Staphylococcus aureus plays a central role in the exacerbation of atopic dermatitis (AD), but the population structure and pathogenic determinants of strains colonizing AD patients remain poorly understood. It is unclear whether these strains mirror those circulating in the general community or whether specific clonal lineages are selectively adapted to the AD skin microenvironment. Data addressing this question are scarce, particularly in Portugal. In this study, we investigated the molecular epidemiology and pathogenic traits of S. aureus colonizing skin lesions in adult patients with AD in Portugal. We found that lesion-associated isolates belonged predominantly to the methicillin-susceptible S. aureus MSSA-ST398 clonal type, a lineage that is widely circulating in the Portuguese community, particularly among vulnerable populations, and that has also been implicated in severe human infections. Notably, isolates from this clonal type in AD harboured specific pathogenicity traits associated with skin barrier disruption, including hemolysin and urease production, which may contribute to their success as colonizers in AD. Our findings highlight that S. aureus colonization in AD arises from a dynamic interplay between community-level molecular epidemiology and disease-specific selective pressures. While circulating lineages provide the genetic background diversity, the AD skin microenvironment appears to shape which clones ultimately become dominant. Such an integrated perspective may help to inform future geographically tailored strategies aimed at limiting bacterial burden and preventing disease exacerbation in AD.

Skin development is a tightly regulated, multistep process that transforms the embryonic surface into a complex organ composed of a stratified epidermis, dermal-epidermal junction, dermis, hypodermis and cutaneous appendages. This review provides a compartment-based, clinically oriented overview of human skin embryology, linking key developmental pathways to normal structure and inherited skin disorders. The epidermis arises from surface ectoderm through the coordinated activity of bone morphogenetic protein (BMP) and Wnt signalling, with p63 maintaining the basal progenitor compartment required for stratification. Subsequent keratinocyte differentiation, cornification and barrier maturation also depend on Notch, lipid-processing and desquamation pathways. Disruption of these sequential events helps explain epidermal mosaic disorders and congenital ichthyoses. In parallel, melanocytes derive from neural crest precursors and colonize the developing epidermis and hair follicles through microphthalmia-associated transcription factor (MITF)-centred melanocytic programmes; abnormal migration, survival or pigment production underlies several congenital pigmentary disorders. The dermal-epidermal junction forms as hemidesmosomes and collagen VII-containing anchoring fibrils progressively secure adhesion between epidermis and dermis; defects in these structures underlie inherited forms of epidermolysis bullosa. The dermis develops from BMP-patterned mesenchyme into papillary and reticular compartments, where fibroblast differentiation, extracellular matrix production, vascularization and foetal repair programmes shape connective tissue strength, vascular patterning and wound-healing behaviour. Disruption of these processes contributes to connective tissue disorders, vascular anomalies and congenital defects of repair. The hypodermis forms through progressive adipogenic differentiation, from early mesenchymal commitment to later peroxisome proliferator-activated receptor gamma (PPARγ)-mediated adipocyte maturation, with disruption reflected clinically by lipodystrophy syndromes. Finally, skin appendages arise through reciprocal epithelial-mesenchymal interactions, initiated by Wnt/β-catenin signalling and refined by ectodysplasin/NF-κB, Sonic Hedgehog, Notch and connexin-mediated pathways. Defects in these shared developmental programmes provide the basis for ectodermal dysplasias. Understanding this developmental trajectory provides a unifying framework for interpreting skin disease, clarifying how timing, context and cellular origin shape clinical outcomes.

Dupilumab, a monoclonal antibody targeting interleukin‐4 receptor alpha, has shown both robust efficacy and safety in pediatric atopic dermatitis (AD), but data on complete disease control (Eczema Area and Severity Index [EASI] 100) are scarce. A retrospective analysis of 25 pediatric patients at our institution with a median treatment duration of 20 months revealed a significant improvement in EASI scores, with 44.0% achieving EASI 100 at a median of 52 weeks of treatment. No significant differences were found between the group of patients achieving EASI 100 and those who did not. While this study provides additional insight into the efficacy and safety of dupilumab in pediatric AD, its limited sample size underscores the need for larger scale studies focusing on EASI 100 to better understand the factors influencing complete disease control and to optimize treatment strategies for pediatric patients with AD.

