A fatal case of hemophagocytic lymphohistiocytosis due to neonatal lupus erythematosus.

Neonatal lupus erythematosus (NLE) is an autoimmune disease caused by the passive transfer of autoantibodies from mother to child during pregnancy. A rare complication of NLE is hemophagocytic lymphohistiocytosis (HLH), a potentially life-threatening hyperinflammatory state more commonly associated with other rheumatologic disorders. Herein, we describe a fatal case of NLE-associated HLH.

Successful Treatment of Keloids and Hypertrophic Scars With Systemic and Intralesional Dupilumab.

Keloids and hypertrophic scars negatively impact the quality of life for millions of people in the world. Unfortunately, though many thera-peutic approaches are used to treat scars, they are often limited in efficacy with high rates of recurrence. Lately, a better understanding of the immune dysregulation of several dermatologic conditions has led to the emergence of multiple cytokine-targeted therapies for numerous conditions. Several studies have implicated T helper 2 (Th2) immune dysregulation in the development of scars and keloids, with interleukins (IL)-4 and -13 identified as pro-fibrotic mediators. Dupilumab is an IL-4 receptor alpha antagonist that inhibits the ex-pression of both IL-4 and -13. Herein, we describe a 44-year-old woman who developed numerous disfiguring hypertrophic scars and keloids after suffering from a severe herpes zoster infection. Given the number of scars, intralesional corticosteroid injections were not feasible. Therefore, treatment with systemic dupilumab was initiated. Many scars flattened, several even developing a cigarette-paper-like texture due to rapid involution. The largest and most recalcitrant keloid was further treated with intralesional dupliumab injec-tions every 2 weeks with an even more dramatic improvement noted in 2 months. To our knowledge, this is the first report of treating multiple keloids and hypertrophic scars with both systemic and intralesional dupilumab. Dermatologists may want to consider treating keloids that cover a large area with systemic dupilumab, a therapy with an established, reassuring safety profile. The most recalcitrant areas may further benefit from concentrating dupilumab by intralesional delivery. J Drugs Dermatol. 2023;22(12):1220-1222.  doi:10.36849/JDD.6385.

Periorbital hypopigmentation and telangiectasias: Clues to diagnosing neonatal lupus in skin of color.

Neonatal lupus erythematosus (NLE) is an autoimmune disease characterized by a periorbital erythematous rash. Although post-inflammatory hypopigmentation and telangiectasias are known possible sequelae, these features may be particularly noticeable in skin of color. Herein, we describe two infants with skin of color in whom periorbital hypopigmentation and telangiectasias were clues to the diagnosis of NLE.

Perineal desquamation: An early sign of the Kawasaki disease phenotype of MIS-C.

Multisystem inflammatory syndrome in children (MIS-C) is a syndrome associated with coronavirus disease 2019. Various phenotypes of MIS-C have been described including Kawasaki disease (KD). Although perineal desquamation is a known early sign of KD, to our knowledge, this rash has not yet been described in the KD phenotype of MIS-C. In this article, we report two patients in whom perineal desquamation was an early clue for the KD phenotype of MIS-C.

Recurrent reactive infectious mucocutaneous eruption (RIME): Insights from a child with three episodes.

Reactive infectious mucocutaneous eruption (RIME) was recently proposed to replace the term Mycoplasma pneumoniae (MP)-induced rash and mucositis to account for the fact that non-MP pathogens may also cause rash and mucositis. In this report, we describe a unique case of recurrent RIME featuring a total of three episodes. As two of the episodes demonstrated contemporaneous infection with MP and group A streptococcus or influenza B, this case lends further support to use of the term RIME. In addition, although RIME typically involves at least two mucous membranes, this case shows that recurrent episodes may fall into the rare exception in which mucositis is limited to one site.

Eosinophilic Fasciitis: an Updated Review on Diagnosis and Treatment.

PURPOSE OF REVIEW: First recognized in 1974, eosinophilic fasciitis (EF) is a fibrosing disorder of the fascia with characteristic cutaneous and hematologic manifestations. This review discusses recent trends in the diagnosis and treatment of EF. RECENT FINDINGS: Although fascial biopsy has classically been considered the gold standard for making a diagnosis of EF, radiologic imaging, particularly magnetic resonance imaging, has been increasingly used for both diagnosis and monitoring of treatment response. Systemic corticosteroids remain the first-line treatment for EF; however, their often prolonged use in the treatment of EF has prompted a search for adjunctive therapies. Methotrexate has emerged as the leading corticosteroid-sparing agent for EF. Since EF was initially described over 40 years ago, important diagnostic and therapeutic progress has been made. Future efforts should be directed at the pursuit of prospective studies including clinical trials and evidence-based guidelines.