bluuebunny/crossref_metadata_2025 huggingface dataset:
Langerhans Cell Histiocytosis Masquerading as Lichen Aureus
Authors: Megahed Mosaad , Schuppe Hans‐Christian , Hölzle Erhard , Jürgens Herbert , Plewig Gerd
Published: March 2008

Abstract: We report a 13‐year‐old boy with localized Langerhans cell histiocytosis. The lesion was restricted to the skin of the mons pubis and clinically resembled lichen aureus. Histopathologic and electron microscopic examinations of a skin biopsy specimen of the lesion showed typical features of Langerhans cell histiocytosis. Extensive examination revealed no other organ involvement.
Sjögren‐Larsson Syndrome: Case Reports
Authors: Levisohn Dianne , Dintiman Brenda , Rizzo William B.
Published: March 2008

Abstract: Sjögren‐Larsson syndrome (SLS) is a rare, autosomal recessive disorder with worldwide distribution. It consists of ichthyosis, spastic diplegia, and mental retardation. An enzymatic defect in fatty alcohol oxidation recently was identified and is thought to be responsible for the disorder. We report two siblings with SLS. In addition to the typical features of the syndrome, the sister had marked hyperkeratosis of the palms and soles, which is rarely seen to this degree. The brother had joint hyperextensibility, which has not been reported previously. Both individuals had documented deficient activity of fatty alcohol:NAD+ oxidoreductase.
Localized Cutis Marmorata Telangiectatica Congenita
Authors: Suarez Sylvia M. , Grossman Marc E.
Published: March 2008

Abstract: Cutis marmorata telangiectatica congenita (CMTC) is a rare, usuaiiy sporadic, congenital, reticulated vascular anomaly, it may be widespread or segmental, with atrophy or ulceration, and may be associated with other congenital abnormalities. We report a case of CMTC with atrophy, restricted in size to a small area on the back.
Variations in the Beading Configuration in Monilethrix
Authors: de Berker D. , Dawber R.P.R.
Published: March 2008

Abstract: Using information from a light microscope integrated with a computer, we quantified the variation in node and internode dimensions in monllethrix. We combined this with rate of growth measurements in individual haris to make the following conclusions. There is considerable variation of node and internode configuration within single halrs. Halrs from the same scalp, sampled at the same time show no mutual coordination of node‐internode formation, even when different growth rates are taken into account. There is no apparent correlation between the day‐night cycle and the node‐internode rhythm. The follicular expression of monilethrix appears largely independent of unifying systemic influences.
Oral‐Facial‐Digital Type 1 Syndrome of Papillon‐Léage and Psaume
Authors: de Luna Margarita Larralde , Raspa María Luisa , Ibargoyen Jesús
Published: March 2008

Abstract: A female infant was classified as having oral‐facial‐digital syndrome (OFDS) type 1, with oral (cleft palate, bifid uvula, lingual cleft, numerous hypertrophic frenula), facial (numerous milia on face, scalp, and ears; frontal bossing; hypertelorism; hypoplasia of nasal alar cartilage; micrognathia), and digital (bilateral brachydactyly of hands) symptoms. She also had diffuse, nonscarring alopecia with wiry, dry hair. Resuits of roentgenographic and ultrasound studies were normal. At her present age of 11 months, her psychomotor development is appropriate for her age.
LICHEN SCLEROSUS IN GIRLS
Authors: RIDLEY C.M.
Published: March 2008

Letters to the Editor are welcomed for publication (subject to ed‐Iting). Letters must be signed by all auhors, 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 materials submitted or published in other Journals. Pre‐publicaations proof wil not be provided.
Alopecia in Children After Cardiac Surgery
Authors: Ben‐Amitai D. , Garty B. Z.
Published: March 2008

Abstract: Postoperative alopecia is an uncommon complication of surgery and is reported mainly in adults who undergo prolonged anesthesia. The disorder occurred In three infants after cardiac surgery. It is easily prevented by frequently changing the position of the head during surgery and the recovery period.
Multiple Juvenile Xanthogranulomas Without Foam Cells and Giant Cells
Authors: Claudy A. L. , Misery L. , Serre D. , Boucheron S.
Published: March 2008

Abstract: A 4‐month‐old boy had numerous nodular lesions. Microscopic findings revealed a histtocytic proliferation without foam cells and giant cells. Immunohistochemical studies showed that the histiocytes belonged to the monocyte‐macrophage system and not to the Langerhans cell system. This case is an unusual variant of juvenile xanthogranutoma that, when congenital, may be difficult to differentiate from HashimotoPrtizker disease.
Juvenile Xanthogranuloma with Central Nervous System Involvement
Authors: Botella‐Estrada R. , Sanmartin O , Grau M. , Alegre V. , Mas C. , Aliaga A.
Published: March 2008

Abstract: An 18‐year‐old men has been followed in our department since age 10 years when he began to develop numerous yellow, papular lesions on the scalp, face, neck, trunk, and upper extremities. The lesions enlarged slowly to form yellow‐brown nodules measuring up to 6 cm. During this period five of the nodules and two small papules were excised. Histopathology was consistent with juvenlle xanthograuloma (JXG). Recently, the patient complained of significant loss of memory, and a computed tomography scan was performed showing several cerebral and cerebellar lesions. The cutaneous lesions In our patient were almost identical to those described elsewhere as being characteristic of progressive nodular histiocytoma. The many clinical and histopathologic similarities between lesions of progressive nodular histiocytoma and JXG suggest that they may represent a continuum rather than two distinct disease processes. Given the fact that there are no histopathologic differences with JXG, as well as the wide range of clinical lesions that JXG may adopt, there Is no reason to separate the entities. Although JXG lesions are usually believed to be benign and self‐healing, targe nodular forms can be associated with visceral lesions. Only four previous cases of cutaneous JXG with central nervous system involvement were found in a review of the literature.
Failure of the Flashlamp‐Pumped Pulsed Dye Laser to Prevent Progression to Deep Hemangioma
Authors: Ashinoff Robin , Geronemus Roy G.
Published: March 2008

