Bernard A. Cohen

Olmsted Syndrome: Case Report and Identification of a Keratin Abnormality

BACKGROUND Olmsted syndrome is a rare disorder characterized by a mutilating palmoplantar keratoderma and periorificial keratotic plaques. It begins in early childhood and is complicated by the development of painful flexion contractures, constrictions, and autoamputations of the digits. Only 11 cases of Olmsted syndrome have been reported to date. However, no biochemical abnormalities in the skin were reported in any of these cases. OBSERVATIONS We report the 12th case of Olmsted syndrome. In addition, we describe a keratin abnormality found in a skin specimen obtained from our patient. The specimen showed a suprabasilar staining pattern with AE1, an antibody that shows only basilar staining in normal skin. CONCLUSION We report the 12th case of Olmsted syndrome, review the literature, and describe a keratin abnormality that was found in our patients skin specimen.

Insect Bite–Induced Hypersensitivity and the SCRATCH Principles: A New Approach to Papular Urticaria

Insect bites and the associated hypersensitivity reactions known as papular urticaria account for a significant number of all referrals from pediatricians and dermatologists to our pediatric dermatology clinic. Unfortunately, children affected by these eruptions are frequently misdiagnosed and often subject to expensive evaluations including invasive and unnecessary procedures. Here we review the course of 4 children with the typical physical findings and natural history of these reactions. On the basis of our clinical findings and experience with this patient population, we propose a set of principles (termed “SCRATCH”) as clinical features to aid clinicians in making an early and accurate clinical diagnosis. We conclude that a more appropriate term for future study and diagnosis of this entity is insect bite–induced hypersensitivity.

Dermatophytid in Tinea Capitis: Rarely Reported Common Phenomenon With Clinical Implications

Tinea capitis may be associated with a dermatophytid, which appears as a disseminated eczematous eruption. This phenomenon may occur before or after initiation of systemic antifungal drug therapy and is not an indication for stopping medication. We present here a series of cases that involve 5 children with tinea capitis who developed a dermatophytid before or during the course of their management. In each child, the eruption resolved despite continuation of oral antifungal therapy. Our experience suggests that dermatophytid secondary to tinea capitis is much more common than reported. Furthermore, parents and clinicians frequently mistake dermatophytid for drug allergy. Recognition of this phenomenon, distinction of dermatophytid from drug allergy, and continuation of systemic treatment is essential for clearing the infection and dermatophytid.

Cardiovascular and blood glucose parameters in infants during propranolol initiation for treatment of symptomatic infantile hemangiomas.

Objectives: We sought to determine the effect of propranolol on cardiovascular and blood glucose parameters in infants with symptomatic infantile hemangiomas who were hospitalized for initiation of treatment, and to analyze adverse effects of propranolol throughout the course of inpatient and outpatient treatment. Methods: A retrospective cohort analysis was performed on 50 infants (age less than 12 months) with symptomatic infantile hemangiomas who were hospitalized for propranolol initiation between 2008 and 2012. Demographic data and disease characteristics were recorded. Systolic and diastolic blood pressures, heart rate, blood glucose values, and adverse events recorded during hospitalization were analyzed. An additional cohort of 200 consecutively treated children was also assessed for adverse events associated with outpatient propranolol use. Results: The median age among the inpatient cohort was 3.4 months (range, 0.8 to 12.0 months). Infants older than 6 months were more likely to exhibit bradycardia than were younger infants (p < 0.001). Hypotensive and/or bradycardic periods were infrequent and were not associated with observable clinical symptoms. The mean systolic and diastolic blood pressures and the mean heart rate decreased significantly from day 1 of hospitalization to day 2 (p = 0.004; p = 0.008; p < 0.001), but not from day 2 to day 3, when the propranolol dose was increased to target. Hypoglycemia was rare (0.3% incidence.) Among the 250 outpatients, 2 infants developed lethargy and hypoglycemia during a viral illness and recovered without sequelae. One infant experienced recurrent bronchospasm with viral illnesses and required concomitant bronchodilator therapy. Conclusions: Frequent deviations from normal ranges of blood pressure and heart rate occur upon initiation of propranolol, but are clinically asymptomatic. These findings support that outpatient initiation of propranolol in healthy, normotensive infants appears to be a relatively safe alternative to inpatient initiation. Hypoglycemia is rare, but can occur throughout the treatment period; parent counseling is of paramount importance.

No biopsy needed for eclipse and cockade nevi found on the scalps of children.

I n the Pediatric Dermatology Clinic at the Johns Hopkins Children’s Center, we evaluate over 5000 children each year, and at least 40% of visits include a full skin examination for assessment of melanocytic nevi. Melanocytic nevi of the scalp constitute a significant subset of referrals, and queries regarding removal of these lesions are often associated with unfounded fear of their malignant potential among primary care providers, dermatologists, and parents. Dermatologists frequently recommend that melanocytic nevi of the scalp be excised, resulting in unsightly football-shaped scars and permanent localized hair loss (Figure 1). Unfortunately, surgery begets surgery, and some patients are subjected to recurrent procedures because parents and pediatricians become involved in notifying dermatologists of new scalp nevi as they appear. Although for darkly pigmented nevi that either fulfill some of the ABCDE criteria for melanoma (asymmetry, border irregularity, color variegation, diameter 6 mm, and/or evolution) or are difficult to follow clinically, removal might be justified, 2 particular clinical variants of scalp nevi that may appear worrisome for having malignant features are actually benign: they evolve in an innocent fashion and require observation only. These nevi, while frequently larger than 6 mm in diameter and multicolored, are notable for their often striking symmetry and distinctively bland and reassuring appearance to the trained eye. We encourage both dermatologists and pediatricians to be aware of the frequency and innocence of these lesions to prevent unnecessary concern, referrals, and surgical procedures.

