bluuebunny/crossref_metadata_2025 huggingface dataset:
Immediate‐ and Delayed‐Type Contact Hypersensitivity in Children Older than 5 Years With Atopic Dermatitis: A Pilot Study Comparing Different Tests
Authors: Oranje Arnold P. , Bruynzeel Derk P. , Stenveld Harma J. , Dieges Paul H.
Published: March 2008

Abstract: We conducted a prospective open study of immediate‐ and delayed‐type contact hypersensitivity to food and other allergens in 33 children with atoplc dermatitis (AD). The design of the study was exploratory and not randomized. Various methods for detecting immediate‐type hypersensitivity were compared. Thirty‐three children age 5 to 15 years with persistent AD were initially enrolled, but 3 dropped out. Nine patients had positive reactions to foods in the patch‐scratch tests, four had positive reactions in the skin application food test, and five had positive reactions to foods in the prick tests. Positive reactions to foods were observed in only three patients on the delayed‐type patch tests. In all tests, but especially the patch‐scratch and prick tests, positive reactions to food allergens were observed without clinically related symptoms. None of these tests gave ideal results. Twenty (67%) of the 30 children had positive reactions to inhalants in prick testing. Fourteen showed positive patch‐test reactions with the European standard series (True Test). The most positive reactions were to nickel (9 patients), cobalt, and balsam of Peru. Restrictive measures led to evident improvement of AD only in some children. The results of this study illustrate that food allergy plays only a limited rote in patients with AD age 5 to 15 years. We could not conclude which of the tests would predict which children might benefit from dietary manipulation.
Neurologic Disease in a Child With Hepatoerythropoietic Porphyria
Authors: Parsons Jessica L. , Sahn Eleanor E. , Holden Kenton R. , Pai G. Shashidhar
Published: March 2008

Abstract: Hepatoerythropoietic porphyria (HEP) is a rare, autosomal recessive disorder due to deficient uroporphyrinogen decarboxylase enzyme activity. Patients exhibit photosensitivity, red urine, hypertrichosis, and characteristic serum and urine porphyrin profiles. Two siblings had the classic clinical and biochemical findings of HEP. The older patient developed a left‐sided hemiparesis accompanied by an abnormal brain magnetic resonance imaging study. Although central nervous system abnormalities are a common feature of other hepatic porphyrias, they have not been previously documented in association with HEP.
Proteus Syndrome
Authors: Darmstadt Gary L. , Lane Alfred T.
Published: March 2008

Abstract: A 10‐month‐old girl had macrodactyly, facial and extremity hemihypertrophy, plantar cerebriform hyperpiasia, a subcutaneous mass on the back, macrocephaly, and lumbar kyphosis. These findings were diagnostic of Proteus syndrome. The clinical features, etiology, management, and points of differential diagnosis are discussed.
Cutaneous Mucinosis of Infancy
Authors: Stokes Kathleen S. , Rabinowitz Linda G. , Segura Annette D. , Esterly Nancy B.
Published: March 2008

Abstract: Cutaneous mucinosis of infancy has been reported only rarely in the literature. We describe a case occurring in a black infant giri. 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.
An Uniisual Case of Infantile Dermal Mucinosis
Authors: Calza A.‐M. , Masouye I. , Saurat J.‐H.
Published: March 2008

Abstract: Dermal mucinosis occurred in a 3‐month‐old and persisted for six years. The features suggest is represents a novel type of childhood mucinosis.
Perforating Milia‐like Idiopathic Calcinosis Cutis and Periorbital Syringomas in a Girl With Down Syndrome
Authors: Schepis Carmelo , Siragusa Maddalena , Palazzo Rosaria , Batolo Dario , Romano Corrado
Published: March 2008

Abstract: An 11‐year‐old girl with Down syndrome had whitish, milia‐like lesions on the acral areas of the limbs, and periorbital syringomas. Calcium deposits were the histologic counterparts of the milia‐like lesions. This is the first European report of this feature in Down syndrome.
Methotrexate in Childhood Psoriasis
Authors: Kumar Bhushan , Dhar Sandipan , Handa Sanjeev , Kaur Inderjeet
Published: March 2008

Abstract: We treated childhood psoriasis with methotrexate (MTX) in seven children (4 boys, 3 girls) over 7.5 years. Their ages and duration of disease varied from 3.5 to 16 years (mean 12.14 yrs) and 4.8 months to 5 years (mean 2.2 yrs), respectively. Psoriatic erythroderma was seen in three patients, generalized pustular psoriasis in two, recalcitrant psoriasis and psoriatic arthropathy in one each. Pre‐MTX liver biopsy performed in 4 children showed grade I changes. Methotrexate was given in a single weekly oral dose of 3.75 to 25 mg (mean 16.6 mg). The duration of treatment necessary to control the disease varied from 6 to 10 weeks (mean 7.9 wks). Total duration of MTX therapy was 31.2 to 46.4 weeks (mean 38.8 wks). Posttherapy disease‐free interval ranged between 14.4 and 16.8 weeks (mean 15.5 wks). Follow‐up after withdrawal of MTX was 16 to 28 weeks (mean 22.3 wks). Total cumulative MTX dose ranged from 390 to 960 mg (mean 683.6 mg). Side effects were nausea and vomiting in three patients.
Childhood Acquired Epidermolysis Bullosa
Authors: Lacour Jean‐Philippe , Bernard Philippe , Rostain Gilles , Baechler‐Sadoul Elisabeth , Pisani Anne , Ortonne Jean‐Paul
Published: March 2008

