Albert C. Yan

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.

Interstitial granulomatous lesions as part of the spectrum of presenting cutaneous signs in pediatric sarcoidosis.

Abstract:u2002 Skin findings in childhood sarcoidosis vary greatly, but only a few occurrences have been published in which the histopathology has been characterized well. We describe a child with sarcoidosis in whom the cutaneous findings were atypical, resembling granuloma annulare. Histologic examination of these cutaneous lesions, however, revealed areas of sarcoid‐like epithelioid cell granulomas, a palisading granulomatous process with features of granuloma annulare, as well as palisading neutrophilic and granulomatous dermatitis and interstitial granulomatous dermatitis. This underscores the variability of skin findings in childhood sarcoidosis—even within the same patient—and suggests that sarcoidosis should be considered in the differential diagnosis of children initially diagnosed with granulomatous skin lesions, such as granuloma annulare, palisading neutrophilic, and granulomatous dermatitis or interstitial granulomatous dermatitis, who demonstrate associated signs of systemic disease.

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.

Mycoplasma-associated Stevens-Johnson syndrome in children: retrospective review of patients managed with or without intravenous immunoglobulin, systemic corticosteroids, or a combination of therapies.

Administration of intravenous immunoglobulin (IVIG) to patients with Stevens–Johnson syndrome (SJS) has been controversial. The objective of this study was to evaluate the effectiveness of IVIG, systemic corticosteroids, or both in treating Mycoplasma pneumoniae–associated SJS (mpSJS). Retrospective series of 10 pediatric mpSJS cases were stratified into four treatment groups: IVIG alone, IVIG and systemic corticosteroids together, systemic corticosteroids alone, and supportive care. The efficacy of therapy was evaluated on the basis of several proxies of disease severity, including hospital length of stay (LOSt) and number of febrile days (Febt) after initiation of therapy. Patients treated with IVIG alone had a longer LOSt and more Febt, despite different baseline characteristics, than patients treated with supportive therapy. Of patients who received IVIG, 50% were treated with corticosteroids concurrently and had similar characteristics of disease severity but showed a non–statistically significant trend toward shorter LOSt and fewer Febt than those who received IVIG alone. A patient treated with corticosteroids alone had the shortest LOSt in this series. Therefore treatment with IVIG alone was associated with a more severe disease course than supportive therapy, although causality cannot be inferred given possible confounding by indication. When systemic corticosteroids were used alone or in conjunction with IVIG, hospital LOSt and Febt trended lower than with the use of IVIG alone, although disease severity at baseline was similar between those treated with IVIG and corticosteroids concurrently and those treated with IVIG alone. It was thus concluded that treatment with systemic corticosteroids as monotherapy or in combination with IVIG may be preferable to IVIG alone. Further large‐scale studies are warranted to evaluate this hypothesis.

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.

Focal Preauricular Dermal Dysplasia: Report of Two Cases and a Review of Literature

Abstract:u2002 We report two cases of focal preauricular dermal dysplasia and review the available literature. Focal preauricular dermal dysplasia is a form of aplasia cutis congenita in which atrophic skin lesions occur in a stereotypical bilateral distribution in the preauricular region. Although focal preauricular dermal dysplasia and membranous cutis aplasia of the scalp share clinical similarities, focal preauricular dermal dysplasia represents a form of aplasia cutis congenita that is not typically associated with extracutaneous anomalies.

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.

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.

A New Variant of Connective Tissue Nevus with Elastorrhexis and Predilection for the Upper Chest.

Localized changes in cutaneous elastic tissue often manifest with flesh‐colored, hypopigmented, or yellow papules, plaques, and nodules. We present five children with clinically similar cobblestone plaques composed of multiple hypopigmented, nonfollicular, pinpoint papules located unilaterally over the upper chest. All lesions first appeared at birth or during early infancy. No associated extracutaneous abnormalities have been identified. Histopathology was remarkable for many, thick elastic fibers with elastorrhexis. We believe that these cases represent a distinct and unique variant of connective tissue nevi.