James Treat

A prospective clinical trial of open-label etanercept for the treatment of hidradenitis suppurativa.

BACKGROUND Medical therapies for hidradenitis suppurativa (HS) are often ineffective. Tumor necrosis factor-alpha inhibitors may be a potential treatment for patients with moderate to severe HS. OBJECTIVES We sought to evaluate the safety and efficacy of etanercept for patients with severe HS. METHODS We conducted a phase II clinical trial of etanercept (50 mg/wk subcutaneously) in patients with moderate to severe HS. Efficacy was measured using a Physician Global Assessment and several secondary physician- and patient-reported outcome measures. Responders were classified as those achieving at least a 50% reduction on the Physician Global Assessment score at week 12 compared with baseline. RESULTS Only 3 of the 15 patients who entered the study were classified as responders (response rate of 20%; 95% confidence interval: 4.3-48.1) based on the intention-to-treat analysis. Dermatology Life Quality Index scores improved slightly from a median of 19 to 15 (P = .02). Comparison of baseline with week-12 Physician Global Assessment scores, and secondary outcome measures of lesion counts and patient pain scores, failed to show statistically significant improvement. Etanercept was generally well tolerated; however, two patients discontinued the study as a result of skin infections at the site of hidradenitis lesions requiring oral antibiotics. LIMITATIONS Lack of a control group and a small number of participants are limitations. CONCLUSIONS Our study demonstrated minimal evidence of clinically significant efficacy of etanercept (50 mg/wk subcutaneously) in the treatment of hidradenitis. Future studies using higher doses of etanercept are indicated; however, patients need to be carefully monitored for infection and other adverse events. Randomized, controlled trials will be necessary to demonstrate the risk-to-benefit ratio of tumor necrosis factor-alpha inhibitors in the treatment of hidradenitis.

Subcutaneous fat necrosis as a complication of whole-body cooling for birth asphyxia

BACKGROUND Subcutaneous fat necrosis (SCFN) of the newborn is a form of panniculitis that affects full-term neonates who often have suffered either birth asphyxia or hypothermia. The induction of hypothermia in newborns is becoming frequently used to reduce the neurologic sequelae associated with birth asphyxia. The risk of SCFN in neonates undergoing this therapy is unknown. Observation We describe a neonate who developed an abscess-like presentation of SCFN and subsequent asymptomatic hypercalcemia after undergoing whole-body cooling for hypoxic-ischemic encephalopathy. CONCLUSIONS Hypothermia protocols may be placing newborns at increased risk for the development of SCFN. Clinicians should recognize this association, and newborns who undergo therapeutic cooling should have frequent dermatologic assessments.

Pediatric Allergic Contact Dermatitis: Lessons for Better Care

Allergic contact dermatitis (ACD) is an immune-mediated condition that is likely underrecognized in children. ACD is the result of primary sensitization and secondary elicitation by allergy-provoking haptens. A detailed clinical history and physical examination may yield diagnosis. Patch testing is the criterion standard diagnostic tool for confirming the diagnosis of ACD in both children and adults. Herein, we present an overview of pediatric ACD, an analysis of relevant current literature on the topic, focused management recommendations, and a discussion of existing knowledge gaps that must be addressed by future research.

Patterned pigmentation in children.

The terms pigmentary mosaicism or patterned dyspigmentation describe a spectrum of clinical findings that range from localized areas of dyspigmentation with no systemic findings to widespread dyspigmentation with associated neurologic, musculoskeletal, and cardiac abnormalities, and other sequelae that can lead to early demise. Given this wide spectrum, these patients must be approached with caution, but with the understanding that most who have localized pigmentary anomalies, such as segmental pigmentary disorder (SegPD) seem to have no systemic manifestations. These patients can be approached in many different ways, but generally children with more widespread dyspigmentation, and any with associated abnormalities or not meeting neurodevelopmental milestones, should be evaluated closely. Children with any red flags warrant subspecialty referral, and all children deserve close clinical follow-up with their primary care physician to ensure they meet all of their developmental milestones. Fortunately, parents can be reassured that most children with SegPD, and many with more widespread patterned pigmentation, are otherwise healthy.

Retrospective Analysis of Beta-Blocker Instituted for Treatment of Hemangiomas (RABBIT Study)

Objective. To evaluate the safety and efficacy of our institutional beta-blocker protocol for treatment of complicated infantile hemangiomas (IH). Study Design. A retrospective descriptive study of 76 infants/children with IH treated with oral propranolol at the Children’s Hospital of Philadelphia between June 2008 and August 2010 was performed, assessing both the safety and efficacy of propranolol. Based on preliminary data showing hemangioma recrudescence off-treatment, we reviewed 9 additional patients with recrudescence between August 2010 and December 2011. Results. Mild adverse events included asymptomatic bradycardia, gastrointestinal symptoms, asymptomatic hypotension, cool hands/feet, asymptomatic hypoglycemia, and sleep disturbance. Sixteen patients had recrudescence of IH off-treatment, with propranolol discontinued at a median age of 14 months (interquartile range 10-15 months). Conclusions. Propranolol appears to be associated with minor, not severe symptomatic adverse events. Propranolol appears to be effective in treating complicated IH. Recrudescence can occur off-treatment, even with discontinuing propranolol as late as 15 months of age.

