James R. Treat

High Prevalence of Aquagenic Wrinkling of the Palms in Patients with Cystic Fibrosis and Association with Measurable Increases in Transepidermal Water Loss

Abstract:u2002 Aquagenic wrinkling of the palms (AWP) is a cutaneous phenomenon marked by the transient formation of edematous, translucent papules and plaques on the palms and fingertips within minutes of water exposure. AWP is anecdotally reported in patients with cystic fibrosis (CF) and several studies have recently confirmed this association. The primary aim of this study was to determine the prevalence of aquagenic wrinkling of the palms in subjects with cystic fibrosis (CF) compared to controls, and secondarily to evaluate for genotype‐phenotype correlations among CF subjects found to have AWP. Fifty‐one children with CF and 25 control children who were being treated for asthma underwent a 5‐minute hand immersion in lukewarm water. The test for AWP was positive if subjects demonstrated >30% wrinkling over the palm. Secondary analyses explored associations with genotype, pancreatic and pulmonary function, body mass index (BMI), and sweat chloride levels. Palmar transepidermal water loss (TEWL) was also measured for all subjects with and without AWP. Forty‐three of the subjects (84%) with CF demonstrated aquagenic wrinkling, in contrast to none (0%) of the controls. These results remained statistically significant when stratified for by age and race. TEWL was significantly higher in CF subjects with AWP compared to CF subjects without AWP and controls. No genotype‐phenotype correlations were detected in patients with AWP, nor were there associations of AWP with other phenotypic features of CF, although these analyses were likely underpowered. Aquagenic wrinkling of the palms is prevalent in children with CF and is associated with increased TEWL.

Leclercia adecarboxylata cellulitis in a child with acute lymphoblastic leukemia.

Abstract:u2002 Leclercia adecarboxylata is a rare, gram‐negative rod that has been infrequently reported in the literature. The organism has been documented to cause solitary infections in immunocompromised hosts and polymicrobial wound infections in the immunocompetent. We present a case of an 8‐year‐old boy with significant past medical history of acute lymphobastic leukemia who developed cellulitis due to local infection by L. adecarboxylata. This case is presented to raise awareness of this rare organism’s ability to cause common cutaneous disease, especially in the immunocompromised.

Febrile ulceronecrotic Mucha-Habermann disease in a 34-month-old boy: a case report and review of the literature.

Abstract:u2002 We report a case of febrile ulceronecrotic Mucha‐Habermann disease (FUMHD) in a 34‐month‐old boy. Our patient had a history of biopsy‐proven pityriasis lichenoides et varioliformis acuta (PLEVA) since age 2. At 34u2003months, his skin lesions rapidly progressed to ulceration and necrosis in the setting of high fever. Skin biopsy revealed an intense lichenoid infiltrate with parakeratosis at the edges of areas of epidermal necrosis consistent with FUMHD, which is the severe variant of PLEVA. Despite initial treatment with prednisolone, his disease progressed to involve more than 50% of his body surface area. In addition to corticosteroids, he was treated with intravenous immunoglobulin, dapsone, and acyclovir, without complete resolution of disease. Methotrexate successfully cleared his skin disease and systemic symptoms. This patient highlights the exceptional response of FUMHD to methotrexate. Based on our review of the literature, this also represents the youngest reported case of FUMHD.

Antibiotic Sensitivity and Resistance Patterns in Pediatric Staphylococcal Scalded Skin Syndrome

Historical resistance patterns often guide empiric antibiotic choices in staphylococcal scalded skin syndrome (SSSS), but little is known about the difference in susceptibility between SSSS and other childhood staphylococcal infections. A retrospective chart review of culture‐confirmed cases of SSSS seen in the inpatient dermatology consultation service at the Childrens Hospital of Philadelphia between 2005 and 2011 was performed. Most cases of SSSS at our institution are due to oxacillin‐susceptible Staphylococcus aureus, and approximately half of the cases are due to clindamycin‐resistant strains. Clindamycin and a penicillinase‐resistant penicillin are suggested as empiric treatment for SSSS until culture susceptibility data are available to guide therapy.

PHACE Syndrome: A Retrospective Review of 23 Patients

We present 23 patients with PHACE syndrome showing similarities in our population with data that already exist while highlighting neurodevelopmental occurrences arising in a subset of these patients.

