Davis, Dawn Marie R.

AbstractBackgroundConflicting data have been published on whether an association exists between atopic dermatitis (AD) and nonmelanoma skin cancer. This study aimed to determine whether individuals with AD had an increased risk of squamous cell carcinoma (SCC) development.MethodsWe conducted a retrospective, case–control study of patients residing in Olmsted County, Minnesota. Cases were selected from patients seen at Mayo Clinic (Rochester, Minnesota) who had an initial SCC diagnosis (either invasive SCC or SCC in situ) from January 1, 1996, through December 23, 2010. Age‐ and sex‐matched controls were selected from patients seen at Mayo Clinic with no history of SCC before the case event date.ResultsThree hundred ninety‐nine individuals with a documented history of SCC were identified and matched with 780 controls who did not have a history of SCC. After adjusting for race, smoking history, ionizing radiation exposure, corticosteroid and cyclosporine use, and non‐SCC skin cancers, the odds ratio for SCC development between patients with history of AD versus patients without history of AD was 1.75 (95% CI, 1.05–2.93).ConclusionsOur findings support an increased risk of SCC development in the setting of AD.

AbstractLinear morphea of the limb primarily affects children, and extracutaneous manifestations are common. Orthopedic surgeons are often essential in the care of patients with linear morphea, yet there are few reports outlining specific orthopedic complications in this population. We sought to improve the understanding of orthopedic complications in linear morphea of the limb. Between 1999 and 2014, 51 children were evaluated for linear morphea of an extremity. Twenty‐six (51%) had documented orthopedic manifestations. Outcome measures included limb length discrepancy, angular malalignment, limb atrophy, and orthopedic surgical intervention. Joint contractures were most common, affecting 88% of patients, followed by limb atrophy, angular deformity, and limb length discrepancy; 14% required surgical intervention. Despite the use of systemic immunosuppressive therapy in many patients, approximately half of patients with linear morphea of an extremity have orthopedic disease. Early orthopedist involvement is crucial to improve limb alignment and preserve function.

Abstract Background Anti–tumor necrosis factor alpha ( TNF ‐α) agents are used to treat a variety of autoimmune and inflammatory conditions, including psoriasis. Paradoxically, numerous reports have documented new‐onset or exacerbation of psoriasis or psoriasiform skin lesions ( PSO ) in patients treated with these agents for conditions other than PSO—particularly in adults with inflammatory bowel disease ( IBD ). Not much is known regarding similar cases in children. Methods A retrospective chart review was performed on children younger than 19 years of age with IBD seen at the Mayo Clinic between 2003 and 2015 who developed new‐onset or recurrent PSO while undergoing anti‐ TNF ‐α therapy. Results Fourteen children developed PSO while undergoing anti‐ TNF ‐α therapy for IBD . All three anti‐ TNF ‐α agents (infliximab, adalimumab, certolizumab) used to treat IBD in this series led to induction or recurrence of PSO lesions. The median time to development of PSO was 11 months (range 0–48 mos), the median age was 15 years (range 12.5–17.5 yrs), and 57% of patients were male. IBD activity was quiescent in 93% of cases at PSO onset. Seven patients (50%) discontinued their initial anti‐ TNF ‐α therapy because of their skin disease. Ultimately, four patients (29%) had to discontinue all anti‐ TNF ‐α therapy to induce PSO resolution. Conclusion TNF ‐α antagonist–induced PSO in children with IBD is a rarely reported adverse reaction. PSO onset has a variable latency, but usually occurs during IBD remission, with a slight male bias. Nearly half of patients required a change in their initial anti‐ TNF ‐α agent despite conventional skin‐directed therapies, and one‐third of patients discontinued all anti‐ TNF ‐α therapy because of PSO .

AbstractBackground/ObjectivesPyoderma gangrenosum (PG) is a neutrophilic dermatosis rarely seen in children. Its features have not been well characterized in children. We sought to characterize the clinical features, etiologic associations, and treatment of PG in children younger than 18 years.MethodsWe performed a retrospective review of children younger than 18 years with PG at the Mayo Clinic from January 1976 to August 2013.ResultsThirteen children with PG were identified (n = 8; 62% female). All had ulcerations, with 62% having pustular lesions. Sites of involvement included the trunk (77%), lower extremities (77%), upper extremities (38%), and head and neck (38%). Nine (69%) had an underlying comorbidity, including seven with Crohn's disease (54%), one with juvenile idiopathic arthritis (8%), and one with pyogenic arthritis, pyoderma gangrenosum, and acne syndrome (8%). Treatments included topical or local care (92%) and systemic therapies (85%) such as oral corticosteroids (62%) and sulfasalazine or related 5‐aminosalicylate drugs (46%). The clinical course did not correlate with that of the underlying systemic disease and response to treatment varied.ConclusionPediatric PG has a more varied anatomic distribution and a greater predominance of pustular lesions than PG in adults and a strong association with inflammatory bowel disease.