Recognizing and treating toilet-seat contact dermatitis in children.

Toilet-seat contact dermatitis is a common condition around the world and is reemerging in the United States. It can be easily recognized and treated. However, few practitioners consider this diagnosis, which results in a delay in treatment and often exacerbation of the skin eruption. In the past, exposure to wooden toilet seats and associated varnish, lacquers, and paints led to the development of an allergic contact dermatitis on the buttocks and posterior thighs. In recent years, most public facilities have changed to plastic seats, resulting in a change in the clinical presentation of toilet-seat dermatitis. We present 5 cases of toilet-seat dermatitis in children from the United States and India and review the history, presentation, and clinical course of the disease. Our findings suggest that toilet-seat dermatitis is more common than previously recognized and should be considered in any child with a dermatitis that involves the buttocks and posterior thighs.

Recognizing and Managing Eczematous Id Reactions to Molluscum Contagiosum Virus in Children

Molluscum contagiosum (MC) is a self-limiting cutaneous viral eruption that is very common in children. MC infection can trigger an eczematous reaction around molluscum papules known as a hypersensitivity or an id reaction. In addition, a hypersensitivity reaction can occasionally occur at sites distant from the primary molluscum papules. These eczematous reactions are often asymptomatic or minimally pruritic. We believe that id reactions represent an immunologically mediated host response to MC virus and a harbinger of regression. Therefore, these reactions often do not require treatment other than emollients. Moreover, topical steroids or immunomodulators may suppress this process and potentiate the spread of the primary MC infection. However, in symptomatic patients, treatment should not be withheld and short-course treatments of topical corticosteroids may be used. In this case series, we describe 3 cases of hypersensitivity reactions in otherwise healthy children with MC. We hope that our report will make clinicians more aware of this common eczematous response to MC and will improve the management and counseling of these patients and their parents.

Destruction of the Nasal Septum by Aspergillus Infection after Autologous Bone Marrow Transplantation

To the Editor: We report the case of a 15-year-old boy with necrosis of the nasal septum due to aspergillus infection after autologous bone marrow transplantation for acute myelogenous leukemia. Tr...

Congenital choroid plexus papilloma and giant nevomelanocytic nevus: Report of a case

Case report. At 6 hoursof age a full-term while female infant was transferred to Childrens Hospital of Pittsburgh for evaluation of a large pigmentednevusand generalizedhypotonia.The pregnancy was complicated only by nausea and vomiting during the first trimester. The patient had no family historyof congenital nevi, central nervous system tumors, or consanguinity. Physical examination revealed multiple pigmented nevi (Fig. I), including a IS X 15cm speckled plaque on the back and numerous brown noduleson the extremities and head. Neurologic examination was remarkable for diffusehypotonia,a poorsuck, and a weak Mora reflex. A head computed tomographic (CT) scan with contrast and magnetic resonance imaging revealeda 2 X 4 cm mass in the atrium of the left lateral ventricle and changes of hydrocephalus (Fig. 2). The infant underwent surgical resection of the tumor 48 hours later and an external ventricular drain was placed. Histologic studies revealed a benign CPP. The patient was given carbamazepine (Tegretol) and phenobarbital for postoperative seizure prophylaxis. When the patient was5 months of age, recurrent hydrocephalus developed. A head CT with intraventricular contrast demonstrated obstruction at the outlet of the fourth ventricleas the result of a posterior fossacyst. There was no evidenceof recurrence of the tumor. A right ventricular peritoneal shunt was placed, and the infant has subsequently done well.

Effect of a Single Application of Pulsed Dye Laser Treatment of Port-wine Birthmarks on Intraocular Pressure

BACKGROUND A new pathophysiologic mechanism has been proposed that indicates that periorbital port-wine birthmarks (PWBs) serve as alternate collateral blood passageways when orbital venous drainage is impaired. The occlusion of such collateral venous channels could, therefore, potentially exacerbate impaired ocular venous flow and trigger the development or worsening of glaucoma in patients with Sturge-Weber syndrome. We investigated to what extent a single application of laser therapy, which occludes only the most superficial portions of a facial PWB, might affect intraocular pressure. Pressures before and after laser treatment were measured to determine pressure difference in 15 patients receiving laser treatment. OBSERVATIONS The greatest pressure differences were observed in patients with a PWB closest to the eye (P = .02). Posttreatment pressures were significantly decreased, relative to pretreatment pressures, only in patients with a PWB on the eyelid compared with patients with a facial PWB not near the eyes (2.33 vs 0.75 mm Hg; P = .004). No correlation was found between change in pressure and patient age, PWB size, or number of previous treatments. CONCLUSIONS A single laser application to a PWB does not appear to show a clinically relevant change in intraocular pressure. Further study is needed longitudinally in a broad range of patients.