Healing of Granulomatous Skin Changes in Ataxia-Telangiectasia After Treatment with Intravenous Immunoglobulin and Topical Mometasone 0.1% Ointment

Ataxia‐telangiectasia (AT) is a rare autosomal recessive disorder characterized by faulty DNA damage repair. The disease affects multiple systems and is noted to be particularly difficult to diagnose in children because of the wide spectrum of clinical presentations. We present an unusual case of a child in whom the primary cutaneous manifestation of AT was noninfectious cutaneous caseating granulomas. A 3‐year‐old girl presented to the emergency department with ataxia, poor growth, and multiple ulcerated plaques on both upper extremities that had been present for 2 years. She had two prolonged hospitalizations and underwent extensive examination to identify an etiology for the skin lesions. She was diagnosed with AT after immunology examinaton and genetic testing. Outpatient intravenous immunoglobulin (IVIG) therapy was initiated and she was prescribed twice‐daily mometasone 0.01% ointment under occlusion. After 6 weeks on this regimen her lesions had completely healed. Twenty‐two cases of AT have been reported in which patients presented with cutaneous granulomas. This report demonstrates the first reported case in which the granulomatous skin lesions of AT healed after aggressive application of topical steroids with concurrent IVIG therapy, without oral steroids. A brief review of cutaneous granulomas in the setting of immunodeficiency is also presented.

Topical Cidofovir for Recalcitrant Verrucae in Individuals with Severe Combined Immunodeficiency After Hematopoietic Stem Cell Transplantation

Verrucae vulgaris in patients with severe combined immunodeficiency (SCID) after hematopoietic stem cell transplantation (HCST) can be challenging to manage. We describe two brothers with X‐linked SCID who had severe, persistent verrucae that did not respond to traditional topical therapies, including liquid nitrogen, imiquimod, salicylic acid, sinecatechins, 40% urea, and 5‐fluorourcil. Both brothers had full response to topical 3% cidofovir, which should be considered in recalcitrant warts in individuals with SCID after HSCT.

Fibrous Hamartoma of Infancy: A Firm Plaque Presenting with Hypertrichosis and Hyperhidrosis

Fibrous hamartoma of infancy (FHI) is a rare, benign, soft tissue tumor. The lesions clinical resemblance to more aggressive soft tissue tumors of childhood makes histologic diagnosis paramount. We report a unique presentation of a FHI associated with localized hypertrichosis and hyperhidrosis.

Herpes Zoster Duplex Bilateralis in an Immunocompetent Adolescent Boy: A Case Report and Literature Review

Simultaneous involvement of herpes zoster in multiple dermatomes is uncommon, and even more so in immunocompetent individuals. We report a case wherein a healthy adolescent boy presented with herpes zoster in two distinct dermatomes, raising concern for immunodeficiency, but he was found to be immunocompetent on further testing. A 14‐year‐old boy with no significant past medical history developed painless vesicular eruptions in two distinct distributions. Varicella zoster virus polymerase chain reaction was positive from unroofed vesicles in both regions. Initial laboratory studies disclosed abnormalities of unknown significance in natural killer (NK) cell percentage and function. The patient was treated with appropriate antiviral therapy. Repeat studies while healthy were not suggestive of an underlying NK cell defect. There are few case reports describing herpes zoster in two or more dermatomes in children. Previously described presentations most commonly occurred in the context of primary immunodeficiency, acquired immunodeficiency, or immunosuppressive medications. Because of the rarity of this presentation in immunocompetent patients, the authors recommend a thorough immune evaluation of all children presenting with isolated multidermatomal zoster.

Pediatric Pemphigus Vulgaris: Durable Treatment Responses Achieved with Prednisone and Mycophenolate Mofetil (MMF)

Abstract:u2002 Pemphigus vulgaris (PV) is a chronic autoimmune blistering disease of the skin and mucous membranes. Most cases occur in adults; cases in children are rare. This report describes the clinical presentations and treatment responses of three children with PV, as confirmed according to histology and indirect immunofluorescence studies. In all three cases, oral prednisone used in conjunction with mycophenolate mofetil (MMF) resulted in complete clinical remission, during which all pharmacotherapy was successfully discontinued. Resolution of the skin and mucosal blistering tended to occur quickly with prednisone, and after initiation of treatment with MMF, discontinuation of all pharmacotherapy was achieved within a range of 10 to 30u2003months in the three patients. One patient experienced a recurrence of genital lesions 19u2003months after discontinuation of therapy, but the condition remitted within 2u2003weeks with topical corticosteroid therapy. At the time of this report, the duration of complete remission ranged from 6 to 19u2003months. In summary, combination therapy with prednisone and MMF for pediatric PV appears to be a safe and effective approach that is associated with durable remission.