Abstract: We present the ninth case of epidermolysis bullosa acquisita (EBA) reported in children. As in most of the other childhood cases, the 7‐year‐old boy described herein had an acute, widespread, inflammatory vesiculobuilous eruption with oral involvement. Indirect immunofluorescence on salt‐split skin as well as Western imrnunobtot confirmed the diagnosis of EBA. The patient responded to combined prednisone and dap‐sone, and was maintained with dapsone alone.
Erythrokeratoderma Variabilis: Case Report and Review of the Literature
Authors: Knipe Ronald C. , Flowers Franklin P. , Johnson Franklin R. , DeBusk Franklin L. , Ramos‐Caro Francisco A.
Published: March 2008

Abstract: A 5‐month‐old boy with erythrokeratoderma variabilis is presented. The parents noted that the asymptomatic erythematous plaques changed over the course of hours to days, Topical therapy with retinoic acid proved ineffectlve. A brief review of the literature is presented.
Apocrine Chromhidrosis Involving the Areolae in a 15‐ear‐Old Amateur Figure Skater
Authors: Saff Deborah M. , Owens Ruth , Kahn Teri A.
Published: March 2008

Abstract: Apocrine chromhidrosis is a rare disease frequently localized to the face or axillae, and rarely has been reported to occur around the breasts. We report a 15‐year‐old amateur figure skater who displayed localized chromhidrosis around her areolae. The most common precipitating event was exercise, She was treated topically with capsaïcin cream 0.025% with a subsequent decrease in symptomatology.
CHILD Syndrome: Analysis of Abnormal Keratinization and Ultrastructure
Authors: Hashimoto Ken , Topper Shari , Sharata Harry , Edwards Mohammad
Published: March 2008

Abstract: A new patient with CHILD syndrome (congenital hemidysplasia, ichthyosiform erythroderma, and limb defects), the thirtieth in the literature, was observed for over three years. Initially, the right‐sided lesion spared the breast area. At 10 months of age the trunk lesion extended to cover the entire area of the right chest At age 20 months the patient developed linear, bandlike, keratotic, brown‐black lesions on her teft thigh that subsided within six weeks, leaving a slight hyperpigmentation. This patient was studied by routine histologic methods as well as with markers of keratinlzation and electron microscopy. In hematoxylin and eosinstained sections, parakeratosis and orthokeratosis atternated. In some parakeratotlc areas, targe granular cells, and in others, ghost granular cells, were present. The latter showed basophilic cytoplasm, and palestaining or vacuolated nucleus and were seen either above the normal granular layer or without it. Although regional variations existed, basat cell‐type keratins as recognized by AE1 continued to be expressed in suprabasal layers. Filaggrin‐ and Involucrin‐positive layers were expanded, particularly the latter, down to the lower prickle cell layer. Ultrastructurally, numerous lamellar or membranous structures were found in upper layers of the epidermis, both intracellulary and intercellularly. Normal cementsomes coexisted with these abnormal lamellar structures, and it was thought that the latter represent modified cementsomes because the discharge of those from the cell periphery was Often detected.
Bilateral Pseudocyst of the Auricle in an Infant Girl
Authors: Santos Anthony D. , Kelley Peggy E.
Published: March 2008

Abstract: A 6‐week‐old girl developed bilateral auricular swelling in the absence of trauma. Clinical and histologic findings were consistent with pseudocyst of the auricle. This case is unusual given the age and sex of the patient and the bilateral presentation of the pseudocysts.
Phacomatosis Pigmentovascularis with a Selective IgA Deficiency
Authors: de Luna Margarita Larralde , Barquin Maria Antonia , Casas Jose Gabriel , Sidelsky Susana
Published: March 2008

Abstract: We report a 1‐year‐old boy with an extensive cutaneous vascular malformation, oculocutaneous pigmentation, and severe neurologic abnormalities from birth, as well as a selective lgA deficiency. Ultrastructural study demonstrated prominent endothelial cells in the lumina of the blood vessels. The diagnosis of phacomatosis pigmentovascularis type llb seemed appropriate for this patient.
Rothmund‐Thomson Syndrome and Addison Disease
Authors: Lapunzina Pablo , Fonseca Eduardo , Gracia Ricardo , Delicado Alicia
Published: March 2008

Abstract: We report an 18‐year‐old man with the unusual combination of Rothmund‐Thomson syndrome (RTS) and Addison disease. He was admitted when he was 26 months old because ot short stature, dehydration, metabolic acidosis, hyperpigmentation, and typical skin lesions. Because his growth remained delayed, at age 10 years he was given a trial of recombinant growth hormone. After six years of treatment no improvement in height, bone, or sexual maturation was observed. Hits fact may be related to a defect in connective tissue metabolism. Chromosomal analysis of peripheral blood lymphocytes revealed increased numbers of breaks and gaps. Fibroblasts cultured from affected skin did not grow. Patients with RTS are prone to developing cancer, but no malignant disease was found in our patient Early diagnosis and treatment of both endocrinologic and malignant complications are essential for survival of patients with this rare syndrome.
Gastric Outlet Obstruction and Gastric Infarct in Junctional Epidermolysis Bullosa
Authors: Zirn Jonathan R. , Scott Rachelle A. , Aronian John M. , Lin Andrew N.
Published: March 2008

Abstract: A newborn boy had functional epidermolysis bullosa, duodenal obstruction, and gastric Infarction, a newly described combination that extends the spectrum of gastrointestinal findings associated with Junctional epidermolysis bullosa. The infant underwent feeding jejunostomy, but died 16 days after birth. This report emphasizes the need for gastrointestinal assessment in any neonate suspected of having epldermotysls bullosa, especially if the pregnancy was complicated by polyhydramnios.
Infantile Acropustulosis in Six Immigrant Children
Authors: Humeau Sophie , Bureau Brigitte , Litoux Pierre , Stalder Jean‐François
Published: March 2008

