bluuebunny/crossref_metadata_2025 huggingface dataset:
Systemic Cytomegalovirus in a Patient with the Keratitis, Ichthyosis, and Deafness (KID) Syndrome
Authors: Helm Klaus , Lane Alfred T. , Orosz John , Metlay Leon
Published: March 2008

We describe a patient with the keratitis, ichthyosis, and deafness (KID) syndrome who also had a generalized cytomegalovirus infection. Patients with the KID syndrome are susceptible to not only bacterial and fungal infections, but also to viral infections.
Dermatofibrosarcoma Protuberans in a 9‐Year‐Old Child: Treatment by MOHS Micrographic Surgery
Authors: Goldberg David J. , Maso Martha
Published: March 2008

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, localiy and deepiy recurring dermal neoplasm that, when it occurs in cosmetically sensitive areas, can be devastating, particuiariy in children. MOHS micrographic surgery is now recognized as the treatment of choice for DFSP. A iesion of DFSP near the breast of a 9‐year‐old girl was treated by this technique. To our knowiedge, this is the first case of DFSP in a chiid in which MOHS micrographic surgery was used as the first‐tine treatment modaitty.
Comparison of Crotamiton 10% Cream (Eurax) and Permethrin 5% Cream (Elimite) for the Treatment of Scabies in Children
Authors: Taplin David , Meinking Terri Lynn , Chen Joaquin A. , Sanchez Regulo
Published: March 2008

Permethrin 5% cream (Elimite) was approved as a treatment for scabies by the U.S. Food and Drug Administration in September 1989. in a doubie‐biinded, randomized study, it was compared with crotamiton 10% cream (Eurax) for the treatment of scabies in children 2 months to 5 years of age. Two weeks after a singie overnight treatment, 14 (30%) of 47 chiidren were cured with permethrin 5% cream, in contrast to oniy 6 of 47 (13%) of subjects treated with Eurax. Four weeks after treatment the figures were 89% and 60% cured for the two agents, respectively. In 10 of the 19 patients whose treatment failed, the condition became worse after therapy. The difference in efficacy in favor of permethrin was significant (P = 0.002). That agent also demonstrated greater effectiveness in reducing pruritus and secondary bacterial infections. Eilmite offers a safe, efficacious, and cosmetically elegant aiternative to Eurax in the treatment of scabies in chiidren.
Lichen Sclerosus et Atrophicus in Children and Young Adults
Authors: Helm Klaus F. , Gibson Lawrence E. , Muller Sigfrid A.
Published: March 2008

Abstract: We performed a retrospective study of 52 children and young adults (average age 18 yrs) with lichen sclerosus et atrophicus. In 56% of patients the eruption was still present after a follow‐up of 7.5 years. Younger patients were most likely to show improvement. The histologic appearance of both groups (patients with persistent lesions and those with resolution of lesions) showed classic features of lichen sclerosus et atrophicus: homogenized collagen with an underlying bandlike lymphocytic infiltrate. Epidermal changes such as atrophy, hyperplasia, and follicular plugging were slightly more prominent in patients with persistent lesions. Menarche, pregnancy, and the presence of extragenital lesions had no effect on the prognosis, but since the condition resolved, on average, during adolescence, unknown developmental factors may be implicated.
Ascher Syndrome: A Case Study
Authors: Navas Jesús , Rodríguez‐Pichardo Antonio , Camacho Francisco
Published: March 2008

Abstract: A case of Ascher syndrome in association with bilateral cryptorchidism is reported, and several features of the syndrome are discussed.
Benign Neonatal Hemangiomatosis with Aggressive Growth of Cutaneous Lesions
Authors: Rothe Marti Jill , Rowse Debra , Grant‐Kels Jane M.
Published: March 2008

Abstract: A patient experienced rapidly proliferating, multiple cutaneous hemangiomas subject to significant hemorrhage, with associated asymptomatic pulmonary involvement. The dramatic features of his cutaneous disease, an overview of the spectrum of neonatal hemangiomatosis, and recent therapeutic options are reviewed.
Diffuse Cutaneous Mastocytosis Mimicking Staphylococcal Scalded‐Skin Syndrome: Report of Three Cases
Authors: Oranje Arnold P. , Soekanto Widowati , Sukardi A. , Vuzevski Vojislav D. , Willigen André van der , Afiani H. M.
Published: March 2008

Abstract: Three cases of diffuse cutaneous mastocytosis (DCM) were at first incorrectly diagnosed as staphylococcal scalded‐skin syndrome. In the first patient, at age 1 day the disease was recognized promptly by simple techniques such as Darier's sign and Tzanck smear. Much delay in making the diagnosis occurred in the other two patients, however: almost 1 year and 15 years, respectively.

Bullous manifestations in mastocytosis occur only in the first two or three years. In the first months the disease can be dangerous and life threatening. To distinguish mastocytosis from vesicular and bullous neonatal disorders, one should perform Darier's sign and a Tzanck smear. The diagnosis is confirmed by histopathologic studies.

Treatment of the bullous manifestations is symptomatic, with zinc oxide paste and oral antihistamines, which may provide some relief. In addition, cimetidine and sodium cromoglycate may be beneficial. At a later age psoralen plus ultraviolet A therapy may also relieve the symptoms.

