bluuebunny/crossref_metadata_2025_split huggingface dataset:
Dermatomyositis and acquired ichthyosis as paraneoplastic manifestations of ovarian tumor
Authors: Roselino Ana Maria F. , Souza Cacilda S. , Andrade Jurandyr M. , Tone Luiz G. , Scares Fernando A. , Llorach‐Velludo Maria Angeles S. , Foss Norma T. 
Published: August 2010

Case 1 A 37‐year‐old white woman was seen at the Dermatology Clinic presenting muscle weakness of the pelvic and scapular girdles, as well as a violaceous erythema and periorbital infiltration (heliotropium) and erythematous‐violaceous lesions on the dorsum of the finger joints, with periungual telangiectasia. A diagnosis of dermatomyositis was confirmed and treatment with prednisone, 1 mg/kg per day, was started, with improvement.One year and two months later she presented ascites, bilateral pleural effusion, and a 7‐kg weight loss. Cytology of the ascitic fluid revealed cells compatible with adenocarclnoma. Exploratory laparotomy demonstrated peritoneal carcinomatosis, and an implant biopsy confirmed a moderately differentiated adenocarcinoma of the ovary of the endometriold type. The patient died 1 year and 4 months after the diagnosis of dermatomyositis (Fig. 1 and Table 1). Case 2 An 8‐year‐old white girl was seen at the Dermatology Clinic presenting generalized ichthyotic skin of a brovi/nish color, perilabial and palmo‐plantar fissures of 5 months duration, and hair fall. For the last 2 months, she had been presenting daily fever (38°C) and lack of appetite, and had lost 7 kg.Abdominal ultrasound revealed a large pelvic mass (9 × 8 × 7 cm) located in a small basin, with possible right renal invasion, as well as periaortic and pericaval adenomegaly, findings that were later confirmed by computerized tomography. Radiologic examinations of the thorax, skull, spine, and long bones were normal. Exploratory laparotomy confirmed the presence of a dysgerminoma of the right ovary as determined by biopsy (Fig. 2).Chemotherapy was started (carboplatin, bleomycin, and vinblastine) and an abdominal ultrasound taken 1 5 days later showed a 67.5% reduction of the pelvic mass, as well as reduction of rectoperitoneal adenomegaly.
T‐cell lymphoma with remarkable muscle involvement
Authors: Saeki Hidehisa , Torii Hideshi , Ogata Fuyuki , Furue Masutaka , Nakagawa Hidemi , Ishibashi Yasumasa 
Published: August 2010

A 31‐year‐old Japanese man presented to our hospital in December 1990 with swelling of the lower lip and cheek which he had noticed 8 months earlier. Before his first visit to us, systemic corticosteroids had been administered for a week. This treatment resulted in remarkable improvement of the swelling of both sites; however, after discontinuation of these corticosteroids, recurrent episodes of the swelling occurred. Our examination revealed an enlarged, soft, nontender lower lip and a swollen cheek (Fig. 1). Bilateral hilar lymphadenopathy was not found by a chest radiograph. Computed tomography of the abdomen was normal. Laboratory investigations disclosed that lactic dehydrogenase was 195 U/L and that angiotensin converting enzyme (ACE) was 29.4 IU/L. The antibody against HTLV‐I was negative and a biopsy of the lower lip revealed a relatively dense infiltration of the inflammatory cells composed mainly of lymphocytes and histiocytes. Atypia of lymphocytes was not prominent and some muscle fibers showed degenerative changes (Fig. 2). A skin biopsy of the cheek disclosed a dense lymphohistiocytic infiltrate in the dermis. Based on the clinical findings, we tentatively diagnosed this case as cheilitis granulomatosa and reinstituted systemic corticosteroid therapy.In December 1991 the patient was admitted for evaluation of muscle weakness of the forearms. Electric myography revealed a myogenic pattern. He showed neither a heliotrope eruption nor Gottron's papules. Laboratory investigation showed that the white cell count was 14 600/mm3 (lymphocytes 27%), creatinine phosphokinase (CPK) was 679 U/L, and ACE was 28.0 IU/L. A biopsy of the deltoid muscle disclosed muscle atrophy and a dense atypical lymphocytic infiltrate around the muscle fibers (Fig. 3). Immunohistochemical findings revealed that most of the Infiltrating cells were CD3(+) and CD4(+). Although molecular biologic investigation did not disclose the monoclonality of the gamma T‐cell receptor gene, this time we diagnosed the case from the histologic findings as T‐cell lymphoma. Needle biopsy of the bone marrow suggested the infiltration of lymphoma cells. In January 1992, chemotherapy of MACOP‐BV (MTX, doxorubicin hydrochloride, CPA, VCR, prednisolone, BLM, and etoposide) was started, but we were unable to prevent the progression of muscle weakness, and in April 1992 the patient died of pneumonia. No autopsy was performed.
Pseudo‐melanoma revealing Laugier‐Hunziker syndrome
Authors: Porneuf Marc , Dandurand Michel 
Published: August 2010