Expanding the differential diagnosis of fetal hydrops: an unusual prenatal presentation of megalencephaly‐capillary malformation syndrome

Division of Human Genetics, Department of Pediatrics, University of Pennsylvania School of Medicine and The Children’s Hospital of Philadelphia, Philadelphia, PA, USA Division of Neonatology, Department of Pediatrics, University of Pennsylvania School of Medicine and The Children’s Hospital of Philadelphia Philadelphia, PA, USA Department of Surgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA Center for Fetal Diagnosis and Treatment, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA Department of Dermatology, University of Pennsylvania School of Medicine and The Children’s Hospital of Philadelphia, Philadelphia, PA, USA Center for Integrative Brain Research, Seattle Children’s Hospital, Seattle, Washington, WA, USA Departments of Neurology and Pediatrics, University of Washington, Seattle, Washington, WA, USA *Correspondence to: Elaine H. Zackai. E-mail: zackai@email.chop.edu

Vesicles and pustules in the neonate

Vesicles and pustules in the neonate can be due to common or life threatening conditions. Recognizing key clinical features and understanding when and how to workup the various different vesiculopustular eruptions that occur in the neonatal period is vital. A rapid diagnosis of an infectious cause of a neonatal vesiculopustular eruption can be life-saving.

Spread of cantharidin after petrolatum use resulting in a varicelliform vesicular dermatitis

To the Editor: Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome emphasizes the connection between skin and bone diseases. Palmoplantar pustulosis and severe acne conglobata or fulminans are well-known features, but pustular psoriasis, dissecting cellulitis of the scalp, hidradenitis suppurativa, Sweet’s syndrome, and Sneddon-Wilkinson disease have all been reported in association with the syndrome. When skin disease is mild or absent, this diagnosis is difficult to make. A 32-year-old Caucasian woman had a 3-year history of recurrent palmar lesions and 2-year history of a painful enlarged left clavicle head. Mild palmoplantar pustulosis (Fig 1) and a firm, nonmobile, tender, subcutaneous 63 4.5-cm nodule with overlying skin erythema along the left clavicular head were present. Magnetic resonance imaging with gadolinium showed faint enhancement along the first costochondral junction suggesting costochondritis. Bone scintigraphy showed isolated increased tracer activity within the medial clavicles, consistent with SAPHO syndrome (Fig 2). After Chamot et al described 85 cases (13 with severe acne, 44 with palmoplantar pustulosis, and 28 with hyperostosis without skin findings), Benhamou et al established diagnostic criteria including: (1) osteoarticular manifestations of acne conglobata, acne fulminans, or hidradenitis suppurativa; (2) osteoarticular manifestations of palmoplantar pustulosis; (3) axial or appendicular hyperostosis with or without dermatosis; and (4) chronic recurrent multifocal osteomyelitis involving the axial or appendicular skeleton with or without dermatosis. Most series report only 60% of patients with skin involvement. Skin and articular manifestations may precede each other with pustular eruptions occurring 2 to 20 years after bone involvement. Skin lesions may be minimal and escape the notice of nondermatologists. Patients often present with the gradual onset of pain and swelling of the anterior chest wall; young adults have disease primarily in the sternoclavicular region (65%-90%) followed by disease of the spine, pelvis, and long bones. Laboratory values are normal or reveal elevated C-reactive protein, erythrocyte sedimentation rate, and white blood cell count. Early stages of SAPHO syndrome are indistinguishable from osteomyelitis and studies to eliminate septic osteomyelitis are appropriate. Initially there is soft-tissue swelling around the joint, then characteristic osteosclerosis, hyperostosis, and bone hypertrophy. Calcification of the sternoclavicular joints is typical. Computed tomography scan and magnetic resonance imaging can identify soft-tissue and bone abnormalities but nuclear Fig 1. Well-demarcated erythematous plaque on palm with associated deep-seated pustules and overlying scale.

Intractable Nodulocystic Acne in a Patient with Trisomy 13

We report the case of a boy with trisomy 13 who developed severe nodulocystic acne with sinus tract formation that has been unresponsive to multiple courses of systemic antibiotics and isotretinoin.

A Case of Exuberant Candidal Onychomycosis in a Child With Hyper IgE Syndrome

An 8-year-old girl with recurrent oral thrush as an infant and toddler, multiple diaper yeast infections, recurrent ear infections that required multiple ear-tube placements, a long history of mild atopic dermatitis, and at least 7 Staphylococcus aureus abscesses on the extremities presented with new-onset thickening of the thumbnail that had failed therapy with 40% urea cream (Figure 1). Of note, she had no history of bronchopulmonary infections. Periodic acidSchiff stained sections of a nail-plate clipping revealed yeast