Abstract: Hemangiomas are common vascular lesions in children. The flashIamp‐pumped pulsed dye laser has shown excellent results in the treatment of port‐wine stains and, more recently, superficial (capillary) hemangiomas. Four patients with clinically evident superficial hemanglo‐mas illustrate the point that early treatment with this laser may not preclude growth of a deeper component of the lesions.
Trichothiodystrophy: Ultrastructural Studies of Two Patients
Authors: Calvieri S. , Rossi A. , Amorosi B. , Giustini S. , Innocenzi D. , Micali G. , Rizzo R.
Published: March 2008

Abstract: An 18‐month‐old and an 8‐year‐old girl had trlchothlodystro‐phy (TTD). Microscopic observation of the hair under polarized light showed typical alternation of bright and dark bands; amlno add analysis of the hair demonstrated a marked reduction of cystine levels. Both patients had skin lesions consisting In the older child of diffuse follicular keratosis since birth, and in the younger of an ichthyosiform darmatitls on the lower legs that appeared at age 4 months. Ultrastructural studies of the skin showed striking similarities in both cases: perinuclear vscuoles with a unit membrane in the keratinocytes, and dispersed, irregularty arranged bundles of tonofilaments particularly at the desmosome junction. The origin of the vacuoles is unknown; the abnormalities of the tonofilaments could be explained on the basis of a generalized abnormality in sulfur‐containing proteins, reflecting a disturbance In the synthesis of keratins. These electron microscopy findings could be considered as a peculiar feature of ichthyotic skin in patients with TTD.
Benign Pigmented Apocrine Vulvar Hamartomas
Authors: Stephens‐Groff Susan M. , Hansen Ronald C. , Bangert Jerry
Published: March 2008

Abstract: Pigmented vulvar lesions were observed In a child during a sexual abuse evaluation. Gross examination of the lesions appeared most consistent with bowenoid papulosis; however, biopsy confirmed the le‐sions to be pigmented apocrine hamartomas. To our knowledge, these rare and benign tumors have never been described as pigmented, but should be added to the differential diagnosis of pigmented vulvar lesions.
Scabies of the Scalp Mimicking Seborrheic Dermatitis in Immunocompromised Patients
Authors: Duran Carols , Tamayo Lourdes , Orozco Ma. de la Luz , Ruiz‐Maldonado Ramon
Published: March 2008

Abstract: Two children with acute tymphoblastic leukemia undergoing chemotherapy had a special form of scabies characterized by fine scaling of the scalp simulating seborrheic dermatitis. Pruritus was mild or absent, immunocompromised children and adults should receive whole‐body and scalp antiscabietic treatment.
Granular Cell Tumor of the Vulva
Authors: Guenther Lyn , Shum David
Published: March 2008

Abstract: Granular cell tumors are uncommon in the vulva. We cared for a child who had a solitary vulvar tumor since infancy.
Linear Nevus Sebaceus of Jadassohn Treated with the Carbon Dioxide Laser
Authors: Ashinoff Robin
Published: March 2008

Abstract: A 10‐year‐old boy from Guiana was born with a linear nevus sebaceus covering his entire nose from the glabella area to the tip. Cosmetic appearance, which was the major concern of the patient and his family, was greatly Improved by the use of the carbon dioxide (C02) laser. The CO2? laser has not been reported previously as a treatment modality for nevus sebaceus.
Cutaneous Amebiasis in Children
Authors: Magańa‐García Mario , Arista‐Viveros Antonio
Published: March 2008

Abstract: A 7‐month‐old girl developed cutaneous amebiasis of the perianal and genital areas in association with amebic dysentery. The diagnosis was based on the identification of Entamoeba histolytica by skin biopsy. She was treated with dehydroemetine and metronidazole with excellent results. This is only the eighth reported case of this disease in a child
Generalized Infestation of a 31/2‐Year‐Old Girl With the Pubic Louse
Authors: Klaus Sidney , Shvil Yigal , Mumcuoglu Kosta Y.
Published: March 2008

Abstract: A 31/2‐year‐oid girl had a Pthirus pubis infestation of the scalp, neck, eyelashes, back, and pubic area. The child's body, Including the scalp, was treated with a 1% permethrln cream rinse formulation for 10 minutes. The treatment was repeated after 10 days. The eyelashes were treated by application of the permetnrin solution with a cotton‐tip swab.
A Survey of Childhood Psoriasis in Kuwait
Authors: Al‐Fouzan Abdulwahab S. , Nanda Arti
Published: March 2008

Abstract: One hundred ninety cases of childhood (age birth‐12 yrs) psoriasis constituted 0.3% of the total of new dermatologic outpatients. Girls outnumbered boys by a ratio of 1.5:1. Plaque psoriasis was the commonest clinical variant (84%), followed by guttate psoriasis. The scalp was the most common site of onset (22%). Sites predominantly affected later were the extensor surfaces of the legs and the scalp (63% and 53%, respectively). A majority of the children had a milder form of disease. Nail involvement was seen in 36%, with pitting the most common feature (87%). Seven percent had mucosal involvement. A positive family history of psoriasis was obtained in 28%. Topical therapy (coal tar, anthralin, steroids) was the main treatment in the majority of patients (92%). Sixteen patients (8%) required more aggressive intervention (ultraviolet B, psoralin‐UVA, retinoids). This is the first study of this kind from the Middle East, the findings are compared with those reported from other parts of the world.
Oral Pemphigus Vulgaris Associated with Inflammatory Bowel Disease and Herpetic Gingivostomatitis in an 11‐Year‐Old Girl
Authors: Prendiville Julie S. , Israel David M. , Wood William S. , Dimmick James E.
Published: March 2008

