bluuebunny/crossref_metadata_2025 huggingface dataset:
Therapeutic Efficacy, Secondary Effects, and Patient Acceptability of 10% Sulfur in Either Pork Fat or Cold Cream for the Treatment of Scabies
Authors: Avila‐Romay Alfoncina , Alvarez‐Franco Maira , Ruiz‐Maldonado Ramon
Published: March 2008

Abstract: Twenty‐six children with scabies and 32 contacts were treated with 10% sulfur In cold cream. A 100% clinical cure rate was observed, although 56.8% of patients experienced some kind of mild, transient cutaneous reaction. An additional 25 children with scabies and 28 contacts were treated with 10% sulfur and 1% salicylic acid in pork fat. Of these, 88% were clinically cured and 73.5% had some cutaneous adverse effects, in both groups, most adverse effects were related to skin dryness or postscabetic reaction. The cold cream base was more acceptable to patients than the pork fat base. However, the pork fat base was significantly cheaper and easier to obtain than the cold cream base, and 238 times less expensive than the cheapest commercial scabicidal medication available in the United States.
Psychologic Aspects of Acne
Authors: Koo John Y. M. , Smith Laura L.
Published: March 2008

Abstract: Psychologic factors may play a significant role in acne in at least three ways. First, as many patients readily report, emotional stress can exacerbate acne. Second, it is common for patients to develop psychiatric problems as a consequence of the conditions, such as those related to low self‐esteem, social phobias, or depression. Finally, primary psychiatric illnesses such as obsessive‐compulsive disorder and psychosis may be based on a complaint that is focused on acne. In this review we hope to facilitate an understanding of how emotional factors and acne connect, and aid the dermatologist in identifying the possible presence of psychiatric problems stemming from or contributing to the disorder.
Oral Acyclovir Therapy for Varicella and Zoster Infections in Pediatric and Pregnant Patients: A Brief Review
Authors: Rothe Marti Jill , Feder Henry M. , Grant‐Kels Jane M.
Published: March 2008

Abstract: Oral acyclovir recently was approved for the treatment of herpes zoster and has been shown to be effective in the treatment of varicella. Studies of the use of high‐dose oral acyclovir in the management of varicella‐zoster infections in immunocompetent pediatric patients are reviewed. Guidelines are provided for the drug s use in immunocompetent children and adolescents, pregnant patients, and pediatric atopic patients receiving systemic steroids.
Lichen Aureus in Childhood
Authors: Gelmetti Carlo , Cerri Danilo , Grimalt Ramon
Published: March 2008

Abstract: Lichen aureus is a rare asymptomatic dermatosis of unknown origin that is now classified in the group of pigmented purpuric dermatoses. The eruption consists of asymptomatic, roundish or irregular, lichenoid erythematous‐purpuric papules with a tendency to coalesce in patches, most prevalent on the limbs. No meaningful laboratory abnormalities have been found. Histologically, the epidermis is normal, with a lymphohistiocytic, bandlike infiltrate with extravasated blood red cells and hemosiderin deposits observed in the dermis. During the last 20 years we have followed eight new patients, which represents 0.05% of our hospitalized patients. In five of eight patients the disease resolved in two to four years. No therapy has been carried out on these children, since lichen aureus has a tendency for slow, spontaneous improvement and resolution.
Infantile Acropustulosis
Authors: Dromy Rachel , Raz Aryeh , Metzker Aryeh
Published: March 2008

Abstract: Infantile acropustulosis is a recurrent, pruritic, vesicopustular eruption appearing mostly on the palms and soles. The present survey of 25 children with infantile acropustulosis is the most extensive reported since this entity was first described in 1979. Our cases conform to the recognized clinical course, but disclaim any male or ethnic origin predominance. Although the etiology of infantile acropustulosis is obscure, it may be ascribed to some infective agent. It is not limited to young children, and has been found in the siblings of patients who were followed. Suppressive therapeutic measures, such as topical corticosteroids under occlusion, were found to be effective. The disorder does not seem to be as rare as one might gather from the literature.
Nevus Comedonicus: Case Report and Brief Review of the Literature
Authors: Cestari Tania F. , Rubim Mercedes , Valentini Berenice C.
Published: March 2008

Abstract: We report extensive nevus comedonicus in a female patient that involved half of her body entirely, with infected cystic lesions as well as typical scars, limited by the midline. The lesions worsened at the beginning of puberty. A brief review of the literature highlights the histopathologic, etiopathogenic, and therapeutic aspects.
Linear IgA Dermatosis of Childhood: Case Report with an Immunoelectron Microscopic Study
Authors: Legrain Valérie , Taieb Alain , Surlève‐Bazeille Jean‐Etienne , Bernard Philippe , Maleville Jean
Published: March 2008

Abstract: A 7‐year‐old girl developed a cutaneous bullous eruption with genital and nasal mucous membrane involvement. Direct immunofluorescence revealed linear deposits of IgA and IgM at the basement membrane zone. No circulating antibasement membrane zone antibodies were detected. Small bowel biopsies showed a partial villous atrophy. The clinical, histologic, and immunopathologic findings were consistent with a diagnosis of linear IgA dermatosis of childhood. Immunoelectron microscopy revealed IgA deposits in the lamina lucida in association with hemidesmosomes, confirming results of two recent studies.
Allergy Consultation in the Management of Atopic Dermatitis
Authors: Apter Andrea J. , Rothe Marti J. , Grant‐Kels Jane M.
Published: March 2008