Particular foods and medicines can liberate histamine and should be restricted as much as possible in extremely affected patients. Special care should be taken when these patients are to undergo anesthesia. The risk of complications during and after anesthesia is also present in other forms of mastocytosis.
Dry Skin of Newborn Infants: Functional Analysis of the Stratum Corneum
Authors: Saijo Shinobu , Tagami Hachiro
Published: March 2008

Abstract: Our clinical observations disclosed that most newborn infants showed scaling on at least some parts of the body. To clarify the mechanism for such xerosis, we performed measurements of highfrequency conductance (Gx), which assesses the skin surface hydration and evaporative water loss from the skin. The skin of newborns showed surprisingly lower Gx values and evaporative water loss than those of adults or 1‐ to 6‐month‐old infants in a warm environment. These findings may be explained partly by low eccrine activity, which has been reported as characteristic of newborn skin. However, defective stratum corneum function, such as reduced water‐holding capacity as demonstrated by the in vivo water sorption‐desorption test, also seems to be responsible for the development of dry skin in newborns.
Severe Febrile Mucha‐Habermann's Disease in Children: Case Report and Review of the Literature
Authors: Luberti Anthony A. , Rabinowitz Linda G. , Ververeli Kathleen O.
Published: March 2008

Abstract: Mucha‐Habermann disease, or pityriasis Itchenoides et varioliformis acuta, is usually a benign, papulosquamous, cutaneous disorder. It has also been reported in a severe form with fever and systemic symptoms both in children and adults. We report a 12‐year‐old boy with the febrile, ulceronecrotic type. A review of similar cases in the literature shows a 16% frequency of acute necrotic lesions, as well as rare complications such as fever, superinfected lesions, bacteremia (most often with Staphylococcus aureus), and rheumatologic manifestations such as arthritis and scieroderma. There is no definitive treatment, but tetracycline, erythromycin, methotrexate, and ultraviolet light are used most frequently. The most common histologic feature is mononuclear perivascular infiltrates. Mucha‐Habermann disease can mimic other common entities such as varicella and insect bites.
Cutaneous and Oral Lesions in 32 Children After Renal Transplantation
Authors: Menni Silvano , Beretta Daniela , Piccinno Roberta , Ghio Luciana
Published: March 2008

Abstract: A variety of skin and oral mucosal lesions appearing in patients after renal transplantation have been reported in the literature. Because most of these data pertain to adults, we studied a group of children with kidney transplants to investigate the occurrence of skin and/or oral mucosal lesions, compare them with those observed in adults, and contribute to the knowledge of the pathogenesis of these disorders.

Our series consisted of 32 patients aged 5 to 18 years, all of whom had skin and/or oral mucosal lesions that were classified as being either drug induced, probably related to uremia, or due to other causes. A possible relationship between drug‐induced lesions and the therapeutic immunosuppressive regimen in use was investigated. In addition, two formerly unreported lesions were seen in these patients: hypertrophy of lingual fungiform papillae and onychoschizia. The former occurred only in children receiving cyclosporine. Compared with adults, the frequency of gingival hypertrophy was higher and that of infectious disease was lower.
Spray‐Induced Frostbite in a Child: A New Hazard with Novel Aerosol Propellants
Authors: Lacour Marc , Coultre Claude Le
Published: March 2008

Abstract: A case of deep frostbite occurred in an 8.5‐year‐old child. The lesion was due to the improper use of a toilet air freshener and was severe enough to require a skin graft. The propellants contained in the spray were propane and butane. We measured the temperature of this aerosol during spraying (−40°) in comparison with an ethyl chloride spray (−3°) widely used for local skin anesthesia. This difference is mainly due to the much lower evaporation temperature of propane (−42.2°) and butane (−0.6Y) compared with ethyl chloride (12.5°). This child aimed the spray directly toward his skin, thus producing a deep frostbite. We wish to draw the attention of clinicians to this potential hazard with new propelltants, since they should soon replace chlorohydrofluorocarbons throughout the world for ecologic reasons.
Juvenile Pemphigus Vulgaris: Efficacy of Moderate Doses of Deflazacort
Authors: Fimiani Michele , Pianigiani Elisa , Castelli Antonio
Published: March 2008

Abstract: A 13‐year‐old girl with pemphigus vulgaris (PV) had clinical features of chronic stomatitis. The histologic and immunologic findings were typical of a diagnosis of PV. Good therapeutic results were obtained with moderate doses of deflazacort, 1 mg/kg/day slowly tapered to 0.1 mg/kg every other day. The patient had no significant side effects.
Papular Acrodermatitis of Childhood (Gianotti‐Crosti Disease)
Authors: Magyarlaki Márta , Drobnitsch Ildikó , Schneider Imre
Published: March 2008

Abstract: An 11‐year‐old boy had lentil‐sized lichenoid papules, localized to the limbs and trunk, together with acute, nonicteric, hepatitis B surface antigen‐positive hepatitis. The clinical picture and course were typical of Gianotti‐Crosti disease. Monoclonal antibodies were used to study the lymphocyte subpopulations and surface antigens in the inflammatory infiltrate in frozen sections of a skin biopsy specimen. The results provide data on the pathogenic mechanism of the papular exanthem.
Piezogenic Papules of the Feet in Healthy Children and Their Possible Relation with Connective Tissue Disorders
Authors: van Straaten E. A. , van Langen I. M. , Oorthuys J. W. E. , Oosting J.
Published: March 2008

Abstract: Piezogenic papules (PP) are pressure‐induced lesions that appear on the heels while bearing weight, due to herniation of fat tissue into the dermis. They are present in the majority of adults. Because of the poor quality of connective tissue in hereditary disorders of connective tissue, such as the Ehlers‐Danlos syndrome, it has been suggested that PP would be larger in number and diameter in this group of disorders. If papules are present, they might be painful as well. In view of this hypothesis 322 heaithy pupils aged 4 to 13 years from a Dutch primary school were examined in order to study the prevalence and characteristics of piezogenic papules and signs of connective tissue disorders (Ehlers‐Danlos) such as hypermobility and skin fragility. Of the 322 children investigated, 72% had one or more PP, the average number being five, with a mean diameter of 3.3 mm. The mean papule diameter increased with age and body weight. None of the papules were painful. Hypermobile joints occurred in 4.3% of the children. Mean body weight was the same in hypermobile and nonhypermobile children of the same age. The numbers of PP were equal in both groups, as was the number of children with and without PP. None of the children showed skin fragility. Our conclusion is that PP are present in the majority of healthy children, and are never painful.
Lichen Planus in Childhood: A Report of 17 Patients
Authors: Kanwar Amrinder J. , Handa Sanjeev , Ghosh Srabani , Kaur Surrinder
Published: March 2008

