhood and adolescent psoriasis. METHODS: Retrospective analysis of a group of children and adolescents (<17 years of age) with moderate to severe plaque psoriasis treated with acitretin between 2010 and 2014 at Italian dermatology clinics. Patients were identified through databases or registries. RESULTS: The study population consisted of 18 patients with a median age of 9.5 years at the start of therapy. The median maintenance dosage per day was 0.41 mg/kg. Eight patients (44.4%) achieved complete clearance or good improvement of their psoriasis, defined as improvement from baseline of 75% or more on the Psoriasis Area and Severity Index at week 16. Three had three or more courses of treatment with short disease-free intervals. In three patients, acitretin treatment was ongoing at the time of data collection. The mean total duration of treatment in responders was 22.7 months. One patient discontinued treatment because of arthralgia. The remaining nine patients (50%) discontinued treatment because it was ineffective. Mucocutaneous adverse effects occurred in all patients, but did not affect therapy maintenance. CONCLUSIONS: In this retrospective case series, acitretin was a moderately effective, well-tolerated treatment in children with moderate to severe plaque psoriasis. Given the small number of patients, statements about long-term safety are not possible.

Advances in Microbiology, Infectious Diseases and Public Health: Refractory Trichophyton rubrum Infections in Turin, Italy: A Problem Still Present.

Dermatophytosis caused by Trichophyton rubrum is the most common cutaneous fungal infection in industrialized countries and worldwide with high recurrence and lack of treatment response. In addition, patients with cutaneous and concurrent toenail lesions are often misdiagnosed and therefore treated with an inappropriate therapy. In this study, we evaluated five previously misdiagnosed cases of T.rubrum chronic dermatophytosis sustained by two variants at sites distant from the primary lesion. Our patients were successfully treated by systemic and topical therapy, and 1 year after the end of therapy follow-up did not show any recurrence of infection.Our data indicate that the localization of all lesions, the isolation and the identification of the causative fungus are essential to establish the diagnosis and the setting of a correct therapeutic treatment to avoid recurrences.

A novel composition for in vitro and in vivo regeneration of skin and connective tissues.

The particular combination of polydeoxyribonucleotides, l-carnitine, calcium ions, proteolytic enzyme and other ingredients acts in a synergetic way in the regeneration of skin and connective tissues. This new formulation of active principles was tested in vitro as a cell and tissue culture medium and in vivo for various preparations in support of tissue regeneration. In vitro, the new blend allowed the maintenance of skin biopsies for more than 1 year in eutrophic conditions. Immunocytochemical analyses of fibroblasts isolated from these biopsies confirmed a significant increase of the epidermal and connective wound-healing markers such as collagen type I, collagen type IV, cytokeratin 1 (CK1), CK5, CK10 and CK14 versus controls. To examine the effects of the new compound in vivo, we studied impaired wound healing in genetically diabetic db/db mice. At day 18, diabetic mice treated with the new composition showed 100% closure of wounds and faster healing than mice treated with the other solutions. This complex of vital continuity factors or life-keeping factors could be used as a tissue-preserving solution or a cosmetic/drug/medical device to accelerate wound healing in the treatment of patients with deficient wound repair to promote the regeneration of cutaneous and connective tissues (injuries-wound, dermatitis) and prevent the recurrent relapses.

Non-dermatophyte moulds as skin and nail foot mycosis agents: Phoma herbarum, Chaetomium globosum and Microascus cinereus.

The increased prevalence of dermatomycoses along with the wide range of organisms now recognized as potential pathogens needs accurate laboratory isolation and identification of the aetiological agents. In this report three cases of foot dermatomycoses due to filamentous fungi commonly present in the environment with ubiquitous distribution are described in immunocompetent subjects. Skin and nail samples were collected, suspended in 20% KOH solution, examined under a light microscope and cultured in Mycobiotic agar and Sabouraud dextrose agar containing chloramphenicol to detect fungal growth. Phoma herbarum, Chaetomium globosum, and Microascus cinereus were isolated and identified.

Iron metabolism markers and haptoglobin phenotypes in susceptibility to HSV-1 or/and HSV-2 lesion relapses.

Different haptoglobin (Hp) phenotypes play a role in several pathologic processes including infectious diseases. In order to evaluate the role of iron storage and metabolism in susceptibility to herpetic manifestations, we studied the frequency of the Hp phenotypes and iron metabolism in patients affected by H. Simplex virus 1 or 2 (HSV-1 or HSV-2), compared with controls. Hp phenotype and iron metabolism were determined in 100 patients with recurrent HSV-1 or HSV-2 manifestations during the relapses, and in 110 healthy subjects. The frequencies of the three Hp phenotypes in the patient group compared to the control group were 18% versus 14.5% p = NS for Hp 1.1, 25% versus 40% p = 0.03 for Hp 2.2 and 57% versus 45.5% p = NS for Hp 2.1. All iron metabolism parameters tested showed significant differences between patients and controls; haemoglobin (Hb), ferritin, and serum iron were lower, while transferrin was higher in the patients than in controls. Reductions in iron availability may be a risk factor for relapsing lesions of HSV-1 or HSV-2. Hp 2.2 phenotype may offer some protection against the recurrence of Herpes labialis or genitalis manifestations.