Abstract: Allergists and dermatologists both care for patients with atopic dermatitis, and their roles may be complementary. Questions regarding food allergy, aeroallergens, and the interpretation of skin and RAST testing are answered by an allergist.
Childhood Rosacea
Authors: Drolet Beth , Paller Amy S.
Published: March 2008

Abstract: Rosacea usually occurs in adults and rarely has been noted in children. We recently observed three children with rosacea, all of whom responded dramatically to systemic and topical antibiotics. Rosacea in childhood must be distinguished from other erythematous facial disorders, most commonly acne, granulomatous perioral dermatitis, and sarcoidosis. The distribution of facial lesions; the presence of telangiectasias, flushing, and pustules; and the appearance of lesional biopsy sections and the ocular lesions, if present, allow differentiation of rosacea from other facial eruptions.
Neurocutaneous Melanosis in Association with the Dandy‐Walker Complex
Authors: Kadonaga Julie N. , Barkovich A. James , Edwards Michael S. B. , Frieden Ilona J.
Published: March 2008

Abstract: An infant had a giant congenital nevus, neurocutaneous melanosis (NCM), and a Dandy‐Walker malformation of the brain. The diagnosis of NCM was suspected at 6 weeks of age when macrocephaly was noted, resulting in the discovery of hydrocephalus and a Dandy‐Walker Malformation. Serial Magnetic resonance imaging scans demonstrated so‐called T1 shortening in the pia or subarachnoid spaces surrounding the cerebellar vermis and in the temporal lobes anterior to the temporal horns. Eventually, a biopsy‐proved melanoma developed in the anterior temporal lobe, in an area previously noted to have T1 shortening. Since meningeal cells have been shown experimentally to play a critical role in cerebellar development, we hypothesize that the association of NCM with a Dandy‐Walker malformation may be due to meningeal melanosis disrupting the normal development of the cerebellum and fourth ventricle.
Imported Fire Ant Stings: Clinical Manifestations and Treatment
Authors: Cohen Philip R.
Published: March 2008

Abstract: Stings by imported fire ants result in local reactions to the insect's venom; less frequently, they induce systemic allergic reactions. A 19‐month‐old child developed a characteristic localized cutaneous reaction at the sites of numerous stings after disturbing an ant mound. He experienced no secondary complications, and his pustular lesions resolved with conservative treatment.
CONGENITAL MALALIGNMENT OF THE GREAT TOENAILS
Authors: ROBERT BARAN
Published: March 2008

Letters to the Editor are welcomed for publication (subject to editing). Letters must be signed by all authors, typewritten double spaced, and must not exceed two pages of text including references. Two copies of all letters should be submitted. Letters should not duplicate material submitted or published in other journals. Pre‐publication proofs will not bet provided.
Nutrition in Dystrophic Epidermolysis Bullosa
Authors: Allman Susan , Haynes Lesley , MacKinnon Petrina , Atherton David J.
Published: March 2008

Abstract: In dystrophic epidermolysis bultosa (EB), a combination of diminished food intake and Increased nutritional requirements commonly leads to mainourishment Adequate nutrition in these patterns could pro‐vide many benefits, including Improved growth, accelerated healing, de‐creased susceptibility to infection, and enhanced well‐being. We as‐sessed nutrition status of children with dystrophic EB, and evaluated the benefits of nutritional advice in those who were considered malnour‐ished. The majority of patients had inadequate Intakes of a wide spectrum of nutrients, Including those who appeared to be relatively mildly af‐fected. We were unable to secure persisting substantial Improvements In dietary intake despite thorough counseling. Adequate nutrition In pa‐tients with dystrophic EB depends on active nutritional support starting from birth. The need for more invasive techniques of nourishment, such as nasogastric and gastrostomy feeding, may have to be considered earlier than Is currently the case.
Accessory Nipples: Any Relationship to Urinary Tract Malformation?
Authors: Armoni Michael , FUk Daniel , Schlesinger Menachem , Pollak Shlomo , Metzker Aryeh
Published: March 2008

Abstract: One hundred two Infants and children age 3 days to 16.5 years, found to have accessory nipples (AN), went enrolled in tills study. They were categorized by ethnic origin, sex, positive family history of AN, and number, site, and shape of AN, to determine factors for Increased risk of anomalies of the urinary tract Physical and ultrasound examinations of the abdomen did not reveal evidence of urinary tract malformation In any of the children. The results of this survey support the contentions that AN are not associated with urinary tract matformatlons, and that no further investigation is required In children with solitary AN.
Painful, Plaque‐like, Pitted Keratolysis Occurring in Childhood
Authors: Shah Anita S. , Kamino Hideko , Prose Neil S.
Published: March 2008

Abstract: 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 top‐ical erythromycln was curative.
Neurothekeoma in a 15‐Year‐Old Boy: Case Report
Authors: Pepine Marci , Flowers Franklin , Ramos‐Caro Francisco A.
Published: March 2008

Abstract: A 15‐year‐old boy experienced a rapidly enlarging nodule on his left nose. Histologic findings and immunoperoxidase stains were consistent with the diagnosis of neurothekeoma. The patient was treated with local excision without evidence of recurrence.
Ultraviolet Light Burn:A Cutaneous Complication of Visible Light Phototherapy of Neonatal Jaundice
Authors: Siegfried Elaine C. , Stone Mary S. , Madison Kathi C.
Published: March 2008