Abstract: An 11‐year‐old girl with recently diagnosed oral pemphigus vulgaris developed a severe exacerbation of mouth ulceration due to superinfection with herpes simplex virus type I. A concurrent diagnosis of chronic inflammatory bowel disease was established to explain symptoms of weight loss and intermittent bloody diarrhea that predated the oral ulceration by several years. Herpes simplex infection is a recognized complication of pemphigus vulgaris that may be mistaken for a recrudescence of the disease. The association of pemphigus with chronic inflammatory bowel disease has been documented in a small number of adults. Its relationship to pyostomatitis vegetans, an acknowledged marker for ulcerative colitis and Crohn disease, remains unclear.
Dermatologic Services Provided to Children and Adolescents by Primary Care and Other Physicians in the United States
Authors: Krowchuk Daniel P. , Bradham Douglas D. , Fleischer Alan B.
Published: March 2008

Abstract: To determine the frequency and types of pediatric dermatologic problems encountered by primary care physicians, dermatologists, and other physicians, we examined data from the 1990 National Ambulatory Medical Care Survey, a continuing study of physician practice in the United States. In 1990, 163·3 million physician office visits were made by patients 18 years of age or younger for all diagnoses; of these, 126·2 million were to primary care physicians. Among visits to these providers, a primary, secondary, or tertiary cutaneous concern or diagnosis was recorded in 12·3 million visits (9·7%). Of these patients with a primary cutaneous concern, 68% of visits were made to primary care physicians, 21% to dermatologists, and 10% to other physicians. For encounters in which primary care physicians reported a primary dermatologic diagnosis (9·6 million visits, 7·6%), the majority of diagnoses (86–93%) were in one of five categories: skin Infections, dermatitis, parasitic infestations, acne, or urticaria. This analysis demonstrates the great frequency with which cutaneous disease occurs in ambulatory medicine, and reinforces the Importance of dermatologic education in the training of primary care physicians.
Congenital Sensory Neuropathy With Anhidrosis
Authors: Domingues J.C. , Moreno A. , Mariano A. , Tellechea O. , Correia C. , Gonçalves O. , Baptista A. Poiares
Published: March 2008

Abstract: A 6‐year‐old girl had congenital sensory neuropathy with anhidrosis (CSNA), one of the five variants of a group of very rare genetic disorders of the peripheral nervous system—hereditary sensory neuropathies (HSN). Clinical, laboratory, and physiopathologic aspects are discussed. Dermatologic findings of anhidrosis and self‐mutilation suggest the diagnosis.
Solitary Mastocytoma With Massive Eosinophilic Infiltration
Authors: Kuramoto Yoko , Tagami Hachiro
Published: March 2008

Abstract: An 11‐month‐old girl had a yellowish brown nodule that histologically had an unexpectedly massive infiltrate of eosinophils in the dermis. This histologic feature made determining the correct diagnosis of cutaneous solitary mastocytoma difficult until we performed specific histochemical staining for mast cells.
A Case of Infantile Febrile Psoriasiform Dermatitis
Authors: Tsuge Ikuya , Fujii Hatsumi , Andou Yoshihiro , Katayama Isao , Kajita Mitsuharu , Haga Yoshiko , Asano Keiko , Mori Osamu , Matsumoto Yoshinari , Ohashi Masaru , Hara Kazuo , Watanabe Kazuyoshi
Published: March 2008

Abstract: An infant suffered from psoriasiform dermatitis complicated by severe constitutional symptoms. During his 12 months of hospitalization, the symptoms exacerbated periodically despite numerous therapeutic trials. Histologie findings revealed lichenoid psoriasiform dermatitis with striking eosinophilic necrosis of epidermal cells and satellite cell necrosis. Immunohisfochemically, CD1 + Langerhans cells had almost disappeared, and CD8+ cytotoxic‐suppressor T cells were predominant over CD4+ helper‐inducer T cells in the epidermis. These findings in the skin biopsy specimens suggested some similarity to graft‐versus‐host disease but no known cause of that disorder was proved. Finally, methotrexate was effective. The patient became afebrile, and his skin lesions Improved, leaving almost no scarring. This patient seems to have had specific clinical features that do not correspond with any of the known dermatitites. Clinical, histoiogic, and laboratory findings did not uncover any etiologic factors.
Rothmund‐Thomson Syndrome in Fraternal Twins
Authors: Tong Melinda
Published: March 2008

Abstract: Fraternal twins of Malay descent had the Rothmund‐Thomson syndrome. This is a rare, autosomal recessive disorder characterized by photosensitivity, poikiloderma, short stature, skeletal defects, and juvenile cataracts. This is the first case report of the syndrome from southeast Asia.
Multiple Agminated Spitz Nevi: Report of Two Cases and Review of the Literature
Authors: Bullen Ricardo , Snow Stephen N , Larson Paul O. , Kircik Leon H. , Nychay Stephen , Briggs Pedro
Published: March 2008

