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.

Rare and common manifestations of COVID-19 in children.

Introduction: It has been almost 2 years since the first reports on cutaneous manifestations of COVID-19. Those reported in children are different and include macular, papular, lichenoid, vesicular, urticarial, and vascular morphologies, among others. The prognosis of isolated cutaneous involvement in COVID-19 in children is usually self-limiting but the extreme variety of clinical presentations complicates the clinical approach. Methods: Numerous reviews have been systematically drafted and edited giving the clinicians a future direction for skin presentations during pandemics. Results and Discussion: Hereby we report the rare and common manifestations of COVID-19 in children and question the recurrence phenomena and age-related distribution of the eruptions.

Papulo-purpuric dermatitis of childhood: a distinct PLEVA-like eruption associated to SARS-CoV-2 infection. Clinical, histopathological and immunohistochemical study of 10 cases.

We observed ten children with a papular eruption with purpuric features during the SARS-CoV-2 pandemic in Northern Italy (May-December 2020). Histological examination showed signs of SARS-CoV-2-related dermatosis. Evidence of nucleocapsid viral proteins using SARS-CoV-2 (2019-nCoV) nucleocapsid antibody revealed cuticular staining of the deep portion of the eccrine glands in all cases.

Intrauterine big toenail imprints: A new cause of congenital skin dimples.

Congenital skin dimples (SD) are small cutaneous depressions that can be noted on any part of the body and may be caused by traumatic, mechanical, metabolic, and genetic factors as well as by exposure to infections or drugs. We describe 3 cases of unrelated healthy newborns displaying SD and discuss as a possible explanation the persistent friction of the big toenail onto the immature skin of the fetus during intrauterine life causing as depression in the skin.

Infantile digital fibromatosis: a rare tumour of infancy. Report of five cases.

Infantile digital fibromatosis (IDF) is a rare tumour of infancy with a typical clinical presentation and characteristic histopathological findings. Despite an alarming appearance, IDF does not cause deep infiltration or metastasis. The traditional approach of surgical resection was recently challenged by increasing evidence of self regression in months or years. We describe the clinical history of five patients with IDF that were followed in our departments for 5 years.

PENS syndrome: a new neurocutaneous phenotype.

BACKGROUND: The central nervous system originates ontogenetically from the ectoderm and therefore numerous neurocutaneous syndromes exist. OBJECTIVE: We describe a novel neurocutaneous syndrome with characteristic epidermal papules and plaques associated with mild psychomotor retardation and epilepsy with a good prognosis. METHODS: We investigated history, clinical features, laboratory parameters including karyotype, histopathological and immunohistological examination, electron microscopy, HPV infection, EEG and neurologic imaging in patients with an unusual symptom complex. RESULTS: Six children are reported with characteristic keratotic papules and plaques of 'gem-like' appearance, randomly distributed on the trunk and the proximal part of the limbs. Four patients were unrelated and two were siblings. The cutaneous lesions appeared congenitally or developed soon after birth. The skin lesions were rounded or of an irregularly polycyclic shape, ranging from 0.5 to 1.5 cm in diameter. A few to eighteen lesions were present. The lesional surface was rough and of white or yellowish color. Histopathological examination showed acanthokeratosis, but immunostaining for keratins and melanocytes, electron microscopy and laboratory results were within normal range. During the natural course of the disease the lesions were stable and asymptomatic. All but one of our six patients developed neurologic signs and symptoms during the first year of life, in the form of epilepsy and psychomotor delay. Five children had low intellectual performance together with EEG abnormalities and some had MRI changes of the brain. One patient had marked language impairment. The central nervous system involvement had a peculiar benign and self-limiting course and total normalization occurred in one case. LIMITATIONS: We present only a small series of patients. CONCLUSION: Cutaneous lesions showing identical clinical and microscopical features, but without any extracutaneous abnormalities, have recently been described by Torrelo et al. [J Am Acad Dermatol 2011;64:888-892] under the designation 'papular epidermal nevus with "skyline" basal cell layer (PENS)'. An association of such nevi with neurologic abnormalities had so far only been published in the 'Atlas of Genodermatoses' edited by Caputo and Tadini. We believe that this unique and otherwise undescribed symptom complex is a new neurocutaneous phenotype that may be called PENS syndrome. Whether this new syndrome can be categorized as a distinct phenotype within the group of epidermal nevus syndromes remains speculative and has to be clarified by further investigations.

Medallion-like dermal dendrocyte hamartoma: a case misdiagnosed as neurofibroma.

Medallion-like dermal dendrocyte hamartoma is a rare congenital lesion, comprised of a benign dermal proliferation of fusiform cells that stain positive for CD34, often positive for factor XIIIa, and negative for S100. It has a highly characteristic clinical presentation consisting of a well-circumscribed atrophic and wrinkled patch located on the upper trunk or neck that remains stable with time. We report a case of an 11-year-old boy with a typical medallion-like dermal dendrocyte hamartoma on the nape of the neck that was previously misdiagnosed as neurofibroma on the basis of initial histological examination that was later reevaluated due to lack of clinical correlation. Three previously-reported cases of medallion-like dermal dendrocyte hamartoma also have had a previous histological misdiagnosis of probable neurofibroma; other reported cases have been misdiagnosed as congenital atrophic dermatofibrosarcoma protuberans. Clinical correlation and immunostaining are particularly important for the recognition of this rare benign lesion.

Innovative therapeutics in pediatric dermatology.

Although clinical trials for new drugs are often limited in children because of safety concerns or restrictions, new therapies or novel strategies with old drugs have recently expanded dermatologic armamentarium for pediatric patients. Oral propranolol is currently the first choice in the treatment of alarming infantile hemangiomas. In atopic dermatitis, proactive strategy with topical calcineurin inhibitors can safely prevent disease exacerbation. Tacrolimus, in particular, is also useful for the treatment of vitiligo occurring in sensitive areas such as the eyelids. Among biologic drugs, use of etanercept is safe and efficient in children and adolescents with moderate-to-severe plaque psoriasis. Engineered tissues with special antimicrobial properties (silver-coated fabrics or engineered silk) are now used to treat eczema and fungal diseases in children. In athlete's foot, the use of 5-finger socks can also be helpful.

Hypertrophic scarring is the usual outcome of non-membranous aplasia cutis of the scalp.

The outcome of aplasia cutis congenita of the scalp, nonmembranous type, in a series of 21 Caucasian patients is reported. In all the patients the congenital skin defect healed with irregular hypertrophic scar formation.

Juvenile xanthogranuloma associated with neurofibromatosis 1: 14 patients without evidence of hematologic malignancies.

The clinical features and natural history of juvenile xanthogranuloma (JXG) in 14 children affected by neurofibromatosis 1 (NF1) are reported. Mean follow-up in 11 of these patients was 4.3 years (range 1-10 years). None of the children developed hematologic malignancies during this period. The onset of JXG was in the first 2 years of life in 13 of the patients. In this series, the association between JXG and six or more café au lait spots more than 5 mm in diameter was a good marker for NF1 in the first few years of life. Overall the JXG in these patients did not show any features distinguishable from those of "classical" JXG.