AbstractBackgroundLeukocytoclastic vasculitis (LCV) in children is a complex group of conditions.ObjectivesThis study presents the demographics, clinical features, direct immunofluorescence (DIF) results and suspected aetiologies of 56 biopsy‐confirmed cases of leukocytoclastic vasculitis in children.MethodsRetrospective review of 56 children seen at Mayo Clinic in Rochester, Minnesota, from 1993 to 2013 with clinical features and cutaneous biopsy consistent with LCV.ResultsTwenty‐seven (48%) cases were found to be due to IgA vasculitis (Henoch‐Schonlein purpura). The remaining cases were found to be due to cutaneous small‐vessel vasculitis (n = 19, 34%), urticarial vasculitis (n = 5, 9%), ANCA‐associated vasculitis (n = 4, 7%) and acute haemorrhagic oedema of infancy (n = 1, 2%). IgA vasculitis was found to be associated with abdominal pain (P = 0.008), whereas the non‐IgA vasculitis group was associated with headache (P = 0.052). Children with IgA vasculitis had palpable purpura (P = <0.001), petechia (P = 0.057), vesicles (P = 0.009) and involvement of the buttock (P = 0.004) more frequently than the non‐IgA vasculitis group. On DIF, perivascular IgA was positive in IgA vasculitis compared to non‐IgA vasculitis cases (P = <0.001), the other conjugates were similar between the two groups.ConclusionThe most common subtype of biopsy‐confirmed LCV in children is IgA vasculitis. Clinical features, exam characteristics and DIF results can be helpful in determining the subtype of cutaneous vasculitis in children.

AbstractBackgroundKeratosis pilaris rubra is a common but rarely reported condition characterized by follicular‐based hyperkeratotic papules on a background of erythema. It can be embarrassing and symptomatic for patients, particularly adolescent boys. We sought to explore the efficacy of pulsed dye laser (PDL) in the treatment of keratosis pilaris rubra.MethodsEight patients were treated with PDL for keratosis pilaris rubra.ResultsAll patients reported noticeable improvement after one to four treatments.ConclusionsPDL is an effective, easily accessible, and underused therapy in the treatment of keratosis pilaris rubra.

AbstractBackground: Penile and scrotal swelling can occur as an extraintestinal manifestation of Crohn's disease (CD) and is thought to be an uncommon form of metastatic CD (MCD). Because of the rarity of this manifestation, much is unknown concerning the presentation, treatment, and response to therapy in children with genital MCD. Methods: Boys ages 1 to 17 years presenting with genital edema and a confirmed diagnosis or strong suspicion of CD who were evaluated at the Mayo Clinic between 1996 and 2014 were included for review. We sought to characterize the clinical and pathologic features of genital MCD and response to treatment in our cohort of patients. Results: Eight patients with genital MCD were identified from our institution (mean age 11.4 yrs, range 7–16yrs). Seven (88%) patients experienced cutaneous symptoms before a formal diagnosis of CD was made, and two of the seven had no adverse gastrointestinal symptoms at that time. Patients were prescribed an average of 3.4 medications (topical and systemic) for management of their gastrointestinal CD and MCD. Conclusions: Penile and scrotal swelling can occur as an extraintestinal manifestation of CD and is thought to be an uncommon form of MCD. In boys, genital swelling typically precedes intestinal CD. Treatment of the underlying CD with systemic medications was most helpful in this series. An evaluation for CD is necessary in all patients presenting with unexplained genital swelling.

AbstractBackgroundThe incidence of melanoma has been rising in the United States, with conflicting evidence regarding trends in children.MethodsWe identified patients 0 to 17 years old with a diagnosis of melanoma from January 1, 1970, through December 31, 2010, in Olmsted County, Minnesota. Information on survival and demographic characteristics was abstracted, and estimates of true incidence were calculated.ResultsThe estimated true incidence of melanoma in children from 1970 to 2010 was found to be 0.62 per 100,000 girls and 0.45 per 100,000 boys. The incidence of melanoma in this population did not increase with time after adjusting for age and sex. Only one case of metastatic disease (lymph node) was identified. Girls were more commonly affected and the mean age of disease onset was 14 years. Five of the seven melanomas in this population arose in association with a nevus, and none involved the trunk. Overall and disease‐specific survival rates were not calculated because all patients studied were alive at the last follow‐up.ConclusionThe estimated true incidence rates of pediatric melanoma from our population‐based study in Olmsted County, Minnesota, appear stable. This finding is in contrast to our prior research showing rapidly increasing incidence rates of melanoma in young and middle‐aged adults from the same population.

AbstractThe porphyrias are a group of disorders characterized by defects in the heme biosynthesis pathway. Many present with skin findings including photosensitivity, bullae, hypertrichosis, and scarring. Systemic symptoms may include abdominal pain, neuropsychiatric changes, anemia, and liver disease. With advances in DNA analysis, researchers are discovering the underlying genetic causes of the porphyrias, enabling family members to be tested for genetic mutations. Here we present a comprehensive review of porphyria focusing on those with cutaneous manifestations. In Part I, we have included the epidemiology, pathogenesis, presentation, diagnosis, and histopathology. Treatment and management options will be discussed in Part II.