Abstract: Infantile acropustulosis is a recurrent, pruriginous, vesiculopustular eruption of the palms and soles first described in 1979. We report six cases of infantile acropustulosis in recently emigrated children treated for scabies. Clinical follow‐up was obtained by questionnaire addressed to patients'families and general practitioners. Our study suggests infantile acropustulosis is frequent in immigrant infants and could be a non‐specific hypersensitivity reaction to Sarcoptes scablel.
Painful Fingers, Heat Intolerance, and Telangiectases of the Ear: Easily Ignored Childhood Signs of Fabry Disease
Authors: Shelley E. Dorinda , Sheelly Walter B. , Kurczynski Thaddeus W.
Published: March 2008

Abstract: Generalized anhldrosls with heat collapse, painful fingers, and angiokeratomas were the presenting signs in a 23‐year‐old man who was shown by enzyme studies and electron microscopy to have Fabry disease. His 6‐year‐old brother had the early diagnostic findings of episodic painful fingers, heat intolerance, and retroaurlcular telangiectasia. Both had an absence of α‐galactosidase, Over 6 years of observation, the older brother developed progressive renal failure and the younger one developed acrodynla and anhidrosls. Their mother had dlminished α‐galactosidase activity and several anglomatous papules on one breast A review of Fabry disease emphasizes the need for skin Inspection for anglomas and telanglectasia, and enzyme assay in patlents with lnexplicable complaints or lindings. Carrier females are most easily recognized by the presence of unique comeal opacities.
Infantile Acute Hemorrhagic Edema: A Variant of Leukocytoclastic Vasculitis
Authors: Ince Erdal , Mumcu Yelda , Suskan Emine , Yalcinkaya Fatos , Tümer Necmiye , Cin Sükrü
Published: March 2008

Abstract: Infantile acute hemorrhagic edema (IAHE) is a leukocytoclastic vasculitis that is contined to the skin without visceral Involvement. Edema and purpuric lesions characterize the disease. The disorder has a dramatic onset, with a short, benign course and spontaneous resolution within several weeks. The clinical similarities between IAHE and Henoch‐Shünlein purpura have been discussed in the literature. We report three Infants with IAHE and discuss the clinical, laboratory, and histopathologic features of the disease. We suggest that it should be regarded as a separate entity for appropriate diagnostic investigations and therapy.
Extensive Symmetric Truncal Aplasia Cutis Congenita without Fetus Papyraceus or Macroscopic Evidence of Placental Abnormalities
Authors: Boente Maria del C. , Frontini Maria del V. , Acosta Maria I. , Saleme César , Barrionuevo Silvia , Asial Raül
Published: March 2008

Abstract: Aplasia cutis congenita is a rare disorder characterized by localized absence of skin at birth. Type V in Frieden's classification, which is associated with fetus papyraceous or placental infarcts, occurs as a large cutaneous defect on the trunk and extremities. The patient we report had a lesion affecting the trunk and extremities symmetrically, with no family history of the disorder or chromosomal abnormalities. In our opinion, despite the absence of fetus papyraceous or placental infarct, this patient's condition can be classified as type V.
Cutaneous Histoplasmosis in a Child with Hyper‐IgM
Authors: Yilmaz Gülsün Gülay , Yilmaz Ertan , Coşkun Mesut , Karpuzoǧlu Gülten , Gelen Tekinalp , Yeǧin Olcay
Published: March 2008

Abstract: Immunodeficiency with hyperlmmunoglobullnemia M is a rare disease characterized by very low levels of IgG and IgA and normal or high levels of serum IgM and IgD. Recurrent and severe systemic infections with pathogenic bacteria are frequent if immunoglobulin replacement therapy is not given. Histoplasmosis is a systemic granulomatous mycosis due to Histoplasma capsutatum and characterized by a particular affinlty for the reticuloendothellal system. Glabrous skin involvement in histoplasmosis is highly unusual except in patients with advanced human Immunodeficiency viral disease. Cutaneous histoplasmosis and granulomatous reaction were diagnosed in a 5‐year‐old boy with hype‐IgM disease. The lesion Improved after oral ketoconazole therapy. To our knowledge, this is the first case of cutaneous histoplasmosis associated with hype‐IgM to be reported.
Epidermolysis Bullosa Junctionalis Associated with Urinary Bladder Exstrophy: A Case Report
Authors: Moretti G. , Mazzaglia E. , D'Anieri A. , Merlino V. , Magaudda L. , Mondello M.R. , Santoro G. , Vaccaro M. , Albanese A.
Published: March 2008

Abstract: We report the second infant of nonconsanguineous parents with epidermolysis bullosa junctionalls associated with urinary bladder extrophy, epispadias, anterlorized anus, and bilateral inguinal hernias. The family history also included the death of a maternal cousin due to epidermolysis bullosa. Our diagnosis was based on electron microscopy and immunofluorescence evidence. This patient is reported because of the rarity of this constallation of findings.
Congenital Glomangioma: Case Report and Review of the World Literature
Authors: Glick Sharon A. , Markstein Ellen A. , Herreid Peter
Published: March 2008

Abstract: Glomangiomas, or multiple glomus tumors, occur in disseminated, localized, or congenital plaquelike forms. The first two cases of congenital plaquelike glomangioma were described in 1990. We report a 9‐year‐old girl with a congenital, violaceous, 75‐cm2 indurated plaque of the left abdomen that showed the classic histologic findings of glomangioma. In our review of the world literature, we found 11 additional, welldocumented cases of glomangioma present at birth. Ten of these patients had violaceous indurated plaques, and the other two had clusters of discrete nodules. The majority of lesions were painless and enlarged with body growth. Many patients developed satellite lesions at sites distant from the original glomangiomas later in life. Family history of glomangioma was positive in 4 of the 12 patients.
Unilateral Beau's Lines in Childhood Reflex Sympathetic Dystrophy
Authors: O'Toole Edel A. , Gormally Siobhan , Drumm Brendan , Monaghan Hugh , Watson Rosemarie
Published: March 2008