Abstract: Lichen planus is a dermatosis of unknown origin with a very limited frequency in children. Over a period of one and a half years we observed 17 cases of childhood lichen planus. The classic form of the disease as seen in adults was the most common in these children; unlike adults, however, mucosal and nail involvement was uncommon. The natural history of the disease was similar to that in adults.
Alpha1‐Antitrypsin Deficiency‐Associated Panniculitis: Case Report and Review of the Literature
Authors: Edmonds Bebe K. , Hodge Julie A. , Rietschel Robert L.
Published: March 2008

Abstract: Alpha1‐antitrypsin is the primary serum proteinase inhibitor. Alpha1‐antitrypsin deficiency, especially the ZZ genotype, has been linked mainly to emphysema and cirrhosis; it is also associated with panniculitis. A case of α‐AT‐associated panniculitis was documented in a 13‐year‐old girl in whom a deficiency of the enzyme was known to be present from infancy. This is unusual, since α‐AT panniculitis previously was described in older patients without prior knowledge of the duration of the deficiency. Our patient developed erythematous, subcutaneous nodules subsequent to trauma, which later developed into deep, painless ulcers. We report this case so that the condition may be suspected in patients with panniculitis. The diagnosis may be confirmed by measuring quantitative α‐AT serum levels and by enzyme genotyping. The treatment of choice is dapsone.
Cutaneous Lesions in Severe Combined Immunodeficiency: Two Case Reports and a Review of the Literature
Authors: Llorente C. Postigo , Amorós J. Ivars , de Frutos F. J. Ortiz , Regueiro J. R. , Martin R. Llamas , Tapia A. Guerra , Diez L. Iglesias
Published: March 2008

Abstract: Two patients with severe combined immunodeficiency (SCID) in whom cutaneous lesions were the first clinical feature were studied. Neither the morphology nor the histology of the lesions was uniform, although we have noted some common findings that can, in subsequent cases, lead us to suspect SCID. The immunologic defects were not uniform, representing the two poles of the spectrum of SCID. We believe that early recognition of the skin lesions is very important, since the patient's life expectancy can be increased by a bone marrow transplantation (1).
Chediak‐Higashi Syndrome in a Black Child
Authors: Anderson Lawrence L. , Paller Amy S. , Malpass David , Schmidt Mary Lou , Berger Timothy G.
Published: March 2008

Abstract: Chediak‐Higashi syndrome (CHS) is an uncommon genetic disorder with a constellation of clinical, pathologic, and immunologic manifestations. It is rarely reported in blacks. Pathognomonic intracellular inclusions in white blood cells are well recognized; however, characteristic abnormal melanin aggregation into giant melanosomes also occurs, as can be readily seen by histologic evaluation of hair. We present a case of CHS in a black child.
Cutaneous Lesions as a Clue to Servere Combined Immunodeficiency
Authors: De Raeve Linda , Song Micheline , Levy Jack , Mascart‐Lemone Francoise
Published: March 2008

Abstract: A 2‐month‐old boy experienced cutaneous lesions that revealed an underlying severe, combined immunodeficiency (SCID). It is important to recognize cutaneous manifestations of primary immunodeficiency disorders, as they may provide the earliest clue to a defect in immune function.
Shwachman Syndrome: A Case Report
Authors: Mortureux Patricia , Taïeb Alain , Bazeille Jean‐Etienne Surlève , Hehunstre Jean Pierre , Maleville Jean
Published: March 2008

Abstract: Cutaneous involvement is frequent in Shwachman syndrome, and includes various degrees of dry skin, and eczematous and ichthyosiform lesions. A 12‐year‐old boy with Shwachman syndrome had cutaneous involvement characterized by dry skin, perioral dermatitis, and follicular keratosis. Polymorphonuclear motility was decreased. A nutrition work‐up showed a decrease in liposluble vitamins, and suggested a causative link with the skin lesions.
Halo Dermatitis in Children
Authors: Shifer Ora , Tchetchik Raphaela , Glazer Oded , Metzker Aryeh
Published: March 2008

Abstract: Halo dermatitis is a pruritic, eczematous eruption on pigmented nevi and halo nevi in young adults. It may be mistaken for nummular eczema, psoriasis, or fungal skin infection. Awareness of halo dermatitis will possibly reveal more cases in children, which have been reported only rarely.
Sweet Syndrome in a Neonate with Aseptic Meningitis
Authors: Dunn Terris R. , Saperstein Harry W. , Biederman Aviva , Kaplan Richard P.
Published: March 2008

Abstract: A 7‐week‐old Infant with antecedent otitis media, upper respiratory infection, and aseptic meningitis was diagnosed as having Sweet syndrome. Although this disease usually affects adults, it has been reported in 17 children. This is the youngest reported patient with the disorder to date, and the first in whom the syndrome was associated with aseptic meningitis.
Aplasia Cutis Congenita, Cleft Palate, Epidermolysis Bullosa, and Ectrodactyly: A New Syndrome?
Authors: Jones Evelyn M. , Hersh Joseph H. , Yusk Janice W.
Published: March 2008