Abstract: Spitz nevus is most commonly a benign solitary lesion. Multiple lesions arranged in clusters (agminated) are very rare. Malignant degeneration has not been reported. Although spontaneous involution may occur, the nevi are frequently treated by surgical excision. We describe two preschool girls with multiple agminated Spitz nevi, and review the current world literature on multiple Spitz nevi.
Congenital Apocrine Hamartoma: An Unusual Clinical Variant of Organoid Nevus with Apocrine Differentiation
Authors: Herrmann James J. , Eramo Lynne R.
Published: March 2008

Abstract: Congenital apocrine hamartomas are rare tumors of the skin composed of mature apocrine glands in the papillary and reticular dermis. Several cases have been reported in the literature but few were of uniform clinical appearance. The term apocrine nevus has been used interchangeably with apocrine hamartoma in the literature, however, based on their distinctive morphologies we believe they are different entities. We describe a 7‐year‐old girl with a congenital apocrine hamartoma of the left cheek. A stained biopsy specimen from the lesion revealed large mature apocrine glands with decapitation secretion in the dermis. The presence of periodic acid‐Schiff‐posltive, diastase‐resistant granular material in the apical cytoplasm of some secretory cells helped differentiate these as apocrine glands. We think this lesion may represent a form of organoid nevus with pure apocrine differentiation.
Cervical Occult Spinal Dysraphism: MRI Findings and the Value of a Vascular Birthmark
Authors: Enjolras O. , Boukobza M. , Jdid R.
Published: March 2008

Abstract: Spinal dysraphism is easily recognized in the overt form as a meningocele or myetomeningocele. The closed form or occult spinal dysraphism (OSD) can be overlooked. It occurs predominantly at the lumbosacral level, but OSD at the cervical level, although very rare, also occurs. The value of magnetic resonance imaging investigations in preparation for surgical treatment is emphasized. We discuss the value of various midline posterior skin anomalies as Indicators of an underlying developmental defect in the neural axis. Hallmarks for OSD in the inferior third of the back are well known. They can also occur at the cervical level. Among these warning cutaneous midline changes, a vascular stain alone is rarely a clue for OSD whatever the spinal level involved, and specifically In the nuchal area.
Congenital Triangular Alopecia (Brauer Nevus)
Authors: García‐Hernández Maria Jose , Rodríguez‐Pichardo Antonio , Camacho Francisco
Published: March 2008

Abstract: Congenital triangular alopecia is manifested at 3 to 5 years of age by unilateral or, less frequently, bilateral patches of alopecia in the frontotemporal region. At this age the differential diagnosis is important, particularly as regards alopecia areata. Only about 47 cases have been reported, probably because the lesion is benign and nonprogressive. In 6200 patients seen in index visits, we found 7 with triangular alopecia, a frequency of 0.11%. We believe that males do not require treatment because of the later development of androgenic alopecia, but in women, surgical treatment is successful.
Congenital Ventral Hernia in Association with Focal Dermal Hypoplasia
Authors: Bhatia Amina M. , Clericuzio Carol L. , Musemeche Catherine A.
Published: March 2008

Abstract: Focal dermal hypoplasia is a rare, X‐linked dominant syndrome characterized by dysplasla of the skin, skeleton, and central nervous system. We report an intant who was born with severe focal dermal hypoplasla and an epigastric hernia. Operative timing and approach to abdominal wall defects in the presence of severe cutaneous dysplasia are discussed.
Congenital Melanoma: A Case Suggesting Rhabdomyogenic Differentiation
Authors: Koyama Teruhisa , Murakami Motomasa , Nishihara Osami
Published: March 2008

Abstract: A newborn girl had some subcutaneous masses within a giant congenital melanocytic nevus in the left parietal area. Microscopic examination revealed melanoma. The tumor consisted of two different patterns, being composed partly of epithelioid cells and partly of spindle shaped cells. Immunohistochemically the epithelloid cells showed a positive reaction for HMB‐45 and NKI/C3, and a negative reaction lor desmin and α‐sarcomeric actin. The spindle‐shaped cells showed a negative reaction for HMB‐45 and NKI/C3, and a positive reaction tor desmin and α‐sarcomeric actln. Ultrastructurally the spindle‐shaped cells had some schwannian features, namely, a basal lamina, interdigitating cell processes, and functional complexes. The pathologic findings of our case suggest possible direct rhabdomyogenic differentiation of melanoma cells.
Congenital Triangular Alopecia
Authors: Armstrong D. K. B. , Burrows D.
Published: March 2008

Abstract: We report a child with bilateral congenital triangular alopecia. Shaving the scalp and inserting intravenous cannulas into scalp vessels during the neonatal period was misconstrued by the parents as the cause of hair loss, and resulted in medicolegal implications.
Transient Cutaneous Vesicles in a Teenager: The Final Piece to a Puzzle
Authors: Solomon Barry A. , Phillips Charlotte , Laude Teresita A.
Published: March 2008

Abstract: Cicatricial pemphigoid is a chronic blistering disease that predominantly affects the mucous membranes. It has a peak occurrence in the seventh decade. Pediatric cicatricial pemphigoid is a rare entity, with fewer than 20 cases reported. We report an 18‐year‐old man who was recently diagnosed with cicatricial pemphigoid after six years of diagnostic uncertainty.
Bullous Mastocytosis in an Infant Associated with the Use of a Nonprescription Cough Suppressant
Authors: Cook Joel , Stith McLean , Sahn Eleanor E.
Published: March 2008