Abstract: Visible light phototherapy is an easily administered and effective treatment for neonatal indirect hyperbilirubinemia. Reported cutaneous side effects include transient rashes and the uncommon bronze baby syndrome. A more hazardous side effect is ultraviolet burn. Two premature infants developed phototherapy‐induced erythema, one associated with a second‐degree burn, after exposure to fluorescent daylight bulbs inadvertently used without Plexiglass shields, thus allowing prolonged ultraviolet A (UVA) exposure. Premature infants, especially during the first two weeks of life, may be significantly susceptible to UVA‐Induced erythema. Plexiglass shields should always be In place during visible light phototherapy, and nursery staff should be made aware of their purpose.
Cutaneous Manifestations of Pediatric HIV Infection
Authors: Prose Neil S.
Published: March 2008

Abstract: 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, vasoulitis, and pyoderma gangrenosum. Kaposi sarcoma is extremely rare in children with the acquired immunodeficiency syndrome.
Sexually Transmitted Diseases in Children
Authors: Lowy Gabriela
Published: March 2008

Abstract: Sexually transmitted diseases (STDs) in children may be transmitted by sexual abuse, by accidental contact, or perinatally. Although only 2% to 10% of abused children become infected, childhood syphilis, gonorrhea, condylomata acuminata, and Chlamydia trachomatis must always be considered. We reviewed data from our hospital regarding the frequency, prevalence, routes of transmission, and clinical features of these infections. Ninety‐five percent of acquired syphilis in children is transmitted by sexual abuse. The perpetrator is usually someone the child knows or trusts. Of our 21 patients under 14 years of age with acquired syphilis, most were 4 to 8 years old. Girls were infected twice as often as boys. Sexual contact was confirmed in 71.4%. A chancre sore wss infrequent in children; condylomata lata was the most frequent cutaneous lesion (80.9%). In the last 10 years, 102 cases of congenital syphilis were diagnosed In our hospital. The main clinical findings were bone involvement (78.7%), hepatosplenomegaly (68.8%), cutaneous lesions (50.8%), and jaundice (15.1%). Gonorrhea was detected in only nine children. Vulvovaginitis was the most common clinical manifestation. Sexual transmission was documented in three patients. Accidental contact with their infected mother occurred in two sisters. Three newborns acquired the disease during delivery. The STDs in children are a worrisome problem. Evaluation for sexual abuse should be done in all cases. Prevention and treatment of adults are the main steps to prevent these infections in children.
Investigation of the Photosensitive Child
Authors: Ferguson James
Published: March 2008

Abstract: Photosensivity disorders in childhood frequently can be diagnosed and managed in the general dermatology clinic. Occasionally, when diagnostic doubt exists, referral to a specialist unit is required for diagnostic phototesttng. Light testing equipment is fickle by nature, making such units uncommon. Phototesting using monochrometor or provocation systems takes approximately 45 minutes. Immediate and delayed readings over the following 48 hours as appropriate are required to cover the diagnostic possibilities. Individual diseases are characterized by particular patterns of wavelength dependency and evolution of the abnormal response. Other investigations that may be required are autoantibodies to exclude lupus erythematosus, a porphyrin scan leading to full studies and, on occasion, cell mutation or survival, and chromosome studies for the rarer genophotodermatoses. Good investigative data frequently help clarify the common clinical variants.
Ichthyosis: Mechanisms of Disease
Authors: Williams Mary L.
Published: March 2008

Abstract: The disorders of cornification (Ichthyoses) comprise acquired and Inherited disorders characterized clinically by generalized sealing and histologically by hyperkeratosls. They may arise through defects In the production or maintenance of a normal cornmed cell compartment, or both. The stratum cornaum is composed of protein‐enriched and llpid‐depleted corneocytes (“bricks”) surrounded by an Intercellular domeln (“mortar”) composed of hydrophoblc, llpid‐enriched membrane bllayers, and containing desmosomes and a limited array of hydrolytfc enzymes. Mechanisms whereby a genetic defect Involving either the bricks or the mortar may result In abnormal stratum comeum retention are diecussed using lehthyosls vulgaria and recessive X‐linked ichthyosis as examples. In addition, epidermal hyperproliferation, which floods the cornfiled cell compartment with incompletely formed units, results In hyperkeratosis. To date, no primary disorders of epidermal hyperproliferation have been defined. Recent work, however, demonstrates that stratum corneum barrier function regulates epidermal DMA synthesis. For example, in essential fatty acid deficiency, barrier dysfunction Is responsible at least In part for the epidermal hyperprollferatlon. Defective barrier function due to detective lamellar body secretion may also underlbe the phenotypic changes after birth In harlequin Ichthyosis; that is, from the massive, constrictive hyperkeratosis of the newborn to an exfoliative erythroderma In survivors. The mechanisms whereby specific detects in cornlification result In generalized scaling disease are only beginning to be defined. Yet, even at this early stage, the view of the stratum corneum as a tightly organized structure whose function Is highly regulated is emerging. Hence, the disorders of comlflcation should provide Important insights into stratum comeum structure and function.
Atopic Dermatitis: Studies of Skin Permeability and Effectiveness of Topical PUVA Treatment
Authors: Ogawa Hideoki , Yoshiike Takashi
Published: March 2008

