Human immunodeficiency virus and the Gianotti-Crosti syndrome.
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Acrodermatitis↗
Biomedical subjects
N B Esterly
Publications and source records attributed to N B Esterly.
Cutaneous telangiectases in neonatal lupus erythematosus.
BACKGROUND: Persistent telangiectases are a feature of neonatal lupus erythematosus (NLE) but have generally been noted in areas of prior inflammatory disease. The occurrence of vascular macules and papules at sites without preceding dermatitis has not been reported in NLE. OBJECTIVE: Our purpose was to emphasize a previously unidentified aspect of cutaneous NLE: the presence of angiomatous or matlike telangiectases in sites without antecedent or concurrent dermatitis. METHODS: We describe seven patients in which telangiectatic macules or angiomatous papules were primary or early features of NLE. RESULTS: Five infants lacked a history of preceding or concurrent inflammatory lesions at sites of telangiectasia development. In four infants findings included vulvar or inguinal angiomatous papules. CONCLUSION: Telangiectases may be a presenting feature of NLE, can be found in sun-protected sites, and may occur independent of "lupus dermatitis."
Child, Preschool↗
Neurocutaneous syndromes associated with pigmentary skin lesions.
Tremendous strides have been made in our understanding of genetic disorders, including those that involve both the skin and the nervous system. The list of well-established neurocutaneous syndromes has increased during a few decades from a few classical phakomatoses to more than 30 entities. The dermatologist has the opportunity to facilitate precise diagnosis and optimal care for these patients by recognition of the cutaneous markers for these diseases. We have attempted to provide an overview of some recent advances in those syndromes manifested by pigmentary skin changes, emphasizing the clinical spectrum of each disorder and providing guidelines for an approach to management.
Humans↗
Lymphomatoid papulosis in children.
BACKGROUND: Although lymphomatoid papulosis is well described in adults, the clinical course, prognosis, risk for lymphoma, and recommendations for follow-up have not been established in children. OBJECTIVE: Our aim was to analyze our data on six children with lymphomatoid papulosis and to analyze available information on reported cases from the literature to characterize better lymphomatoid papulosis in childhood and to compare it with adult-onset lymphomatoid papulosis. METHODS: Clinical records, laboratory studies, and histopathologic evaluation of skin biopsy specimens from six children with lymphomatoid papulosis were reviewed. A literature search was also performed and disclosed detailed information on 17 childhood cases. RESULTS: In most cases childhood lymphomatoid papulosis is clinically and histologically similar to lymphomatoid papulosis in adults, but three unusual patterns were identified in our children: first, after initial outbreak, dwindling outbreaks (both in frequency and number of lesions) until the eruption ceased completely; second, lymphomatoid papulosis localized to one area for years before generalizing, and third, presentation of lymphomatoid papulosis with hundreds of lesions. In our children and in those previously reported, response to systemic antibiotics and potent topical steroids was variable, as in adults. All our children to date have remained healthy; the longest period of follow-up is 9 years. However, in previously reported cases two patients with childhood-onset lymphomatoid papulosis had lymphoma as adults. CONCLUSION: Childhood lymphomatoid papulosis may be more likely to resolve spontaneously than adult lymphomatoid papulosis; nevertheless these children may still be at risk for lymphoma and thus need lifelong follow-up.
Adolescent↗
Nevoid basal cell carcinoma syndrome. Report of a case with associated Hodgkin's disease.
PURPOSE: Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal-dominant multisystem disorder characterized by various congenital anomalies, multiple cutaneous basal cell carcinomas (BCCs), and increased risk for other benign and malignant tumors. We report a 13-year-old girl with NBCCS who developed cutaneous BCCs 2 years after radiotherapy for Hodgkin's lymphoma. PATIENTS AND METHODS: Review of clinical and laboratory observations of patients with NBCCS suggest a close relationship between radiotherapy and the early emergence of numerous BCCs. However, in vitro studies of the effect of radiosensitivity on cells cultured from patients with NBCCS show inconsistent results. RESULTS: Clarification of the role of radiotherapy in the induction of BCCs in NBCCS requires additional studies. Nevertheless, recognition of the syndrome is important because whenever possible radiotherapy for secondary tumors might be effectively replaced by chemotherapy.
Adolescent↗
U1RNP positive neonatal lupus erythematosus: association with anti-La antibodies?