A 47‐year‐old Arabic woman presented with a few months history of a 20 × 10 mm brown‐black interdigital macule, irregular in shape and outline (Fig. 1). The patient had no previous personal or familial history of dysplastic nevi, melanoma, or gastrointestinal polyposis. She was receiving fluindione, furosemide, digoxin. and lisinopril dihydrate for chronic atrial fibrillation. She had a mitral valvular bioprothesis secondary to rheumatic heart disease.On physical examination there were no enlarged lymph nodes. Multiple pigmented macules were noted on the lower lip and buccal mucosa (Fig. 2). Genital mucosa showed no pigmentary abnormalities. There were also many pigmented longitudinal streaks involving five finger‐ and two toenails without Hutchinson's sign, two brownish interdigital macules of the fingers (Fig. 3), and dark brown spots around the nails. Clinical examination of her two sisters and three children did not demonstrate mucocutaneous pigmentation.Investigations showed normal renal, hepatic, and thyroid function. Human immunodeficiency virus serology was negative. Two‐punch biopsy 4×4 mm of the right darkness and left edges of the interdigital lesion confirmed the diagnosis of Laugier‐Hunziker syndrome with basal hypermelanosis without melanocytic proliferation or cytologic atypia (Fig. 5). After 2 years follow‐up, clinical and histologic examination was not modified.
Omenn's syndrome
Authors: Nazzari Guido , Drago Francesco , Crovato Franco 
Published: August 2010

A 7‐month‐old boy presented with a 6‐month history of a skin eruption of the scalp and the diaper area (Fig. 1) resembling seborrheic dermatitis. Since the age of 3 months, he had developed high grade fever, diffuse pruritic skin lesions, stomatitis (Fig. 2), chronic diarrhea, diffuse lymphadenomegaly, and failure to thrive. The parents were nonconsanguineous and no similar disease had ever been reported in the family.Physical examination showed alopecia, generalized lymphadenopathy, and hepatosplenomegaly. The patient's skin was dry with generalized erythematous, scaling pruritic eruptions and small violaceous nodules on the extremities.Laboratory investigations revealed: slight normochromic anemia, leukocytosis (18,000/mm3) with lymphocytosis (42%), and mild eosinophilia (12%); decreased levels of all classes of serum immunoglobulins were observed with normal IgE levels. Examination of phagocytosis, opsonization, and granulocyte chemotaxis revealed no abnormalities. The number of circulating T cells was within normal limits, whereas that of B cells was low. Recall antigens and T‐ and B‐lymphocyte proliferative response to mitogens and Candida albicans proteins resulted in poor stimulation. Chromosomal analysis of mononuclear ceils from the peripheral blood and skin fibroblasts showed a normal male Kariotype, and HLA typing did not reveal any maternal chimerism.A skin biopsy specimen showed satellite cell necrosis of Keratinocytes and a dense lymphohistiocytic and eosinophilic perivascular infiltrate in the dermis. A specimen from a lymph node showed a picture consistent with dermatopathic iymphadenopathy, with an increase of cells with histiocytic appearance and a dense infiltrate of eosinophils. On electron microscopic examination, no cells with Birbeck's granulations were found in the skin infiltrate and lymph nodes. A bone marrow biopsy was normal.One month later the patient developed generalized exfoliative erythroderma with recurrent cytomegalovirus infections and staphylococcal septicemia. Despite antimicrobial and prednisone therapy, the patient died of toxic shock at the age of 9 months. An autopsy showed diffuse lymphohistiocytic infiltration involving the skin and lymph nodes and extreme lymphocytic depletion in the thymus. Meningitis and pneumonia were also present.
The Olmsted syndrome
Authors: Santos Omar Lupi R. , Amorim José Hunaldo , Voloch Karen , Goes Marcia C. , Silva Maria R. , Pereira Antônio Carlos 
Published: August 2010