Abstract: Uttraviolet light is effective treatment for patients with atoptc dermatitis that is resistant to conservative therapy, or complicated by adverse effects of extended steroid use. We designed a protocol using topical paoraton chemotherapy with ultraviolet A (PUVA) to treat atopic dermatitis In 114 patients. Clinical results were excellent, with complete clearing in 50% of patients receiving dally treatment. Histologic and immunologic values correlated with the clinical response, including reduced epidermal thickness, and decreased numbers of epidermal Lang‐erhans cells and dermal mast and mononuclear cell Infiltrates. The pattern of keratin 14‐positive keratinocytes returned toward normal. In addition, the water‐holding capacity of the stratum corneum increased to near normal levels. We also studied stratum comeum permeability in lesional and nonlesional skin using the dimethyl sulfoxide wheallng test and theophy line absorption studies. Compared wtth controls, permeabuIity was markedly increased in lesional skin and mildly Increased In nonlesional skin in patients with atopte dennatatis. These results suggest that Immune abnormalities and barrier dysfunction participate in the patho genesis of atopic dermatitis.
Self‐Healing Collodion Baby: Evidence for Autosomal Recessive Inheritance
Authors: Frenk Edgar , De Techtermann Francois
Published: March 2008

Five spontaneously healing collodion babies were recorded in a large Swiss kindred. They all had consanguineous parents. Their distribution in the family indicates autosomal recessive inheritance. At birth they had the typical features of collodion babies. The collodion‐like membrane was shed within the first month, leaving a slightly scaly skin for a few weeks. Thereafter the skin remained normal without any evidence of a disorder of cornification.
Ki‐1 Large Cell Lymphoma with Regressing Lesions in a Child
Authors: Bittencourt Achiléa Lisboa , Mendonça Nubia , De Freitas Luiz Antonio Rodrigues , Schnitzer Bertram
Published: March 2008

An 8‐year‐old boy was seen with a cutaneous Ki‐1 anaplastic, large cell lymphoma with multiple lesions. Some of the lesions showed spontaneous regression. During more than seven years of disease no systemic Involvement was observed, but recurrent, self‐healing lesions did appear. Histopathologic examination of five lesions revealed a variety of findings, from an inflammatory infiltrate to a highly anaplastic pattern. The neoplastic cells expressed Ki‐1 and leukocyte common antigens. Ultrastructurally, those cells showed ruffled indentations. The differential diagnosis includes microvillous malignant lymphoma. The patient has had a four‐year follow‐up without relapses.
Correspondence
Authors: LEUNG ALEXANDER K. C. , WHEELER BRUCE H. , ROBSON WM. LANE M. , KOSSAKOWSKA ANNA E.
Published: March 2008

Letters to the Editor are welcomed for publication (subject to editing). Letters must be signed by all authors, typewritten double spaced, and must not exceed two pages of text including references. Two copies of all letters should be submitted. Letters should not duplicate material submitted or published in other journals. Pre‐publication proofs will not be provided.
Giant Mast Cell Granules in a Solitary Mastocytoma
Authors: Kawai Shuzou , Okamoto Hiroyuki
Published: March 2008

Abstract: A 4‐month‐old girl had an isolated, reddish brown nodule on her back that blistered repeatedly. Toluidine blue staining of a biopsy specimen showed a dense infiltration of mast ceils in the upper dermis. In addition, large granules that stained similarly to the normal mast cell granules were observed. Electron microscopic studies disclosed that these giant granules (2–4 pm diameter) had the characteristic substructures of mast cell granules, that is, lamellar and scroll‐like forms. Some giant mast cell granules lost their electron density, suggesting a degran‐utatton process. Aggregates of normal‐size granules with varying degrees of electron density were also observed. We assume that trie variations in morphology of these giant granules represent their maturation process.
Acute Alopecia: Clue to Thallium Toxicity
Authors: Feldman Jerry , Levisohn Dianne R.
Published: March 2008

Abstract: The combination of rapid, diffuse alopecia, and neurologic and gastrointestinal disturbance is pathognomonic for thallium toxicity. The hair mount, showing a tapered or bayonet anagen hair with black pigmentation at the base, may be highly diagnostic before the onset of alopecia. We saw a 10‐year‐old boy who suffered from thallium poisoning.
Congenital Dermatofibrosarcoma Protuberans
Authors: Annessi Giorgio , Cimitan Augusto , Girolomoni Giampiero , Giannetti Alberto
Published: March 2008

Abstract: Congenital dermatofibrosarcoma protuberans occurred in a 16‐year‐old girl. The lesion was a hard cutaneous plaque on the abdomen. It was present at birth and slowly enlarged during the patient's lifetime. Histologic examination was essential to establish the diagnosis and to differentiate it from other congenital fibrohistiocytic proliferations.
Supraumbilical Midabdominal Raphe, Sternal Atresia, and Hemangioma in an Infant: Response of Hemangioma to Laser and Interferon Alfa‐2a
Authors: Blei Francine , Orlow Seth J. , Geronemus Roy G.
Published: March 2008

Abstract: We cared for an Infant girl with the clinical constellation of supraumbllical midabdomlnal raphe, sternal atresia, and cutaneous facial and upper trunk hemangioma. This is the first report of this clinical association in the dermatologic literature. The vascular component of the disorder responded to fiashlamp‐pumped pulsed dye laser therapy and to systemic Interferon alfa‐2a (Roferon‐A).
Childhood Condyloma Acuminatum: Association with Genital and Cutaneous Human Papillomaviruses
Authors: Obalek Slavomir , Misiewicz Joanna , Jablonska Stefania , Favre Michel , Orth Gerard
Published: March 2008

Abstract: We studied 25 children, age 7 months to 12 years 6 months, with anogenltal warts, and their parents. In most children the warts were localized in the anal area, In 3 of 18 girls perianally and on the vulva, and in 4 girls exclusively on the vulva. Southern blot hybridization studies disclosed an association of condylomata with human papllormavlruses (HPV) 6 and 11 in 74% and HPV 2 In 17.4% ol patients. The clinlcal features were similar in warts Induced by genital and cutaneous HPVs. Even the HPV 2‐associated warts in the vulva of two girls were typical of condyloma acuminatum.

