Anne W. Lucky

Five Cases of Anti-Tumor Necrosis Factor Alpha–Induced Psoriasis Presenting with Severe Scalp Involvement in Children

Abstract:u2002 Although anti‐tumor necrosis factor alpha (TNF‐α) agents are commonly used to treat psoriasis and other inflammatory diseases in adults and children, numerous reports have documented new‐onset or flaring psoriasis in adults treated for the other conditions. Individual case reports have documented similar observations in three children. We report a series of anti‐TNF‐α‐induced psoriasis in children with juvenile idiopathic arthritis or inflammatory bowel disease treated at a large children’s hospital. All five patients presented with severe scalp involvement. One child was treated with adalimumab for juvenile idiopathic arthritis, and four received infliximab for inflammatory bowel disease. The five patients developed psoriasis 2 to 10u2003months after initiating anti‐TNF‐α therapy. They presented with erythematous, scaly, crusted scalp lesions. Three of the five patients were initially treated with griseofulvin for presumed tinea capitis. The anti‐TNF‐α agent was discontinued at the time of diagnosis in two cases. Topical steroids were the mainstay of psoriasis therapy, with improvement in four of five patients. Anti‐TNF‐α agents have been associated with the onset or worsening of psoriasis in adults, but this has rarely been reported in children. We describe five pediatric cases of anti‐TNF‐α‐induced psoriasis presenting with severe scalp involvement and review their subsequent management. We hope that clinicians caring for patients receiving anti‐TNF‐α agents will consider psoriasis from the onset of cutaneous symptoms and institute appropriate therapy or referral.

Growth, Bone Mineral Accretion, and Adrenal Function in Glucocorticoid-Treated Infants with Hemangiomas – A Retrospective Study

Abstract:u2002 Hemangiomas, common proliferative vascular tumors, can grow rapidly in the first months of life. Although therapy with high‐dose oral glucocorticoids is standard for lesions that threaten vital functions or are disfiguring, little is known about the endocrine consequences of this treatment. Using retrospective data, we examined growth velocity, changes in bone mineral density, and adrenal function in infants with hemangiomas treated with systemic glucocorticoids. Treatment consisted of oral prednisolone 2 to 4 mg/kg/day or dexamethasone 1 mg/kg/day. Mean growth velocity Z score on glucocorticoid therapy was −1.41 standard deviations in 13 patients. In four infants with adequate follow‐up, growth velocity increased to +1.90 standard deviations after glucocorticoid treatment (Δ growth velocity +3.31 standard deviations). Mean lumbar spine bone mineral density Z score was −2.46 standard deviations before glucocorticoid treatment and −1.08 standard deviations at the end of treatment in six infants. Adrenal function after glucocorticoid therapy was assessed by low‐dose adrenocorticotropic hormone stimulation test in 10 infants. Eight had a normal cortisol response, and one had a borderline response. One infant, who had been treated with dexamethasone, had an abnormal test result. In conclusion, systemic glucocorticoid treatment in infants with hemangiomas slowed linear growth, but “catch‐up” growth was observed after treatment ceased. Glucocorticoids did not affect bone mineralization adversely. Only 1 of 10 glucocorticoid‐treated infants had clear evidence of adrenal insufficiency after therapy was stopped.

Progressive Macular Hypomelanosis in a 16-Year Old

Abstract:u2002 Progressive macular hypomelanosis of the trunk is a disease of unclear etiology that often goes unrecognized in the clinical setting. We present a Caucasian adolescent girl with hypopigmented macules coalescing into patches on her trunk, initially diagnosed as tinea versicolor. Upon further evaluation, the patient was found to have progressive macular hypomelanosis that demonstrated improvement with sunlight exposure and doxycycline. We report this patient to make physicians more aware of this entity and discuss the literature.

Pseudoacne of the nasal crease: a new entity?

Abstract:u2002 In this article we describe a clinical entity appearing in seven preadolescent patients who presented with chronic red papules within a prominent nasal crease. Milia were also noted in the nasal crease, but there was no evidence of acne vulgaris. The duration of symptoms was 4 months to 2 years, and lesions ranged from inflamed red papules, which were treated with topical antiinflammatory medications, to scarred white papules requiring excision. Histologic evaluation of two lesions revealed keratin granulomas that were likely derived from ruptured, inflamed milia. Due to its similarity in appearance to acne vulgaris, but different pathogenesis and clinical course, we suggest naming this newly described entity “pseudoacne of the nasal crease.”

Prevalence and characterization of pruritus in epidermolysis bullosa.

Qualitative data suggest that pruritus is a burdensome symptom in patients with epidermolysis bullosa (EB), but the prevalence of pruritus in children and adults with EB and factors that contribute to pruritus are unknown. The objective of the current study was to quantitatively identify and to characterize pruritus that EB patients experience using a comprehensive online questionnaire. A questionnaire was developed to evaluate pruritus in all ages and all types of EB. Questions that characterize pruritus were included and factors that aggravate symptoms were investigated. Patients from seven North American EB centers were invited to participate. One hundred forty‐six of 216 questionnaires were completed (response rate 68%; 73 male, 73 female; median age 20.0 years). Using a 5‐point Likert scale (1 = never, 2 = rarely, 3 = sometimes, 4 = often, 5 = always), itchiness was the most bothersome EB complication (mean 3.3). The average daily frequency of pruritus increased with self‐reported EB severity. Pruritus was most frequent at bedtime (mean 3.8) and interfered with sleep. Factors that aggravated pruritus included healing wounds, dry skin, infected wounds, stress, heat, dryness, and humidity. Pruritus is common in individuals with EB and can be bothersome. Future studies will need to investigate the most effective treatments given to individuals with EB for pruritus.