Abstract: Aptasia cutis congenita (ACC) is a skin disorder in which localized or widespread areas of skin are absent at birth. It has been associated with numerous anomalles and recognizable syndromes. We report a newborn infant with ACC of the scalp, multiple facial abnormalities including cleft palate but not cleft lip, hypoplasia of the distal phalanges of the hands, ectrodactyly of the feet, and epidermolysis bullosa of the extremities and lower trunk. Although this patient had some features that overlapped with ectrodactyly‐ectodermal dysplasia‐clefting syndrome, the absence of cleft lip and presence of additional skin abnormalities made that diagnosis unlikely. This could represent a newly recognized syndrome of numerous malformations in which ACC is associated with a constellation of previously undescribed structural anomalies.
Food Hypersensitivity and Dietary Management in Atopic Dermatitis
Authors: Sampson Hugh A.
Published: March 2008

Abstract: Although immune dysfunction it known to play an integral rote In the development of atopic dermatitis, no clear delineation of the underlying pathogenic mechanism(s) responsible for this disorder has been proposed. Several factors are known to trigger flares of ttople dermatitis, in the extrinsic form of this disorder, food and airborne allergans may provoke Hares of eczema. Research Implicating food hypersensitivity in the pathogenesis of atopic dermatitis includes studies of food sliergen avoidance In newborn Infants at high risk for atopic disease, Investigations of children with blinded food challenges, and therapeutic trials of allergen‐elimination diets. Taken together, these studies demonstrate a significant pathogenic role for food hypesensltivity In about one‐third of children with atopic dermatitis.
Dyskeratosis Congenita Associated with Elevated Fetal Hemoglobin, X‐Linked Ocular Albinism, and Juvenile‐Onset Diabetes Mellitus
Authors: Reichel Martin , Grix Arthur C. , Isseroff R. Rivkah
Published: March 2008

An 11‐year‐old boy had dyskeratosis congenita, elevated fetal hemoglobin level, X‐linked ocular albinism, and juvenile‐onset diabetes mellitus. A review of the international literature revealed that elevated fetal hemoglobin has been noted in 15 reported cases of dyskeratosis congenita. It is a previously unrecognized, commonly associated finding in dyskeratosis congenita that may provide insight into the location and function of the gene for dyskeratosis congenita.
Anesthesia and/or Sedation for Pulsed Dye Laser Therapy
Authors: Rabinowitz Linda G. , B, Esterly Nancy , Frieden Ilona J. , Garbin Gregory S. , Grekin Roy C. , Stafford T. J. , Tan O. T. , Rabinowitz Linda G. , Goldberg Gerald N. , F, Rothman Karen , Roberts Lynne J. , Geronemus Roy G. , Mallory Susan B. , Garden Jerome M. , Morelli Joseph G. , L, Levy Moise , Cohen Bernard A.
Published: March 2008

One of the most exciting developments in pediatric dermatology has been the use of the flashlamp‐pumped, 585‐nm, pulsed dye laser for treatment of vascular birthmarks. In many cases the results are miraculous. The increase in self‐esteem and happiness of many children and adolescents has been overwhelming; for some, depression has been lifted, stuttering has ceased, social involvement has increased, and antidepressants have been discontinued. There are many success stories to tell.

Despite the remarkable effects of the pulsed dye laser and the medical and psychosocial indications for its use, the issue of pain control remains significant. We have no perfect outpatient pediatric anesthetic. Most methods carry either some risk or, if not hazardous, often are not very effective for controlling pain. Needless to say, a diversity of opinions exist on how to manage discomfort from this treatment modality. Therefore, we thought it would be useful to share the experiences and opinions of several dermatologists who have extensive experience with the pulsed dye laser.
Cutis Marmorata Telangiectatica Congenita: Long‐Term Follow‐up, Review of the Literature, and Report of a Case in Conjunction with Congenital Hypothyroidism
Authors: Pehr Kevin , Moroz Brenda
Published: March 2008

Abstract: Cutis marmorata telanglectatlca congenlta is an uncommon, generally congenital, cutaneous condition. The major skin findings are persistent, fixed cutts marmorata, telangiectasia, and phlebectasia; often, there is associated skin atrophy and ulceratton as well. Significantly, two‐thirds of patients have other congenital anomalles, although often minor ones. We report a series of eight children with cutis marmorata tefangiectatica congenlta, including one with associated congenital hypothyroidism, a relationship that has never before been noted.
Nonprogressive Scrotal Hair Growth in Two Infants
Authors: Slyper Arnold H. , Esterly Nancy B.
Published: March 2008

Abstract: Two infant boys developed scrotal hairs within the first three months of life. There was no other clinical or biochemical evidence of excessive androgen production, and no further progression of hair growth.
Rapp‐Hodgkin Syndrome with Pili Canaliculi
Authors: Camacho Francisco , Ferrando Juan , Pichardo Antonio R. , Sotillo Ismael , Jorquera Enrique
Published: March 2008

Abstract: A 20‐year‐old woman and her 12‐year‐old brother had hypohidrotic ectodermal dysplasia, cleft lip and palate, midfacial hypoplasia with narrow nose from the nasal bridge to the tip, narrow dysplastic nails, and conical teeth and hypodontia, and hypospadias and hypoplastic uvula in the boy. The woman had major underdevelopment of intellectual capacity. The most important hair anomalies in both siblings were sparse eyebrows, pili torti, and pili canaliculi. Some of the pili canaliculi had two canals (pili bicanaliculi), and the cross section for scanning electron microscopy had a quadrangular aspect. This is the seventh family reported with Rapp‐Hodgkin ectodermal dysplasia.
Dermoid Cyst Connected with the Lacrimal Canaliculum
Authors: Orozco‐Covarrubias MA. De La Luz , Salazar‐Leon Jose A. , Tamayo‐Sanchez Lourdes , Duran‐McKinster Carola , Ruiz‐Maldonado Ramon
Published: March 2008