In all children with HPV 2‐induced condytomata, cutaneous common warts coexisted, also induced by HPV 2. However, three mothers had cutaneous warts, and the children's condylomata were associated with HPV 6. Thus, the mere presence of skin warts in family members does not rule out other sources of infection. Sexual abuse was suspected in four girls and two boys, but was not confirmed in any. Nonsexual transmission could occur by persons with the lesions taking care of children. Perinatal transmission also appears to be an important route of infection In small babies. Infection In utero was probable In one girl in whom anal warts appeared in the first week of life and whose mother had cervical condylomata during pregnancy. This study provides further confirmation of possible nonsexual transmission of genital HPVs and the not infrequent association of childhood condylomata with HPV 2.
Trichothiodystrophy and Associated Anomalies: A Variant of SIBIDS or New Symptom Complex?
Authors: Hersh Joseph H. , Klein Laura R. , Joyce Margie R. , Hordinsky Maria K. , Tsai Michael Y. , Pafler Amy , Hyzer Resa , Zax Robert H.
Published: March 2008

Abstract: Trichothiodystrophy is characterized by sparse, short, sulfur‐deflclent hair. Numerous symptom complexes have been described in which the hair abnormaiity represents a constant feature. We report a boy with trichothiodystrophy, ichthyotic skin changes, onychodystrophy, chronic neutropenia, osteoscierosis, hypothyroidism, nystagmus, growth and mentai retardation, and microcephaiy, who developed a progressive encephalopathy with ataxia and optic atrophy at 2.5 years of age. in addition to a deficient cystine ievei identified on a hair sampie, a disturbance in the composition of other amino acids was present. Ai‐though features were reminiscent of osteoscierosis, ichthyosis, brittie hair due to trichothiodystrophy, impaired inteliigence, decreased fertiiity, and short stature (SIBIDS) and couid represent a variant of this disorder, findings in our patient may refiect a new trichothiodystrophy symptom compiex that carries a poor prognosis for survivai beyond chiidhood.
Erythema Elevatum Diutinum Associated with Celiac Disease: Response to a Gluten‐Free Diet
Authors: Rodriguez‐Serna Mercedes , Fortea Jose‐Miguel , Perez Amparo , Febrer Isabel , Ribes Carmen , Aliaga Adolfo
Published: March 2008

Abstract: An 11‐year‐old girl with a history of Insulin‐dependent diabe‐tes melitus had erythema elevatum diutinum (EED) associated with a ce‐liac disease related to a possible kidney disease. Dapsone did not im‐prove the skin manifestations. However, the tesions disappeared after a gluten‐free diet was begun. To our knowledge, this report describes the first case of EED In a patient with celiac disease.
Lichen Planus in Children: A Case Report
Authors: Cottoni F. , Ena P. , Tedde G. , Montesu M. A.
Published: March 2008

Abstract: Lichen planus (LP) Is rare in children. A review of the literature reveals that it has some peculiarities with respect to sex, localization, clinical aspect, race, and family history. We present an Indian child with the documented peculiarities of infantile LP. A comparison of LP and graft‐versus‐host disease points to the Importance of thymic Involution in the pathogenesis of the former, which could explain the rarity of this disorder In infants.
Case Report of Darier Disease Localized to the Vulva in a 5‐Year‐Old Girl
Authors: Salopek Thomas G. , Krol Alfons , Jimbow Kowichi
Published: March 2008

Abstract: A 5‐year‐old girl with Darier disease had numerous verrucous papules coalescing into plaques on both labia major a of approximately one year's duration. She was diagnosed as having genital warts, raising the suspicion of sexual abuse. Subsequently, a biopsy specimen from one of the vulvar lesions revealed histologic characteristics consistent with a diagnosis of Darier disease. The case is unusual for the age of onset and the site of initial involvement, and stresses the importance of including acantholytic disorders such as Darter disease in the differential diagnosis of a child with clinically verrucous lesions.
Neonatal Lupus Erythematosus and Maternal Lupus Erythematosus Mimicking HELLP Syndrome
Authors: Paton Sarah , Wiss Karen , Lyon Nancy , Baden Lynn , Gellis Stephen E.
Published: March 2008

Abstract: A 4‐month‐old boy had a faint, confluent, macular erythema involving the forehead, nose, and philtrum, and matllke telangiectases over the forehead and eyelids. The clinical features, course, and laboratory studies were characteristic of neonatal lupus erythematosus. He was otherwise healthy and the product of a preterm twin pregnancy complicated by the development of maternal HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome. The patient had elevated Ro(SSA) and La(SSB) antibody titers, and studies performed on maternal blood disclosed positive antinuclear antibodies and elevated Ro and La antibody titers.
Electron Microscopic Studies of Harlequin Fetuses
Authors: Hashimoto Ken , Dobbeleer G. De , Kanzaki Tamotsu
Published: March 2008

