RESUMEN
BACKGROUND: Paediatric scalp naevi may represent a source of anxiety for practitioners and parents, as the clinical and dermoscopic features of typical naevi have yet to be defined. Prompted by concern about the large size, irregular borders and colour variation of scalp naevi, clinicians and parents may request unnecessary excision of these naevi. OBJECTIVES: To establish the typical clinical and dermoscopic patterns of scalp naevi in children younger than 18 years old to help optimize clinical care and management. METHODS: Scalp naevi were imaged with a camera (Canon Rebel, XSi; Canon, Tokyo, Japan) and dermoscopic attachment (Dermlite Foto, 30 mm lens; 3Gen, San Juan Capistrano, CA, U.S.A.) to the camera. The clinical and dermoscopic images were reviewed and analysed. Both acquired and congenital scalp naevi were included but were not further differentiated from each other. RESULTS: We obtained clinical and dermoscopic images of 88 scalp naevi in 39 white children. Two subjects had received chronic immunosuppressive medication. Nineteen children had a family history of melanoma. Boys (18/39 subjects, 46%) possessed 68% (60 naevi) of scalp naevi imaged. Younger (< 10 years old) subjects (24/39 subjects, 62%) possessed 42% (37 naevi) of scalp naevi. The main clinical patterns included eclipse (n=18), cockade (n = 3), solid brown (n=42) and solid pink (n=25) naevi. Solid-coloured naevi showed the following dermoscopic patterns: globular (57%), complex (reticular-globular) (27%), reticular (9%), homogeneous (6%) and fibrillar (1%). The majority of naevi had a unifying feature - perifollicular hypopigmentation, which caused the appearance of scalloped, irregular borders if occurring on the periphery, or variegation in pigmentation, if occurring within the naevi. CONCLUSIONS: Older subjects and boys tend to harbour a larger proportion of scalp naevi. The main clinical patterns include solid-coloured and eclipse naevi. The most common dermoscopic pattern of scalp naevi is the globular pattern. Perifollicular hypopigmentation is a hallmark feature of signature scalp naevi. Dermoscopy is a noninvasive tool in the evaluation of cutaneous melanocytic lesions in children and may decrease the number of unnecessary excisions.
Asunto(s)
Neoplasias de Cabeza y Cuello/patología , Nevo Pigmentado/patología , Cuero Cabelludo/patología , Neoplasias Cutáneas/patología , Adolescente , Distribución por Edad , Niño , Preescolar , Femenino , Color del Cabello , Neoplasias de Cabeza y Cuello/epidemiología , Humanos , Masculino , Nevo Pigmentado/epidemiología , Distribución por Sexo , Neoplasias Cutáneas/epidemiología , Población BlancaRESUMEN
Trichothiodystrophy (TTD) is a rare group of autosomal recessive disorders of DNA repair unified by the presence of sulfur-deficient brittle hair. We report a 3-year-old boy with classic clinical features of TTD, including ichthyosis, alopecia, developmental delay, and tiger-tail banding of the hair shaft on polarizing microscopy. Brain MR imaging showed both diffuse dysmyelination and osteosclerosis, findings that, in combination, may be specific for TTD.
Asunto(s)
Enfermedades Desmielinizantes/complicaciones , Osteosclerosis/complicaciones , Síndromes de Tricotiodistrofia/complicaciones , Preescolar , Enfermedades Desmielinizantes/diagnóstico , Humanos , Imagen por Resonancia Magnética , Masculino , Osteosclerosis/diagnósticoRESUMEN
Waxy keratoses of childhood is a rare genodermatosis previously noted in both familial and isolated presentations. Three previous cases have been described in which the lesions appeared over the trunk and extremities. We report a case in which the waxy keratoses of childhood lesions appeared in a segmental distribution along a single lower extremity, differing from the more extensive patterns reported previously. We suggest a possible postzygotic mutation hypothesis for this unique segmental distribution, addressing both the possibility of a genomic mosaicism and loss of heterozygosity.
Asunto(s)
Queratosis/genética , Niño , Diagnóstico Diferencial , Femenino , Humanos , Queratosis/diagnóstico , Queratosis/patologíaRESUMEN
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.
Asunto(s)
Conjuntiva/patología , Disqueratosis Congénita/patología , Mucosa Bucal/patología , Adolescente , Adulto , Diagnóstico Diferencial , Disqueratosis Congénita/genética , Femenino , HumanosRESUMEN
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.