AbstractEpidermal growth factor receptor (EGFR) is a transmembrane glycoprotein that exhibits tyrosine kinase signaling activity, playing a pivotal role in various cellular processes. Overexpression or overactivity of EGFR is significantly associated with inflammatory and malignant diseases, particularly lung cancer. Conversely, germline loss-of-function mutations in EGFR have been distinctly linked to a rare condition known as neonatal nephrocutaneous syndrome. We report a case of a preterm female neonate, born to consanguineous parents and from a pregnancy that was complicated by polyhydramnios. From birth, she displayed distinctive clinical features, including fragile skin and widespread erythema, sparse scalp hair, bilateral ankyloblepharon, thick eyebrows, and arachnodactyly. Subsequently, she developed progeroid features marked by skin desquamation, along with failure to thrive, diarrhea, and severe electrolyte and acid–base imbalances. Additional complications comprised anemia, generalized mucocutaneous candidiasis, external otitis, and recurrent septic episodes. Genetic analysis confirmed a homozygous EGFR mutation c.1283G > A p.(Gly428Asp). The infant's neurological status remained stable, albeit requiring pain management through oral morphine and gabapentin. Palliative care became imperative, given the dismal prognosis. The identified mutation is exceedingly rare, with only 24 reported cases known thus far. Each of these instances resulted in a complex multisystemic condition marked by a wide range of challenging symptoms and signs. This clinical journey highlights the intricate interplay among genetic mutations and complex medical conditions, and underscores the critical significance of multidisciplinary care.

Lymphoplasmocytic plaque in children (LPC) is a rare and distinctive skin disorder primarily affecting the pediatric population. Characterized by its unique histopathological features, the condition manifests as well-defined plaques with a predominance of lymphocytes and plasma cells infiltrating the dermis. Despite its limited prevalence, recognizing this entity is crucial for accurate diagnosis and appropriate management of affected patients. We report the case of a 10-year-old male presenting with LPC in the extensor surface of the upper arm, a rarely reported location, treated with both topical and intralesional corticosteroids resulting in partial improvement.

Introduction: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe and life-threatening mucocutaneous conditions characterized by fever and necrosis of the epidermis. Up to 90% of cases are drug-induced. Antiseizure drugs (ASD) are known to cause SJS, but only a very few ethosuximide-induced SJS cases have been reported in the literature. Case presentation: We report a case of ethosuximide-induced SJS/TEN overlap in a 4-year-old boy, confirmed by patch tests, with improvement with intravenous immunoglobulin (IVIG) and systemic corticosteroid therapy cycle. Conclusion: This case highlights the need to expand knowledge about the optimal treatment strategy for these rare conditions, in addition to supportive treatment.

Newborns may develop blisters and erosions due to multiple entities, including infections, congenital or hereditary diseases. In this report we describe a male newborn who presented with pustular lesions on the first day of life and progressively developed blisters and erosions. Due to disease progression even with intravenous antibiotic therapy after suspicion of bullous impetigo, other diagnoses had to be considered and a multidisciplinary approach with the support of dermatology was necessary. A skin biopsy and genetic study were performed, which allowed the definitive diagnosis of Epidermolysis Bullosa, a group of rare hereditary bullous diseases. Because of the absence of curative therapy, supportive treatment was performed, which did not prevent the patients death at 2 months of life. It is therefore essential for pediatricians to identify this entity as early as possible in order to improve the prognosis

