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Mary Wu Chang

Publications and source records attributed to Mary Wu Chang.

11 recordsLinked to original sources
PubMed2004

Genetic association of cutaneous neonatal lupus with HLA class II and tumor necrosis factor alpha: implications for pathogenesis.

OBJECTIVE: Cutaneous neonatal lupus resembles subacute cutaneous lupus erythematosus (SCLE), and photosensitivity is a common symptom. Tumor necrosis factor alpha (TNFalpha) release by ultraviolet light-exposed keratinocytes may be exaggerated in SCLE patients who have the haplotype TNFalpha -308A;DRB1*03. Accordingly, this study was undertaken to seek genetic and histologic evidence for a role of TNFalpha in the pathogenesis of cutaneous neonatal lupus. METHODS: DNA was isolated from 83 children (22 with rash, 35 with congenital heart block [CHB], 26 unaffected siblings) and 58 mothers from the Research Registry for Neonatal Lupus. RESULTS: The -308A allele (associated with higher TNFalpha production), HLA-DRQB1*02, and HLA-DRB1*03 were each present in the majority of children with rash (64%, 68%, and 64%, respectively). The frequency of all 3 6p alleles occurring together in 1 individual was greater in children with rash than in children who had either CHB or no manifestation of neonatal lupus (59% versus 30%; P = 0.02). This association with neonatal lupus rash was equivalent to published findings in a cohort of patients with SCLE, but significantly greater than the association in patients with discoid lupus erythematosus. Prominent TNFalpha staining in the epidermis was observed in lesional skin from 3 children with rash, but not in skin from a healthy neonate. CONCLUSION: Taken together, the finding of a genetic predisposition to generate increased levels of TNFalpha following tissue injury and the histologic demonstration of TNFalpha in the target organ support the notion that this inflammatory cytokine plays a role in the pathogenesis of cutaneous neonatal lupus. Furthermore, the results of these studies provide evidence of a biologic link between neonatal lupus and the rash of SCLE.

Epidermis↗
PubMed2004

Infantile granular parakeratosis: recognition of two clinical patterns.

Granular parakeratosis is an acquired, idiopathic disorder of keratinization typified by retention hyperkeratosis. It usually occurs in women at intertriginous sites. There have been only 2 reports of infants with granular parakeratosis to our knowledge. We describe 3 additional infants with granular parakeratosis. We demonstrate that infantile granular parakeratosis exhibits 2 clinical patterns: bilateral linear plaques in the inguinal folds; and erythematous geometric plaques underlying pressure points from the diaper. A thick, flakelike scale is present in both forms and is characteristic. Diaper wearing appears to play an important role in the genesis of infantile granular parakeratosis but the mechanisms are unclear. Therapeutic responsiveness to topical agents is ambiguous, however, spontaneous clearance after months to 1 year appears to be the rule.

Biopsy↗
PubMed2004

Infantile systemic hyalinosis.

Infantile systemic hyaloinosis is a rare, progressive, and fatal disease that is inherited in an autosomal recessive fashion. We describe 2 patients in whom thickened skin; small nodules of the perianal region, face, and neck; joint contractures; growth failure; diarrhea; and frequent infections developed within the first few weeks of life. Both patients died before 2 years of age.

Buttocks↗
PubMed2003

Updated classification of hemangiomas and other vascular anomalies.

Vascular anomalies comprise a widely heterogenous group of tumors and malformations. Great confusion has arisen because of the term hemangioma has been and is continued to be used to represent a multitude of vascular entities. This review presents the updated classification of vascular anomalies with the goal of clarifying the term hemangioma. In addition, newer clinical concepts in hemangiomas and other vascular tumors is presented. Hemangioma subtypes and hemangioma variants are also discussed, and a brief review of pyogenic granuloma and Kaposiform hemangioendothelioma is provided. Finally, the immunohistochemical marker GLUT1 is reviewed, a marker that heralds a new era in vascular anomalies research.

Cardiovascular Abnormalities↗
PubMed2002

Cutaneous anthrax associated with microangiopathic hemolytic anemia and coagulopathy in a 7-month-old infant.

