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N B Esterly

Publications and source records attributed to N B Esterly.

At least 19 recordsLinked to original sources
PubMed1992

Crohn's disease presenting as unilateral labial hypertrophy.

We describe a child who had unilateral hypertrophy of the vulva, a rare extraintestinal manifestation of Crohn's disease. A biopsy specimen revealed a noncaseating granuloma. Flexible sigmoidoscopic examination revealed punctate 1 mm lesions with surrounding erythema in the rectum and sigmoid colon. Response to treatment with sulfasalazine was excellent. Gastrointestinal symptoms developed 1 year later when sulfasalazine was discontinued.

Child↗
PubMed1992

Tissue expansion in a patient with extensive nevus comedonicus.

Nevus comedonicus is a rare developmental abnormality of the pilosebaceous apparatus that presents as an aggregation of dilated follicular orifices filled with pigmented keratinous material. Occurrence of extensive or giant lesions is even more rare. Indications for treatment include recurrent infections and cosmetic reasons. Therapeutic approaches include topical keratolytic agents, manual extraction of comedones, dermabrasion, and excision of smaller lesions. Increasing clinical experience with tissue expansion has suggested that it might be used effectively in the definitive treatment of an extensive nevus comedonicus previously considered too large to excise without the use of a skin graft, despite the potential risk of infection within the lesion during the course of expansion. We report the first patient with giant nevus comedonicus to be treated using tissue expansion. This patient demonstrates that prompt treatment of cellulitis and abscesses within the lesion will keep this process isolated from the adjacent expanders and will not compromise a planned excision and reconstruction.

Buttocks↗
PubMed1992

Isotretinoin for acne in Apert syndrome.

Apert syndrome is a congenital disorder consisting primarily of deformities of the skull, hands, and feet. These individuals are also prone to severe acne, particularly involving the upper arms and forearms. Conventional therapy is often ineffective. We report the successful use of isotretinoin for acne in a patient with Apert syndrome.

Acne Vulgaris↗
PubMed1992

Perianal pseudoverrucous papules and nodules in children.

BACKGROUND: Pseudoverrucous papules and nodules were originally described as a reaction to irritation in association with urostomies. These changes have not been described as occurring on the perianal skin or around colostomies. OBSERVATIONS: Five children had remarkably similar-appearing papules and nodules of the perianal and suprapubic skin. The rash consisted of more than a dozen 2- to 8-mm shiny, smooth, red, moist, flat-topped, round lesions. This peculiar and striking reaction appears to be the result of encopresis or urinary incontinence. Biopsy specimens reveal reactive acanthosis or psoriasiform spongiotic dermatitis. The lesions regress when the irritating factor is removed. CONCLUSIONS: Recognition of this entity is important because pseudoverrucous papules and nodules may mimic more serious dermatoses and unnecessary workup may be initiated. Although this reaction involving perianal skin has not been reported previously, we believe it is not uncommon.

Adolescent↗
PubMed1991

Impetigo.

Explore the source record for details and available documents.

Administration, Topical↗
PubMed1991

Revised clinical and laboratory criteria for subtypes of inherited epidermolysis bullosa. A consensus report by the Subcommittee on Diagnosis and Classification of the National Epidermolysis Bullosa Registry.

Inherited epidermolysis bullosa encompasses a number of diseases, with the common finding of blister formation after minor mechanical trauma to the skin. In some forms significant, if not eventually fatal, extracutaneous disease activity may occur. In recent years application of newer technologies has contributed substantially to an overall understanding of this collection of inherited diseases. Concurrently, many new phenotypes have been recognized, in part the result of ongoing prospective patient registries in the United States and abroad. Unfortunately, this has resulted in a massive literature that may appear to be confounded by seemingly excessive or arbitrary subdivision of epidermolysis bullosa variants. With these concerns in mind a subcommittee was established by the National Epidermolysis Bullosa Registry to summarize the current literature and to make recommendations as to the best clinical and laboratory criteria for the practical diagnosis and subclassification of patients with inherited epidermolysis bullosa.

Epidermolysis Bullosa↗
PubMed1991

McCune-Albright syndrome with multiple bilateral café au lait spots.

A 7-week-old infant developed hyperpigmented lesions at 5 days of age that gradually progressed to sharply demarcated, medium-brown macules of unusual configuration involving the neck, trunk, buttocks, upper arms, and right upper thigh. A biopsy specimen of a representative lesion showed changes of epidermal melanosis consistent with café au lait spot. At age 15 months the patient developed a limp. Roentgenographic evaluation showed widespread, bilateral changes of fibrous dysplasia, most severe in the right pelvis, femur, and tibia. At the present time he has no evidence of precocious puberty or other endocrinopathies. Despite the absence of endocrine abnormalities, these findings are consistent with a diagnosis of McCune-Albright syndrome with extensive bony and cutaneous lesions.

Fibrous Dysplasia, Polyostotic↗
PubMed1991

Screening for psychosocial dysfunction in pediatric dermatology practice.

The Pediatric Symptom Checklist, a brief psychosocial screening questionnaire, was used in a multi-center study of pediatric dermatology clinics (n = 377). Overall rates of positive screening indicated that approximately 13% of patients screened positive, a rate similar to findings in primary care pediatric settings. Examining the sample in greater detail demonstrated that children whose dermatologic disorder is perceived to have a greater impact on their appearance are at higher risk for psychosocial dysfunction.

Child↗
PubMed1991

Normal peroxisomal function and absent skeletal manifestations in Conradi-Hünermann syndrome.

We describe a child with the classic cutaneous and ocular manifestations of Conradi-Hünermann syndrome in whom repeated roentgenographic studies during the first 2 years of life revealed no evidence of epiphyseal stippling. The findings in this case and others from the literature suggest that skeletal changes may be absent or show limited expression in patients with this condition and that chondrodysplasia punctata should not be considered an invariable feature of Conradi-Hünermann syndrome. Studies of peroxisomal function in our patient failed to confirm two previous reports of a significant reduction in activity of the peroxisomal enzyme dihydroxyacetone phosphate acyltransferase in this disorder.

Acyltransferases↗
PubMed1990

Cutaneous T-cell lymphoma (parapsoriasis en plaque). An association with pityriasis lichenoides et varioliformis acuta in young children.

Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC) are related benign disorders without recognized association with cutaneous T-cell lymphoma (CTCL). We report the cases of two children with documented PLEVA evolving into CTCL over several years. One child had the clinical lesions of PLC but the dermatopathologic findings of PLEVA at age 2 years. At age 12 years, he had skin changes of poikiloderma atrophicans vasculare and dermatopathologic findings consistent with parapsoriasis en plaque. The second child presented at age 7 years with scaling dermatitis and dermatopathologic findings of PLEVA. At age 12 years, the histologic diagnosis was parapsoriasis. Monoclonal antibody studies performed on biopsy specimens from both patients revealed 70% to 100% cells staining with CD5, 80% to 90% staining with CD4, 30% to 50% staining with CD8, and an increase in CD1-staining cells in the papillary dermis, indicating a predominantly helper T-cell infiltrate. We believe that PLC and PLEVA may be part of the spectrum of CTCL. Furthermore, CTCL may be more common in young children than once thought.

Child↗