Systemic Involvement (systemic + involvement)

Distribution by Scientific Domains


Selected Abstracts


The periodontal abscess (I).

JOURNAL OF CLINICAL PERIODONTOLOGY, Issue 6 2000
Clinical, microbiological findings
Background/aims: Little information is available regarding the diagnosis and microbiology of periodontal abscesses. The aim of this descriptive clinical and microbiological study was to provide more information in order to help in the characterisation of the periodontal abscess associated to periodontitis. Method: 29 consecutive patients with a periodontal abscess were studied by the assessment of clinical variables, including both subjective (pain, edema, redness and swelling) and objective (bleeding on probing, suppuration, probing pocket depth, tooth mobility and cervical lymphadenopathy) parameters. Microbiological samples were taken for anaerobic microbiology and processed by means of culture. Systemic involvement was also studied through the analysis of blood and urine samples using conventional laboratory standards. Results: 62% of the abscesses affected untreated periodontitis patients, and 69% were associated with a molar tooth. More than 75% of the abscesses had moderate-severe scores related to edema, redness and swelling, and 90% of the patients reported pain. Bleeding occurred in all abscesses, while suppuration on sampling was detected in 66%. Mean associated pocket depth was 7.28 mm, and 79% of teeth presented some degree of mobility. Cervical lymphadenopathy was seen in 10% of patients, while elevated leucocyte counts were observed in 31.6%. The absolute number of neutrophils was elevated in 42% of the patients. High prevalences of putative periodontal pathogens were found, including Fusobacterium nucleatum, Peptostreptococcus micros, Porphyromonas gingivalis, Prevotella intermedia and Bacteroides forsythus. Conclusions: The periodontal abscess has clear clinical characteristics and is usually associated with severe periodontal destruction. This condition may cause systemic involvement and the lesion generally has a large bacterial mass with a high prevalence of well-recognised periodontal pathogens. [source]


Clinicopathologic profile of normocomplementemic and hypocomplementemic urticarial vasculitis: a study from South India

JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 7 2008
CVP Dincy
Abstract Background, This study aims to study the clinical and histopathological characteristics of hypocomplementemic and normocomplementemic urticarial vasculitis (HUVS and NUV) among dermatology clinic attendees in a tertiary care hospital in South India. Patients and methods, A prospective study was conducted in the dermatology department from February 2003 to May 2004. Seventy-five patients met the inclusion criteria for UV. Sixty-eight patients in whom complement levels were available were classified into either NUV or HUVS groups. Clinical features, laboratory parameters and histological features were compared, and the significance of differences was established using Pearson's Chi-squared test. Results, There was a female preponderance among patients with HUVS. Wheals > 24 h were seen in 90% of patients, and in 54.4% of patients, the wheals were partially blanching or non-blanching. Angioedema was more prevalent in patients with NUV than HUVS (44.4% vs. 21.4%). Systemic involvement was seen in 64.3% of patients with HUVS and 44.4% of patients with NUV. Fever, ANA positivity and systemic lupus erythematosus (SLE) were significantly associated with HUVS. In most cases of UV, a provoking factor could not be identified. Neutrophilic small vessel vasculitis was seen in 42.9% of patients with HUVS and 16.6% patients with NUV. Direct immunofluorescence test showing immunoreactants at the dermo-epidermal junction were present in 60% of patients with HUVS and 33.3% patients with NUV. Conclusion, The clinical features of Indian patients with UV were similar to those reported from the West. Fever, ANA positivity and SLE were significantly associated with HUVS. [source]


Surgical repositioning of a traumatically intruded permanent incisor in a patient with rheumatic fever: case report

DENTAL TRAUMATOLOGY, Issue 1 2009
Rosana Sales Dias
However, it is one of the most severe types of dentoalveolar trauma. By definition, intrusive luxation consists of the axial displacement of the tooth into the alveolar bone, accompanied by comminution or fracture of the alveolar bone. Here we report the treatment management of a traumatically intruded immature permanent central incisor by surgical repositioning undertaken in a 10-year-old child with rheumatic fever 10 days after sustaining a severe dentoalveolar trauma. The intraoral examination showed the complete intrusion of the permanent maxillary right central incisor and the radiographic examination revealed incomplete root formation. Prophylactic antibiotic therapy was prescribed and the intruded tooth was surgically repositioned and endodontically treated thereafter. The postoperative course was uneventful, with both clinically and radiographically sound conditions of the repositioned tooth up to 3 years and 2 months of follow-up. These outcomes suggest that surgical repositioning combined with proper antibiotic prophylaxis and adequate root canal therapy may be an effective treatment option in cases of severe intrusive luxations of permanent teeth with systemic involvement. [source]


Detection of a subset of CD30+ anaplastic large cell lymphoma by interphase fluorescence in situ hybridization