Neonatal lupus erythematosus (NLE) is an antibody-mediated disorder most commonly associated with autoantibodies to Ro and/or La antigens. There have been five previous reports describing eight NLE patients with anti-U1RNP antibody in the absence of anti-Ro and anti-La autoantibodies. We report two cases of anti-U1RNP antibody-positive NLE, and briefly review the five previous reports. The diagnosis of NLE was based on physical examination and serological studies by ELISA, immunodiffusion, and immunoblotting. By conventional immunodiffusion and ELISA, our cases were negative for anti-Ro and anti-La antibodies, and positive for anti-U1RNP antibody. However, one of the mothers had anti-La antibody detected by immunoblot assay only. All anti-U1RNP antibody-positive infants had classic cutaneous lesions of NLE, but it is of interest that none had congenital heart block (CHB). Although these infants were negative for anti-Ro and anti-La antibodies with immunodiffusion and ELISA techniques, these antibodies might be detectable by immunoblotting, as was the case in one of the mothers.
Adult↗
'Membranous aplasia cutis' with hair collars. Congenital absence of skin or neuroectodermal defect?
BACKGROUND: The skin and the nervous system are both derived from ectoderm. Separation of neural ectoderm from epithelial ectoderm occurs concurrently with the closure of the neural tube. This chronologic association may explain the cutaneous abnormalities often found overlying neural tube defects. A ring of dark long hair encircling a congenital scalp lesion (the hair collar sign) is one such marker and is often associated with encephaloceles, meningoceles, and heterotopic brain tissue. OBSERVATIONS: We describe six children with aplasia cutis who displayed the hair collar sign. Aplasia cutis is a relatively heterogeneous disorder; however, these lesions had a unique and strikingly similar appearance. This subtype of aplasia cutis, which we have termed membranous aplasia cutis, shares several clinical and histologic features with cranial neural tube defects. CONCLUSIONS: We propose that membranous aplasia cutis is a form fruste of a neural tube defect and may be derived from a similar embryological defect. Recent advances in the understanding of cranial neural tube closure may provide support for this hypothesis.
Atrophy↗
Recurrent palmoplantar hidradenitis in children.
BACKGROUND: There are several reports of tender, erythematous plantar nodules occurring in pediatric patients. Despite similar morphological features, the histologic findings in these lesions have been quite diverse. A new entity called idiopathic plantar hidradenitis (also termed neutrophilic eccrine hidradenitis in children), which is characterized by tender, red nodules on the feet and unique histopathologic features, was recently described. OBSERVATIONS: We describe two children with these unusual cutaneous lesions and histopathologic findings and compare them with patients with idiopathic plantar hidradenitis. Our patients, in contrast to those with idiopathic plantar hidradenitis, had involvement of the palms as well as the soles. Both children had self-limited recurrent lesions; in one child, the lesions were associated with low-grade fever. Biopsy specimen findings in both cases demonstrated dense neutrophilic infiltrates localized to the eccrine units. CONCLUSIONS: Our patients had clinical and histologic findings similar to those recently reported as idiopathic plantar hidradenitis (neutrophilic eccrine hidradenitis in children). Because palms and soles can be affected and the lesions typically recur, we suggest that this condition be referred to as recurrent palmoplantar hidradenitis.
Child↗
Familial scleroderma-like deformity of the fingers.
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Adolescent↗
The hair collar sign: marker for cranial dysraphism.
OBJECTIVE: To call attention to a cutaneous marker for neural tube closure defects of the scalp, the "hair collar" sign. This finding consists of a ring of long, dark, coarse hair surrounding a midline scalp nodule. METHODS AND RESULTS: Four children with small congenital scalp nodules and the hair collar sign were studied from the standpoint of clinical findings, radiologic scans, and histology of the excised nodules. All four had an overlying vascular stain in addition to the hair collar. Patients 1 and 2 were found to have encephaloceles, and one had heterotopic brain tissue. The fourth family refused surgery, but the clinical and radiologic findings were consistent with a diagnosis of atretic encephaloceles. One infant had agenesis of the corpus callosum and a Dandy-Walker malformation as associated findings. CONCLUSIONS: The "hair collar" sign should alert the pediatrician to the possibility of ectopic neural tissue in the scalp and/or underlying central nervous system malformations.
Agenesis of Corpus Callosum↗
X-linked dominant ichthyosis with peroxisomal deficiency. An ultrastructural and ultracytochemical study of the Conradi-Hünermann syndrome and its murine homologue, the bare patches mouse.