Abstract: Reflex sympathetic dystrophy is characterized by severe pain and autonomic dysfunction in a limb, usually after an injury. We describe a patient with childhood reflex sympathetic dystrophy with unilateral Beau's lines on the nalls of the affected hand. Unilateral Beau's lines have not been described previously In this condition to our knowledge, and we discuss their possible pathogenesis.
Extramediastinal Bronchogenic Cysts in Children
Authors: Jona Juda Z.
Published: March 2008

Abstract: Three children with subcutaneous masses were found to have extramediastinal bronchogenic cysts. The suprasternal area is by far the most common location of this highly unusual embryonic rest cyst. In one of our patients a cyst was located in the scapular region, which is a particularly rare site. To date only 41 subcutaneous bronchogenic cysts have been reported. Preoperative diagnosis is difficult, although the presence of clear mucoid material within the cyst may suggest the diagnosis. The histologic presence of ciliated, pseudostratified, columnar or cuboidal epithelium, mucus‐secreting glands, smooth muscles, and occasional cartilage establishes the diagnosis.
Subepidermal Calcified Nodule in Children: a Clinicopathologic Study of 21 Cases
Authors: Evans M. J. , Blessing K. , Gray E. S.
Published: March 2008

Abstract: Twenty‐one cases of subepidermal calcific nodule are presented. These lesions occur twice as commonly in males compared to females, with the head (particularly the ear), and neck as favored locations. No case was identified correctly clinically and only 1 case had a history of trauma, which appeared unrelated to the lesion. Histologically, there was a variety of appearances, with two‐thirds of specimens exhibiting a warty architecture. Younger lesions were composed of large, amorphous, calcific dermal deposits and were frequently associated with epidermal ulceration. The older lesions were not ulcerated and the calcium deposits were in small spherules. There was no evidence of pilomatrixoma or pre‐existing nevus. It is hypothesized that subepidermal calcified nodule represents dystrophic calcification secondary to dermal injury‐several in our series showed the architectural changes of verruca vulgaris.
Acanthosis Nigricans: A Marker for Hyperinsulinemia
Authors: Brockow Knut , Steinkraus Volker , Rinninger Franz , Abeck Dietrich , Ring Johannes
Published: March 2008

Abstract: A 12‐year‐old girl developed acanthosis nigricans due to increased levels of serum insulin. Hyperinsuiinemia has been recognized as the underlying cause in most cases of acanthosis nigricans. In this paper we outline a typical clinical case with special regard to the proposed pathogenesis.
Multiple Familial Pilomatricomas: A Cutaneous Marker for Gardner Syndrome?
Authors: Pujol Ramon M. , Casanova Josep M. , Egido Ramon , Pujol Josep , de Moragas Jose M.
Published: March 2008

Abstract: A 40‐year‐old man and his 6‐year‐old only son had numerous, firm papulonodular lesions on their faces. Their medical histories were unremarkable and no family consanguinity was recorded. Surgical excision of several lesions was performed on each patient. All the lesions were solid tumors with the characteristic histopathologic features of pilomatricoma. A gastrointestinal radiologic and fibroscopic survey disclosed numerous adenomatous colonic polyps in the father. Additional studies revealed that he also had minor dental abnormalities, a small osteoma on the right mandible, and unilateral, ocular, pigmented retinal macules. The diagnosis of multiple adenomatous colonic polyposis was established only after the well‐known association of pilomatricoma‐like changes in epidermal cysts in patients with Gardner syndrome was considered. Possibly, multiple familial pilomatricomas could be considered a cutaneous marker of Gardner syndrome.
Cutis Marmorata Telangiectatica Congenita Associated with a Double Aortic Arch
Authors: O'Toole Edel A. , Deasy Patrick , Watson Rosemarie
Published: March 2008

Abstract: We report a patient with cutis marmorata telangiectatica with the hitherto unreported anomaly of a double aortic arch. The presence of two major vascular anomalies in this patient may be secondary to a developmental defect of the mesoderm during embryogenesis.
Congenital Cutaneous Candidiasis: Case Report and Review
Authors: Raval D. S. , Barton L. L. , Hansen R. C. , Kling P. J.
Published: March 2008

Abstract: This report describes a preterm infant with congenital cutaneous candidiasis associated with paronychia, dystrophy of the nail plates, and marked, transient leukocytosis.
Cutaneous Congenital Candidiasis: A Case Report
Authors: Gibney Mary Davis , Siegfried Elaine C.
Published: March 2008

Abstract: Candida albicans is a frequent pathogen of the female genital tract, especially during pregnancy. Congenital candidiasis can occur as cutaneous or disseminated infection. We report a case of congenital cutaneous candidiasis, which may occur more frequently than is indicated by the literature. This is followed by a discussion of the pathogenesis. clinical presentation, diagnosis, and treatment of this infection.
Zunich Neuroectodermal Syndrome: Migratory Ichthyosiform Dermatosis, Colobomas, and Other Abnormalities
Authors: Tinschert Sigrid , Anton‐Lamprecht Ingrun , Albrecht‐Nebe Helga , Audring Heike
Published: March 2008

Abstract: We report a 21‐month‐old girl with symptoms consistent with the Zunich neuroectodermal syndrome, an apparently rare condition first described in 1983, Common features of all previously reported patients as well as in this child are characteristic craniofacial dysmorphism, bilateral colobomas of the retina, sparse and fine hair, hearing loss, Ichthyosiform erythroderma, mental retardation, ear anomalies, brachydactyly, and broad second toes. Light microscopic and uttrastructural investigations of the affected skin showed characteristic but nonspecific changes. The structural hair shaft abnormalities as well as the dysplastic nails in our patient have not been described before and are consistent with the previous assumption of an ectodermal dysplasia syndrome.
Cutaneous Tuberculosis in a Child: Case Report and Review
Authors: Paul Maria A. , Williford Phillip M.
Published: March 2008