A 15-year-old male, with personal history of limb-girdle muscular dystrophy, was admitted to the paediatric ward for a 1-month history of pruritic, generalised, polymorphous eruption and severe painful mucositis. Two months earlier he had been diagnosed with SARS-CoV-2-induced mucositis and treated with prednisolone 40 mg/day for 2 weeks with improvement. At home, due to further therapeutic non-compliance by the patient, mucous lesions recurred associated with a de novo skin eruption, leading him to the emergency department and subsequent hospitalisation. Physical examination revealed shiny, erythematous, flat-toped papules, scarce tense blisters and erosions, scattered over the trunk and extremities, sparing the face. There were multiple palmoplantar blisters and peri-ungual erythema, erosions and crusts. There were widespread erosions of the oral mucosa extending to the vermillion, where there were also pseudomembranes and haemorrhagic crusts (Fig. 1). The glans penis and conjunctiva showed erosions and hyperaemia, respectively. Nikolsky sign was negative. Neither lymphadenopathy nor organomegaly was noticed. Biopsy of a blister demonstrated foci of acantholysis in the mid and superficial epidermis and vacuolization of the basal layer with few apoptotic keratinocytes (Fig. 2). In the superficial dermis, there was moderate perivascular lymphocytic infiltrate. Direct immunofluorescence of perilesional skin revealed intercellular deposition of immunoglobulin G (IgG) and C3. Immunoprecipitation showed anti-desmoglein-3 and anti-desmoglein-1 antibodies. Clinical, histological and immunofluorescence findings established the diagnosis of paraneoplastic pemphigus (PNP). The patient started methylprednisolone 80 mg/day (1 mg/kg/day). Further investigation, including complete blood count, renal and liver functions, protein electrophoresis and anti-nuclear antibodies, was unremarkable. Normal serologies for herpes simplex virus and Mycoplasma pneumoniae, ruled out other possible causes of mucositis. Positron emission tomography/computed tomography (PET/CT) showed multiple retroperitoneal lymphadenopathies and a 62 × 105 × 118 mm hypermetabolic, lobulated mass (Fig. 3), of which biopsies were inconclusive, exhibiting inflammatory infiltrate alone. During this time, due to lack of improvement with corticosteroid therapy alone, azathioprine 50 mg/day was added and an eight-session treatment with plasmapheresis was performed. Following this, the patient underwent incomplete resection of the tumour, whose histologic examination revealed a hyaline vascular variant of Castleman's disease. Given the involvement of multiple lymph nodes, the patient was diagnosed with multicentric Castleman's disease. Considerable improvement over the first month following surgery allowed a reduction of methylprednisolone to 16 mg/day. Since a second procedure to remove the remaining tumour was not feasible, and the patient still had few painful skin lesions with significant mucositis, he started monthly cycles of intravenous immunoglobulin (IVIg) 1 g/kg. During the next 6 months, he showed global improvement of the dermatosis (Fig. 4), despite maintaining refractory oral mucositis. Currently, he is being treated with methylprednisolone 16 mg/day, azathioprine 100 mg/day and monthly IVIg, showing stable disease, but recurrent infectious complications requiring hospitalisation and multiple antibiotic regimens. He has now completed 10 months of follow-up. PNP is a multisystemic autoimmune disease characterised by: painful mucositis refractory to therapy; polymorphic skin lesions (lichenoid, targetoid, blistering, erosive or confluent erythema); acantholysis and lichenoid or interface dermatitis on histological examination; and immunoprecipitation of various proteins, namely envoplakin, periplakin, plectin, desmoglein-1 and desmoglein-3.1, 2 Direct immunofluorescence of the perilesional skin demonstrates intercellular deposition of IgG and C3, and additional staining along the dermal-epidermal junction.3 As the name implies, it occurs in the context of neoplasms, usually of lymphoproliferative origin, including chronic lymphocytic leukaemia and non-Hodgkin lymphoma, and less frequently thymoma, sarcomas and carcinomas.4, 5 PNP is an uncommon condition in paediatric patients, with few case reports and small case series described in literature, representing a diagnostic challenge. Due to the oral blistering and polymorphic skin eruption, differential diagnoses include herpes simplex virus infection, Mycoplasma pneumonia-induced rash and mucositis, reactive infectious mucocutaneous eruption, erythema multiforme major, Stevens-Johnson syndrome/toxic epidermal necrolysis, lichen planus or other autoimmune blistering diseases.6 In fact, our