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Viral exanthems are frequent in children and are mostly self-limited. Early recognition and differentiation from other childhood illnesses are important to direct further investigations and treatment initiation. The clinical presentation of viral exanthems in children includes a polymorphic spectrum of skin eruptions ranging from classic viral exanthems to "atypical" presentations that can mimic nonviral diseases; thus, viral exanthems of childhood can be readily diagnosed on clinical grounds, but not rarely do they represent a diagnostic challenge. In this review, we focus on viral diseases in children that may be difficult to diagnose due to their clinical similarities with nonviral diseases, and we offer clues for the differential diagnosis and proper diagnostic testing in such cases., (Copyright © 2019 Elsevier Inc. All rights reserved.)

The skin is one of the most frequent tissues affected in patients with mastocytosis, but cutaneous lesions are highly heterogeneous in shape, size, color, number, localization, and distribution. The World Health Organization recognizes 3 subtypes of cutaneous mastocytosis (CM): maculopapular CM (MPCM), diffuse CM, and mastocytoma of skin. An international task force of experts in mastocytosis has recently proposed subdividing MPCM into monomorphic and polymorphic, which could predict the duration of the disease in children. More research is warranted to develop an improved classification of CM that ideally should incorporate robust factors with prognostic impact on disease behavior., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Pimecrolimus 1% cream is an effective, non-corticosteroid, topical anti-inflammatory treatment for atopic dermatitis (AD). The aim of this article was to review published clinical data that have examined how pimecrolimus can address the medical needs of AD patients. Clinical studies have demonstrated that early treatment with pimecrolimus decreases the progression to disease flares, rapidly improves pruritus and significantly enhances quality of life. Patients find the formulation easy to apply, which may result in improved adherence with the treatment regimen. Pimecrolimus, in contrast to topical corticosteroids (TCSs), does not induce skin atrophy or epidermal barrier dysfunction and is highly effective for the treatment of AD in sensitive skin areas. Furthermore, pimecrolimus reduces the incidence of skin infections compared with TCSs and is not associated with other TCS-related side effects such as striae, telangiectasia and hypothalamic-pituitary-adrenal axis suppression. An additional benefit of pimecrolimus is its substantial steroid sparing effect. On the basis of these data, a new treatment algorithm for patients with mild-to-moderate AD is proposed in which pimecrolimus is recommended as a first line therapy for patients with established mild AD at the first signs and symptoms of disease. Pimecrolimus is also recommended for mild-to-moderate AD after initial treatment with a TCS. After resolution of lesions, maintenance treatment with pimecrolimus may effectively prevent subsequent disease flares. In conclusion, the clinical profile of pimecrolimus suggests that it may be considered the drug of choice for the treatment of mild-to-moderate AD in children as well as adults and particularly in sensitive skin areas.

Clustered or agminated juvenile xanthogranuloma (JXG) is an unusual form of JXG characterized by multiple reddish-brown or yellowish papules and nodules in a coalescent pattern. We report a case of clustered JXG involving the left lower back of a 5-month-old boy., (© 2012 Wiley Periodicals, Inc.)

Gorham-Stout syndrome is a rare disease characterized by progressive osteolysis leading to disappearance of the bone. Vascular proliferations have been implicated in the pathogenesis of this syndrome. The case of a 7-year-old girl with a prominent invasive lymphatic malformation on the lumbosacral area and massive osteolysis of the pelvic girdle is reported., (© 2012 Wiley Periodicals, Inc.)

Background: Epidermal keratinization disorders comprise a heterogeneous group of skin diseases that share the common feature of abnormal epidermal maturation, often leading to a disturbed stratum corneum., Objective: To describe two cases of an unusual disorder of epidermal keratinization., Methods: The clinical features of two unrelated patients with a long-standing widespread cutaneous eruption are described. Histopathologic examination and immunohistochemical studies were performed on skin biopsy specimens., Results: The eruption was characterized by symmetric erythematous, flat, discrete papules with a polygonal shape and fine scaling. The papules covered most of the skin surface and, in some areas of the trunk, they were arranged along the lines of cleavage, parallel to the ribs. There was no facial, mucosal, nail, or palmoplantar involvement; the teeth and hair were normal. The first patient had a sister with an identical eruption, and a brother of the second patient was said to have similar skin lesions. Histopathology revealed well-demarcated areas of compact eosinophilic orthokeratotic hyperkeratosis overlying a slightly acanthotic epidermis. Lesional skin showed weaker immunoexpression for connexin 43 compared with normal skin., Limitations: Only two patients and one sibling were investigated., Conclusion: We propose the name "saurian papulosis" to describe this newly described clinicopathologic entity., (Copyright © 2011 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Juvenile xanthogranuloma is a benign and self-limited disease which usually appears in the skin of children. Visceral involvement has been rarely reported, as has fatal outcome in some affected individuals. We report a case of systemic juvenile xanthogranuloma in a female newborn with mainly skin, bone marrow, and liver involvement, leading to death at the age of 2 months.

