European Journal of Pediatric Dermatology

European Journal of Pediatric Dermatology, issue 2, vol. 36, 2026

STAFF STAFF — 2026-04-13

none

Clinical phenotypic spectrum of Schimmelpenning-Feuerstein-Mims syndrome: a systematic review.

U. Joshi — 2026-04-13

Background. Schimmelpenning–Feuerstein–Mims syndrome (SFMS) is a rare mosaic neurocutaneous disorder caused by postzygotic mutations affecting the RAS-MAPK signaling pathway. It is characterized by a linear epidermal sebaceous nevus associated with variable neurological, ocular, skeletal, and metabolic involvement. Despite nearly seven decades having elapsed since its initial description, comprehensive phenotypic data remain limited.

Methods. A systematic literature review (1957-2025) was conducted in accordance with PRISMA guidelines. Of 182 records identified, 28 studies met the inclusion criteria. One index case (a 5-year-old male with extensive epidermal sebaceous nevus, skeletal dysplasia, and severe hypophosphatemia – 0.58 mmol/L –) was added to the previously reported cohort.

Results. Epidermal–sebaceous nevus was present in 100% of cases. Extracutaneous involvement included neurological abnormalities (50-75%), ocular defects (40-60%), and skeletal anomalies (15-30%). FGF23-mediated hypophosphatemic rickets represents a distinct metabolic subgroup (5-15%) requiring specialized management. The historically overestimated risk of basal cell carcinoma (10-20%) reflects misclassification of benign trichoblastomas; the true risk of malignancy in childhood is <1%.

Interpretation. SFMS exists along a phenotypic continuum reflecting the timing of postzygotic RAS mosaicism. A modular diagnostic approach (skin findings plus neurological, ocular, or skeletal features) should replace the now-obsolete classic triad. Early multidisciplinary evaluation enables anticipatory management of neurological, ophthalmologic, and metabolic complications

The impact of dermatology educational programs on school-aged children. Systematic review.

B. Sa — 2026-04-13

Background/Objectives. Dermatologic conditions, particularly those affecting adolescents, can significantly impact mental health, self-esteem, and social development. Acne, eczema, and psoriasis are frequently associated with emotional distress, social isolation, bullying, and an increased risk of suicide. Although early interventions aimed at reducing stigma, educating on skin care, and fostering empathy are critical, time constraints during healthcare visits often limit the delivery of comprehensive education. This systematic review evaluates the effectiveness of dermatology educational programs targeting school-aged children, focusing on their impact on dermatologic knowledge, behavior change, and empathy.

Methods. A systematic review was conducted using studies from diverse geographic regions that examined school-based or community dermatology education programs for children. Inclusion criteria focused on interventions reporting outcomes related to dermatologic knowledge, behavioral changes, and psychosocial effects such as empathy or stigma reduction.

Results. The reviewed programs demonstrated improvements in dermatologic knowledge, adoption of healthier skin care behaviors, and increased empathy toward individuals with visible skin conditions. Some interventions also reported reductions in bullying and stigmatizing attitudes. However, challenges such as cultural barriers, inconsistent program implementation, and knowledge decay over time were noted.

Conclusions. Dermatology educational programs for school-aged children show promise in enhancing dermatologic literacy, promoting empathetic behavior, and reducing stigma associated with skin conditions. To maximize impact, culturally tailored, ongoing initiatives should be integrated into school curricula and community settings. These efforts may contribute to improved dermatologic, psychological, and social outcomes, addressing health disparities among pediatric populations.

A unique presentation of reactive infectious mucocutaneous eruption (RIME) and the importance of acute diagnosis.

E. Laughlin — 2026-04-13

Reactive Infectious Mucocutaneous Eruption (RIME) is a constellation of systemic upper respiratory infectious symptoms preceding mucocutaneous eruption. This case describes RIME following amoxicillin initiation with an otherwise unidentified pathogenic etiology. A previously healthy, fully vaccinated, adolescent male presented for five days of upper respiratory symptoms and was started on empiric amoxicillin therapy due to a family member diagnosed with strep pharyngitis earlier on the same day. Two days later, he developed an oral mucocutaneous eruption, and amoxicillin was discontinued. Within 48 hours of discontinuing amoxicillin, he developed conjunctivitis, stomatitis, and penile lesions. The patient was then hospitalized for mucositis with dehydration. Through exclusion of other etiologies, the patient was diagnosed with mucositis secondary to RIME. RIME has commonly been linked to M. pneumoniae infection; however, our patient had negative infectious assay testing and titers. Limited documentation of post-amoxicillin, aseptic RIME exists, prompting support for an expanded spectrum of disease recognition and timeline documentation.

Probably drug-induced toxic epidermal necrolysis in an adolescent.

Darmani Endang H — 2026-04-13

Toxic epidermal necrolysis (TEN) is a rare, life-threatening mucocutaneous adverse reaction, most commonly triggered by medications. Although uncommon in children and adolescents, TEN carries significant morbidity and mortality, requiring prompt diagnosis and multidisciplinary management.

This work aims to report a diagnostic challenge case of drug-induced TEN in an adolescent and to highlight the importance of early recognition and timely intervention in achieving favorable outcomes. A 13-year-old girl developed persistent high-grade fever followed by rapidly progressive generalized erythematous-to-dusky macules evolving into extensive epidermal detachment involving more than 90% of the total body surface area. Severe mucosal involvement affected the oral, ocular, and genital surfaces. Symptoms occurred approximately two weeks after initiation of amoxicillin, dexchlorpheniramine maleate, dexamethasone, and mefenamic acid for an upper respiratory tract infection. Laboratory evaluation revealed mild anemia, hypoalbuminemia, and electrolyte imbalance. The SCORTEN at admission was 1, corresponding to a predicted mortality of 3.2%. All suspected medications were discontinued immediately. The patient received intensive supportive care, multidisciplinary management, and early high-dose systemic corticosteroids. Rapid clinical improvement was observed, with cessation of new blister formation, progressive re-epithelialization, and complete remission without sequelae at follow-up.