Abstract: An infant had a 3 × 1.5‐cm congenital, slow‐growing, lobulated tumor below the right inferior eyelid. The mass transilluminated, and needle aspiration yielded tears. Dacryocystography showed a large cystic area connected with the lower lacrimal canailcuium. Histoiogicaily, the tumor proved to be a dermoid cyst. This association has not been reported previoualy. Surgical excision and suture of the pedicuie resulted in permanent cure.
Papular Xanthoma in Children: Report and Immunohistochemical Study
Authors: Fonseca E. , Contreras F. , Cuevas J.
Published: March 2008

Abstract: Papular xanthoma was diagnosed in a 14‐month‐old boy. The eruption cleared spontaneously within four years, immunohistopathologic study revealed that the predominant foamy histiocytic cells had the phenotypic features of the dermal dendrocyte. Giant multinucleated cells, which showed characteristics of monocyte‐derived macrophages, were also present. We suggest classifying diseases derived from dermal dendrocytes into a separate group of histiocytoses.
Congenital Smooth Muscle Hamartoma of the Skin
Authors: Gagné Eric J. , Daniel Su W. P.
Published: March 2008

Abstract: : A 2‐month‐old white girl had a congenital, tan‐colored, slightly elevated, 3 × 2‐cm plaque on the left midback. Clinically, the lesion was suspected to be a solitary masiocytoma. Rubbing the lesion produced a transient erythema and edema that was similar to Darier sign seen in mastocytoma. Analysis of a skin biopsy specimen revealed a smooth muscle hamartoma; special stains did not show evidence of increased numbers of mast cells. This relatively uncommon condition can be confused with a variety of other cutaneous diseases. Light microscopic examination of a skin biopsy specimen establishes the diagnosis. A review of the approximately 50 cases reported in the literature showed that there is no known associated systemic involvement or malignant transformation. The clinical lesions usually become less prominent with time.
Linear and Whorled Nevoid Hypermelanosis
Authors: Alvarez J. , Peteiro C. , Toribio J.
Published: March 2008

Abstract: A case of hyperpigmentation distributed in streaks and whorls was recently delineated and separated from a confusing number of pigmentary disorders, A 13‐year‐old boy had hyperpigmented, reticu‐late spots on his trunk and neck in a linear and whorted distribution fol‐lowing the lines of Blaschko. Onset was at age 1 year. Histologically, the spots showed increased melanin In the basal layer of the epidermis, but no increase In melanocytes, Incontinence of pigment, or melanophages were seen in the dermis. The karyotype was 46, XY and there was no evi‐dence of chromosomal mosaicism or chlmerlsm. This entity represents a disorder separated recently from other pigmentary alterations that follow Blaschko lines, such as Bioch‐Sulzberger incontinentia pigmentl, hy‐pomelanosis of ito, early systematized epidermal nevus, and chimerism.
Encephalocraniocutaneous Lipomatosis: Case Report and Review of the Literature
Authors: Grimalt R. , Ermacora E. , Mistura L. , Russo G. , Tadini G. L. , Triulzi F. , Cavicchini S. , Rondanini G. F. , Caputo R.
Published: March 2008

Abstract: Encephalocraniocutaneous Hpomatosis is a congenital disorder characterized by unilateral cerebral malformations and ipsilateral scalp, face, and eye lesions. Distinguishing histopathologic features are dysgenesis and neoplasia of the adipose tissue. A Caucasian boy had soft tumors and elastic papules on his head since birth, associated with atrophic areas, and a bllobed lesion on the upper right eyelid. On the bulbar conjunctiva of the right eye, an oval 6‐mm lesion was present. Ultrasonogram, computerized tomographic scan, and magnetic resonance imaging revealed a dilation of the right lateral ventriculus, a mass on the pontocerebellar angle, agenesia of the corpus callosum, an arachnoldal cyst on the right hemisphere, microcalcifications, and pachygyria. The histology of a soft cutaneous tumor was consistent with a fibrollpoma, and dispersed extracellular lipid globules in the upper dermis were found on electron microscopy. The diagnosis suggested by these findings was encephalocraniocutaneous lipomatosis. Even in view of the rarity of the syndrome (11 cases described in the literature), this patient seems unusual because of the bilateral distribution of the cutaneous lesions and because of the agenesia of the corpus callosum. The peculiar ultrastructural findings require further confirmation.
Recurrent Giant Aphthous Ulcers in a Child: Protracted Treatment With Thalidomide
Authors: Menni Silvano , Imondi Dario , Brancaleone Wilma , Croci Simona
Published: March 2008

Abstract: A child affected by glant recurrent aphthous ulcers was treated successfully over the long term with thalidomide, with no adverse reactions or reduction of therapeutic efficacy. The use of thalidomide in children for serious aphthosis is proposed.
Perniosis in Association With Anorexia Nervosa
Authors: White Kathleen P. , Rothe Marti J. , Milanese Ann , Grant‐Kels Jane M.
Published: March 2008

Abstract: Several dermatologic abnormalities have been described In anorexia nervosa, but only rare associations have been made with pernlosis. We recently saw two teenage girls and one woman with anorexia nervosa who had symptoms of perniosis. We suggest that altered ther moregulation and a hyperreactive peripheral vascular response to cold in anorexia nervosa may predispose these patients to permiosis.
Pyoderma Gangrenosum in Infants and Children
Authors: Graham Jean A. , Hansen Kathryn Kay , Rabinowitz Linda G. , Esterly Nancy B.
Published: March 2008

