Bilateral symmetric facial epidermal nevus.
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Adolescent↗
Biomedical subjects
Paul J Honig
Publications and source records attributed to Paul J Honig.
Methicillin-resistant Staphylococcus aureus skin abscesses in a pediatric patient with atopic dermatitis: a case report.
Recent trends indicate an increasing incidence of community-acquired methicillin-resistant Staphylococcus aureus (CAMRSA) in the general population, which is especially worrisome for clinicians caring for patients with atopic dermatitis (AD). Patients with AD are heavily colonized with S aureus and have impaired skin integrity and abnormal immune responses, which predisposes them to more invasive cutaneous infections (eg, cellulitis, furuncles, abscesses). In this report, we describe a child with severe AD who presented with CAMRSA skin abscesses. The presence of an atypical skin infection in patients with AD, particularly those unresponsive to conventional penicillinase-resistant penicillins and cephalosporins, should alert the clinician to the possibility of MRSA as the underlying etiology, and intervention should be directed accordingly.
Child↗
Two novel TP63 mutations associated with the ankyloblepharon, ectodermal defects, and cleft lip and palate syndrome: a skin fragility phenotype.
BACKGROUND: Ankyloblepharon, ectodermal defects, and cleft lip and palate (AEC) syndrome is a rare autosomal dominant disorder caused by mutations in the sterile alpha motif region of TP63, a homologue of the tumor suppressor TP53. Recent structure-function studies have identified complexities in the genotype-phenotype correlation of the p63 syndromes. OBSERVATIONS: We report 2 sporadic cases of AEC syndrome in infants. Both patients demonstrated skin erosions with prominent scalp involvement. Histologic studies demonstrated mild basal layer vacuolization and rare dyskeratotic keratinocytes, with evidence of both acantholysis and cytolysis at the blister edge. Immunohistochemistry using anti-p63 monoclonal antibody demonstrated basal epidermal nuclear staining in both healthy control and patient tissue samples. Ultrastructural studies showed focal disruption of anchoring fibrils near the blister edge of one patient and normal desmosomes, hemidesmosomes, and basement membrane zone in the nonblistered skin of the other patient. The DNA analysis of each patient revealed 2 novel missense mutations in the TP63 gene that resulted in L514S and R555P amino acid substitutions within the sterile alpha motif region of the p63 protein. CONCLUSIONS: We report 2 novel TP63 mutations resulting in AEC syndrome. The R555P mutation is the most carboxy-terminal of all the reported AEC missense mutations of p63. The presence of skin fragility, manifested as erosive skin lesions in body areas in addition to the scalp, is postulated to be an important diagnostic feature of AEC syndrome.
Abnormalities, Multiple↗
Extensive aplasia cutis congenita and the risk of sagittal sinus thrombosis.
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Abdomen↗
Rice nightmare: Kwashiorkor in 2 Philadelphia-area infants fed Rice Dream beverage.
A 14-month-old girl and a 7-month-old boy each presented with a diffuse dermatitis, whole body edema, and hypoalbuminemia. The diets of both infants consisted almost entirely of Rice Dream, a rice-based, protein-poor beverage. Both infants were diagnosed with kwashiorkor, which resolved with protein supplementation. Clues from the physical examination, a diet history, appropriate laboratory examinations, and an index of suspicion are crucial in promptly diagnosing and treating infants with kwashiorkor. Manufacturers of rice beverages should appropriately warn parents about the dangers of using their products as infant nourishment.
Dermatitis↗
Neonatal cutaneous fungal infections.
PURPOSE OF REVIEW: Cutaneous fungal infections are not uncommon in newborns and are seen in premature or otherwise immunocompromised neonates as well as in healthy full-term neonates. Healthy newborns can develop clinical manifestations as a result of infection with Candida species or as a result of skin colonization with Malassezia species; cutaneous infection with other fungal pathogens is rare. Immunocompromised and premature neonates, however, are susceptible to infection with opportunistic pathogens and are also at higher risk for invasive infection with common pathogens such as Candida. This review discusses the fungal species associated with cutaneous fungal infection in neonates, emphasizes the relevant clinical features, and also reviews the use of newer antifungal agents, including lipid-associated amphotericin B, voriconazole, and caspofungin. RECENT FINDINGS: Neonatal cutaneous infections with opportunistic fungal pathogens, including Aspergillus and the Zygomycetes, have been reported with increasing frequency as advances in neonatal care have improved the survival rate in very low birthweight neonates. Although these infections are frequently fatal, survival in some neonates has been reported with the use of aggressive surgical debridement and systemic antifungal therapy. Newer antifungal agents, including voriconazole and caspofungin, show promise in the treatment of potentially fatal fungal infections in neonates. SUMMARY: Cutaneous fungal infections in neonates range from generally benign conditions such as congenital candidiasis and neonatal cephalic pustulosis to potentially fatal infections with opportunistic pathogens in very low birthweight or immunocompromised neonates. The prompt recognition and appropriate treatment of cutaneous fungal disease in neonates is critical to the prevention of adverse outcomes.