Abstract: Four cases of harlequin fetus of various estimated gastational ages (16, 20, 21, 24 weeks) were examined by light and electron microscopy. When the epidermis was keratinized the following features were commonly found: hyperkeratosis with or without granular cells; dilated hair follicles with plugged keratin; a large number of dense or particle‐cored granules In the upper malpighlan layer; absence of cementsomes (lamellar bodies); large vacuotes with peripherally located laminations; large mitochondria with vesicular or membranous cristae; and early formation of the marginal band in keratinocytea and abnormal formotion of the same in luminal cells of the acrosyringlum. A 16‐week specimen had no sign of karatinization, which made It difficult to detect these abnormalities; however, it did have large mitochondria in the keratlnocytes. The mucous membrane of the lip was thickened but not keratinized.
Ultrastructural Study of Two Patients With Both Piebaldism and Neurofibromatosis 1
Authors: Chang Tsuling , McGrae John D. , Hashimoto Ken
Published: March 2008

Abstract: Piebaldism was associated with neurofibromatosis 1 (NF‐1) in two patients, an association not previously reported. Dopa staining (tyroslnase) and electron microscopy were performed: no melanocytes or melanosomes were found in hypomelanotic skin of patient 2 and in the white forelock skin of patient 1; in patient 2, normal melanocytes and melanosomes were present in the white forelock epidermis but absent from the cortex, cuticles, and inner root sheath of the while forelock hair. Because these structures receive melanosornes from melanocytes in the hair bulb, it was assumed that there were no melanocytes in the hair matrix. Melanocytes and melanosornes were normsl by ultrastructural criteria and in terms of their distribution in a normally pig men ted macule within a hypomelanotic patch of patient 2. These and earlier report findings led to three conclusions: subtypes of piebaldism exist, Including our patients showing a combination of piebaldism and NF‐1; the most commonly reported subtype has no melanocytes in the white forelock and hypomelanotic skin, although microscopic islands of melanocytes may exist within hypomelanotic skin; and the infrastructure of white forelock skin and hair of patient 2 is consistent with a mouse model of piebaldism, in which the hair follicle has no active melanocytes, but the interfollicular epidermis is normally melanized.
Sporadic Cases of Heck Disease in Two Polish Girls: Association With Human Papillomavirus Type 13
Authors: Obalek Slavomir , Jaaniger Camila , Jablonska Stefania , Favre Michel , Orth Gerard
Published: March 2008

Abstract: Two sporadic cases of Heck disease in Polish girts were associated with human papillomavirus 13. No other children and nobody from their surroundings had similar lesions. The course of the disease was chronic in both patients. The lesions regressed spontaneously in one girl within several years after some of the papules were excised. The second girl's warts persist after eight years.
Trichoepitheliomatous Infiltration of the Skin Simulating Leprosy
Authors: de la Luz Orozco‐Covarrubias Ma. , Uribe‐Rea Consuelo , Tamayo‐Sánchez Lourdes , Durán‐McKinster Carola , Ruiz‐Maldonado Ramon
Published: March 2008

Abstract: A 13‐year‐old girl had a six‐year history of infiltrated erythematous plaques on the face, alopecia of the eyebrows, diffuse alopecia at the scalp, and absence of body hair. Histologicaily, the lesions on the face and body corresponded to trichoepitheliomas. The lesions on the face clinically simulated lepromatous leprosy. This case probably represents an entity not previously described.
Hemorrhagic Complications in a Rapidly Growing, Congenital Hemangiopericytoma
Authors: Resnick Steven D. , Lacey Stuart , Jones Gary
Published: March 2008

Abstract: A 3‐week‐old infant had a massive hemangioma‐like vascular neoplasm that had enlarged rapidly since being noted at birth. Less than a day after this initial evaluation the tumor underwent spontaneous ulceratlon and the infant had a near‐fatal hemorrhage. Histoiogically, the tumor was a spindle cell neoplasm most consistent with the diagnosis of congenital hemangiopericytoma. Local excision appears to have been curative after almost two years of follow‐up. The case is instructive in pointing out the importance of considering nonhemangioms vascular neoplasms in the evaluation of newborns with vascular tumors.
Congenital Hemangioma of Eccrine Sweat Glands
Authors: Rositto Alicia , Ranalletta Maria , Drut Ricardo
Published: March 2008

Abstract: We present six patients with congenital hemangioma of eccrine sweat glands. In every one the lesion was congenital, clinically angiomatous, painless, and nonsweating, with progressive involution over months. Histotogically all specimens showed many dilated capillaries with prominent endothelial cells associated with trie eccrine sweat gland coils.
An X‐Linked Reticulate Pigmentary Disorder With Systemic Manifestations: Report of a Second Family
Authors: Adès Lesley C. , Rogers Maureen , Sillence David O.
Published: March 2008

Abstract: Another family manifesting an X‐linked, reticulate, pigmentary dermatosis, previously familial cutaneous amyloidosis, has been observed. The disorder is characterized in males in this family by onset in the first year of recurrent episodes of respiratory illness including pneumonia, a progressive reticulate pigmentation of the skin, hypophidrosis, and photophobia. The absence of amyloid deposits in the skin in both the mother and sons confirms that less emphasis should be given to the word “amyloidosis” in naming the disorder.
Dyskeratosis Congenita or Chronic Graft‐versus‐Host Disease? A Diagnostic Dilemma in a Child Eight Years After Bone Marrow Transplantation for Aplastic Anemia
Authors: Ivker Rachel A. , Woosley John , Resnick Steven D.
Published: March 2008