Abstract: Pyoderma gangrenosum is an uncommon ulcerative skin disorder that occurs in all age groups. Approximately 4% of patients are infants and children. There are several notable differences between the childhood and adult manifestations of the disease, including the distribution of lesions and associated disorders. We reviewed the childhood cases (≤18 yrs of age) of unequivocal pyoderma gangronosum in the English literature and tabulated the trends in clinical features, associated disorders, and therapy. We report our 3‐week‐old patient, the youngest documented case. Of the 46 patients, only 4 were less than 1 year of age. A systemic Illness was present in 74% of the older children, most commonly, ulcerative colitis. Only one Infant had an associated problem (HIV+) at the time of onset. Infants appear to have an unusual distribution of perianal and genital lesions not often described in other age groups. Our review suggests that pyoderma gangrenosum in children has a similar clinical appearance to that in adults. It Is associated with some of the same underlying disorders, but with different frequencies. The distribution of lesions in children Is similar, often involving the lower extremities, but pyoderma gangrenosum of the head and face appears to be more common in children. Infants may have ulcers in genital and perianal areas. The most frequently prescribed treatment for children is systemic corticosteroids, which generally are very effective.
Infantile Systemic Hyalinosis in a Black Infant
Authors: Sahn Eleanor E. , Salinas Carlos F. , Sens Mary Ann , Key Janice , Swiger Frederick K. , Holbrook Karen A.
Published: March 2008

Abstract: A black girl was born with flexion contractures and experlenced pain on movement by 1 week of age. She subsequently developed perioral papules, gingival hyperplasla, perianal nodules, torticollis, dlarhea, rectal prolapse, and inability to open her mouth. Her skin became increasingly sclerodermatous, and velvety, hyperpigmented plaques arose over bony prominences. A skin biopsy specimen snowed hyallne material in the papillary dermis with tack of elastic fibers. Ultrastructural examination revealed fibrilIogranular material around fibroblasts and blood vessels, This child had the clinical, histologic, and ultrastructural features of Infantile systemic hyalinosis. This disorder has not been described in a black infant. Previous case reports of infantile systemic hyalinosis are reviewed and unusual features of our case are discussed.
Management of a Young Patient With Xeroderma Pigmentosum
Authors: Leal‐Khouri Susana , Hruza George J. , Hruza Luciann L. , Martin Ann G.
Published: March 2008

Abstract: Xeroderma pigmentosum is a group of rare autosomal recessive disorders with defective DNA repair that provide insight into the basic mechanism of carcinogenesis. It is the best human model linking clinical abnormalities and neoplasla to carcinogen exposure. We describe a patient with xeroderma pigmentosum and numerous basal cell carcinomas, squamous cell carcinomas, and melanomas treated with radiation therapy, Mohs micrographic surgery, dermabrasion, and isotretinoin prophylaxis.
Unusual Presentation of a Third Ventricular Cyst in a Patient with Basal Cell Nevus Syndrome
Authors: Snider Rebecca L.
Published: March 2008

Abstract: Patients with basil roll nevus syndrome often have neurologic manifestations that occasionally cause complications. I report a case of a third ventricular cyst causing unusual symptoms in a 16‐year‐old boy. The discussion includes a review of the various neurologic signs and symptoms in this syndrome.
Cutaneous Histiocytosis with Langerhans Cell eatures Induced by Scabies: A Case Report
Authors: Talanin Nickolai Y. , Smith Stuart S. , Shelley E. Dorinda , Moores William B.
Published: March 2008

Abstract: An infant with biopsy‐proven scabies developed nodular lesions. Histopathology revealed atypical histiocytes with Langerhans cell features. Within six months after treatment all skin lesions gradually disappeared. We suggest that the nodules In scabies can be due to Langerhans cell proliferation.
Toxic Epidermal Necrolysis Associated with Klebsiella Pneumoniae Sepsis
Authors: Picard Elie , GUlis David , Klapholz Laurent , Schreiber Laetitia , Engelhard Dan
Published: March 2008

Abstract: Toxic epidermal necrolysis (TEN) is a rare condition In childhood usually attributed to drugs. We describe a 4‐month‐old infant who developed typical clinical and histologic findings of TEN concomltantly with Klebsiella pneumoniae sepsis. We emphasize that in cases of acute, severe exfoliative disease in infants, apart from staphylococcal infection, gram‐negative bacterial sepsis must also be considered.
Prevalence of Nail Abnormalities in Children With Alopecia Areata
Authors: Tosti Antonella , Morelli Rossella , Bardazzi Federico , Peluso Anna Maria
Published: March 2008

Abstract: We assessed the prevalence of nail abnormalities in 272 children with alopecia areata who were seen in our department during an eight‐year period. Of these, 126(46%; 50 girls, 76 boys) had nail abnormalities that were related to alopecia areata. Nail pitting was detected in 92 patients, Including 37 with alopecia totalis or alopecia universalis. Three patients experienced an onychomadesis of all 20 nails during the acute onset of alopecia areata universalis. Thirty‐two (11.7%) had nail thinning and severe nail plate surface abnormalities that were consistent with a diagnosis of trachyonychia.
Epidemiology of Skin Disease Among Children in a Referral Skin Clinic in Singapore
Authors: Goh C.L. , Akarapanth R.
Published: March 2008

Abstract: This retrospective epidemiologic study compared data of skin disease in children less than 16 years old with those of adults attending the same tertlary referral outpatient clinic. of the 74,589 patlents seen in this clinic, 9273 (12.4%) were children. There were significant differences in the types of skin disease between children and adults. In children, eczema was the most common (50%), followed by viral infection (7%), bacterial infection (5%), and insect bite reaction (5%), significantly higher proportions than in adults. in contrast, acne vulgaris (3%), fungal infection (3%), and psoriasis (1.1%) rates were significantly lower in children than in adults. Atopic eczema (51%), followed by endogenous hand foot eczema (9.6%) was the most common type of eczema in children. The number of children with atopic (including asteatotic eczema) and sebor rhoeic eczema decreased with age, suggesting that these conditions tend to clear with age, whereas other endogenous eczemas, including hand foot eczema, nummular eczema, and lichen simplex chronicus, tend to increase with age. Epidemiologic data are useful in monitoring changes in disease trends in children and planning health care programs for them.
Atypical Varicella Exanthems Associated With Skin Injury
Authors: Belhorn Thomas H. , Lucky Anne W.
Published: March 2008