Asunto(s)
Epidermis/patología , Enfermedades del Prematuro/patología , Enfermedades de la Piel/patología , Resultado Fatal , Femenino , Herpes Simple/complicaciones , Humanos , Recién Nacido , Necrosis , Enfermedades de la Piel/complicacionesRESUMEN
Two infants, one with a T-cell-signaling defect resulting in a primary immunodeficiency syndrome and the other with severe combined immunodeficiency (SCID), are described. Both infants developed cutaneous infections secondary to their bacillus Calmette-Guérin (BCG) vaccinations. Both patients were from countries where BCG is routinely administered in infancy. The infant with the T-cell-signaling defect developed a disseminated infection involving the skin, while the infant with SCID developed a localized cutaneous infection at the site of his BCG immunization. These two cases resemble other reported cases of cutaneous BCG infection following routine vaccination in immunocompromised patients. Mycobacterium bovis infection should be considered in patients with cutaneous eruptions who have received BCG vaccination, especially those who are immunocompromised.
Asunto(s)
Vacuna BCG/efectos adversos , Síndromes de Inmunodeficiencia/inmunología , Mycobacterium bovis , Inmunodeficiencia Combinada Grave/inmunología , Tuberculosis Cutánea/etiología , Tuberculosis/prevención & control , Vacunación/efectos adversos , Humanos , Lactante , MasculinoRESUMEN
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.
Asunto(s)
Anemia de Células Falciformes/tratamiento farmacológico , Antidrepanocíticos/efectos adversos , Hidroxiurea/efectos adversos , Hiperpigmentación/etiología , Enfermedades de la Uña/etiología , Enfermedades de la Piel/etiología , Adolescente , Niño , Femenino , Humanos , Hiperpigmentación/patología , Masculino , Enfermedades de la Uña/patologíaRESUMEN
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.
Asunto(s)
Hamartoma/genética , Enfermedades Cutáneas Vesiculoampollosas/genética , Enfermedades de la Piel/genética , Adolescente , Adulto , Niño , Preescolar , Femenino , Genes Dominantes , Hamartoma/patología , Humanos , Masculino , Persona de Mediana Edad , North Carolina , Linaje , Fenotipo , Enfermedades de la Piel/patología , Enfermedades Cutáneas Vesiculoampollosas/patología , SíndromeAsunto(s)
Enfermedades de la Boca/diagnóstico , Pénfigo/diagnóstico , Tiña del Cuero Cabelludo/complicaciones , Niño , Diagnóstico Diferencial , Extremidades , Femenino , Humanos , Enfermedades de la Boca/complicaciones , Enfermedades de la Boca/patología , Pénfigo/complicaciones , Pénfigo/patologíaRESUMEN
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.
Asunto(s)
Granuloma Anular/diagnóstico , Imagen por Resonancia Magnética , Enfermedades de la Piel/diagnóstico , Biopsia , Preescolar , Colorantes , Tejido Conectivo/patología , Medios de Contraste/administración & dosificación , Femenino , Células Gigantes/patología , Granuloma Anular/patología , Histiocitos/patología , Humanos , Inyecciones Intravenosas , Masculino , Mucinas/análisis , Rótula/patología , Estudios Retrospectivos , Enfermedades de la Piel/patología , Tibia/patologíaAsunto(s)
Pueblo Asiatico , Población Negra , Enfermedades de la Piel/patología , Alopecia/patología , Asiático , Niño , Dermatitis/patología , Humanos , Trastornos de la Pigmentación/patología , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/etiología , Enfermedades de la Piel/terapia , Neoplasias Cutáneas/patología , Pigmentación de la PielRESUMEN
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.
Asunto(s)
Fracturas Espontáneas/etiología , Osteomielitis/complicaciones , Psoriasis/complicaciones , Adolescente , Enfermedad Crónica , Femenino , Fracturas Espontáneas/patología , Humanos , Osteomielitis/tratamiento farmacológico , Osteomielitis/patología , Psoriasis/tratamiento farmacológico , RecurrenciaRESUMEN
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.
Asunto(s)
Angiomatosis/patología , Enfermedades Cutáneas Vasculares/patología , Mejilla , Niño , Endotelio Vascular/ultraestructura , Extremidades , Humanos , Lactante , Masculino , Microscopía ElectrónicaRESUMEN
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.
Asunto(s)
Pénfigo/congénito , Adulto , Diagnóstico Diferencial , Femenino , Humanos , Recién Nacido , Pénfigo/tratamiento farmacológico , Pénfigo/patología , Prednisona/uso terapéuticoRESUMEN
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.
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Infecciones por VIH , Síndrome de Inmunodeficiencia Adquirida/diagnóstico , Síndrome de Inmunodeficiencia Adquirida/tratamiento farmacológico , Síndrome de Inmunodeficiencia Adquirida/epidemiología , Síndrome de Inmunodeficiencia Adquirida/historia , Síndrome de Inmunodeficiencia Adquirida/patología , Síndrome de Inmunodeficiencia Adquirida/transmisión , Infecciones por VIH/diagnóstico , Infecciones por VIH/tratamiento farmacológico , Infecciones por VIH/epidemiología , Infecciones por VIH/historia , Infecciones por VIH/patología , Infecciones por VIH/transmisión , Historia del Siglo XX , HumanosRESUMEN
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.