Abstract: Bullous mastocytosis is an unusual expression of mastocytosis typically seen in young children, and many causes of the acute mast cell degranulatlon with bulla formation have been identified. We report a 6‐month‐old boy with urticaria pigmentosa and an extensive bullous eruption associated with the ingest ion of a non prescription cough suppressant containing dextrotmethorphan. The pathogenesis of mastocytosis and the care of patients with this disease are discussed.
Treatment of Childhood Cutaneous T‐Cell Lymphoma with Alpha‐Interferon Plus PUVA
Authors: Tay Yong‐Kwang , Weston William L. , Aeling John L.
Published: March 2008

Abstract: All forms of cutaneous T‐cell lymphoma are rare in childhood. We describe an 8‐year‐old boy with plaque‐stage mycosis fun‐goides stage HA whose cutaneous eruption had been present for 5 years. Histologic examination revealed the presence of a granulomatous infiltrate together with atypical lymphocytes within the dermis. The child had an excellent response to combination psoralen‐UVA (PUVA) with interferon‐α2a treatment and is currently in remission.
Linear Sebaceous Nevus Syndrome: Report of a Patient with Unusual Associated Abnormalities
Authors: Palazzi P. , Artese O. , Paolini A. , Cazzato C. , Cucchiarelli S. , Iezzi D. , Amerio P.
Published: March 2008

A 9‐year‐old girl was diagnosed as having a linear sebaceous nevus syndrome (LSNS). The nevus sebaceus was located on the face, and the girl also had nevoid hypertrichosis on the neck, sensorineural deafness, partial anodontia, blocked tear ducts, labiopalatoschisis, and an area of micropolygyria in the left encephalic (cerebral) hemisphere. Electroencephalographic alterations were detected, but they were not accompanied by a history of seizures; furthermore, the child was not mentally retarded. This phenotypic pattern of LSNS is unusual for the rarity of associated abnormalities.
Metastatic Crohn's Disease: A Case Report
Authors: Cummins Rebecca E. , Mullins Diane , Smith Linda J. , Ford Michael J.
Published: March 2008

Metastatic Crohn's disease (MCD) is a rare extraintestinal manifestation of Crohn's disease characterized by the histologic finding of granulomatous dermatitis at a site noncontiguous to the gastrointestinal tract. An adolescent had MCD of the face that was initially mistaken for severe, treatment‐resistant acne. Histopathologic and microbiologic evaluation, combined with clinical examination and response to therapy, ultimately led to the correct diagnosis. Mycobacterium paratuberculosis is a suspected cause of Crohn's disease. Polymerase chain reaction was used to detect genomic DNA specific for the organism in biopsy specimens from the patient's cutaneous lesions. This study failed to demonstrate M. paratuberculosis in the specimens.
Loose Anagen Hair in Hypohidrotic Ectodermal Dysplasia
Authors: Azón‐Masoliver Antoni , Ferrando Juan
Published: March 2008

We report a 4 year‐old boy affected with hypohidrotic ectodermal dysplasia in whom loose anagen hair syndrome (LAHS) was suspected clinically. The diagnosis was confirmed by examination of hair by optic and scanning electron microscopy. Loose anagen hairs have not been previously described in the ectodermal dysplasias. It is possible that our patient had real LAHS or an ectodermal dysplasia with loose anagen hair as an epiphenomenon.
Leukemia Cutis in Three Children: Clinical and Immunohistochemical Studies
Authors: Koga Mayumi , Furukawa Susumu
Published: March 2008

Abstract: We report 3 children with leukemia cutis observed at the initial diagnosis of systemic leukemia. Leukemia subtypes in the three children were congenital monocytic, acute undifferentiated, and acute monocytic, respectively. The patients were girls age 10 days, 14 years, and 11 months, respectively, at diagnosis. We describe the clinical features of the cases and the results of immunohistochemical studies on paraffin‐embedded skin biopsy specimens. The skin lesions were tumors and areas of reddish purple erythema in the first child, pigmented erythema in the second, and bright red erythema in the third. In the first two patients skin lesion biopsy specimens had dense leukemic infiltrates in the dermis with reactive T lymphocytes scattered among them. In the third patient, the infiltrating cells were almost all reactive T lymphocytes, with a few leukemic cells. A relationship between the leukemic‐reactive cell ratio and the prognosis was suggested; dense leukemic cell infiltrates may be associated with a poor prognosis.
Congenital Smooth Muscle Hamartoma Presenting as a Linear Atrophic Plaque: Case Report and Review of the Literature
Authors: Grau‐Massanes M. , Raimer S. , Colome‐Grimmer M. , Yen A. , Sanchez R. L.
Published: March 2008

Abstract: Congenital smooth muscle hamartoma usually manifests as a well‐circumscribed, hyperpigmented plaque, frequently hypertrichotic, on the trunk or extremities. We report such a lesion in a 7‐month‐old girl that presented as a linear, mottled, purplish red plaque appearing In areas to be atrophic, involving her right buttock, posterior thigh and leg, and fifth toe. Although the clinical appearance suggested linear morphea, a biopsy specimen had numerous haphazardly oriented bundles of smooth muscle in the reticular dermis. Masson trichrome staining, smooth muscle specific actin, and electron microscopic studies confirmed the smooth muscle nature of the cells. A diagnosis of smooth muscle hamartoma was made. To our knowledge, this linear clinical presentation has not been described previously.
Herpes Zoster in Childhood: Case Report and Review of the Literature
Authors: Smith Corinne G. , Glaser Dee Anna
Published: March 2008

Abstract: Herpes zoster in childhood is uncommon even in the setting of known risk factors such as primary varicella zoster infection before 12 months of age and immunocompromised states. We report a 7‐year‐old, otherwise healthy girl with shingles, and review the risk factors, prognosis, and treatment of pediatric zoster.
A Reappraisal of Topical Steroid Potency
Authors: Hepburn David J. , Aeling John L. , Weston William L.
Published: March 2008