Abstract: varicella is a common childhood disease with a typical exanthem. We present four children with severe, localized disease, all associated with some form of trauma to the skin during the incubation period: a 3½‐year‐old boy sustained wasp stings on the hand, a 5‐year‐old boy received extensive sun exposure, a neonate had latrogenic trauma to her arm, and a 13‐year‐old boy underwent knee arthroscopy and was wearing a cast. It is postulated that such injuries to the skin either allowed more virus‐infected cells to enter the skin at the sites, or that factors such as insect venom and ultraviolet light altered local immunity to varicella zoster virus.
Benign Cephalic Histiocytosis: Case Report and Literature Review
Authors: Peña‐Penabad Carmen , Unamuno Pablo , Garcia‐Silva Jesús , Ludeña M. Dolores , Armijo Miguel
Published: March 2008

Abstract: A 3‐year‐old boy had maculopapules on his face and neck since age 6 months. These were yellow‐brown, asymptomatic, and clinically similar to flat warts. Histopathologic study revealed a fibrohistiocytic infiltrate in the superficial dermis. Ultrastructurally, comma‐shaped bodies, desmosome‐like junctions, and coated vesicles were seen; there were no lipid droplets or Birbeck granules. With these data, a diagnosis of benign cephalic histiocytosis was made. Twenty‐five cases are reported in the literature: 17 males and 8 females (male:female ratio 2:1). Sixteen patients had lesions on parts of the body other than the head, neck, and shoulders.
Perianal Streptococcal Dermatitis Associated With Guttate Psoriasis and/or Balanoposthitis: A Study of Five Cases
Authors: Patrizi Annalisa , Costa Angela Maria , Fiorillo Loretta , Neri Iria
Published: March 2008

Abstract: Perianal streptococcal dermatitis (PSD) is a recently described cutaneous entity caused by group A β‐hemolytic streptococci. It is characterized by perianal erythema, sometimes associated with functional disturbances. We describe four children (2 boys, 2 girls) who had acute guttate psoriasis and also PSD. One of these patients also had balanoposthitis. A fifth patient experienced an association of PSD and balanoposthitis without psoriasis. To our knowledge, the association between guttate psoriasis and PSD has only been reported in five children, and the one with balanitis has not been previously reported.
Ulerythema Ophryogenes and Keratosis Pilaris in a Child with Monosomy 18p
Authors: Zouboulis Ch. C. , Stratakis C. A. , Rinck G. , Wegner R. ‐D. , Gollnick H. , Orfanos C.E.
Published: March 2008

Abstract: We report a 13‐year‐old boy with deletion of the short arm of chromosome 18 and follicular, partialy inflammatory, keratotic papules of the eyebrows, foreskin, and cheeks (ulerythema ophryogenes) as well as the shoulders, upper back, upper arms, and thighs (keratosis pilaris), initially diagnosed as atopic dermatitis. Over 100 patients with this genetic defect have been reported, and the 18p‐ syndrome is considered one of the most frequently occurring deletion syndromes. However, ulerythema ophryogenes and keratosis pilaris have not been described in any of these patients, although the association of the latter with other genetic abnormalities is well known. Keratosis pilaris is a relatively common genodermatosis of ectodermal origin, frequently occurring with ichthyosis or atopy; concomitance with ulerythema ophryogenes has also been reported. The association of chromosome 18p deletion defect and ulerythema ophryogenes may be helpful in future attempts to localize the gene defect responsible for follicular genokeratoses.
Giant Congenital Juvenile Xanthogranuloma
Authors: Magana Mario , Vazquez Ruth , Fernández‐Díez Jorge , Flores‐Villa Rebeca , Cazarín Jorge
Published: March 2008

Abstract: Congenital giant juvenile xanthogranuloma is an uncommon lesion that apparently has benign biologic behavior in most reported patients. In one of our three patients it was necessary to perform histochemical reactions with CD 1 (‐), MAC 387, and electron microscopy to rule out Langerhans cell histiocytosis. The other patients had a typical histologlc picture, with foamy histiocytes and Touton cells in association wtth lymphocytes and eosinophils. Comments about treatment are also made.
Porokeratosis of Mibelli: Rapid Recurrence of a Large Lesion After Carbon Dioxide Laser Treatment
Authors: McCullough Toni Lewis , Lesher Jack Leiter
Published: March 2008

Abstract: Porokeratosis of Mibelli was diagnosed in a 9‐year‐old Afro‐American girl. The lesion was rather large (4.1 x 2.0 cm) and had developed rapidly over the course of six months. Considering the potential for malignant transformation without neglecting the importance of cosmesis, carbon dioxide laser vaporization with curettage was chosen to ablate the lesion. Within two months the entire treated area revealed recurrent porokeratosis. Due to the aggressive nature of this lesion, complete excision was performed.
Golf Tee Hairs in Netherton Disease
Authors: de Berker David A. R. , Paige David G. , Ferguson David J. P. , Dawber Rodney P. R.
Published: March 2008