BACKGROUND AND DESIGN: The bare patches (Bpa) mouse expresses an X-dominant disorder that may be homologous to the Conradi-Hünermann (CH) syndrome in man; ie, both express ichthyosis, cataracts, and skeletal defects. To confirm their homology, we compared the light and electron microscopy of involved (I) vs uninvolved (U) skin from an infant with CH syndrome to Bpa mice during and after resolution of the scaling disorder. The peroxisomal content of Bpa and CH skin was evaluated by diaminobenzidine (DAB) ultracytochemistry (Bpa only) and by assessment of catalase (Bpa only) and dihydroxyacetone phosphate-acyltransferase (DHAP-AT) activities in cultured I vs U fibroblasts. RESULTS: Both CH and Bpa I epidermis exhibited psoriasiform features. In addition, ultrastructurally both exhibited the following: (1) vacuolated lamellar bodies, (2) dilatation of intercellular spaces by vesicular structures and amorphous debris, and (3) abnormal mitochondria. Stratum corneum interstices were filled with vesicular structures, and no lamellar unit structures were evident using ruthenium tetroxide postfixation. Peroxisomes were poorly stained by DAB in I Bpa epidermis and dermis during the eruptive phase. Moreover, catalase and DHAP-AT activities in cultured I Bpa fibroblasts were decreased to 40% and 30% of U Bpa levels, respectively; DHAP-AT activity in cultured I CH fibroblasts was also reduced (60% of U CH). With resolution of the scaling disorder, the ultrastructural and ultracytochemical features of I and U Bpa skin became indistinguishable. CONCLUSIONS: These studies provide evidence for a self-resolving defect involving multiple organelles, including lamellar bodies, peroxisomes, and mitochondria in the I skin of CH syndrome and the Bpa mouse.
Acyltransferases↗
Urticaria pigmentosa and Nager syndrome.
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Abnormalities, Multiple↗
Cutaneous polyarteritis nodosa of childhood.
BACKGROUND: The clinical presentation of childhood polyarteritis nodosa (PAN) can range from isolated cutaneous findings to widespread multisystem involvement. Both the systemic and cutaneous forms are known to occur after streptococcal infection. OBJECTIVE: Our purpose was to emphasize the frequent association of childhood cutaneous PAN with antecedent streptococcal infection. METHODS: We discuss four cases of cutaneous PAN that were associated with streptococcal infection and briefly review the pertinent literature. RESULTS: All patients had evidence of preceding streptococcal infection at the onset of their illness. Although mild systemic symptoms were present in all, their course was benign. The use of nonsteroidal antiinflammatory agents or corticosteroids resulted in clinical improvement. Antibiotic therapy was also used in the treatment of these patients. CONCLUSION: In children with PAN, evaluation should include laboratory studies to detect streptococcal infection.
Adolescent↗
Acrally occurring dermatofibrosarcoma protuberans in children and adults.
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) arising on the hands and feet is unusual and, at any site, is extremely rare in children. OBJECTIVE: We report a 5-year-old girl who developed DFSP on her hand at 9 months of age. We review the clinical features and treatment of childhood DFSP and of acrally occurring DFSP at all ages. CONCLUSION: DFSP in children develops most often on the back (22.2%) and at acral sites (14.8%). It occurs on the hands and feet of children more commonly than in adults. This may be related to the increased frequency of trauma at these sites in children. Mohs micrographic surgery is the treatment of choice for DFSP.
Adult↗
Pyoderma gangrenosum in infants and children.
Pyoderma gangrenosum is an uncommon ulcerative skin disorder that occurs in all age groups. Approximately 4% of patients are infants and children. There are several notable differences between the childhood and adult manifestations of the disease, including the distribution of lesions and associated disorders. We reviewed the childhood cases (< or = 18 yrs of age) of unequivocal pyoderma gangrenosum in the English literature and tabulated the trends in clinical features, associated disorders, and therapy. We report our 3-week-old patient, the youngest documented case. Of the 46 patients, only 4 were less than 1 year of age. A systemic illness was present in 74% of the older children, most commonly, ulcerative colitis. Only one infant had an associated problem (HIV+) at the time of onset. Infants appear to have an unusual distribution of perianal and genital lesions not often described in other age groups. Our review suggests that pyoderma gangrenosum in children has a similar clinical appearance to that in adults. It is associated with some of the same underlying disorders, but with different frequencies. The distribution of lesions in children is similar, often involving the lower extremities, but pyoderma gangrenosum of the head and face appears to be more common in children. Infants may have ulcers in genital and perianal areas. The most frequently prescribed treatment for children is systemic corticosteroids, which generally are very effective.
Adolescent↗
Firm black exophytic tumor on the foot of a black child.
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Black People↗
Cutaneous mucinosis of infancy.
Cutaneous mucinosis of infancy has been reported only rarely in the literature. We describe a case occurring in a black infant girl. Although no associated abnormalities have been described previously, our patient had a history of developmental delay, congenital cataracts, bilateral inguinal hernias, and an accessory tragus. The significance of these features is unclear.
Cataract↗
Atopic dermatitis and ichthyosis vulgaris.
Atopic dermatitis remains a common skin problem in the pediatric age group. General approaches to management focus on reducing inflammation and pruritus as well as preventing xerosis. Ichthyosis vulgaris is the most common form of the ichthyoses and often is associated with atopic dermatitis. Recognition of these conditions is necessary to institute therapy that will alleviate the discomfort experienced by affected individuals.
Child, Preschool↗