Cross-sectional characteristics of pediatric-onset discoid lupus erythematosus: Results of a multicenter, retrospective cohort study.

BACKGROUND: The incidence of systemic lupus in children with discoid lupus is unknown. OBJECTIVE: This study assessed the baseline characteristics of patients with pediatric discoid lupus erythematosus (pDLE). METHODS: Medical records at 17 sites were reviewed for pediatric dermatology and rheumatology patients with discoid lupus erythematosus. The inclusion criteria were clinical and/or histopathologic diagnosis of discoid lupus erythematosus with an age at onset of <18 years. Baseline data were collected at the first documented visit. Outcomes included diagnosis of systemic lupus erythematosus (SLE) at the baseline visit using the 1997 American College of Rheumatology (primary) and the 2012 Systemic Lupus International Collaborating Clinics (secondary) criteria. RESULTS: Of the >1500 charts reviewed, 438 patients met the inclusion criteria. The cohort was predominantly female (72%) and racially/ethnically diverse. A diagnosis of SLE at the baseline visit (pDLE + SLE) was rendered in 162 (37%) patients using the American College of Rheumatology and in 181 (41%) patients using the Systemic Lupus International Collaborating Clinics criteria. Patients with pDLE + SLE were older at the time of rash onset (median, 12.9 vs 8.9 years; P < .001), with shorter time from discoid lupus erythematosus onset to diagnosis, compared with patients with pDLE-only (median, 2 vs 7 months; P < .001). Patients with pDLE + SLE were more likely to be female (P = .004), with generalized discoid lupus erythematosus and clinically aggressive disease, including end-organ involvement, positive serologies, and higher- titer levels of antinuclear antibodies (P < .001). LIMITATIONS: Retrospective study. CONCLUSION: A diagnosis of discoid lupus erythematosus in adolescence should prompt thorough screening for SLE.

Recent advances in hidradenitis suppurativa in pediatrics.

Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition that can cause significant physical, mental, and socioeconomic burden. There remains a paucity of literature on HS in the pediatric population. This systematic review highlights recent advances in pediatric HS in epidemiology, presentation, comorbidities, and management. PubMed, Embase, Google Scholar, and Clinicaltrials.gov databases were used to identify trials and articles published on HS in pediatric patients between January 2015 and October 2019. A total of 39 articles were included. Current evidence suggests that pediatric onset HS may be associated with genetic factors along with endocrine and metabolic abnormalities. Delayed diagnosis in children with HS contributes to poor outcomes. Overall, children and adults with HS share similar lesion types and involved areas. Pediatric HS is associated with a number of comorbid conditions including acne, obesity, inflammatory joint disease, Down syndrome, inflammatory bowel disease, and diabetes. There are currently no pediatric treatment guidelines. Adalimumab is approved for the treatment of moderate-to-severe HS in children 12 and older. Other targeted immunomodulators and hormonal modulators are under investigation. Although the number of studies concerning HS are increasing, further investigation is warranted to better characterize HS, facilitate early diagnosis, and determine the best management for children.

Segmental pigmentation disorder.

BACKGROUND: There is little published information about segmental hypo- and hyperpigmentation pigmentation disorder (SegPD) although it is a relatively common problem in paediatric dermatology. OBJECTIVES: To define the spectrum of disease, clinical presentation and associations in cases of SegPD and to clarify further the terminology in defining patterned hypo- and hyperpigmentation in children. METHODS: This was a retrospective review of cases in an academic paediatric dermatology practice. Thirty-nine patients referred for dermatological evaluation were diagnosed with SegPD. Demographic and clinical features, and distribution and frequency of extracutaneous abnormalities were measured. RESULTS: Twenty female and 19 male patients were included in the study; 33 out of the 39 were referred specifically for a pigmentation abnormality. The mean age at onset was 3·4 months (median age 0·25 months). Family history was positive in two patients. Most (30/39; 77%) had segmental hyperpigmentation whereas nine of 39 (23%) had hypopigmentation. Patches were more often delineated at the ventral midline (32/39) than on the dorsal midline (7/39). The distribution of lesions was as follows: areas of the torso were most often affected (77%) and when the face, neck, arms and legs were affected pigmentation usually extended onto the torso; six patients had SegPD localized to the face. Only three of the 39 patients had extracutaneous abnormalities - atrial septal defect, strabismus with retinal hypopigmentation and a bronchogenic cyst - but the relationship to SegPD was uncertain and none had neurological abnormalities. CONCLUSIONS: SegPD is a relatively common pigmentary anomaly and most affected individuals are otherwise healthy. We propose reviving the term 'segmental pigmentation disorder' coined by Metzker and colleagues to describe children with segmental and block-like hypo-/hyperpigmentation with midline demarcation.