AbstractBackground/ObjectivesPediatric lichen planus (LP) is rare with variable prevalence and atypical presentations compared to adults. Data on LP are lacking for the pediatric population in the United States. We present demographics, presentations, and treatments for a pediatric LP cohort.MethodsWe reviewed 26 patients diagnosed with LP at 20 years or younger. Treatment responses were defined as no response, partial response, and complete response.ResultsDemographics included 54% females and median diagnosis age of 16 years (range 6–20). Most patients presented with cutaneous LP (65%), with fewer having associated oral (23%), nail (7.7%), or genital (3.8%) involvement. Some had cutaneous‐only LP (38%) or strictly mucosal LP (oral‐only 19% and genital‐only 15%). LP lesions were pruritic (50%), painful (19%), and/or asymptomatic (35%). Complete/partial responses occurred with medium‐potency topical corticosteroids in cutaneous (n = 7; 64%), oral (n = 3; 75%), and genital LP (n = 3; 100%), with high/ultra‐high potency topical corticosteroids in oral LP (n = 6; 86%), and with topical calcineurin inhibitors in genital LP (n = 2; 100%). Side effects were clobetasol‐related oral candidiasis and biopsy‐related penile depressed scar. Most patients with available follow‐up achieved remission (n = 17; 81%).ConclusionsPediatric LP usually presents in adolescence with cutaneous involvement and is symptomatic. However, patients frequently can have oral, genital, or nail lesions or may be asymptomatic, so they need thorough examinations and follow‐up. Long‐term remission is common due to treatment or natural disease course. Medium‐potency corticosteroids are recommended for cutaneous, oral, and genital LP. Various other local and systemic therapies exist with successful treatment responses.
Histopathological features of pemphigoid gestationis and polymorphic eruption of pregnancy: A blinded retrospective comparative study of 31 cases
Abstract Background Pemphigoid gestationis (PG) and polymorphic eruption of pregnancy (PEP) are pregnancy‐related dermatoses. Definitive diagnosis often relies upon histopathology and direct immunofluorescence (DIF). PG is associated with fetal and neonatal risks, while PEP confers minimal risk. Objective We aimed to compare histopathologic features to determine key differentiators. Methods A retrospective cohort study of PG and PEP cases, with accompanying DIF, conducted from 1995 to 2020. Skin biopsies were examined independently in a blinded fashion by two dermatopathologists for a list of histopathological features. Results Twenty‐one cases of PG and 10 cases of PEP were identified. PG had significantly denser eosinophils than PEP (mean 155 vs. 48 cells/5 hpf; p < 0.018). PG was also noted to have eosinophilic spongiosis and eosinophils at the dermal–epidermal junction more frequently compared to PEP (80% PG vs. 10% PEP; p < 0.001). A mean cutoff value of 86 eosinophils and a mean optimal sensitivity and specificity of 81% and 83%, respectively, for eosinophils density's diagnostic power of PEP versus PG were achieved. Subepithelial separation was exclusively seen in PG (40% vs. 0%; p < 0.007). Conclusion Eosinophilic spongiosis, eosinophilic epitheliotropism, and dense superficial dermal eosinophils were diagnostic of PG. Given overlapping clinicopathologic features, however, DIF results with clinicopathologic correlation, remain the gold standard.
Autoimmune progesterone dermatitis: a retrospective case series
Background: Autoimmune progesterone dermatitis (APD) is a rare hypersensitivity disorder characterized by recurring dermatologic manifestations during the luteal phase of the menstrual cycle in women. Well-defined clinical and diagnostic criteria, outcomes measurements, and standard treatments are lacking. Methods: We performed a single-institution retrospective review of adult patients (older than 20 years at the time of diagnosis) with APD. Results: Fourteen patients were included with mean age of clinical onset of 34.3 ± 7.7 (range 24-54) years. There was a delay of 3.9 ± 5.5 (range 0.4-20) years between the onset of disease symptoms and diagnosis. The onset of APD was after exposure to exogenous progesterone in 9 of 14 patients. Progesterone skin test was performed in 9 patients and 6 were positive. Patients frequently presented with urticaria (9/14, 64.3%) and dermatitis (4/14, 28.6%). Continuous combined oral contraceptives (4/14, 28.6%), gonadotropin-releasing hormone agonist (3/14, 21.4%), and hysterectomy with bilateral salpingo-oophorectomy (2/14, 14.3%) were the most common attempted treatments with reliable outcomes. Conclusions: APD is a rare disorder which lacks universal diagnostic measures and criteria, contributing to a significant delay in diagnosis. Large-scale multicenter studies are needed to develop accurate tests, establish diagnostic criteria, and define treatment outcomes.
An investigation of metabolic disturbances, including urinary stone disease, hypothyroidism, and osteoporosis in basal cell nevus syndrome
AbstractBackground/ObjectivesBasal cell nevus syndrome (BCNS) is an autosomal dominant skin cancer predisposition syndrome associated with abnormal mineral metabolism, a risk factor for urinary stone disease (USD). However, no research investigating the association between BCNS and USD or other manifestations of abnormal mineral metabolism has been conducted. The objective of this study is to investigate the association between BCNS and conditions associated with disordered mineral metabolism including USD, hypothyroidism, and osteoporosis and compare them to prevalence in the general population to elucidate potential unknown manifestations of the condition.MethodsThis retrospective study examined medical records of adult and pediatric patients with confirmed BCNS from the Mayo Clinic database from 1 January 1995 to 12 January 2020. Records were surveyed for evidence of USD and other comorbidities potentially related to BCNS. The studied cohort included 100 adult patients and 5 pediatric patients.ResultsA total of 105 patients were included in this analysis, 10 of whom experienced confirmed USD, representing a prevalence of 10%. Six adult patients were identified with a diagnosis of osteoporosis, representing a prevalence of 6%. Thirteen adult patients were identified with a diagnosis of hypothyroidism, representing a prevalence of 13%.ConclusionsThis study identified a prevalence of USD in BCNS patients comparable to estimates of national prevalence, indicating that known abnormalities in mineral metabolism likely do not increase the incidence of USD in BCNS patients. Additional findings included increased prevalence of hypothyroidism and decreased prevalence of osteoporosis in the BCNS cohort compared to national averages.
Granulomatous cheilitis in children: three cases successfully treated with intralesional corticosteroids
AbstractBackground/ObjectiveCurrent knowledge about usage of effective, but non‐first‐line topical acne medications in the United States is limited. We aimed to investigate utilization patterns and temporal trends for such acne medications in the US ambulatory care.MethodsPediatric (≤18 years old) and adult (>18 years old) data from the 2012 to 2016 (inclusive) cycles of the US National Ambulatory Medical Care Survey were extracted. Utilization patterns of six non‐first‐line topical acne medications (ie, azelaic acid, salicylic acid, glycolic acid, sulfur, resorcinol, and zinc) were compared and followed over time.ResultsData from 218 410 US office–based sampled visits during 2012‐2016 were included in the analysis. Across all acne visits (n = 1542), salicylic acid (1.58%), azelaic acid (1.22%), and glycolic acid (0.52%) were the most frequently used agents, while zinc and resorcinol were not used. Sulfur (0.52%) and salicylic acid (0.33%) were the only medications used in preadolescents, and none of these medications were used in the neonatal or infantile group. Temporal trends for using at least one of these medications were insignificant among both pediatric and adult age groups (P = .825 and .136, respectively).ConclusionsSalicylic acid and azelaic acid are the most frequently used of the studied second‐line medications to treat acne, although the use of these and the other non‐first‐line topical medications overall is uncommon, especially among younger groups of US pediatric patients.