A 35‐year‐old man had a hyperkeratotic disorder of the palmoplantar skin since the age of 6 months. The palmoplantar keratoderma progressed to thick and warty hyperkeratotic plaques, which enlarged and formed verrucous lesions and deep fissures. The acral keratoderma gradually involved the dorsal surface of the hands and feet with flexion contractures of the fingers and toes (Fig. 1). Since the age of 2 years, the patient also showed universal alopecia and small sharply marginated hyperkeratotic plaques around the nose and mouth, in the groin, and in the intergluteal area (Fig. 2). All of these keratotic lesions were strikingly symmetric.Examination of the oral mucosa revealed a white plaque over the left lateral border of the tongue; crusted white verrucous plaques were found at the labial commissures and underneath the lower lip. There was complete absence of the nails and the roentgenographic examination of the hand showed pronounced osteolysis of the distal phalanx of each of the fingers. The palmoplantar keratoderma has been complicated by the development of constricting bands of keratin around the bases of the fingers, leading to their strangulation and autoamputation (Fig. 1). The mental and physical appearance were normal. A family history of similar verrucous lesions was lacking. Histopathologic examination of the palmar skin showed massive parakeratotic hyperkeratosis and considerable acanthosis. In the thickened horny layer, many cells showed perinuclear edema. The patient was treated with topical retinoic acid cream, 0.1%, at night. This produced an improvement in the plaques around the nose and mouth.
Facial bullous systemic lupus erythematosus
Authors: MD Adriana Lalova , Pramatarov Kyrill , Vassileva Snejina 
Published: August 2010

A 37‐year‐old woman presented with a 6‐month history of malaise, fever, arthralgia, and mild butterfly facial erythema. A diagnosis of systemic lupus erythematosus (SLE) was established, based on clinical, laboratory, and histologic findings, and treatment with 6‐methylprednisolone (80 mg daily) was administered. A week later, a blistering eruption appeared in and around the erythematous areas on the face.On physical examination, infiltrated erythematous plaques were present on the sun‐exposed areas: the arms, dorsa of the hands, and upper chest. In and around the erythematous areas on the face, there were numerous tense vesicles (Fig. 1). A few ulcerations were observed on the lower lip and the hard palate.Laboratory findings revealed a reduced white blood cell count (WBC) (2500/mm3), with normal values of hemoglobin, red blood cell count (RBC), hematocrit, platelets, creatlnine, urea, total protein, blood glucose, and urinalysis. Antinuclear antibodies (ANA) were detected in titre of 1:320 on rat liver substrate. Anti‐DNA, Ro, La‐antibodies and LE‐cells were positive. Anti‐BMZ‐antibodies were not present on the indirect immunofluorescence (IIF) testing on normal human skin substrate. Histopathologic examination showed a subepidermal blister containing polymorphonuclears and eosinophils. There was a mixed Inflammatory infiltrate in the upper dermis (Fig. 2). Direct immunofluorescence (DIF) of perilesional skin from the face showed homogenous linear deposits of IgG and IgA at the basement membrane zone (BMZ); DiF from non‐sun‐exposed normal skin demonstrated granular deposits of IgA and IgM at the BMZ.Besides the systemic steroid therapy with 6‐methylprednisolone, a treatment with dapsone at an initial dosage of 50 mg, and later 100 mg, daily was applied. The condition was resistant to the steroid therapy, but after 4 weeks of treatment with dapsone the lesions completely resolved.
Lipodermatosclerosis: a postphiebitic syndrome
Authors: Sheth Rekha , Poonevala Vera 
Published: August 2010