Abstract: We present a case of r4etherton disease, where the hairs lacked the characteristic microscopic feature of Uichorrhexis invaginata. In its piace were certain hairs with a goif tee tnorphoiogy. These represent the proximal half of the invaginate node seen in typicai Netherton disease. Scanning eiectron microscopy demonstrated the three‐dimensiona) quality of this abnormaiity, which is subtie when assessed by iight microscopy aione. We describe this sign so that it might be recognized when seen in isolation, as here, and aiiow tire diagnosis of Netherton disease to be confirmed.
Branchio‐Oculo‐Faeial Syndrome and Dermal Thymus: Case Report and Review of the Literatere
Authors: Rizzo Renata , Micali Giuseppe , Calvieri Stefano , Sorge Giovanni , Mazzone Domenico
Published: March 2008

Abstract: We report a patiënt affected by the rare branchio‐oculo‐facial syndrome. Histologic study of the branchial clefts showed thymic remnants. A scanning electron microscopic study of the hair was also performed. The clinical features of the patiënt were compared with those of other patients reported in the literature.
Pyoderma Gangrenosum and Sterile Multifocal Osteomyelitis Preceding the Appearance of Takayasu Arteritis
Authors: Dagan Ovadia , Barak Yigal , Metzker Aryeh
Published: March 2008

Abstract: A 9‐month‐old infant had pyoderma gangrenosum (PG) and sterile osteomyelitis. Three years later the patient developed Takayasu arteritis (TA). Sterile osteomyelitis was reported in approxitnately 30 patients with different skin lesions, but never together with pyoderma gangrenosum. The association of PG and TA has been reported rarely, and then maiitly in adults or in children over age 9 years. This rare association should alert physicians with a case of infantile PG of unknown etiology to consider the possible diagnosis of TA.
Subcutaneous Fat Necrosis, Hypercalcemia, and Prostaglandin E
Authors: Sharata Harry , Postellon Daniel C. , Hashimoto Ken
Published: March 2008

Abstract: We present two patients with subcutaneous fat necroses (SCFN) in whom endocrinologie studies revealed an association with elevated prostaglandin E (PGE) levels. A boy born after prolonged labor complicated by meconium aspiration developed erythematous, indurated plaques over the back, arrns, buttocks, and cheeks at 4 days of age. A biopsy specimen of involved skin showed panniculitis with foei of necrotic adipocytes containing radially arranged, needle‐shaped clefts and a granulomatous infiltrate in the septae. Laboratory studies revealed hypercalcemia of 13.6 mg/dl (normal 8.8–10,1 mg/dl), eievated 1,251,25(OH)2D3, and increased urinary excretion of PGE2. The child was hospitalized and treated with systemic steroids and diuretics, with resolution of SCFN and hypercalcemia. The second patient was a girl born with cyanotic heart disease. A diagnosis of Ebstein anomaly was made, and intravenous PGE, was started to keep patent the ductus arteriosus. Four days later erythematous, indurated plaques were noted on the knee, back, and anterior chest. A skin biopsy specimen revealed SCFN. There was no associated laboratory abnormality. On discontinuing PGE, no new tesions formed and the existing panniculltis resolved. These two cases demonstrate the association between SCFN and eievated PGE levels (endogenous in patiënt 1, exogenous in patiënt 2). No previous reports of SCFN after the administration of PGE, have appeared in the literature.
Treatment of Childhood Dermatophyte Infections with Oral Terbinafine
Authors: Goulden V. , Goodfield M. J. D.
Published: March 2008

Abstract: Over the past four years terbinafine has become established as an effective systemic antimycotic agent with an excellent safety profile. However, experience with its use in children is very limited. We report the effective treatment of five children wïth oral terbinafine.
Ichthyosiform Eruption in a Patient with Dubowitz Syndrome
Authors: Kato Taizo , Komatsu Hiromi , Sakakibara Akihiro , Tagami Hachiro
Published: March 2008

Abstract: Dubowitz syndrome is a rare autosomal recessive disorder characterized by intrauterine and postnatal growth retardation, microcephaly, moderate mental retardation, typical craniofacial anomalies, and eczematous skin lesions. We report a case of Dubowitz syndrome associated wtth ichthyosiform skin changes.
Infantile (Desmoid‐Type) Fibromatosis
Authors: Keltz Miriam , DiCostanzo Damian , Desai Panna , Cohen Steven R.
Published: March 2008

Abstract: Infantile (desmoid‐type) fibromatosis is an uncommon, locally aggressive tumor that is seldom reported in the dermatologic literature. We describe a case of Infantile (desmoid‐type) fibromatosis in a 2‐year‐old girl. Surgical disposition entailed amputation of the affected digit Our report is intended to heighten awareness of this potentially fatal tumor, and emphasize the importance of timely diagnosis and treatment.
Mothers' Knowledge and Practices Related to Sun Protection in Greece
Authors: Kakourou Talia , Bakoula Chryssa , Kavadias G. , Gatos A. , Bilalis L. , Krikos X. , Matsaniotis N.
Published: March 2008

Abstract: We attempted to estimate the level of Greek mothers' knowledge relating to the harmful effects of sunlight and whether this knowledge led to protective measures for them and their children. Between September and November 1993, 315 mothers were randomly selected from the outpatient department of our hospital and interviewed by questionnaire about themselves and their children (56% boys, 44% girls, ages 1–12 yrs). Knowledge was estimated by an index score that for 28% of the mothers was considered poor, for 50% moderate, for 16% good, and for only 6% very good or excellent. The score was positively associated with parent education, urban residence, mother's job relevant to the cosmetics industry or the mass media, and history of sunburn in one or both parents. Scores were also established for sunlight‐protective measures taken for themselves (28% poor, 45% moderate, 27% just good) and for their children (24% poor, 46% moderate, 30% just good). These scores were significantly associated only with mothers' knowledge of sun protection. Mothers who used sun protection for themselves also applied it to their children. This study shows that mothers in Greece should be encouraged both to increase their knowledge of sun protection and steadily incorporate it into their knowledge of sun protection and steadily incorporate it into their lifestyle.