Abstract: We present a 5‐month‐old Vietnamese boy who had several erythematous nodules and localized adenopathy that on biopsy revealed Mycabacterium tuberculosis. Complete systemic evaluation failed to reveal any abnormality. Three months after initiation of antituberculosis therapy, there was marked improvement of his existing lesions and no new lesions. This form, as well as the other forms of cutaneous tuberculosis, are discussed.
Phototherapy for Children
Authors: Atherton David J. , Cohen Bernard L. , Knobier Elizabeth , Garzon Maria , Morelli Joseph G. , Tay Yong‐Kwang , Weston William L. , Taieb Alain , Morison Warwick L. , Rasmussen James E.
Published: March 2008

Many dermatologists are uncomfortable with the prospect of administering phototherapy to children. We asked several physicians who prescribe phototherapy for the pediatric age group to discuss their indications, choice of modalities, and modus operandi.
Erythema Nodosum in Children: A Study of 27 Patients
Authors: Labbé Laurence , Perel Yves , Maleville Jean , Taïeb Alain
Published: March 2008

Abstract: Erythema nodosum (EN) seems to occur in children more rarely than in adults. It still remains the most frequent acute panniculitis, for which the diagnosis is almost always clinical. In a retrospective study of 27 pediatric patients, we have attempted to clarify the clinical spectrum and prognosis of this disease and discuss the differential diagnosis of nodular eruptions on the lower limbs of children. In almost half the patients of our series, the cause of EN remained undetermined. Streptococ‐cal infections (usually of the pharynx) were the most common cause of EN in children (22% of patients in our series), followed by Yersinia infection in about 15% of patients. Tuberculosis, an important cause in the past, was never found, but must always be excluded. A benign course was noted in all patients. Erythema nodosum is easily recognized clinically, but other subcutaneous lesions, especially nodular vasculitis and Schönlein‐Henoch purpura, have to be excluded by pathologic study, in cases of atypical presentation or long duration.
Disseminated Cutaneous Eruption after BCG Vaccination
Authors: Rositto Alicia , Molinaro Liliana , Larralde Margarita , Ranalletta Maria , Drut Ricardo
Published: March 2008

Abstract: We present nine infants (3 to 10 months of age) with numerous small, papular, papular‐lichenoid, and papulo‐pustular lesions predominantly on the upper and lower limbs associated with local (axillary) lymphadenopathy which appeared after BCG vaccination. Histopathology of the lesions showed small tuberculoid granulomas mainly in the papillary dermis. The presence of BCG bacillus was demonstrated in five out of seven samples from the lymph nodes after culture and in one skin biopsy specimen. All cases, whether treated or not, evolved to complete resolution of the skin lesions. We believe that this peculiar association results from hematogenous spread of the bacillus, which regresses after an adequate immune system reaction.
Tegumentary Leishmaniasis in Childhood
Authors: Bittencourt Achiléa L. , Barral Aldina , Costa Jackson M. L.
Published: March 2008

Abstract: Very little has been published about tegumentary leishmani‐asis in children and there are many controversies about this disorder in the literature. Therefore, we discuss the pathogenesis, clinical aspects, means to diagnosis, and treatment of this endemic disease.
Partial Lipodystrophy Associated with Juvenile Dermatomyositis: Report of Two Cases
Authors: Quecedo Esther , Febrer Isabel , Serrano Gabriel , Martinez‐Aparicio Antonio , Aliaga Adolfo
Published: March 2008

Abstract: A 27‐year‐old woman and a 13‐year‐old girl diagnosed with juvenile dermatomyositis in childhood developed clinical findings of partial lipodystrophy 10 years after diagnosis. Exhaustive clinical and laboratory examinations showed an association with other abnormalities: hy‐pertrichosis, steatohepatitis, and an abnormal insulin response to the glucose loading test in the first patient. Hypertrichosis, steatohepatitis, insulin‐resistant diabetes mellitus, and acanthosis nigricans were observed in the second patient. Renal function was normal in both patients. Although a localized form of lipodystrophy has been reported associated with connective tissue disease (connective tissue lipoatrophy), the partial form has been infrequently described in association with juvenile dermatomyositis.
Successful Treatment of Onychomycosis with Fluconazole in Two Patients with Hyperimmunoglobulin E Syndrome
Authors: Aihara Yukoh , Mori Masaaki , Yokota Shumpei
Published: March 2008

Abstract: We report two patients with hyperimmunoglobulin E syndrome who had fingernail onychomycosis and were successfully treated with fluconazole. Relapse of oral candidiasis, but not of onychomycosis, was observed within three months of stopping fluconazole. There were no adverse effects from this therapy. Based on our experience, we think that fluconazole may be useful in the treatment of onychomycosis in patients with hyper‐lgE syndrome as well as in those with other primary immunodeficiency disorders.
Lymphomatoid Papulosis: Successful Weekly Pulse Superpotent Topical Corticosteroid Therapy in Three Pediatric Patients
Authors: Paul Maria A. , Krowchuk Daniel P. , Hitchcock Michael G. , Jorizzo Joseph L.
Published: March 2008