patient had initially been diagnosed with a SARS-CoV-2-induced mucositis at another clinic, which may have represented the initial stage of PNP. Most reported cases in children are associated with Castleman's disease.2, 3, 6-8 Other less frequently reported neoplasms in this age group include T-cell lymphoblastic lymphoma,9 follicular dendritic cell sarcoma10 and myofibroblastic tumour.11 Castleman's disease is a rare, non-clonal neoplasm of lymphatic origin, most commonly developing in the retroperitoneal space, namely the renal fossa or the base of the bladder, and the chest.1 Clinically, it can be classified as unicentric or multicentric, according to the involvement of lymph nodes (multicentric if two or more lymph nodes or regions involved). There are three recognised pathological variants: hyaline vascular, plasma-cell and mixed cellular.1 The tumour may be present for several months before autoimmunity develops. The underlying mechanisms are not completely understood, but the autoimmune phenomenon may result from tumoral deregulated cytokine production, especially interleukin-6, and/or an attack against plakin antigens, presented by dendritic follicular cells of the tumour.1 A high index of suspicion is necessary to diagnose Castleman's disease, not only for its rarity but also because it can have benign histological features on biopsy, not demonstrating clonality.7 In the setting of a suspected PNP, if a tumour is not readily detectable, continued surveillance is indicated. PNP is associated with respiratory disease, more specifically bronchiolitis obliterans. This may be related to the expression of plakin proteins in the respiratory epithelium, which are recognised by PNP antibodies. In fact, IgG has been shown to be deposited on the affected bronchial epithelium.12 Respiratory involvement usually predicts a complicated course of the disease and bronchiolitis obliterans may be the leading cause of mortality in children with PNP associated with Castleman's disease.13 A relationship between PNP and myasthenia gravis has also been reported, especially in patients with associated thymoma or Castleman's disease, although there are no studies restricted to paediatric population.14, 15 In contrast to bronchiolitis obliterans, myasthenia gravis does not seem to change the overall survival in these patients.15 Generally, PNP has a poor prognosis and a progressive course, regardless of the patient's age.7 When associated with Castleman's disease, the 3-year survival rate is less than 60%.14 Whether prognosis differs between unicentric and multicentric disease is uncertain.6 The eradication of the underlying tumour may have some benefit in the mucocutaneous lesions; nonetheless, even after complete resection, the autoimmune process may remain active for many years.16 Moreover, there are reports where the pulmonary involvement not only persisted but progressed after tumour resection.13, 14 Systemic therapy with thalidomide, lenalidomide, bortezomib, cytotoxic chemotherapy, rituximab or siltuximab (anti-interleukin-6 monoclonal antibody) may be considered for treatment of residual disease.6, 14 Post-operative adjunctive immunosuppression is, therefore, necessary for long periods of time and diverse agents have been used, despite their low efficacy.3 These include oral corticosteroids, cyclosporin, cyclophosphamide, azathioprine, mycophenolate mofetil, rituximab, IVIg, plasmapheresis and immunopheresis.3, 14 Data regarding the efficacy of these agents in paediatric population are lacking, in part due to the short follow-up periods resulting from elevated mortality. In our patient, IVIg plus azathioprine and low-dose methylprednisolone showed some benefit, with improvement of the cutaneous eruption, but persistence of relapsing oral mucositis. This is in line with the results found in a case series of PNP associated with Castleman's disease,1 showing that patients with unresectable disease may transiently benefit from combined regimens, despite the inevitability of symptomatic recurrence. Besides, the follow-up period of our patient is still very short. The diagnosis of PNP in paediatric population may not be easy, mainly for its rarity. The recognition of the characteristic polymorphic skin eruption, alongside its histological features and immunoprecipitation profile, is important since it can represent the first sign of a haematological or visceral malignancy. When PNP is diagnosed in children, Castleman's disease must always be considered. The authors would like to thank Dr Nelson Neves, Dr Paula Estanqueiro, Dr Manuel Salgado and Dr João Nascimento (Department of Pediatrics, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal) for their essential support in the management of the patient.