Abstract: Topical corticosteroids have been one of the cornerstones of dermatologic treatment for nearly 40 years. At present the most widely used topical steroid ranking system is the vasoconstrictor assay. To determine whether this is a satisfactory measure of the agents’ potency in clinical settings, we examined rankings from the published literature achieved by four different methods: vasoconstriction, clinical outcome, therapeutic index (a ratio of efficacy to systemic safety), and cost, safety, and efficacy. Overall clinical outcome (efficacy) rankings in this study corresponded in only 11 (62%) of 17 topical steroid preparations with the expected vasoconstrictor rankings. The therapeutic index rankings did not correspond with the clinical outcome (33%) or the vasoconstrictor assay (33%) rankings. It was difficult to compare studies because of lack of standardization of clinical trials. We urge that topical steroid rankings not be based solely on vasoconstrictor assays, and that a standard method be developed for clinical trials of the drugs to allow for comparison among preparations.
Tyrosinemia Type 11: A Challenge for Ophthalmologists and Dermatologists
Authors: Benoldi Donata , Orsoni Jelka B. , Allegra Fulvio
Published: March 2008

Abstract: Tyrosinemia type 11 was suspected in a 13‐month‐old chiid with recurrent photophobia, tearing, and hyperkeratotic lesions on the paims and soles. Laboratory tests revealed high tyrosine leveis in blood and urine, AH the symptotns promptly Improved after the institution of a iow tyrosine diet. We emphasize the importance of an early diagnosis In order to avoid the risk of mental retardation in these patients.
Mal de Meleda: A Report of Four Cases from the United Arab Emirates
Authors: Lestringant Gilles G. , Frossard Philippe M. , Adeghate Ernest , Qayed Khalil I.
Published: March 2008

Abstract: Mal de Meleda (MDM), or recessive transgressive palmoplantar keratoderma, is a rare disorder, MDM may have originated as a founder mutation that occurred on the island of Meleda, now Mljet, in Croatia, where it was first described. However, the condition has also been observed in countries distant from Mljet. The presentation of the disease in young patients has not been reported and the progressiveness of the lesions is debated. We examined four young United Arab Emirates nationals patients (ages 7 months to 12 years) who presented with keratoderma palmoplantaris (KPP) and transgressive pachyderma (TP) that had both been present before 1 year of age, KPP and TP were more pronounced in the two oldest patients. Family histories were consistent with autosomal recessive inheritance. The development of MDM lesions appears to be age‐related. However, environment and individual factors may also play a role in the development and persistence of the lesions. Molecular genetic studies are necessary to establish whether the broad clinical presentation of the disease is due to allelic or genetic heterogeneities.
Acute Annular Urticaria in Infants and Children
Authors: Tamayo‐Sanchez Lourdes , Ruiz‐Maldonado Ramon , Laterza Amelia
Published: March 2008

Abstract: Characteristic features of acute annular urticaria in 34 infants and small children were large, erythematous annular and polycyclic lesions with violaceous centers, eyelid, hand, and foot edema, absence of angioedema of the airway, absence of pruritus, spontaneous resolution in 8 to 10 days, and frequent history of furazolidone medication for diarrhea. Nondermatologists often misdiagnose acute annular urticaria as erythema multiforme and unnecessarily overtreat patients. The differential diagnosis with other conditions presenting with annular lesions in children is discussed.
Lack of Efficacy of 6‐Week Treatment with Oral Terbinafine for Tinea Capitis Due to Microsporum canis in Children
Authors: Dragoš Vlasta , Lunder Majda
Published: March 2008

Abstract: Twenty‐two children aged 2 to 9 years with noninflammatory tinea capitis due to Microsporum canis were evaluated in an open clinical pilot study run from January 1994 to July 1995. Each child was given oral terbinafine according to body weight for 6 weeks. Mycologic evaluation was done at the end of treatment and after follow‐up periods of 4 and 8 weeks. None of the patients achieved complete mycologic cure by the end of the treatment period. Four weeks later complete mycologic cure was established in nine patients, and on final evaluation in seven. The treatment was very well tolerated by all the children. No systemic adverse effects were noted. According to our data, a 6‐week course of oral terbinafine is inadequate for tinea capitis due to M. canis in children. Further study is needed to determine the most appropriate duration of therapy.
Prevalence of Cutaneous Findings in Hospitalized Pediatric Patients
Authors: Hubert Jason N. , Callen Jeffrey P. , Kasteler J. Scott
Published: March 2008

Abstract: Cutaneous findings can be useful in establishing the diagnosis and treatment of hospitalized patients. Observation and identification of cutaneous abnormalities can improve the accuracy of diagnosis and result in improved patient care. We set out to determine the prevalence of cutaneous abnormalities in hospitalized pediatric patients in a hospital and how often these findings were noted and properly diagnosed by the admitting team of physicians. Children with medical problems admitted to Kosair Children's Hospital during the month of January 1995 were randomly selected for a skin examination, which was performed within 24 to 28 hours of admission. Parental consent was required prior to admission into the study. Of 117 patients offered participation, 110 accepted. Physical findings were noted and in addition the completeness of charting by the admitting physician and the relationship of any cutaneous findings to admitting diagnosis were noted. One hundred five of the 110 patients (95%) had cutaneous findings consisting of either a “rash” or a “congenital lesion.” Fifty‐one had more than one cutaneous finding noted on examination. Dermatitis was the most common diagnosis made, followed by pigmented lesions and congenital vascular malformations of all types. In 35 of the 105 patients with a dermatologic diagnosis, the dermatologic diagnosis related directly to the admitting diagnosis. In only 22 of these 35 (63%) was the cutaneous involvement noted by the admitting physician. In 9 of the 110 cases (8%), the findings on dermatologic examination altered the primary diagnosis and/or treatment. Cutaneous findings are very common in the hospitalized pediatric patient. The diagnosis, charting, and treatment of dermatologic conditions by the primary pediatric team were often incomplete, although an expert cutaneous examination can be critical to patient care.
Henoch‐Schönlein Purpura: What does the ‘Rash” Look Like in the Gastrointestinal Mucosa?
Authors: Gunasekaran T. S.
Published: March 2008