Abstract: Lymphomatoid papulosis is a T‐cell proliferation that occurs primarily in adults but has been well described in children. Lesions may regress spontaneously but often leave residual scarring and, as a result, intervention frequently is considered. Therapeutic modalities commonly employed for adults with lymphomatoid papulosis may be poorly tolerated by pediatric patients. We present a series of three children with lymphomatoid papulosis treated with superpotent topical corticosteroids (halobetasol or clobetasol propionate). When applied twice daily for 2 to 3 weeks followed by weekly pulsed application, this treatment resulted in complete resolution of nearly all cutaneous lesions. Three ulcerated lesions, occurring in two patients, required adjuvant therapy with intrale‐sional triamcinolone. To date one patient remains free of cutaneous disease and two children experience occasional new lesions that respond to renewed treatment with topical clobetasol propionate. None of the children have evidence of systemic disease. We conclude that pulsed application of a superpotent topical corticosteroid is efficacious and safe in the management of cutaneous lesions of lymphomatoid papulosis and avoids the risks often associated with more aggressive interventions. Since these agents do not alter the risk of subsequent malignancy, careful ongoing surveillance of children with lymphomatoid papulosis is imperative.
Usefulness of Histopathologic Examination of Thick Scales in the Diagnosis of X‐Linked Dominant Chondrodysplasia Punctata (Happle)
Authors: Yanagihara Makoto , Ueda Keiichi , Asano Naomi , Ozawa Takeshi , Nakatani Akemi , Hirose Michiyo
Published: March 2008

Most patients with X‐linked dominant chondrodysplasia punctata (X‐DCDP) have whorled, thick, or spiky adherent hyperkeratosis on their whole body. Histopathologically, there are large keratotlc plugs of hair follicles where calcium deposits. This is the characteristic finding in this disease. We performed histopathologic examination of skin specimens from two newborn babies with X‐DCDP. The desquamated thick scales from the hyperkeratotic lesions had calcium in the center of the keratotic plugs. Histopathologic examination of thick scales is easy and helpful in diagnosing X‐DCDP.
Frequency and Significance of Minor Clinical Features in Various Age‐Related Subgroups of Atopic Dermatitis in Children
Authors: Nagaraja  , Kanwar Amrinder J. , Dhar Santlipan , Singh Surjit
Published: March 2008

The frequency and significance of minor clinical features for the diagnosis of atopic dermatitis (AD) were evaluated in different age‐related subgroups of 100 children (up to 12 yrs) with AD and an equal number of controls. Additional features studied were infraauricular fissuring, diffuse scaling of the scalp, and Hertoghe sign. Features found to be nonspecific were ichthyosis, nipple eczema, cheilitis, keratoconus, anterior subcapsular cataract, pityriasis alba, anterior neck folds, and food intolerance. Other minor features were of variable significance in different age groups. Seasonal variation of AD was observed in 44% of patients, with worsening during winter in 29% and in summer in 15%. Of the additional features, diffuse scaling of the scalp and infraauricular fissuring were significant, but Hertoghe sign was not. It was therefore concluded that minor clinical features for the diagnosis of AD need modification, with deletion of some of the present features and addition of newer ones.
Phakomatosis Pigmentovascularis
Authors: Van Gysel Dirk , Oranje Arnold P. , Stroink Hans , Simonsz Huibert J.
Published: March 2008

We report a patient with phakomatosis pigmentovascularis lib and numerous iris hamartomas. Phakomatosis pigmentovascularis lib is characterized by the simultaneous occurrence of a nevus flammeus, a mongolian spot, and sometimes a nevus anemicus in the same individual, with systemic involvement. To our knowledge, the association with multiple iris hamartomas has been reported only once. This second patient suggests that the association might be more common. Additional reports will indicate if such an association is more frequent than is now assumed.
Juvenile Generalized Pustular Psoriasis
Authors: Kalla G. , Goyal Archana M.
Published: March 2008

A 4‐year‐old boy had a generalized, confluent, pustular eruption on an erythematous base of three weeks’ duration. He was febrile and toxic. He had had a similar problem at age 5 months that lasted for about four weeks. The child was initially treated conservatively, but later developed erythroderma and became increasingly toxic. This necessitated administration of oral methotrexate 2.5 mg/day at weekly intervals. After two pulses there was marked improvement in his psoriasis.
An Unusual Presentation of Incontinentia Pigmenti in a 4‐Month‐Old Girl
Authors: Pearlman Joel , Griego Robert D. , Levy Moise L. , Friedman Jeffrey
Published: March 2008

A four month old girl initially presented with a crusted nodule on her right index finger. Evaluation was unremarkable for infectious causes or malignancy. She ultimately developed similar hyperkeratotic plaques and papules of the right hand and ultimately linear hyperpig‐mented patches of her arms and legs. The diagnosis of Incontinentia Pigmenti (IP) was then made. The diagnosis of IP and the differential diagnosis of IP‐like lesions is presented.
Topical Treatment of Skin Ulcers in Prolidase Deficiency
Authors: Jemec Gregor B. E. , Moe April T. T.
Published: March 2008

Prolidase deficiency is a hereditary enzyme deficiency characterized dermatologically by chronic recurrent ulcers and scarring due to increased skin fragility. It has been speculated that the enzyme deficiency causes a relative deficiency of proline in the wounds of these patients and negatively affects clinical healing. Two ulcers in a 17‐year‐old girl with established prolidase deficiency were treated for 12 weeks with ointments containing amino acids in an open study comparing the effects of 5% proline and a combination of 5% proline plus 5% glycine. Both ointments caused significant reduction of the ulcer size (p < 0.02), but the 5% proline‐5% glycine mixture caused a more rapid reduction (0.01 < p < 0.02). The results confirm previous findings in this rare inborn error of metabolism.
Severe Congenital Generalized Exfoliative Erythroderma in Newborns and Infants: A Possible Sign of Netherton Syndrome
Authors: Hausser Ingrid , Anton‐Lamprecht Ingrun
Published: March 2008