A 7-month-old infant with cutaneous anthrax developed severe systemic illness despite early treatment with antibiotics. The infant displayed severe microangiopathic hemolytic anemia with renal involvement, coagulopathy, and hyponatremia. These findings are unusual with cutaneous anthrax, but have been described in illness resulting from spider toxin and may delay correct diagnosis. The systemic manifestations of the disease persisted for nearly a month despite corticosteroid therapy, but resolved.

Anemia, Hemolytic↗
PubMed2002

Berloque dermatitis mimicking child abuse.

Berloque dermatitis is a type of photocontact dermatitis. It occurs after perfumed products containing bergamot (or a psoralen) are applied to the skin followed by exposure to sunlight. Striking linear patterns of hyperpigmentation are characteristic, corresponding to local application of the scented product. In the acute phase, erythema and even blistering can be seen. We report a case of berloque dermatitis in a 9-year-old girl that was initially reported as child abuse. To our knowledge, this is the first report of berloque dermatitis mimicking child abuse. Questioning to elicit a history of perfume application coupled with sunlight exposure should help to prevent this misdiagnosis in children.

Child↗
PubMed2002

Extensive Riga-Fede disease of the lip and tongue.

Riga-Fede disease presents in early infancy and is characterized by firm, verrucous plaques arising on the oral mucosal surfaces. These histologically benign lesions occur as a result of repetitive trauma of the oral mucosal surfaces by the teeth. Early recognition of this entity is important, because it may be the presenting sign of an underlying neurologic disorder. We report the case of a 10-month-old boy with extensive Riga-Fede disease involving the lip and tongue that prompted a diagnosis of congenital autonomic dysfunction with universal pain loss.

Autonomic Nervous System Diseases↗
PubMed2002

Fever and rash in a 3-year-old girl: Rocky Mountain spotted fever.

Initial symptoms of Rocky Mountain spotted fever (RMSF), a tick-borne illness caused by Rickettsia rickettsii, are nonspecific and include headache, gastrointestinal disturbances, malaise, and myalgias, followed by fever and rash. The classic triad of fever, rash, and history of tick exposure is uncommon at presentation. Clinical manifestations of RMSF range from virtually asymptomatic to severe. Because of the potentially fatal outcome of RMSF, presumptive clinical diagnosis and empiric antimicrobial therapy can be critical. We present the case of a 3-year-old girl from New York State who presented with fever and rash.

Child, Preschool↗
PubMedDate not supplied

Congenital primary cutaneous rhabdomyosarcoma in a neonate.

We report a case of congenital primary cutaneous rhabdomyosarcoma, solid alveolar type, presenting as a solitary skin lesion on the right upper lip of a 2-week-old infant boy. Rhabdomyosarcoma originates from the embryonic mesenchyme precursor of striated muscle. Histologically it belongs to the group of "small round cell tumors." Its myogenic origin is ascertained by immunohistochemical studies positive for myogenin, muscle-specific actin, desmin, and myoglobin. Malignancy in the neonatal period is uncommon and the clinical management presents considerable challenges. Congenital alveolar rhabdomyosarcoma is a highly malignant tumor with no record of long-term survivors. Treatment options include chemotherapy, excision, and radiotherapy. This infant's tumor was responsive to chemotherapy and surgery and he was free of disease at the 6-month follow-up.

Humans↗
PubMedDate not supplied

Confluent and reticulated papillomatosis associated with tinea versicolor in three siblings.

We describe three teenage siblings with confluent and reticulated papillomatosis, all presenting during a 6-month period. Two of the three patients had confirmed tinea versicolor, with positive potassium hydroxide scrapings, in association with this entity. This is the largest series of siblings with confluent and reticulated papillomatosis, and the only report describing family members having both confluent and reticulated papillomatosis and tinea versicolor. This report lends further evidence to the hypothesis that confluent and reticulated papillomatosis may be etiologically linked to tinea versicolor, and also suggests a genetic predisposition for it.

Adolescent↗