A 12-year-old boy had lesions clinically and histopathologically consistent with lymphomatoid papulosis (LP) which were persistent and appeared grouped in a circumscribed area of normal looking background skin on the right abdomen. Staging work-up did not disclose systemic disease. Persistent agmination of lymphomatoid papulosis (PALP) is somewhat different from classical LP because it involves a circumscribed, patch-sized area of the skin and the papules within never resolve completely despite some waxing and waning. PALP might be considered a localized or regional form of LP or a distinct lymphoproliferative disorder; in any case, at least a cautious long-term follow up is recommended, with attention to the possible development of true lymphoma.

The panniculitides include a group of disorders of varied etiology that manifest as inflamed nodules in the subcutaneous tissue. They are rarely seen in infants and children. The panniculitides of the newborn represent a unique response of the infant's fat to different injuries, and are a specific type of panniculitis that is only seen in neonates and very young infants. These specific panniculitides of children include subcutaneous fat of the newborn, poststeroid panniculitis, sclerema neonatorum, and cold panniculitis. This article reviews in detail the specific types of panniculitis of the newborn and discusses the pediatric aspects of the panniculitis that is often seen in adults.

Atopic dermatitis (AD) is a distressing dermatological disease, which is highly prevalent during infancy, can persist into later life and requires long-term management with anti-inflammatory compounds. The introduction of the topical calcineurin inhibitors (TCIs), tacrolimus and pimecrolimus, more than 10 yr ago was a major breakthrough for the topical anti-inflammatory treatment of AD. Pimecrolimus 1% is approved for second-line use in children (≥2 yr old) and adults with mild-to-moderate AD. The age restriction was emphasized in a boxed warning added by the FDA in January 2006, which also highlights the lack of long-term safety data and the theoretical risk of skin malignancy and lymphoma. Since then, pimecrolimus has been extensively investigated in short- and long-term studies including over 4000 infants (

During examination of cases of chilblains in children and adolescents, we identified four patients who also showed skin lesions similar to erythema multiforme (EM). They had no other known triggers for EM. One of them had a positive PCR for SARS‐CoV‐2, while the other three were negative. Skin biopsies from two patients showed features not typical of EM, such as deep perivascular and perieccrine infiltrate and absence of necrosis of keratinocytes. Immunohistochemistry for SARS‐CoV/SARS‐CoV‐2 spike protein showed granular positivity in endothelial cells and epithelial cells of eccrine glands in both biopsies. All patients had an excellent outcome, and had minimal or no systemic symptoms. The coincidence of EM, a condition commonly related to viruses, and chilblains in the setting of COVID‐19, and the positivity for SARS‐CoV/SARS‐CoV‐2 spike protein by immunohistochemistry strongly suggest a link between EM‐like lesions and SARS‐CoV‐2. [ABSTRACT FROM AUTHOR]

Background: Different skin manifestations of COVID‐19 are being reported. Acral lesions on the hands and feet, closely resembling chilblains, have been recognized during the peak incidence of the COVID‐19 pandemic. Material and methods: A retrospective review of 22 children and adolescents with chilblain‐like lesions seen over a short period of time in the Emergency Department of a children's hospital during the peak incidence of COVID‐19 in Madrid, Spain. Results: All patients had lesions clinically consistent with chilblains of the toes or feet, with three also having lesions of the fingers. Pruritus and mild pain were the only skin symptoms elicited, and only 10 had mild respiratory and/or GI symptoms. None had fever. Coagulation tests, hemogram, serum chemistry, and lupus anticoagulant were normal in all patients tested. One out of 16 tested cases had elevated D‐dimer results, but without systemic symptoms or other laboratory anomalies. SARS‐CoV‐2 PCR tested in 19 cases was positive in just one case. Skin biopsies obtained in six patients were consistent with chilblains. On follow‐up, all cases showed spontaneous marked improvement or complete healing. Conclusion: Acute chilblains were observed during COVID‐19 pandemic in children and teenagers. It is a mildly symptomatic condition with an excellent prognosis, usually requiring no therapy. Etiopathogenesis remains unknown. [ABSTRACT FROM AUTHOR]