Abstract: A 12‐year‐old boy had striking reticulate hyper pigmentation of the neck and upper chest, dystrophic nails, patchy alopecia, and a white streak on the buccai mucosa. He was diagnosed as having chronic graft‐versus‐host disease (GVHD) based on clinical findings, skin biopsy findings, and his history of a bone marrow transplantation for apiastic anemia eight years earlier. Dyskeratosis congenita (DC) was not a diagnostic consideration, although the clinical findings and history of aplastic anemia made it a compelling possibility. This case highlights the clinical similarities between DC and chronic GVHD and the difficulty in arriving at an unequivocal diagnosis.
Topical Lidocaine for Anesthesia in Patients Undergoing Pulsed Dye Laser Treatment for Vascular Malformations
Authors: Mallory Susan B. , Lehman Paul A. , Vanderpool Douglas R. , Franz Thomas J.
Published: March 2008

Abstract: Pulsed dye laser treatment of vascular malformations is moderately painful. Twenty‐five percent lidocaine in 70% dimethyl sulfoxide‐ethanol was used to achleve anesthesia in patients undergoing the procedure. Thirteen of 14 patients had some degree of anesthesia (average of 51%) ranging from 10% to 100%, with 8 of them experiencing good results (>50% anesthesia). Diminishment of pain was assessed by comparison of laser therapy on Lidocaine‐treated versus untreated sites within the vascular malformation using verbal and visual analog scales. In vitro permeation demonstrated good absorption of Lidocaine, peaking at one hour. Penneation of lidocaine was significantly greater then that observed from acid mantle cream or EMLA. Topical lidocaine at 25% concentration in dimethyl sulfoxide 70% was well tolerated and had no side effects except transient mild erythema.
Partial Improvement of Olmsted Syndrome With Etretinate
Authors: Ueda Masato , Nakagawa Koichi , Hayashi Kazuhiro , Shimizu Ryosuke , Ichihashi Masamitsu
Published: March 2008

Abstract: An 11‐year‐old Japanese boy with Olmsted syndrome was seen at our clinic. He had a sharply marglnated, painful keratoderma with a red border on his palms and soles. Flexion contractures of the fingers were also observed. Hyperkeratotic plaques were present below the lower lip, on the elbows and knees, and In the sacral area. Localized alopecia, leukokeratosis on the tongue, shortness of stature, and laxity of the large joints corresponded to the clinical features of Olmsted syndrome. Treatment with etretinate was effective for the palms and fingers, but resulted in no improvement of the keratoderma of the soles. Because of periosteal thickening of the tibia, presumably caused by etretinate, therapy was terminated one year after it began.
Association of Skin Wetness and pH With Diaper Dermatitis
Authors: Berg Ronald W. , Milligan Michael C. , Sarbaugh Frank C.
Published: March 2008

Abstract: The strength of the association between diaper dermatitis and measurements of skin wetness and skin pH was evaluated by statistical analysis of four diaper trials involving 1601 infants. Although the strength of the association between skin wetness and diaper dermatitis was greater than that between skin pH and diaper dermatitis, increases in wetness and pH were both significantly associated with elevated mean grades for diaper dermatitis. The skin environment least likely to be associated with diaper dermatitis is one In which Increases In both skin wetness and skin pH are minimized.
Multiple Pilomatricomas in Rubinstein‐Taybi Syndrome; A Case Report
Authors: Cambiaghi Stefano , Ermacora Elisa , Brusasco Alberto , Canzi Lia , Caputo Ruggero
Published: March 2008

Abstract: Rubinstein‐Taybl syndrome is a multisystem developmental disordar due to an autosomal dominant mutation. It Is clinically defined by the presence of peculiar facles, mental retardation, and bread thumbs and first toes. Important dermatologic findings include hirsutism, keiolds, hemanglomas, and dermatoglyphic abnormalities. We report a 12‐year‐old girl with the typical phenotype of Rubinsteln‐Taybi syndrome, associated wtth numerous ptlomatrloomas. These are benign epithelial neoplasms with hair eel differentiation that may have a familial transmission. Pilomatricomas have not been reported in patterns with Rubinstem‐Taybl syndrome, although their association with myotonlc dystrophy, another autosomal dominant disorder, is well known. Possibilities to explain the association Include contiguous gens syndrome, the action of a piefotropic gene, predisposition to malformations, and mere coincidence.
Diagnostic Features, Differential Diagnosis, and Treatment of Suhungual Osteochondroma
Authors: Schulze Keith E. , Hebert Adelaide A.
Published: March 2008

Abstract: Osteochondromas are among the most common cartilaginous neoplasms, but are rarely seen on the fingers and toes. We report a 10‐year‐old girl who had a dystrophic fingernail since Infancy that was identified as a subungual Osteochondroma radiologically and histopathologically. The clinical, radiographic, and histopathologic features, as well as the pertinent differential diagnoses and treatment options are discussed.
Nevoid Hypertrichosis: Multiple Patches Associated With Premature Graying of Lesional Hair
Authors: Rupert Linda S. , Bechtet Mark , Pellegrini Arthur
Published: March 2008

Abstract: A 23‐month‐old Caucasian girt had congenital circumscribed areas of deeply pigmented terminal hairs that gradually lost their pigment over the next two years. Physical examination revealed no other abnormalities. Histologlc examination demonstrated terminal hair in otherwise normal skin consistent with nevold hypertrichosis. The clinical and histologic characteristics of nevoid hypertrichosis are compared with other types of hypertrichotic patches.
Sequestrated Meningocele of the of Scalp:Diagnostic Value of Hair Anomalies
Authors: Khallouf R. , Fétissof F. , Machet M. C. , Stephanov E. , Lechrist J. , Lorette G.
Published: March 2008

