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Biomedical subjects

N S Prose

Publications and source records attributed to N S Prose.

At least 19 recordsLinked to original sources

Intrauterine epidermal necrosis.

BACKGROUND: Epidermal necrosis in a neonate is an uncommon event with a variety of potential cases. RESULT: We report a case of intrauterine epidermal necrosis in a preterm infant, with death occurring soon after birth. The histopathology of the denuded skin revealed full-thickness epidermal necrosis and calcification within both the epidermis and follicular structures. CONCLUSION: We believe this represents the fourth reported case of lethal intrauterine epidermal necrosis and follicular calcification.

Epidermis↗

Skin and nail changes in children with sickle cell anemia receiving hydroxyurea therapy.

Skin and nail changes from long-term hydroxyurea therapy are reported in adults. Skin and nail changes, including nail hyperpigmentation, longitudinal bands, and hyperpigmentation of the palms and other skin surfaces, developed in 7 children with sickle cell anemia after 6 to 16 weeks of hydroxyurea therapy. Cutaneous and nail changes may occur in children receiving hydroxyurea.

Adolescent↗

Hereditary benign intraepithelial dyskeratosis: Report of two cases with prominent oral lesions.

Hereditary benign intraepithelial dyskeratosis is a rare autosomal dominant disorder of the oral and ocular mucosa initially described in the Haliwa-Saponi Native American tribe of North Carolina. We describe 2 sisters with the characteristic oral and ocular findings. This entity should be distinguished from several other diseases that cause white lesions in the mouth including white sponge nevus.

Adolescent↗

Autosomal dominantly inherited generalized basaloid follicular hamartoma syndrome: report of a new disease in a North Carolina family.

BACKGROUND: An 8-year-old girl presented with hundreds of milia, measuring 1 to 2 mm; comedone-like lesions; skin-colored and hyperpigmented papules on the face, scalp, ears, neck, upper trunk, and lower arms along with diffuse scalp hypotrichosis; and pinpoint palm/sole pits. Onset was in early childhood and the disease was historically present in 6 generations. OBJECTIVE: Our objectives were to delineate the clinical and histopathologic features and mode of inheritance as a base for gene studies. METHODS: Eighteen family subjects were studied. Twenty-six skin biopsy specimens were examined. A detailed pedigree was constructed. A complete literature search was done concerning diseases with generalized basaloid follicular hamartomas. RESULTS: The lesions were basaloid follicular hamartomas and other folliculocentric abnormalities. Inheritance was autosomal dominant. Extensive literature search confirmed the finding of a unique genodermatosis. CONCLUSION: A new genodermatosis termed dominantly inherited generalized basaloid follicular hamartoma syndrome was defined by delineating its clinical and histopathologic features and mode of inheritance and by extensive literature review.

Adolescent↗

Subcutaneous granuloma annulare: MR imaging features in six children and literature review.

PURPOSE: To describe the magnetic resonance (MR) imaging findings of subcutaneous granuloma annulare (SGA), a rare mass of early childhood. MATERIALS AND METHODS: MR imaging studies and clinical records in six children aged 2 1/2-4 years in whom SGA was diagnosed between 1993 and 1997 were retrospectively reviewed. All MR imaging examinations included T1-weighted and fast spin-echo T2-weighted sequences. Three children received intravenous contrast material. The diagnosis was established by using excisional biopsy results, with confirmation by means of characteristic features in mucin-stained specimens. RESULTS: All children presented with a nontender, nonmobile mass. The lesion in all six children was confined to the pretibial (n = 5) or prepatellar (n = 1) soft tissues. MR imaging characteristics were uniform. Masses were subcutaneous in location and had ill-defined margins. On T1-weighted images, the lesion was isointense or slightly hyperintense to muscle. On T2-weighted images, the signal intensity was more heterogeneous, but the lesion was predominantly hyperintense. In three children who received contrast material, the lesion enhanced nearly homogeneously. CONCLUSION: Although SGA can often be diagnosed on the basis of clinical characteristics, MR imaging may be requested by practitioners unfamiliar with the lesion. In these cases, with recognition of typical MR imaging features, a limited biopsy and specific histologic preparation that includes mucin staining can be recommended.