Abstract: We examined skin biopsy specimens from 17 of 19 newborns or infants with generalized ichthyosiform, exfoliative, seborrheic, or psoriasiform erythroderma. The specimens showed similar characteristic but nonspecific and therefore, at first sight, uninformative histologic features. Morphologically, the skin was affected overall with a persistent outbreak of eczema‐like eruptions of subacute or chronic dermatitis. Pronounced dermal inflammatory processes were obvious by their perivascular and interstitial presence as well as exocytosis of lymphocytes, macrophages, and neutrophils. Epidermal barrier function was impaired by the highly suppressed terminal differentiation, with thin or in part completely absent stratum corneum, decrease of keratin filaments, decrease or lack of kera‐tohyalin granules, and of keratinosomes containing stacks of lipid membranes. As a result, the formation and discharge of epidermal barrier lipids from the keratinosomes that normally provide intercellular lamellar sheets at the granular‐horny layer interface contributing to the epidermal barrier, was highly disturbed. The concomitant loss of water, electrolytes, and proteins by fluid exudation caused the patients severe metabolic problems and recurrent infections. The suspicion of Netherton syndrome was eventually confirmed in 18 patients by light microscopic demonstration of bamboo hairs (trichorrhexis invaginata), mostly from the scalp, but also in velius hairs and eyelashes. Atopy actually belongs to the symptom triad defining Netherton syndrome and is, in our opinion, primarily responsible for the pathologic events within the skin and of the keratinizing parts of the growing hair shafts. Differential expression of the atopic condition determines the appearance of the keratinization disorder of the skin, namely, severe, generalized, exfoliative erythroderma or milder forms of ichthyosis linearis circumflexa Comèl. Retinoid treatment seems to be contraindicated in these conditions since their biopharmacologic effects involve suppression of terminal differentiation, which is the proper pathognomonic event. In six patients the condition had a fatal course within months because of hypernatremia, recurrent infections, failure to thrive, and sepsis. Our aim is to call attention to and reaffirm that in congenital or early infantile cases of generalized exfoliative erythroderma, Netherton syndrome should be suspected as the underlying disease.
Epidermolysis Bullosa of the Dowling‐Meara Type: Clinical and Ultrastructural Findings in Five Patients
Authors: Puddu P. , Angelo C. , Faraggiana T. , Muda A. Onetti , Colonna L. , Paradisi M.
Published: March 2008

Abstract: Clinical and pathologic features of five cases of epidermolysis bullosa simplex, Dowling‐Meara type (EBS‐DM), are described. Four patients were children, and two were related (father and daughter). Clinical history revealed blistering at birth in three patients; in all of them the signs and symptoms improved with age. Histopathologic and ultrastructural examinations showed cytolysis of the basal cells and clumping of the tonofilaments within the cytoplasm of keratinocytes. Two distinct types of clumps were observed: round (3 patients) and whisklike (2 patients). Two patients had both types of clumps. The presence of both types in the same patient suggests that subtyping of the disease is still premature.
Congenital Psoriasis Following the Lines of Blaschko
Authors: Lehners‐Weber Christiane , De La Brassinne Michel , Dezfoulian Bita , Richert Bertrand , Bonardeaux Chantal , Willemaers Valentine
Published: March 2008

Abstract: Congenital psoriasis without an underlying nevus following the lines of Blaschko is described In a 6‐year‐old girl. In addition to this special type of psoriasis, she suffered from atopic dermatitis. The lesions were aggravated by sun exposure and phototherapy treatments, as well as by upper airways infections, without any clear explanation of these trigger factors. We propose to call this form of psoriasis congenital blaschkoid psoriasis.
Periauricular Eczematization in Childhood Atopic Dermatitis
Authors: Kim Kyu Han , Hwang Ji Hwan , Park Kyoung Chan
Published: March 2008

Abstract: We investigated the prevalence of periauricular eczematization (PAE) in children with atopic dermatitis (AD) in Korea, and evaluated the clinical significance of the lesion in terms of Its treatment in relation to a possible pathogenetic role for Staphylococcus aureus. Tentatively, PAE was classified as infraauricular and postauricular fissuring, which were observed in 62 (57,9%) and 37 (34.6%) of 107 patients, respectively. Sixty eight patients (64.5%) had infraauricular or postauricular fissures, or both, and this prevalence was significantly (p < 0.01) higher than that in controls (1.3%). Staphylococcus aureus was cultured from oozing material from infraauricular fissures in 12 of 14 patients, and topical application of erythromycin cream to the ear lesions led to faster and more definite improvement. Our study shows that PAE is a characteristic clinical manifestation of AD, probably induced by S. aureus. Topical antibiotics are worth trying for effective management of this disorder.
Amelanotic Spitzoid Melanoma in the Burn Scar of a Child
Authors: Ghorbani Zahra , Dowlati Yahya , Mehregan Amir H.
Published: March 2008

Abstract: We report a 3‐year‐old girl with a rapidly growing, amelanotic, nodular melanoma developing within a burn scar over the dorsal aspect of the left foot. Histologically, the lesion was spindle cell and exhibited lack of maturation, cellular atypia, and scattered mitotic figures. Twenty five percent of the tumor cells were positive for proliferating cell nuclear antigen by immunohistochemical stain. A left inguinal lymph node was also positive for regional metastasis. The child died with disseminated metastases 13 months after the lesion was removed.
Pachydermodactyly in Two Young Girls
Authors: Bardazzi Federico , Neri Iria , Fanti Pier Alessandro , Patrizi Annalisa
Published: March 2008

Abstract: Pachydermodactyly is a benign, superficial fibromatosis frequently observed in young males. We describe the condition in two young girls affected by Ehlers‐Danlos syndrome (EDS) and tuberous sclerosis, respectively. We hypothesized that in the patient with tuberous sclerosis, pachydermodactyly is a clinical manifestation of the associated systemic disease, whereas obsessive‐compulsive behavior is probably the main cause in the girl with EDS.
A New Case of Hairy Elbows Syndrome (Hypertrichosis Cubiti)
Authors: Escalonilla Pilar , Aguilar Antonio , Gallego Miguel , Piqué Enric , Fariña M. Carmen , Requena Luis
Published: March 2008