Idiopathic Facial Aseptic Granuloma: Review of an Evolving Clinical Entity

Idiopathic facial aseptic granuloma (IFAG), originally termed pyodermite froide du visage, describes a generally asymptomatic facial nodule presenting in childhood with clinical resemblance to pyoderma or cystic, granulomatous, or vascular lesions. Clinical understanding is constantly evolving, with recent observations indicating that IFAG may represent a subtype of childhood rosacea. We present a case of IFAG associated with eyelid chalazions in a 19‐month‐old boy. Although his clinical course paralleled previously reported IFAG cases, we observed a unique ultrasound variation during initial diagnostic examination. Further delineation of clinical, imaging, and histologic properties of IFAG may reveal insights into etiologic associations and ideal management.

Evaluation of Treatments for Pruritus in Epidermolysis Bullosa

Pruritus is a common complication in patients with epidermolysis bullosa (EB). There is limited published data about the treatments that individuals with EB use for pruritus. The objective of the current study was to determine quantitatively which treatments individuals with EB have used for pruritus and to evaluate the perceived effectiveness of these treatments in pruritus relief. A questionnaire was developed to evaluate the treatments and therapies used for pruritus in patients of all ages and for all types of EB. Questions about bathing products, moisturizers, topical products, oral medications, dressings, and alternative therapies were included. A 5‐point Likert scale (−2 = relieves itch a lot, −1 = relieves itch a little, 0 = no change, 1 = increases itch a little, 2 = increases itch a lot) was used to evaluate perceived effectiveness. Patients from seven North American EB centers were invited to participate. Greasy ointments (53.4%), lotions (45.2%), creams (40.4%), and oral hydroxyzine (39.0%) were the most frequently used treatments for pruritus. Treatments that were used frequently and perceived to be the most effective included creams (mean = −1.1), topical prescription corticosteroids (mean = −1.0), oils (mean = −0.9), oral hydroxyzine (mean = −0.9), topical diphenhydramine (mean = −0.9), and vaporizing rub (menthol, camphor, eucalyptus) (mean = −0.9). Systemic opioids (mean = 0.3), adherent bandages (mean = 0.3), and bleach baths (mean = 0.2) slightly increased pruritus. Randomized controlled trials of therapies will be necessary to develop evidence‐based recommendations for control of pruritus in individuals with EB.

Hand Function and Quality of Life in Children with Epidermolysis Bullosa

Patient‐reported outcomes are becoming increasingly important to clinical care. Epidermolysis bullosa (EB), a rare genetic skin disorder, can result in severe hand impairment, but parent and patient perceptions of hand function have never been assessed. This study aimed to quantify parent‐ and patient‐reported hand function and assess its relationship with quality of life (QOL) in children with EB. This cross‐sectional study included children with EB treated at an interdisciplinary EB center. Hospital records were searched for demographic characteristics and medical history. Eligible families were invited to complete two surveys by mail or telephone. The ABILHAND‐Kids questionnaire assessed manual hand ability for 21 functions. The Quality of Life in Epidermolysis Bullosa questionnaire assessed EB‐related QOL. Hand function and QOL of various subtypes were compared using Mann–Whitney tests. Seventy‐one parents and patients ages 2 to 18 years with EB from 20 states in the United States completed questionnaires. Children with recessive dystrophic EB reported the worst hand function and QOL. Bimanual functions involving finger mobility were the most challenging for all EB subtypes. QOL was highly related to the degree of hand function, being correlated with 20 of the 21 individual hand functions and most associated with the ability to perform unimanual functions. Parent‐ and patient‐reported hand function can be measured in children with EB using the ABILHAND‐Kids questionnaire. Hand impairment is strongly associated with worse QOL, probably due to difficulty performing daily activities. The effect of interventions such as hand surgery could be prospectively evaluated using this questionnaire.

Junctional epidermolysis bullosa incidence and survival: 5-year experience of the Dystrophic Epidermolysis Bullosa Research Association of America (DebRA) nurse educator, 2007 to 2011.

Junctional epidermolysis bullosa (JEB) is a particularly devastating type of epidermolysis bullosa, especially in the newborn period. Data about the number of new cases of JEB in the United States were collected from the records of the Dystrophic Epidermolysis Bullosa Research Association of America (DebRA) nurse educator. Seventy‐one children with JEB were reported to have been born in the 5 years between 2007 and 2011, reflecting an incidence of at least 3.59 per million per year, significantly higher than previously estimated (2.04 per million). There was a high prevalence of morbidity and infant mortality of at least 73%, as 52 of the 71 cases proved fatal by June 2012. These data emphasize the need for future research to develop treatment and ultimately a cure for this disorder.

Hemorrhagic, Bullous Henoch Schonlein Purpura in a 16-Year-Old Girl with Previously Undiagnosed Dominant Dystrophic Epidermolysis Bullosa

Abstract:u2002 A 16‐year‐old girl presenting with systemic and cutaneous symptoms of hemorrhagic Henoch‐Schonlein purpura continued to develop bullae on top of old scars. Past history was significant for bullae on the feet and legs after trauma. Based on history, physical examination, disease course, and biopsy, the patient, her mother, and other family members were diagnosed with dominant dystrophic epidermolysis bullosa, explaining the severe course and complications of her Henoch‐Schonlein purpura.