Abstract: Two patients are reported with Henoch‐Schönlein purpura, one with the typical presenting sign of purpuric skin lesions, the other whose diagnosis was made on the basis of endoscopic examination in the absence of an Initial cutaneous eruption.
A Case of Hypopigmented Mycosis Fungoides in a Young Caucasian Boy
Authors: Landro Anna Di , Marchesi Lorenzo , Naldi Luigi , Motta Teresio , Cainelli Tullio
Published: March 2008

Abstract: Hypopigmented mycosis fungoides is a variant of mycosis fungoides characterized by the presence of hypopigmented patches as the sole manifestation of the disease. It has been described aimost always in young black or dark‐skinned patients. The only white patient described was a 64‐year‐oid woman who not oniy had hypopigmented lesions, but also nodular lesions with lymphadenopathy. We describe hypopigmented lesions arising in a white boy 12 years of age, born in northern Italy, without any foreign ancestors. The microscopic alterations, with epidermotropism, the immunoiogic markers, the negativity of T‐cell receptor gene rearrangement, and the good response to PUVA therapy correspond to the main findings in black patients with this disease. Long‐term follow‐up of these patients is important to obtain better knowledge of the natural history of the disorder, Hypopigmented mycosis fungoides must now be included in the differential diagnosis of hypopigmented macular lesions not only in black or dark‐skinned patients but also in white patients.
Urticaria Pigmentosa Presenting with Massive Peripheral Eosinophilia
Authors: Stern Roy L. , Manders Steven M. , Buttress Sharon H. , Heymann Warren R.
Published: March 2008

Abstract: A 5‐month‐old Hispanic boy had a diffuse eruption and massive peripheral eosinophilia. Mastocytosis was diagnosed by skin biopsy and Giemsa stain. Other causes of eosinophilia were ruled out by bone marrow examination and negative cultures. The patient's course improved with antihistamines and the removal of mast cell degranulating agents. Clinicians need to be aware that mastocytosis should be considered in the differential diagnosis of eosinophilia.
Autosomal Recessive Inheritance of Erythrokeratoderma Variabilis
Authors: Armstrong D. K. B. , Hutchinson T. H. , Walsh M. Y. , McMillan J. C.
Published: March 2008

Abstract: Erythrokeratoderma variabilis is a rare genodermatosis conentionally regarded as autosomal dominant in inheritance. We describe the clinical features and light and electron microscopic findings in two affected siblings born to unaffected parents and suggest an autosomal recessive mode of inheritance in this famiiy. We also briefly review the literature on this disorder.
OLIGONUCLEOTIDE PRIMERS FOR THE DETECTION OF BIOLUMINESCENT DINOFLAGELLATES REVEAL NOVEL LUCIFERASE SEQUENCES AND INFORMATION ON THE MOLECULAR EVOLUTION OF THIS GENE1
Authors: Baker Andrea , Robbins Ian , Moline Mark A. , Iglesias‐Rodríguez María Débora
Published: March 2008

Bioluminescence is reported in members of 18 dinoflagellate genera. Species of dinoflagellates are known to have different bioluminescent signatures, making it difficult to assess the presence of particular species in the water column using optical tools, particularly when bioluminescent populations are in nonbloom conditions. A “universal” oligonucleotide primer set, along with species and genus‐specific primers specific to the luciferase gene were developed for the detection of bioluminescent dinoflagellates. These primers amplified luciferase sequences from bioluminescent dinoflagellate cultures and from environmental samples containing bioluminescent dinoflagellate populations. Novel luciferase sequences were obtained for strains of Alexandrium cf. catenella (Whedon et Kof.) Balech and Alexandrium fundyense Balech, and also from a strain of Gonyaulax spinifera (Clap. et Whitting) Diesing, which produces bioluminescence undetectable to the naked eye. The phylogeny of partial luciferase sequences revealed five significant clades of the dinoflagellate luciferase gene, suggesting divergence among some species and providing clues on their molecular evolution. We propose that the primers developed in this study will allow further detection of low‐light‐emitting bioluminescent dinoflagellate species and will have applications as robust indicators of dinoflagellate bioluminescence in natural water samples.
The Sankey Diagram in Energy and Material Flow Management
Authors: Schmidt Mario
Published: March 2008