Skin lesions in children with proven COVID‐19 are not frequent in the literature apart from those associated with multisystem inflammatory syndrome. Fortunately, microbiologic testing for SARS‐CoV‐2 has become widely available not only for admitted patients but also for mild cases. We present a series of 6 children with mild erythema and desquamation of the fingertips and/or toes as the only skin manifestation of COVID‐19. As all children had asymptomatic to mildly symptomatic disease, it is reasonable to consider this a sign of benign disease and favorable outcome. [ABSTRACT FROM AUTHOR]

A growing number of skin lesions during the COVID‐19 pandemic are being recognized. Acral ischemic lesions identical to chilblains are most typical in children and young adults. We report an infant girl, aged 1 month and 29 days, with a peculiar reticulated purpuric eruption on her soles, with positive immunohistochemistry for SARS‐CoV‐2 in the endothelia of dermal blood vessels. The patient had an excellent outcome without specific therapy. [ABSTRACT FROM AUTHOR]

Objective Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE syndrome) is an autoinflammatory syndrome recently described in children. We undertook this study to investigate the clinical phenotype, genetic cause, and immune dysregulation in 9 CANDLE syndrome patients. Methods Genomic DNA from all patients was screened for mutations in PSMB8 (proteasome subunit β type 8). Cytokine levels were measured in sera from 3 patients. Skin biopsy samples were evaluated by immunohistochemistry, and blood microarray profile and STAT-1 phosphorylation were assessed in 4 patients and 3 patients, respectively. Results One patient was homozygous for a novel nonsense mutation in PSMB8 (c.405C>A), suggesting a protein truncation; 4 patients were homozygous and 2 were heterozygous for a previously reported missense mutation (c.224C>T); and 1 patient showed no mutation. None of these sequence changes was observed in chromosomes from 750 healthy controls. Of the 4 patients with the same mutation, only 2 shared the same haplotype, indicating a mutational hot spot. PSMB8 mutation-positive and -negative patients expressed high levels of interferon-γ (IFNγ)-inducible protein 10. Levels of monocyte chemotactic protein 1, interleukin-6 (IL-6), and IL-1 receptor antagonist were moderately elevated. Microarray profiles and monocyte STAT-1 activation suggested a unique IFN signaling signature, unlike in other autoinflammatory disorders. Conclusion CANDLE syndrome is caused by mutations in PSMB8, a gene recently reported to cause 'JMP' syndrome (joint contractures, muscle atrophy, microcytic anemia, and panniculitis-induced childhood-onset lipodystrophy) in adults. We extend the clinical and pathogenic description of this novel autoinflammatory syndrome, thereby expanding the clinical and genetic disease spectrum of PSMB8-associated disorders. IFN may be a key mediator of the inflammatory response and may present a therapeutic target. [ABSTRACT FROM AUTHOR]

Nevus psiloliparus is a distinct type of mesodermal nevus of the scalp characterized by absence or paucity of hair, and presence of an excessive amount of fatty tissue. It is considered a hallmark of encephalocraniocutaneous lipomatosis, a rare disorder comprising a variety of cutaneous, ophthalmologic, and neurologic defects. We report two infants with encephalocraniocutaneous lipomatosis with nevus psiloliparus on the scalp in close association with aplasia cutis congenita. This unusual association may be considered a further example of didymosis, for which we propose the term, didymosis aplasticopsilolipara. [ABSTRACT FROM AUTHOR]

Aplasia cutis of the scalp is often a sporadic condition, but familial occurrences with an autosomal dominant inheritance have been documented. Aplasia cutis of the scalp may be seen in two main clinical variants: oval-shaped membranous aplasia cutis and irregular, larger defects. We report six families in whom more than one member has aplasia cutis of the scalp, all of them with large irregular defects located over the vertex or anterior to the vertex along the sagittal suture. We review previous reports of this entity with clinical pictures and note that in most instances, the defects are of the nonmembranous variant. [ABSTRACT FROM AUTHOR]

ABSTRACT Down syndrome (DS) is a common chromosomal disorder with distinct phenotypes and skin markers. This study reports 39 DS patients, aged 1–44  months, who presented from birth with rough, brownish skin in the dimples of their knees and/or elbows. Dermoscopy revealed hyperkeratosis with brownish scales, and biopsies in six cases showed papillomatosis and mild hyperkeratosis. In the cases followed, the condition resolved spontaneously. Hyperkeratosis in the skin dimples on the knees and elbows seems to be a congenital and perhaps characteristic finding in patients with DS. [ABSTRACT FROM AUTHOR]