A 30‐year‐old businessman, weighing 94 kg, presented with pigmentation and skin changes extending from the calf to the dorsum of the left foot. One year ago, the patient noticed erythema around the medial malleolus and edema of the ankle, following trauma while starting a bike. The patient was afebrile during the event. He gave no history suggestive of muscle weakness, cramping calf pains at rest, intermittent claudication while walking, paresthesia, or increased sensitivity to cold. No history suggestive of systemic involvement was obtained.On examination, the affected part showed pigmentary color changes varying from brown to bluish black (diffuse around the ankle and mottled towards the knee). The overlying skin was warm, indurated, brawny, and stony hard. Along with patchy hair loss, there was a painless decrease in the mobility of the left ankle. The circumference of the left calf and ankle measured approximately 2.5 cm less than the corresponding right side. The skin on the thigh above was hypertrophied and pachydermatous. There was no evidence of varicose veins.The blood count, urine analysis, erythrocyte sedimentation rate, bleeding, clotting, and prothrombin time were all within normal limits. Blood sugar levels were moderately elevated. Antinuclear‐antibodies were absent.Histopathology of the skin biopsy showed uniform thinning of the epidermis, with focal areas of increased pigmentation in the stratum basale. A sparse perivascular lymphocytic infiltrate was seen around the superficial and deep vessels of the retioular dermis. The collagen bundles were closely packed, homogeneous, thickened, and deeply stained. The subcutaneous tissue was replaced with collagen fibers, thus resembling a scleroderma‐like picture.Color Doppler showed evidence of segmental thrombosis in the left posterior tibial and peroneal veins.Before the dermatologic referral, the patient had been treated with intravenous heparin followed by tablet aspirin. We advised dietary restrictions for weight reduction and control of diabetes. In addition, we recommended vitamin supplements and compression stockings. The patient was taught limb care with special emphasis on leg elevation and exercise (to hasten the development of collaterals).
Papular umbilicated granuloma annulare in association with Alagille syndrome
Authors: Kibarian Maral A. , Mallory Susan B. , Keating James , Shitabata Paul 
Published: August 2010

A 10‐year‐old white boy with Alagille syndrome was referred to the dermatology clinic for evaluation of an eruption on the lower extremities. The lesions were mildly pruritic, gradual in onset, and increased in number over several months. The patient reported no insect bites, no unusual contacts, no history of chronic dermatologic disorders, and no recent febrile illness or systemic complaints. He had Alagille syndrome with cholestatic liver disease, hyperlipidemia, pulmonary artery stenosis, retinal pigment abnormalities, and myopia. His medications included rifampin and phenobarbital. There was no family history of Alagille syndrome; however, the patient's father had retinal cone abnormalities and a half‐sister had died of Aicardi syndrome.Physical examination revealed typical facies with hypertelorism, deep set eyes, and a sharply pointed chin. Dermatologic examination showed firm umbilicated papules symmetrically distributed on the dorsa of both feet and ankles (Fig. 1).The differential diagnosis included xanthomas, prurigo papules, and molluscum contagiosum. A skin biopsy revealed palisading necrobiotic granulomas consistent with granuloma annulare (Fig. 2).
Cytochrome P450 polymorphisms in patients with Behcet's disease
Authors: Tursen Umit , Tamer Lulufer , Api Hale , Yildirim Hatice , Baz Kiymet , İkizoglu Guliz , Atik Ugur 
Published: August 2010

AbstractObjectives Although the etiopathogenesis of Behcet's disease (BD) remains unknown, increased neutrophil functions such as chemotaxis, phagocytosis and excessive production of reactive oxygen species, including superoxide anion, may be responsible for the oxidative tissue damage observed in BD. Cytochrome P‐450 are a multigene family of enzymes involved in the detoxification and occasional activation of a wide variety of chemicals. Our aim was to investigate CYP2C9 and CYP2C19 polymorphisms in patients with BD.Methods Sixty‐two subjects with BD and 107 healthy control subjects were enrolled in the study. Polymorphisms of CYP2C9 and CYP2C19 were performed by real‐time PCR with a LightCycler instrument. We researched associations between CYP polymorphisms and BD.Results The frequencies of wild‐type and heterozygous CYP2C19*2 genotypes were 66.1% and 33.9% in the patients and 83.2% and 16.8% in the controls, respectively. There was a 2.53‐fold increased risk of Behcet's disease in individuals with the CYP2C19*2 heterozygous genotype (OR = 2.53; 95% CI, 1.22–5.25) when compared with the control group. But the CYP2C9*2, CYP2C9*3 and CYP2C19*3 gene polymorphisms were not related to an increased risk of developing BD.Conclusions We observed that patients with BD presented with a higher prevalence of the heterozygous CYP2C19*2 genotype. Hereditary deficiencies of this enzyme activity may lead to an imbalance between pro‐ and antioxidant systems, resulting in the formation of excessive reactive oxygen species.
Indigenous leishmaniasis in Taiwan: report of a case
Authors: Wang Jia‐Ru , Lee Sho‐Tone , Juan Wei‐Hsin , Chuang Wei‐Lin , Hung Shuen‐Iu , Chung Wen‐Hung , Hong Hong‐Shang 
Published: August 2010