Newborns may develop blisters and erosions due to multiple entities, including infections, congenital or hereditary diseases. In this report we describe a male newborn who presented with pustular lesions on the first day of life and progressively developed blisters and erosions. Due to disease progression even with intravenous antibiotic therapy after suspicion of bullous impetigo, other diagnoses had to be considered and a multidisciplinary approach with the support of dermatology was necessary. A skin biopsy and genetic study were performed, which allowed the definitive diagnosis of Epidermolysis Bullosa, a group of rare hereditary bullous diseases. Because of the absence of curative therapy, supportive treatment was performed, which did not prevent the patients death at 2 months of life. It is therefore essential for pediatricians to identify this entity as early as possible in order to improve the prognosis.

International Journal of DermatologyEarly View Correspondence Dupilumab in the treatment of dystrophic epidermolysis bullosa: off-label use in a pediatric patient Joana Xará MD, Corresponding Author Joana Xará MD [email protected] Dermatology Department, Coimbra University Hospital Center, Coimbra, PortugalSearch for more papers by this authorMaria Relvas MD, Maria Relvas MD Dermatology Department, Coimbra University Hospital Center, Coimbra, PortugalSearch for more papers by this authorCarolina Figueiredo MD, Carolina Figueiredo MD Dermatology Department, Coimbra University Hospital Center, Coimbra, PortugalSearch for more papers by this authorJoana Calvão MD, Joana Calvão MD orcid.org/0000-0002-1161-269X Dermatology Department, Coimbra University Hospital Center, Coimbra, PortugalSearch for more papers by this authorMariana Batista MD, Mariana Batista MD orcid.org/0000-0003-3442-615X Dermatology Department, Coimbra University Hospital Center, Coimbra, PortugalSearch for more papers by this authorLeonor Ramos MD, Leonor Ramos MD Dermatology Department, Coimbra University Hospital Center, Coimbra, PortugalSearch for more papers by this author Joana Xará MD, Corresponding Author Joana Xará MD [email protected] Dermatology Department, Coimbra University Hospital Center, Coimbra, PortugalSearch for more papers by this authorMaria Relvas MD, Maria Relvas MD Dermatology Department, Coimbra University Hospital Center, Coimbra, PortugalSearch for more papers by this authorCarolina Figueiredo MD, Carolina Figueiredo MD Dermatology Department, Coimbra University Hospital Center, Coimbra, PortugalSearch for more papers by this authorJoana Calvão MD, Joana Calvão MD orcid.org/0000-0002-1161-269X Dermatology Department, Coimbra University Hospital Center, Coimbra, PortugalSearch for more papers by this authorMariana Batista MD, Mariana Batista MD orcid.org/0000-0003-3442-615X Dermatology Department, Coimbra University Hospital Center, Coimbra, PortugalSearch for more papers by this authorLeonor Ramos MD, Leonor Ramos MD Dermatology Department, Coimbra University Hospital Center, Coimbra, PortugalSearch for more papers by this author First published: 25 August 2023 https://doi.org/10.1111/ijd.16821 Conflict of interest: None. Funding source: None. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. References 1Clawson RC, Duran SF, Pariser RJ. Epidermolysis bullosa pruriginosa responding to dupilumab. JAAD Case Rep. 2021; 16: 69–71. 2Papanikolaou M, Onoufriadis A, Mellerio JE, Nattkemper LA, Yosipovitch G, Steinhoff M, et al. Prevalence, pathophysiology and management of itch in epidermolysis bullosa. Br J Dermatol. 2021; 184(5): 816–825. 3Beck LA, Deleuran M, Bissonnette R, de Bruin-Weller M, Galus R, Nakahara T, et al. Dupilumab provides acceptable safety and sustained efficacy for up to 4 years in an open-label study of adults with moderate-to-severe atopic dermatitis. Am J Clin Dermatol. 2022; 23(3): 393–408. 4Hendricks AJ, Yosipovitch G, Shi VY. Dupilumab use in dermatologic conditions beyond atopic dermatitis—a systematic review. J Dermatolog Treat. 2021; 32(1): 19–28. 5Oetjen LK, Mack MR, Feng J, Whelan TM, Niu H, Guo CJ, et al. Sensory neurons co-opt classical immune signaling pathways to mediate chronic itch. Cell. 2017; 171(1): 217–228.e13. Early ViewOnline Version of Record before inclusion in an issue ReferencesRelatedInformation

Margarida Gonçalo provided lectures and advisory boards for AbbVie, Leo, Lilly, Novartis, Pfizer, Sanofi and Takeda. The other authors declare that they have no conflict of interest. APPENDIX S1 Supporting Information Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