Biopsy↗

Pustular psoriasis with chronic recurrent multifocal osteomyelitis and spontaneous fractures.

Chronic recurrent multifocal osteomyelitis is rarely associated with pustular psoriasis in children. We describe a child with severe pustular psoriasis in whom multiple fractures, bone deformities, and severe growth failure from chronic recurrent multifocal osteomyelitis developed. The recurrent fractures led to an incorrect assumption of child abuse. Both the bone disease and pustular psoriasis responded to treatment with etretinate.

Adolescent↗

Progress in the understanding of HIV infection: an overview.

The worldwide epidemic of human immunodeficiency virus (HIV) infection will likely be considered the most important public health event of the twentieth century. During the past 15 years, a wealth of information relating to the epidemiology, diagnosis, natural history, and treatment of HIV infection has accumulated. This article details the recent progress in each of these areas.

Acquired Immunodeficiency Syndrome↗

Neonatal pemphigus vulgaris in a child born to a woman in remission.

We describe the tenth reported case of neonatal pemphigus that mimicked Bart's syndrome and review previously published cases. Unlike previous cases, the child was born with significant blistering to a mother who was in complete remission throughout the pregnancy. High antepartum maternal titers of anti-intercellular space antibodies, increased maternal disease activity, and maternal disease that requires high doses of corticosteroids or use of combined therapy correlate with poor fetal outcome, including intrauterine death.

Adult↗

Eruptive pseudoangiomatosis: a unique childhood exanthem?

We describe three children with an acute onset and spontaneous resolution of angioma-like papules during an apparent viral illness. A biopsy specimen from one patient revealed a unique histologic appearance that consisted of dilated dermal blood vessels with plump hobnail-shaped endothelial cells. On the basis of the natural history and histopathologic features of this exanthem, we suggest the name eruptive pseudoangiomatosis.

Angiomatosis↗

Infectious disorders of the skin.

Cutaneous infections are common occurrences in clinical practice. Plastic surgeons need to recognize the varied presentations of cutaneous infections because they may mimic cutaneous malignancies or be a harbinger of underlying disease. We have reviewed the diagnosis and management of some of the more common bacterial, viral, and fungal infections of the skin.

Humans↗

Bacillary angiomatosis in a child undergoing chemotherapy.

Bacillary angiomatosis is an infectious disease of the skin and viscera characterized by vascular lesions, originally described in patients with human immunodeficiency virus infection. There are also case reports of bacillary angiomatosis occurring in immunocompetent patients and in noninfected patients with suppressed immune function. We report a case of bacillary angiomatosis in a child undergoing chemotherapy for acute leukemia.

Angiomatosis, Bacillary↗

Painful, plaque-like, pitted keratolysis occurring in childhood.

Pitted keratolysis is a superficial infection of the soles of the feet that is almost always asymptomatic. A painful variant of this disorder was reported to occur in adult males during military service. We report painful, plaque-like, pitted keratolysis in two children. Treatment with topical erythromycin was curative.

Adolescent↗

Cutaneous manifestations of pediatric HIV infection.

Children who are infected with human immunodeficiency virus may develop a wide variety of mucocutaneous manifestations, such as skin infections, tumors, and inflammatory skin disorders. The most significant infectious diseases are candidiasis, dermatophytosis, herpes simplex, herpes zoster, and pyoderma. Inflammatory disorders include seborrheic dermatitis, vasculitis, and pyoderma gangrenosum. Kaposi sarcoma is extremely rare in children with the acquired immunodeficiency syndrome.

AIDS-Related Opportunistic Infections↗