Amphotericin B↗
Necrolytic acral erythema: response to combination therapy with interferon and ribavirin.
Necrolytic acral erythema is a papulosquamous and sometimes vesiculobullous eruption bearing clinical and histologic similarity to other necrolytic erythemas such as necrolytic migratory erythema, pseudoglucagonoma, and nutritional deficiency syndromes. Necrolytic acral erythema is distinguished by its association with hepatitis C infection and its predominantly acral distribution. We describe a pediatric patient with necrolytic acral erythema whose eruption resolved with hyperalimentation and combination interferon and ribavirin therapy, despite the persistence of detectable viral load and continued hepatic and renal insufficiency.
Acrodermatitis↗
Nonpseudomonal ecthyma gangrenosum.
Ecthyma gangrenosum is a cutaneous infection associated most commonly with pseudomonal sepsis in the patient who is immunocompromised. We describe an 8-month-old girl with acute myelocytic leukemia who developed perineal ecthyma gangrenosum caused by Citrobacter freundii, a gram-negative pathogen that has been rarely associated with cutaneous disease. We also review the literature to categorize the range of pseudomonal and nonpseudomonal pathogens associated with ecthyma gangrenosum.
Biopsy↗
What's news in pediatric dermatology: an update.
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Child↗
Streptococcal intertrigo: an underrecognized condition in children.
Group A beta-hemolytic streptococci have been implicated in a variety of common childhood cutaneous infections. Infants and young children may be particularly susceptible to a form of streptococcal intertrigo that has heretofore been underrecognized in this population. Manifesting as intense, fiery-red erythema and maceration in the intertriginous folds of the neck, axillae, or inguinal spaces, the condition is characterized by a distinctive foul odor and an absence of satellite lesions. Specific clinical features help differentiate this condition from its clinical mimics. Topical and oral antibiotic therapy with or without concomitant low-potency topical steroid application is generally curative.
Diagnosis, Differential↗
The clonal nature of pityriasis lichenoides.
BACKGROUND: Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC) are benign lymphocytic infiltrates of the skin that classically present as either a recurrent papulonecrotic eruption (PLEVA) or a persistent, scaling, papular eruption (PLC). Observations of both types of lesions present on individual patients have led to speculation that both entities are related. Previous studies evaluating the DNA of biopsy specimens from patients with PLEVA and PLC revealed clonal T-cell receptor beta gene rearrangements. OBJECTIVE: To analyze and compare the T-cell populations between lesions of PLEVA and PLC. DESIGN: Retrospective and prospective analysis of patient tissue samples, classified by histologic analysis. Extracted DNA from 13 skin biopsy specimens with the diagnosis of PLC and 14 skin biopsy specimens with the diagnosis of PLEVA was analyzed by polymerase chain reaction/denaturing gradient gel electrophoresis (PCR/DGGE). SETTING: Molecular diagnostic laboratory at an academic medical center. PATIENTS: Twenty-seven tissue samples were obtained from patients with a histologic diagnosis of PLEVA or PLC. These samples were analyzed by PCR/DGGE. MAIN OUTCOME MEASURE: The presence or absence of T-cell receptor gene rearrangements on PCR/DGGE analysis corresponding to a clonal population of T cells. RESULTS: Of 14 PLEVA specimens, 8 (57%) demonstrated monoclonal T-cell receptor gene rearrangements; 1 (8%) of 13 PLC specimens showed a gene rearrangement (P =.008, Fisher exact test). CONCLUSIONS: Our results demonstrate the polyclonal nature of the lymphocytic infiltrate found in almost all of the PLC specimens, which contrasts with the monoclonal nature found in most of the PLEVA specimens. These differences may represent different stages of the clinical evolution of a single entity that results from varying host immune responses to pathogenic factors. Specifically, we propose that PLEVA is a benign clonal T-cell disorder in which the clone arises from a subset of T cells in lesions of PLC. The host immune response to this clone determines the clinical and histologic findings in PLEVA.
Adolescent↗
Diaper rash in a young infant.
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Diaper Rash↗
Congenital infantile fibrosarcoma: a masquerader of ulcerated hemangioma.
Congenital infantile fibrosarcoma, a rare malignant tumor of childhood, may present as a highly vascularized mass that is clinically difficult to distinguish from a hemangioma. When ulcerated, significant hemorrhage, anemia, and thrombocytopenia may occur in children with these lesions. This report describes three infants with ulcerated congenital infantile fibrosarcomas of the hand. As appropriate medical and surgical management hinges on timely and appropriate diagnosis, we review the clinical manifestations of these lesions.
Diagnosis, Differential↗