Abstract: Hypertrichosis cubiti, also named hairy elbows syndrome (HES), is an uncommon variety of congenital, circumscribed hypertrichosis in which a remarkable amount of long vellus hair is localized on the extensor surfaces of the upper extremities. Usually, this condition appears in infancy, increases during early childhood, and regresses spontaneously at puberty. The mode of inheritance is not elucidated, and the condition presents in a sporadic or familial form. In most patients HES is not associated with any other physical abnormalities, although short stature and other developmental disorders have been described in some. An 8‐year‐old girl affected with HES is reported. The literature on this subject is reviewed, and some clinical and etiopathogenic aspects are discussed.
Epidermolysis Bullosa Simplex Herpetiformis of Dowling‐Meara with Mottled Pigmentation: The Relationship Between EBS Herpetiformis and EBS with Mottled Pigmentation
Authors: Tay Yong‐Kwang , Weston William L.
Published: March 2008

Abstract: A 6–year‐oid girl with epidermolysis bullosa simplex (EBS) is described. Clinical features include generalized herpetiform blistering of the skin, healing without scars, intraoral blistering, nail dystrophy, palmo‐plantar keratoderma, and improvement with age. An unusual feature was the presence of a striking mottled pigmentation involving the arms, trunk, and legs. Histology, immunofluorescence, and electron microscopy of a fresh lesion showed an intraepidermal split within the basal kerati‐nocytes, focal hyperpigmentation of the basal cells without an inflammatory infiltrate, and tonofilament clumping. Our patient illustrates the clinical features of Dowling‐Meara EBS with the pigmentary changes of EBS with mottled pigmentation. The relationship between EBS of Dowling‐Meara and EBS with mottled pigmentation is discussed.
Itraconazole Responsive Tinea Capitis in an HIV‐infected Child
Authors: Smith Rebecca L. , Levy Moise L.
Published: March 2008

Abstract: Tinea capitis has not been reported to be a prevalent or serious affliction in pediatric patients infected with the human immunodeficiency virus (HIV). We report tinea capitis due to Microsporum canis in a boy Infected with HIV. After falling conventional therapy, he was treated successfully with a four‐week course of itraconazole. His immunologic status improved during therapy. The possible relationship of this response to the ultimate resolution of disease is discussed.
Exfoliative Dermatitis Due to Immunologically Confirmed Carbamazepine Hypersensitivity
Authors: Troost Roger J. J. , Oranje Arnold P. , Lijnen Raf L. P. , Benner Robbert , Prens Errol P.
Published: March 2008

Abstract: A 6–year‐old Caucasian girl experienced a generalized erythematous skin rash during carbamazepine therapy. Over the next four days the eruption worsened into erythroderma with fever and generalized lymphadenopathy. Routine laboratory studies revealed increased serum levels of liver enzymes and eosinophilia. Immunologic reactivity to the anticonvulsant carbamazepine and its analogs was investigated both in vivo and in vitro by patch tests and lymphocyte proliferation assays, respectively.
Management of Congenital Melanocytie Nevi: A Decade Later
Authors: Williams Mary L. , Frieden Ilona J. , Suzuki Tadashi , Izaki Seiichi , Ikeda Shigeo , Kitamura Keijiro , Rhodes Arthur R. , Gelmetti Carlo , Rivers Jason K. , Grin Caron M. , Grant‐Kels Jane M. , Enjolras Odile , Ellis Darrel L. , Oranje Arnold P. , Peer Charlotte D. , Paul Eberhard , Lucky Anne W. , Tamayo Lourdes
Published: March 2008

INTRODUCTION

Twelve years ago we published a symposium entitled “The management of congenital melanocytie nevi.” This subject was addressed because of its controversial nature and because of the tremendous uncertainty as to how these lesions should be handled. In the interim between that symposium and this, considerable numbers of publications have dealt with various aspects of this dilemma. We wondered what impact these data might have on current practices. The responses of several colleagues are printed below.
CTCL in Patients Under 20 Years of Age: A Series of Five Cases
Authors: Hickham Patricia Rice , McBurney Elizabeth I. , Fitzgerald Rebecca L.
Published: March 2008

Abstract: The diagnosis of cutaneous T‐cell lymphoma in patients under 20 years of age is extremely rare. We report five patients diagnosed before 20 years of age who illustrate the striking variations in clinical and histologic features as well as disease progression. We feel this information stresses the importance of multiple biopsies In young patients with chronic dermatoses.
Bilateral Abdominal Aplasia Cutis Congenita Associated with Atrial Septal Defect: A Case Report
Authors: Tekinalp Gülsevin , Yurdakök Murat , Kara Ates , Gököz Aytaç , Şahin Sedef , Saçkesen Cansin , Hindioḡlu Uḡur
Published: March 2008

Abstract: A full‐term infant girl who had bilateral, symmetrical, sharply defined, triangular‐shaped ulcers on both sides of the umbilicus is described. A punch biopsy specimen showed histologic findings of aplasia cutis. Echocardiographic examination revealed primum‐type atrial septal defect. To our knowledge, this association is the first of its kind to be reported in the literature.
A New Case of Trichothiodystrophy Associated with Autism, Seizures, and Mental Retardation
Authors: Schepis Carmelo , Elia Maurizio , Siragusa Maddalena , Barbareschi Mauro
Published: March 2008

Abstract: We report a patient with trichothiodystrophy associated with autism, mental retardation, and seizures. The diagnosis was based on the presence of brittle hair, with a marked decrease in sulfur‐rich amino acid content, and characteristic features such as “tiger tail” under polarizing microscopy and trichoschisis under scanning electron microscopy. Macroscopic alterations were mostly observed in the frontal and occipital hair, with only microscopic abnormalities in the occipital hair. We consider this an unusual expression of this disease.