Pediatric Dermatology最新文献

Cutaneous Legionella infection is rare and primarily seen in immunosuppressed patients. Herein, we present a case of cutaneous and pulmonary legionellosis presenting with fever and erythematous subcutaneous nodules in a neonate with severe combined immunodeficiency. This case underscores the importance of considering this diagnosis and highlights the use of modern testing modalities to promptly diagnose and treat infections in immunocompromised patients.

Juvenile pityriasis rubra pilaris is a rare inflammatory skin disorder currently without any FDA-approved treatments, and lesions can be refractory to conventional treatment with topical corticosteroids, methotrexate, and oral retinoids. We herein present a case of a 6-year-old boy who attained clearance of extensive juvenile pityriasis rubra pilaris within 2 weeks of starting ixekizumab therapy. Therapeutic effect has been durable at 6 months, and patient continues on therapy without adverse effects. Our case highlights a new, rapidly effective treatment option for pediatric patients with this rare condition.

A 10-year-old boy presented with persistent genital erythematous plaques unresponsive to traditional topical treatments. Apremilast, an underexplored option in pediatric cases, was initiated and resulted in a significant reduction in pruritus and resolution of the lesions. This case provides insight into the potential efficacy of apremilast in refractory pediatric inverse psoriasis and underscores the necessity for further research in this specific population.

Purpose: Data on acute hemorrhagic edema of infancy (AHEI) are derived from small case series or case reports. We report a 20-year experience at a national referral center.

Methods: We performed a single-center retrospective study including patients who were diagnosed with AHEI from January 1, 2004, to June 30, 2023.

Results: We identified 21 patients (57.1% females) with a median age of 18 months (range 7-33 months). Thirteen (61.9%) patients were admitted to the pediatric ward, the remaining eight (38.1%) presented to the emergency department and were discharged for outpatient management. The median length of hospitalization was 5 days (range 3-9 days). Twenty patients (95.2%) had prodromal symptoms. The most common cutaneous findings were targetoid purpuric plaques. The lesions were most localized on the face (13, 61.9%) and on the upper limbs (18 patients, 85.7%). Sixteen (76%) patients presented with nonpitting and tender edema, localized on the feet (9/16, 56%) and hands (6/16, 37.5%). Systemic involvement was rare, and no patients experienced complications or sequelae. Twelve (57.1%) patients underwent infectious disease investigations, with positive results in only four (33.3%). None of the patients diagnosed after the SARS-CoV-2 outbreak (March 2020) had positive nasopharyngeal swabs for the virus. For the 13 patients who were admitted to the pediatric ward, the median length of hospitalization was five days (3-9 days).

Conclusions: The 21-patient single-center cohort of children affected by AHEI confirmed a generally benign course of AHEI, despite a 62% rate of hospitalization.

Background: Cutaneous (or "Metastatic") Crohn disease (CCD) is a rare and underrecognized disease characterized by cutaneous granulomatous inflammation. We describe patient demographics, clinical characteristics, histology, and treatment of 89 pediatric cases of CCD, including 78 previously reported and 11 new cases seen at four academic institutions. We emphasize the efficacy of biologic mono- and dual therapy.

Methods: PubMed identified cases using keywords including "metastatic Crohn disease" and "cutaneous Crohn disease". Patients were identified by retrospective review of the electronic health record including histopathologic diagnosis consistent with CCD. Chart review collected demographic, clinical, and histologic data.

Results: Most pediatric patients with CCD are male 55% (49/89), present with edema (73/89, 82%) and erythema (47/89, 53%) of the genitals (33/49, 67%), and have intestinal Crohn disease (69/89, 78%). Oral corticosteroids (53/75, 71%) and metronidazole (29/75, 39%) are the most frequently prescribed medications. Of the 17 patients treated with tumor necrosis factor (TNF)-blockade, 94% (16/17) had partial or total clearance. Ustekinumab resulted in clearance of cutaneous disease in two patients (2/3, 67%) and partial clearance in one patient (1/3, 33%). Two cases achieved total clearance with the use of dual biologic therapy defined as the use of two biologic therapies with differing mechanisms of action or the use of a biologic therapy and small molecule inhibitor.

Conclusions: TNF blockade is an effective treatment for pediatric CCD, and interleukin-12/23 inhibitors may be similarly effective. Consideration of dual biologic therapy may be useful in pediatric patients requiring discordant therapies for their intestinal and cutaneous CD.

PLACK syndrome (OMIM 616295) is a rare genodermatosis associated with peeling skin, leukonychia, acral punctate keratosis, cheilitis, and knuckle pads and is caused by loss-of-function mutations in the CAST gene, which encodes calpastatin, a calcium-dependent protease. This case report highlights a case of PLACK syndrome presenting with the unique findings of striate hyperkeratosis on the palms as well as life-threatening cardiomyopathy. We review why CAST mutations might impact cardiac function and raise awareness of the potential association between PLACK syndrome and cardiac manifestations.

Calcinosis cutis (CC) is characterized by the deposition of calcium salts in the skin and subcutaneous tissues. CC involving the vulva or foreskin (prepuce) is uncommon. We present a 9-year-old female with vulvar CC and a 15-year-old male with preputial CC. Microscopic review of excisional specimens revealed calcification associated with follicular cysts in the vulvar case and lichen sclerosus in the preputial case, suggesting a dystrophic origin to a subset of cases of genital CC that might otherwise be classified as idiopathic. The clinical implication of these findings is the need for close histopathologic scrutiny and ongoing clinical surveillance of patients with genital CC initially deemed idiopathic.

Although many clinical variants of Staphylococcus aureus infection are well-recognized, atypical presentations may mimic other conditions. We describe two cases of atypical S. aureus infections in pediatric patients: a S. aureus infection presenting with a vesicopustular rash mimicking varicella zoster virus and a case of multifocal panniculitis. Both of these cases were specifically caused by methicillin-resistant S. aureus (MRSA). Additional cases of atypical S. aureus infections and presenting features from the current literature are also discussed.