An 8-year-old girl, with a past personal history of idiopathic neutropenia, presented with asymptomatic lesions of the lower limbs for the previous 3 months, which appeared during winter. The patient denied any systemic symptoms. Physical examination revealed multiple erythematous to violaceous, annular, infiltrated plaques, symmetrically distributed over the buttocks and upper lateral aspects of the thighs (Fig. 1). There were no erosions or ulceration. The remainder of the examination was unremarkable. Laboratory workup, including a complete blood count, peripheral blood smear, coagulation, renal and liver functions, C-reactive protein, erythrocyte sedimentation rate, complement, and antinuclear antibodies, was normal, except for the previously known neutropenia of 600/μl, which has been stable. Histological examination of a skin biopsy demonstrated a moderately dense lymphocytic infiltrate of the superficial and deep dermis, with a predominantly perivascular and focally perieccrine distribution (Fig. 2). Cold-associated perniosis of the thighs. Since the histological findings were consistent with perniosis and given the topography of the lesions, the patient was diagnosed with cold-associated perniosis of the thighs. The lesions spontaneously evolved into subtle hyperpigmented macules, which ended up disappearing after 1 month. When questioned, the patient denied practicing horse riding or other outdoor activities on a regular basis, although she used to wear tight-fitting jeans. Cold-associated perniosis of the thighs corresponds to the entity classically known as “equestrian cold panniculitis.” It was originally described in 1980 in young, healthy women, who frequently rode horses wearing tight-fitting, uninsulated pants.1 Clinically, it presents as red-to-violaceous, indurated plaques or nodules characteristically localized on the upper lateral portions of the thighs.1 The lesions may be pruritic or painful and show central ulceration or necrosis.2, 3 Young women are, indeed, the most affected group, but in many reported cases, they did not specifically practice horse riding. Any outdoor activity, for example, hiking, cycling, or river crossing, especially if wearing tight-fitting clothes in cold weather, may originate identical lesions.2, 3 Underlying pathogenic mechanisms may encompass impaired blood circulation on the skin originating a subacute vascular and/or perivascular damage. This is different from the acute cold-induced panniculitides (Haxthausen's adiponecrosis e frigore and ice popsicle panniculitis), in which there is a primary cold-induced injury with fat necrosis.4 The main histologic feature is the presence of a superficial and deep perivascular and periadnexal lymphocytic infiltrate, similar to idiopathic perniosis. Lobular panniculitis, if present, is usually very subtle.4 Since most of the patients with cold-associated perniosis of the thighs do not practice horse riding and the histological findings are not compatible with primary panniculitis, the replacement of “equestrian cold panniculitis” with “cold-associated perniosis of the thighs” has been proposed.4 Particularly in our case, considering the morphology of the lesions, clinical differential diagnosis included chilblain lupus erythematosus, lupus tumidus, granuloma annulare, interstitial granulomatous dermatitis, or urticarial vasculitis. In fact, the clinical and histological overlap between cold-associated perniosis of the thighs and both chilblain lupus and lupus tumidus has been reported.3, 4 Despite this, the first is a benign entity, with favorable clinical course and spontaneous resolution, although potent topical corticosteroids may have a role in symptomatic treatment.2 The recognition of a classic presentation of cold-associated perniosis of the thighs, namely in young, healthy, female patients during winter, may prevent unnecessary complementary studies, which should be reserved only for atypical cases.

Capillary malformation-arteriovenous malformation syndrome (CM-AVM) is a rare autosomal dominant disorder caused by heterozygous mutations in RASA1 and EPHB4 that associates multifocal capillary malformations (CMs) with an increased risk for fast-flow vascular malformations (FFVMs) which can cause life-threatening complications. We report the case of a 6-year-old girl with lip telangiectasia as well as multiple (>10) erythematous to brownish irregular macules and patches on the face, neck, trunk, and superior extremities from the age of 18 months with no local or systemic complaints. Several family members had similar lesions. Genetic testing revealed a heterozygous mutation in the EPHB4 gene, confirming the diagnosis of CM-AVM type 2. EPHB4 gene mutation was also present in her father and brother. No associated arteriovenous malformations (AVMs) were found after a thorough evaluation by cardiology and neurology, neither in the patient nor in the closest relatives. The present case is a characteristic presentation of CM-AVM type 2 syndrome, with gradually appearance of multifocal CMs, lip telangiectasias, and a positive family history, without associated extracutaneous FFVMs. An increasing number of atypical CMs on the skin may be the only sign of CM-AVM and should alert the clinician to further investigate the presence of AVMs which can be life-threatening.