AbstractTaiwan is not considered an endemic area of leishmaniasis. Imported cases are encountered infrequently, and only two cases of indigenous cutaneous leishmaniasis have been reported.1 We found one new case in the past 20 years. The patient presented with erythematous plaques on the nasal bridge and right thumb. Skin biopsy specimens from both sites revealed numerous Leishman–Donovan bodies in macrophages. There was no history of travel outside the country, and the diagnosis of indigenous cutaneous leishmaniasis was made. Polymerase chain reactions (PCR) identified the species as Leishmania tropica. The route of infection in this patient is unclear. Because pentavalent antimony, the drug of choice for leishmaniasis, is not available in Taiwan, the patient was treated with levamisole and potassium iodide, with an excellent response.
Solitary and multifocal oral melanoacanthoma
Authors: Yarom Noam , Hirshberg Avraham , Buchner Amos 
Published: August 2010

AbstractBackground  Oral melanoacanthoma is a rare pigmented lesion characterized by sudden appearance and rapid radial growth, mimicking malignant melanoma. Oral melanoacanthoma may present as a solitary or multifocal lesion; however, the characteristics of these two clinical variants have never been addressed. In this study, we present an unusual case of multifocal oral melanoacanthoma and analyze cases of oral melanoacanthoma reported in the literature, with special emphasis on multifocal lesions.Methods  A thorough MEDLINE search of the literature for cases of oral melanoacanthoma was performed. The demographic and clinical data, histologic features, and immunohistochemical findings were extracted from the full‐text articles.Results  The literature search yielded 52 patients with 67 lesions. The addition of our case increased this number to 53 patients with 72 lesions, 43 of whom had solitary lesions and 10 of whom had multifocal lesions. There was a female predominance amongst the patients with solitary oral melanoacanthoma (3 : 1), whereas multifocal oral melanoacanthoma showed an equal gender distribution (1 : 1). Multifocal lesions tended to occur more frequently on the palate, and solitary lesions on the buccal mucosa.Conclusions  Multifocal oral melanoacanthoma appears to demonstrate some demographic and clinical variations from the solitary type of lesion.
Are age‐specific high serum IgE levels associated with worse symptomatology in children with atopic dermatitis?
Authors: Hon Kam‐lun Ellis , Lam Man‐ching Adrian , Leung Ting‐fan , Wong Kin‐yee , Chow Chung‐mo , Fok Tai‐fai , Ng Pak‐cheung 
Published: August 2010

AbstractBackground Atopic dermatitis (AD) is a distressing disease associated with excoriations, pruritus, sleep disturbance, and elevation of serum total immunoglobulin E (IgE) levels.Objective To evaluate whether serum IgE levels correlate with the symptomatology and plasma chemokine levels in children with AD.Methods AD patients aged younger than 18 years were recruited from the pediatric dermatology clinic of a university teaching hospital, and the AD severity was evaluated using the SCORing Atopic Dermatitis (SCORAD) index. Concentrations of serum total IgE, eosinophil count, and plasma AD‐associated chemokines [cutaneous T‐cell‐attracting cytokine (CTACK), thymus and activation‐regulated chemokine (TARC)] were measured.Results One hundred and seventeen Chinese children with AD (64 boys and 53 girls), with an age (mean ± standard deviation) of 10.7 ± 4.4 years, were recruited. Their overall SCORAD index (mean ± standard deviation) was 51.1 ± 22.8. The total serum IgE level divided by the age‐specific upper limit (Æ) correlated well with the extent and intensity of AD, except for oozing/crusting, which was significant only in males. There was a significant correlation between Æ and pruritus or sleep loss only in females. Levels of IgE, CTACK, and TARC, and eosinophil count, differed significantly between patients with mild, moderate, and severe disease. Æ correlated well with TARC (r = 0.50,P < 0.001) and eosinophil count (r = 0.41,P < 0.001), but not with CTACK (r = 0.11,P = 0.270). The prediction of moderate to severe eczema by Æ gave an area under the receiver‐operating characteristic curve of 0.76 (95% confidence interval, 0.65–0.86;P = 0.004). An optimum positive predictive value of 94.2% was achieved with a cut‐off point of Æ of 2.95, sensitivity of 75.0%, and specificity of 66.7%.Conclusion Æ correlates significantly with various objective clinical scores and chemokine markers of AD, and is a useful indicator for predicting moderate to severe AD in children.