Procedural pediatric dermatology.

Due to many factors, including parental anxiety, a child's inability to understand the necessity of a procedure and a child's unwillingness to cooperate, it can be much more challenging to perform dermatologic procedures in children. This article reviews pre-procedural preparation of patients and parents, techniques for minimizing injection-related pain and optimal timing of surgical intervention. The risks and benefits of general anesthesia in the setting of pediatric dermatologic procedures are discussed. Additionally, the surgical approach to a few specific types of birthmarks is addressed.

Denuded congenital lesions: recessive dystrophic epidermolysis bullosa.

Recessive dystrophic epidermolysis bullosa (Hallopeau-Siemens type) (RDEB-HS) is a rare severe mechanobullous disorder resulting from a defect in collagen VII. Patients with RDEB-HS present with generalized blistering and denudation of the skin at birth and have mucosal involvement. The repeated blistering leads to scarring, which may be deforming and result in serious complications. Transmission electron microscopy is currently the gold standard for diagnosis of RDEB-HS.

Burow's advancement flap closure of adjacent defects.

When two surgical defects are closely approximated, primary closure may be difficult because of tension on the tissue between the defects. We outline a technique using a Burow's-triangle advancement flap in which the advanced Burow's triangle contains the second defect. The defects are easily closed with a single flap that utilized the second defect. This flap is useful when there are two closely approximated surgical defects of which primary closure is limited by tension on the tissue between the defects.

Congenital erosive and vesicular dermatosis healing with reticulated supple scarring.

Congenital erosive and vesicular dermatosis healing with reticulated supple scarring is a rare disorder of unknown etiology first reported in three patients in 1985. Nine patients have subsequently been reported, helping to further characterize this unique dermatosis. We describe another patient and further describe the histologic and electron microscopic findings of this entity. This case is unique in that histologic distinction is made between vesicular and scarred lesions.

Response of ulcerated perineal hemangiomas of infancy to becaplermin gel, a recombinant human platelet-derived growth factor.

BACKGROUND: Hemangiomas of infancy are the most common tumors of childhood, and ulceration is the most common complication. Many treatments have been used for hemangioma ulceration, although none are uniformly effective. A recent report described the successful use of 0.01% becaplermin gel, a recombinant human platelet-derived growth factor, for an ulcerated hemangioma refractory to standard care. We sought to further assess the responsiveness of hemangioma ulceration to 0.01% becaplermin gel and to compare its cost to that of conventional modalities. OBSERVATIONS: We report a case series of 8 infants treated with becaplermin gel for ulcerated perineal hemangiomas of infancy. All infants were seen between January and June 2003 in the pediatric dermatology clinic at Texas Children's Hospital. Six female and 2 male infants were included. All of the hemangiomas were large (> or =6 cm(2)), and of superficial or mixed superficial and deep morphology. Rapid ulcer healing occurred in all patients within 3 to 21 days (average, 10.25 days). CONCLUSIONS: In this small series, 0.01% becaplermin gel was a safe and effective treatment for perineal hemangioma ulceration. The rapid healing achieved with 0.01% becaplermin gel allows a reduction in the risk of secondary infection, pain, and need for hospitalization, as well as in the costs that often accumulate from multiple follow-up visits and long-term therapy.

Linear IgA dermatosis with IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180): evidence for a distinct subtype.

BACKGROUND: Autoantibodies in linear immunoglobulin A (IgA) disease (LAD) are reported to be of IgA class and directed against a 97-120 kDa epidermal antigen. METHODS: We report a 39-year-old woman with clinical features of LAD and with circulating IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180). RESULTS: Histopathology of lesional skin revealed a subepidermal blister with mixed inflammatory cell infiltrate. Direct immunofluorescence of perilesional skin showed linear deposits of IgA along the dermal-epidermal junction. The antigen specificity of the patient's circulating antibodies was determined by Western blotting and enzyme-linked immunoabsorbent assay (ELISA) using various antigen sources, including cultured human keratinocytes, dermal protein lysates, and purified laminin-5, as well as proteins corresponding to BP180, the 230 kDa bullous pemphigoid antigen (BP230), laminin-5 subunits, and collagen IV alpha1-alpha6 chains. IgA and IgG antibodies in the patient's serum were directed against BP180, and no IgA or IgG reactivity was found against the other skin antigens. CONCLUSIONS: These data provide evidence for the presence of a subtype of LAD with dual IgA and IgG autoimmune response to BP180.