Journal of Paediatrics and Child HealthVolume 58, Issue 1 p. 213-214 Letter to the EditorFree Access Not everything in dermatology is treated with corticosteroids Dr Marta Vidal, Dr Marta Vidal orcid.org/0000-0002-3950-552X Pediatric Dermatology Service, Hospital and University Centre of Coimbra, Coimbra, PortugalSearch for more papers by this authorDr Joana Calvão, Dr Joana Calvão orcid.org/0000-0002-1161-269X Pediatric Dermatology Service, Hospital and University Centre of Coimbra, Coimbra, PortugalSearch for more papers by this authorDr Leonor Ramos, Dr Leonor Ramos Pediatric Dermatology Service, Hospital and University Centre of Coimbra, Coimbra, PortugalSearch for more papers by this author Dr Marta Vidal, Dr Marta Vidal orcid.org/0000-0002-3950-552X Pediatric Dermatology Service, Hospital and University Centre of Coimbra, Coimbra, PortugalSearch for more papers by this authorDr Joana Calvão, Dr Joana Calvão orcid.org/0000-0002-1161-269X Pediatric Dermatology Service, Hospital and University Centre of Coimbra, Coimbra, PortugalSearch for more papers by this authorDr Leonor Ramos, Dr Leonor Ramos Pediatric Dermatology Service, Hospital and University Centre of Coimbra, Coimbra, PortugalSearch for more papers by this author First published: 18 November 2021 https://doi.org/10.1111/jpc.15833Citations: 1 Conflict of interest: None declared. AboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Dear Editor, NOT EVERYTHING IN DERMATOLOGY IS TREATED WITH CORTICOSTEROIDS A 2-year-old infant male with a previous diagnosis of atopic eczema and multiples episodes of acute wheezing illness, managed with oral montelukast, inhaled salbutamol and an inhaled combination of salmeterol 25 mcg and fluticasone 50 mcg once daily through a spacer chamber since 18 months, developed facial skin lesions with no pruritus associated which evolved for 5 months. Initially, small erythematous micropapules were observed only on his lower eyelids and treated with topical mometasone furoate, methylprednisolone aceponate and mupirocin, but they worsened and spread. The lesions included small papules and pustules, some with overlying yellow crusts located on the infant's face, including eyelids and cervical area. The perioral vermilion border was spared (Fig. 1). Fig. 1Open in figure viewerPowerPoint Infant with extensive periorificial dermatitis. He was sent to the Dermatology Department who diagnosed extensive periorificial dermatitis (POD) due to inhaled and topical corticosteroids. Treatment was initiated including soap and water facial wash post-inhaled corticosteroids, topical erythromycin and topical methylprednisolone aceponate (only for the first 2 weeks) and a course of oral erythromycin 40 mg/kg/day. After 1 month of treatment, there was an improvement, but multiple erythematous and brown coloured extensive perioral papules remained. Treatment with oral erythromycin was prolonged for 15 more days, topical erythromycin was stopped and topical ivermectin initiated, once a day. After 3 months of treatment, the patient fully recovered from the skin lesions. POD is an inflammatory disorder of the facial epidermis. Patients typically present with multiple small inflammatory papules, papulovesicles or papulopustules and scaling around the mouth, eyes or nose1 Clinical history and physical examination are usually sufficient for the diagnosis. A history of topical corticosteroid use is commonly reported in association with POD and there has also been notable correlation between the development of POD after oral use of inhaled corticosteroids.2 Fluorinated toothpaste, chewing gum and physical sunscreens can also be associated with POD.2 The differential diagnosis includes allergic contact dermatitis, Demodex folliculitis, irritant cheilitis, rosacea, among others. The discontinuation of corticosteroids and other skin irritants are important measures to be taken for POD treatment. Pharmacological therapy is usually used to aid in lesion resolution.2, 3 For children with mild POD, topical therapy using erythromycin, metronidazole, pimecrolimus or tacrolimus is recommended. Topical ivermectin also considerably improves skin lesions and is well tolerated because of its low skin irritation potential. For those who are under 8 years old, with moderate to severe POD or topical treatment failure the use of oral erythromycin or azithromycin is recommended. Oral tetracyclines should not be used in young children due to its adverse effects.3, 4 Written informed consent for publication was obtained from the parents of the patient. References 1Tolaymat L, Hall MR. Perioral dermatitis. StatPearls Publishing, 2021 Avaible from: https://www.ncbi.nlm.nih.gov/books/NBK525968/ [accessed 30 March 2021]. 2Dubus JC, Marguet C, Deschildre A et al. Local side-effects of inhaled corticosteroids in asthmatic children: Influence of drug, dose, age, and device. Allergy 2001; 56: 944– 8. 3Tempark T, Shwayder TA. Perioral dermatitis: A review of the condition with special attention to treatment options. Am. J. Clin. Dermatol. 2014; 15: 101– 13. 4Charnowski S, Wollenberg A, Reinholz M et al. Perioral dermatitis successfully treated with topical ivermectin. Ann Dermatol 2019; 31: S27– 8. Citing Literature Volume58, Issue1January 2022Pages 213-214 FiguresReferencesRelatedInformation

CLOVES syndrome is a rare overgrowth syndrome caused by an activating somatic mutation in the PIK3CA gene. We report the case of a six-month-old male that presented with a complex dermatosis with evolution since birth. Physical examination revealed a reticulated erythematous patch that affected the right anterior chest wall, where it showed sharp demarcation at the midline. He also had gigantism of the hands and feet, with an acral furrowing of the skin. Later on, he developed papillomatous plaque lesions in the cervical region, suggestive of epidermal nevi. Genetic study performed on lesional skin confirmed the PIK3CA mutation, establishing the diagnosis of CLOVES syndrome. The present case stands out given the rarity of this entity. Early differentiation between other overgrowth syndromes is important, namely because of the worse prognosis of Proteus. Moreover, we emphasize the importance of cutaneous findings and the dermatologist’s role in the diagnostic approach to such cases.