The Sankey diagram is an important aid in identifying inefficiencies and potential for savings when dealing with resources. It was developed over 100 years ago by the Irish engineer Riall Sankey to analyze the thermal efficiency of steam engines and has since been applied to depict the energy and material balances of complex systems. The Sankey diagram is the main tool for visualizing industrial metabolism and hence is widely used in industrial ecology. In the history of the early 20th century, it played a major role when raw materials were scarce and expensive and engineers were making great efforts to improve technical systems. Sankey diagrams can also be used to map value flows in systems at the operational level or along global value chains. The article charts the historical development of the diagrams. After the First World War the diagrams were used to produce thermal balances of production plants for glass and cement and to optimize the energy input. In the 1930s, steel and iron ore played a strategic role in Nazi Germany. Their efficient use was highlighted with Sankey diagrams. Since the 1990s, these diagrams have become common for displaying data in life cycle assessments (LCAs) of products. Sankey diagrams can also be used to map value flows in systems at the operational level or along global value added chains. This article, the first of a pair, charts the historical development. The companion article discusses the methodology and the implicit assumptions of such Sankey diagrams.
What Is Resource Consumption and How Can It Be Measured?
Authors: Gößling‐Reisemann Stefan
Published: March 2008

When analyzing the metabolism of our economy, the usual choice for a measure of resource consumption is the throughput of matter and energy. This, however, cannot be sufficient, since consumption by definition is always relating to the destruction or transformation, and hence a change in quality, not only in quantity, of material or energy flows. Here, an approach is presented that takes the entropy production associated with any process as a measure for the resource consumption of that process. Entropy production is thereby used to approximate the intuitive notion of consumption, which can best be described by the term loss of potential utility. This article delivers theoretical evidence for the validity of this choice, and a second article in a future issue will present an application taken from the metallurgical sector. The related concept of exergy analysis is discussed and compared against the entropy approach.
In Vitro Changes in Hardness of Sealed Resilient Lining Materials on Immersion in Various Fluids
Authors: Mante Francis K. , Mante Mamle O. , Petropolous Vicki C.
Published: March 2008

Purpose: During clinical use, resilient lining materials undergo changes in hardness that make them ineffective. The aims of this investigation were (1) to determine the effect of a resilient lining sealer on the hardness of four resilient denture liners; and (2) to determine the effect of the sealer on hardness after immersion in various solutions.

Materials and Methods: Two sets of specimens of four resilient liners, Coe‐comfort (CC), PermaSoft (PS), Tokuyama soft reline (TK), and Total‐Soft (TS), 6‐mm diameter by 4‐mm thickness, were fabricated. Two coats of Permaseal, a soft reline sealant, were applied to one set of specimens of each material according to manufacturers' instructions. Sealed and unsealed samples were divided into four groups (n = 10). Each group was immersed in one of the following solutions: artificial saliva at 37°C, Efferdent, Efferdent with once daily scrubbing with a soft toothbrush, and 50% ethanol. Shore A hardness numbers were obtained at 0, 1, 3, 7, 30, and 90 days. A two‐way ANOVA test was performed using materials (treated and untreated) and immersion solution as independent variables. The percentage change in hardness after the 90‐day immersion period was the dependent variable.

Results: The results show that the application of sealant significantly improved the durability of CC, PS, and TS in immersion solutions by maintaining hardness close to preimmersion values or delaying the softening effect of the solutions. The hardness of sealed and unsealed TK showed the significantly (p < 0.05) lowest change after immersion in the test solutions. Ethanol caused the most severe decrease in hardness of all solutions, followed by saliva. Immersion in Efferdent and daily brushing after immersion in Efferdent showed only a mild effect on the hardness of the soft reline agents.

Conclusion: The use of a sealer can play an important role in the preservation of the hardness of some resilient lining materials.
Developing Geostatistical Space–Time Models to Predict Outpatient Treatment Burdens from Incomplete National Data
Authors: Gething Peter W. , Noor Abdisalan M. , Gikandi Priscilla W. , Hay Simon I. , Nixon Mark S. , Snow Robert W. , Atkinson Peter M.
Published: March 2008

Basic health system data such as the number of patients utilizing different health facilities and the types of illness for which they are being treated are critical for managing service provision. These data requirements are generally addressed with some form of national Health Management Information System (HMIS), which coordinates the routine collection and compilation of data from national health facilities. HMIS in most developing countries are characterized by widespread underreporting. Here we present a method to adjust incomplete data to allow prediction of national outpatient treatment burdens. We demonstrate this method with the example of outpatient treatments for malaria within the Kenyan HMIS. Three alternative modeling frameworks were developed and tested in which space–time geostatistical prediction algorithms were used to predict the monthly tally of treatments for presumed malaria cases (MC) at facilities where such records were missing. Models were compared by a cross‐validation exercise and the model found to most accurately predict MC incorporated available data on the total number of patients visiting each facility each month. A space–time stochastic simulation framework to accompany this model was developed and tested in order to provide estimates of both local and regional prediction uncertainty. The level of accuracy provided by the predictive model, and the accompanying estimates of uncertainty around the predictions, demonstrate how this tool can mitigate the uncertainties caused by missing data, substantially enhancing the utility of existing HMIS data to health‐service decision makers.
Symptoms of Instability in Models of Spatial Dependence
Authors: Mur Jesús , López Fernando , Angulo Ana
Published: March 2008

In recent years, there has been a growing interest in the problems caused by the existence of instability in cross‐sectional regressions. The results about local autocorrelation measures are part of this debate, as are the proposals concerning the concept of geographically weighted regressions. This article also deals with the problem of stability (or the lack thereof), but focusing the discussion on the supposition of constancy in the parameter of spatial dependence. In most cases, this assumption is treated, with the risks that this involves, as a maintained hypothesis, which should be ascertained before continuing with the modeling exercise. In the article, we present a simple heterogeneity test for this type of parameters, based on the Lagrange Multiplier principle. To illustrate its use, we take the distribution of per capita income among the European regions as our discussion case. According to our results, there are clear signs of structural breaks in the spatial distribution of this variable and the scale factor and the autocorrelation coefficient appear to be principal actors.