This case underscores that TEN may occur in adolescents following exposure to commonly prescribed medications and may present with extensive mucocutaneous involvement without internal organ failure. The presence of multiple potential culprit drugs may complicate etiological identification, representing a diagnostic challenge. Early recognition, immediate withdrawal of suspected agents, and comprehensive supportive management are critical to achieving complete recovery and preventing life-threatening complications.

Dupilumab-induced osteomuscular syndrome in a child with atopic dermatitis.

C.A. Gonzalez Montealegre — 2026-04-13

Atopic dermatitis (AD) is a chronic inflammatory skin disease that in some cases requires systemic treatment to achieve adequate disease control. Dupilumab is a fully human monoclonal antibody that blocks interleukin-4 (IL-4) and interleukin-13 (IL-13) signaling by acting as an antagonist of the interleukin-4 receptor alpha subunit (IL-4Rα). However, several adverse events have been reported, including musculoskeletal symptoms.

We report a case of dupilumab-induced non-inflammatory arthralgia. A 13-year-old girl with moderate AD was treated with dupilumab. Four months after treatment initiation, she developed left knee pain and low back pain. She was treated with nonsteroidal anti-inflammatory drugs and physical therapy without clinical improvement. Imaging and laboratory investigations showed no abnormalities. Based on these findings, an osteomuscular syndrome secondary to dupilumab administration was suspected. The drug was discontinued, and clinical improvement was observed four months after treatment withdrawal.

Acute generalized exanthematous pustulosis induced by amoxicillin-clavulanate in a child: the diagnostic utility of ultraviolet dermoscopy.

Patil Shrinivas — 2026-04-13

Acute generalized exanthematous pustulosis (AGEP) is a rare, acute pustular drug reaction, most commonly triggered by β‑lactam antibiotics. In children, early clinical features may overlap with infectious exanthems, leading to diagnostic uncertainty. We report a case of amoxicillin–clavulanate–induced AGEP developing in temporal succession after a scarlet fever–like illness in a six‑year‑old girl, with emphasis on the diagnostic contribution of ultraviolet (UV) dermoscopy. The child initially presented with fever, diffuse erythema with flexural accentuation, and strawberry tongue. Following initiation of amoxicillin–clavulanate, she developed multiple non‑follicular sterile pustules predominantly involving the bilateral axillae and groin within 24 hours. Dermoscopy revealed whitish to yellowish structureless pustules, while UV dermoscopy demonstrated multiple discrete to confluent yellow‑green fluorescent pustules on a dark background. Withdrawal of the offending drug resulted in rapid resolution with superficial desquamation. This report highlights the role of UV dermoscopy as a useful bedside adjunct in the early recognition of AGEP in pediatric patients.

Phylloid-Type Pigmentary Mosaicism with Transient Mast Cell Enrichment and a Darier-Like Reaction

G. Okan — 2026-04-13

We report a 6-month-old infant with phylloid-type pigmentary mosaicism presenting with a localized Darier-like reaction. Histopathologic examination revealed an increased scattered dermal mast cells, the absence of features consistent with mastocytosis and laboratory findings showed mildly elevated histamin level. Clinical reactivity and biochemical abnormality resolved during follow-up. Such findings suggest that transient mast cell enrichment within mosaic skin during infancy may represent a reactive process rather than clonal mast cell disease.

Langerhans cell histiocytosis in two infants.

A. Ramani — 2026-04-13

Langerhans cell histiocytosis (LCH) is a rare histiocytic neoplastic disorder that mainly affects the skin and bones, with dermatological manifestations that can be easily confused and misdiagnosed with other dermatological conditions, such as seborrheic dermatitis, fungal infections, atopic dermatitis, psoriasis, and cutaneous lymphomas. Here, we report two cases Langerhans cell Histiocytosis. We report these cases to highlight importance of early diagnosis and multidisciplinary approach, as each of the two cases had different outcomes.

Deep circumscribed morphea in a 3-year-old child.

N.S. Haq — 2026-04-13

Morphea, or localized scleroderma, is a rare inflammatory disorder characterized by fibrosis of the skin and underlying tissues. In pediatric patients, delayed diagnosis may lead to functional impairment and growth disturbances, particularly when deeper structures are involved. We report a case of a three-year-old boy who presented with progressive skin induration and hypopigmented macules on the left lower limb, along with hyperpigmented plaques on the abdomen. Over the course of one year, the lesions became firm and retracted, resulting in mild limb asymmetry. Histopathological examination revealed epidermal atrophy, dermal fibrosis, and lymphocytic infiltration. Laboratory evaluation showed elevated antinuclear antibodies and anti–double-stranded DNA levels, while magnetic resonance imaging demonstrated subcutaneous and muscle atrophy with a mild limb length discrepancy. Based on clinical, histological, and radiological findings, a diagnosis of deep circumscribed morphea was established. The patient was treated with systemic methotrexate in combination with topical emollients, leading to lesion softening and stabilization without further disease progression after six months of follow-up. This case highlights the importance of early recognition and appropriate systemic therapy in pediatric morphea to prevent long-term functional and growth-related complications.

Contents, issue 2, vol. 36, 2026

STAFF STAFF — 2026-04-13

none