Summary:Background. Atopic dermatitis is a prevalent condition in the pediatric population, with affected children exhibiting a susceptibility to cutaneous sensitization due to skin barrier dysfunction and immune dysregulation. Recent studies have highlighted an increased prevalence of certain allergens, which identifi-cation may be clinically relevant, with direct implications for the management of atopic dermatitis. Methods. We retrospective reviewed pediatric patients patch tested due to suspected contact dermatitis. Patients were divided according the diagnosis of AD, with subsequent comparison of positive results for both groups. Results. A total of 145 pediatric patch testing were analyzed, 44.1% (n = 63) with the diagnosis of atopic dermatitis. There were notable differences in sensitization rates of relevant allergens between groups and when compared to other European studies. Based on the most prevalent and relevant allergens, we proposed an adapted hapten series for assessing portuguese pediatric patients with AD and suspicion of concomitant allergic contact dermatitis. Conclusions. Our findings confirmed the geograph-ic sensitization variability and emphasize the need for pediatric adaptation and "individualized baseline series".

Dermatologic TherapyVolume 35, Issue 9 e15678 LETTER Losartan as therapy for recessive dystrophic epidermolysis bullosa: Report of three cases Maria Relvas, Corresponding Author Maria Relvas [email protected] orcid.org/0000-0002-2639-7494 Dermatology Department, Coimbra University Hospital Centre, Coimbra, Portugal Correspondence Maria Relvas, Praceta Prof. Mota Pinto, 3004-561 Coimbra, Portugal. Email: [email protected]Search for more papers by this authorAna Carolina Figueiredo, Ana Carolina Figueiredo Dermatology Department, Coimbra University Hospital Centre, Coimbra, PortugalSearch for more papers by this authorRebeca Calado, Rebeca Calado Dermatology Department, Coimbra University Hospital Centre, Coimbra, PortugalSearch for more papers by this authorJoana Calvão, Joana Calvão orcid.org/0000-0002-1161-269X Dermatology Department, Coimbra University Hospital Centre, Coimbra, PortugalSearch for more papers by this authorLeonor Ramos, Leonor Ramos Dermatology Department, Coimbra University Hospital Centre, Coimbra, PortugalSearch for more papers by this author Maria Relvas, Corresponding Author Maria Relvas [email protected] orcid.org/0000-0002-2639-7494 Dermatology Department, Coimbra University Hospital Centre, Coimbra, Portugal Correspondence Maria Relvas, Praceta Prof. Mota Pinto, 3004-561 Coimbra, Portugal. Email: [email protected]Search for more papers by this authorAna Carolina Figueiredo, Ana Carolina Figueiredo Dermatology Department, Coimbra University Hospital Centre, Coimbra, PortugalSearch for more papers by this authorRebeca Calado, Rebeca Calado Dermatology Department, Coimbra University Hospital Centre, Coimbra, PortugalSearch for more papers by this authorJoana Calvão, Joana Calvão orcid.org/0000-0002-1161-269X Dermatology Department, Coimbra University Hospital Centre, Coimbra, PortugalSearch for more papers by this authorLeonor Ramos, Leonor Ramos Dermatology Department, Coimbra University Hospital Centre, Coimbra, PortugalSearch for more papers by this author First published: 30 June 2022 https://doi.org/10.1111/dth.15678Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume35, Issue9September 2022e15678 RelatedInformation

Figure 1. A) Eruption of brownish-grey, non-confluent macules in a Christmas tree pattern over the trunk. B) Multiple macules involving the trunk, neck and arms. C) Histological examination shows mild acanthosis, increased pigmentation of the basal layer, pigmentary incontinence and melanophages in the upper dermis; there is also a mild perivascular lymphohistiocytic infiltrate in the papillary dermis (haematoxylin and eosin; x40). 10. Joshi R, Minni K. Genitogluteal porokeratosis: a clinical review. Clin Cosmet Investig Dermatol 2018; 11: 219-29.