Abstract: Clinical aspects of sequestrated meningocele can be varied, causing difficulties in histopathological diagnosis. The meningeal tissue is scanty and appears as nonspecific connective tissue, therefore it may be overlooked. One classical and two unusual clinical presentations of sequestrated meningocele are described: one of the latter presenting as two atrophic midline bald patches, and the other as a tiny occipital midline hair tuft. In the unusual cases, the pathologic diagnosis was made possible only with the combination of clinical, histologic, and immunohistochemical studies. Sequestrated meningocele should be included in the differential diagnosis whenever hair abnormalities are observed in neonates and young children. We believe that there is a causal relationship between anomalies of the central nervous system and the cutaneous adnexal malformations.
Epidermolysis Bullosa Simplex Associated With Muscular Dystrophy: A New Case
Authors: Patrizi Annallsa , Di Lernia Vito , Neri Iria , De Giorgi Lucilla Badiali , Mast Massimo
Published: March 2008

Abstract: We report an infant with a rare form of epidermolysis buliosa simplex characterized by an autosomal recessive pattern of inheritance, severe cutaneous involvement, oral and nail lesions, association with muscular dystrophy, and a poor prognosis, due to extracutaneous disease. In addition to the usual presentation of this disease, our patient had severe anemia, with immature circulating white cells, and bone marrow histology suggestive of a pre‐leukemic state, a finding which has not before been reported in the literature.
Disseminated Rhizopus Infection in a Premature Infant
Authors: Craig Nona M. , Lueder Fredrick L. , Pensler Jay M. , Bean Bonnie S. , Petrick Marcia L. , Thompson Richard B. , Eramo Lynne R.
Published: March 2008

Abstract: A 16‐day‐old premature male infant developed rapidly expanding necrotic facial plaques. Histology and cultures of tissue biopsy specimens revealed infection with Rhizopus arrhizius. The patient was treated aggressively with surgical debridement, amphotericin B, and rifampin, but had a progressive spread of the cutaneous infection and a fatal clinical course. He had gastrointestinal and pulmonary involvement on autopsy. Zygomycoses are rapidly progressive infections which generally affect immunocompromised hosts. Premature infants should be included as individuals at risk for disseminated zygomycotic disease.
Urticaria Pigmentosa: A Review of 67 Pediatric Cases
Authors: Azaña J. Manuel , Torrelo Antonio , Mediero Imelda G. , Zambrano Antonio
Published: March 2008

Abstract: Mastocytosis is a disorder of mast cell proliferation that may appear during infancy, childhood, or adulthood. We studied 67 consecutive patients (33 males, 34 females) with urticaria pigmentosa and assessed them fully to determine the presence of systemic involvement. Ages at onset of lesions ranged from birth to 11 years, with most developing in the first year of life. Pruritus was the primary symptom. Hematologic and serum chemistry profile, radiologic skeletal surveys, and bone marrow aspirations were performed. Slight anemia was present in three patients. Radiologic bone lesions were observed in eight. Bone marrow aspirates showed slight changes in six patients, with only an increased number of mast cells in an additional patient. The disease tended to resolve spontaneously. This prospective study emphasizes the benign nature of pediatric urticaria pigmentosa.
Linear IgA Bullous Dermatosis of Childhood with Autoantibodies to a 230 kDa Epidermal Antigen
Authors: Kanitakis Jean , Mauduit Gilles , Cozzani Emanuele , Badinand Pierre , Faure Michel , Claudy Alain
Published: March 2008

Abstract: Linear IgA bullous dermatosis (LABD) is an autoimmune, subepidermal disease defined on the basis of direct immunofluorescence findings. However, more recent techniques used to study bullous dermatoses suggest that LABD may be heterogeneous. A patient with LABD of childhood (chronic benign disease of childhood, CBDC) was studied by indirect immunofluorescence on salt‐split skin and by Western blot in an attempt to characterize the involved autoantigen. This young girl's periorificial (mouth, genitalia), erythematovesicular lesions were diagnosed initially as herpes simplex. Histologic examination revealed eosinophilic spongiosis, suggesting the diagnosis of an autoimmune blistering disease. Direct immunofluorescence showed an exclusive linear IgA deposit at the dermoepidermal junction. Indirect immunofluorescence revealed circulating IgA autoantibodies that reacted with the epidermal side of saltsplit skin; these reacted by Western blot with a 230 kDa epidermal antigen, as in bullous pemphigoid. This case, fulfilling the diagnostic clinical and direct immunofluorescence criteria for LABD/CBDC, seems to represent IgA bullous pemphigoid. It further underscores the nosologic heterogeneity of LABD, which probably includes, apart from bullous pemphigoid, epidermolysis bullosa acquisita and cicatricial pemphigoid.
Persistent Erythema Infectiosum‐Like Rash as a Prodrome of Acute Lymphocytic Leukemia
Authors: Lee Seung Min , Kim Dong Gun , Bang Dongsik
Published: March 2008

Abstract: A 7‐year‐old boy had erythema infectiosum with typical manifestations. over more than 40 days, these lesions showed no sign of regression, and chronic anemia became progressively more severe.Electron microscopic examination performed 20days after onset showed abnormal Sezary‐like lymphocytes. Bone marrow biopsy, which was performed to evaluate the anemia, was consistent with acute lymphocytic leukemia. Persistent parvovirus B 19 infection may be connected with immunosuppression. Therefore, early electron microscopic study and bone marrow biopsy may be helpful for early diagnosis of hematologic malignancies.