DIAGNOSTIC CYTOPATHOLOGY, Issue 2 2003
Hyung Ju C. Shin M.D.
Abstract T/null-cell anaplastic large cell lymphoma (ALCL) is a morphologically and clinically heterogeneous group of non-Hodgkin's lymphoma; to date several morphologic variants have been described on histologic specimens. However, the cytologic features of these variants in the fine-needle aspiration (FNA) specimens have not been well evaluated. The t(2;5)(p23;q35) has been identified in a subset of T/null-ALCL and is known to be associated with a favorable prognosis. We reviewed the cytomorphologic characteristics in 24 FNA specimens of ALCL. In all cases, the diagnosis was confirmed on histologic specimens, and immunohistochemical studies for anaplastic lymphoma kinase (ALK) protein expression were performed on the aspirates. The presence of ALK breakpoints were evaluated in nine cases, using a DNA break-apart probe on chromosome 2 covering the ALK gene by fluorescence in situ hybridization (FISH) techniques. Two hundred cells per case were examined. The results were expressed as the percentage of cells containing more than two signals of chromosome 2 to the total number of cells counted. FNA sites included lymph nodes (20), lung (2), breast (1), and soft tissue (1). The median age of the patients was 56 yr (range, 17,75 yr). Twenty cases had systemic involvement; in four cases, skin was the primary site with secondary involvement of the lymph nodes. All cases were CD30+ by immunohistochemistry; 20 were of T-cell phenotype and 4 were null cell type. The cytologic evaluation revealed typical anaplastic morphology (common type) with many "hallmark cells" in 16 (67%) cases. Other morphologic variants identified were small cell pattern in five cases, monomorphic pattern in two cases, and lymphohistiocytic pattern in one case. FISH studies showed that six (66.7%) of nine cases had at least two signals of chromosome 2, consistent with ALK breakpoints. With careful cytomorphologic evaluation in conjunction with appropriate immunohistochemical studies, a diagnosis of ALCL can be confidently made in the FNA specimens in the cellular aspirates and its morphologic variants also can be recognized. Furthermore, the FNA specimen is suitable in detecting ALK breakpoints by FISH study, permitting rapid identification of a subset of patients with ALCL, who may have a favorable prognosis. Using a commercially available probe, detection of ALK breakpoints in the FNA specimens is simple and can be a useful diagnostic adjunct in cases where distinction from other lymphomas or lymphoid lesions is morphologically difficult. Diagn. Cytopathol. 2003;29:61,66. © 2003 Wiley-Liss, Inc. [source]


Acquired localized cutis laxa confined to the face: case report and review of the literature

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 12 2004
Claudia Jimena Perafán Riveros MD
Background, Cutis laxa is an uncommon entity characterized by laxity of the skin, which hangs in loose folds, producing the appearance of premature aging. It can be subdivided into congenital and acquired. This latter variant is rare and the skin involvement varies from generalized to localized. We report a case of a localized acquired cutis laxa confined to the face, without preceding inflammatory lesions or systemic compromise. Four similar cases have been reported to date. The etiology remains unknown and there is no definitive treatment. Methods, A 27-year-old White woman came to our hospital with a wrinkled face, pendulous earlobes and drop eyelids. Changes began 5 years prior, and she appeared much older than her age. Results, Histological analysis and ultrastructural examination of skin biopsy revealed reduction and fragmentation of elastic fibers, confirming the diagnosis of cutis laxa. No systemic involvement was diagnosed. The patient was submitted to plastic surgery for repair, with satisfactory results to date. Conclusions, Acquired localized cutis laxa confined to the face without preceding inflammatory lesions is extremely rare. The etiology remains unknown. Clinical features and histopathologic findings confirm the diagnosis. Surgical repair seems to be the only therapeutic choice, but the results are variable and temporary. [source]


A case of necrobiotic xanthogranuloma without paraproteinemia presenting as a solitary tumor on the thigh

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 6 2003
Sung Eun Chang MD
A 82-year-old Korean woman had had a 6-month history of an asymptomatic, flat, hard, red to brown tumor on her right thigh. This lesion had been slowly enlarging with an advancing margin. She had noted gradually developing pain associated with necrosis and ulceration on the lesion. Examination revealed a solitary, 8 × 7.5 cm, yellow to dark red, telangiectatic tumor with multiple areas of punched out ulceration and a peripheral elevated yellowish margin on the right inner upper thigh (Fig. 1). No clinically similar lesions on the periorbital area or other sites were seen. Histologic examination revealed a massive palisading granulomatous infiltration with several layers of extensive bands of necrobiotic zone in the entire dermis and deep subcutaneous tissue (Fig. 2a). In the granulomatous infiltrate in the dermis and subcutis, many various-shaped, some bizarre, angulated, foreign-body type multinucleated giant cells, many Touton giant cells, and a few Langhans giant cells were found to be scattered (Fig. 2b). There were numerous xanthomatized histiocytes. Dense infiltration of lymphoplasma cells was seen in the periphery of the granuloma and perivascularly. Conspicuous granulomatous panniculitis composed of lymphoplasma cells, polymorphonuclear cells, foam cells, and Touton and foreign-body giant cells was also seen. However, cholesterol clefts and lymphoid follicles were not seen. Subcutaneous septae were widened by necrobiotic change and fibrosis with thrombosed large vessels. Gram, Gomeri-methenamine silver and acid-fast stains were negative. The necrobiotic areas were positive to alcian blue. Laboratory investigation revealed elevated white blood cell counts, anemia and elevated erythrocyte sedimentation rate. The following parameters were within the normal range: lipids, glucose, renal and liver function tests, serum complements, serum immunoglobulins, cryoglobulins and antinuclear antibodies. The findings of chest X-ray, skull X-ray and ectorcardiography were normal. Serum electrophoresis and serum immunoelectrophoresis revealed no abnormality. The patient was diagnosed as having necrobiotic xanthogranuloma without paraproteinemia. She was treated with oral steroid (0.5,0.6 mg/kg) and NSAIDS for 1 month with partial improvement of pain and the lesion ceased to enlarge. In the following 1 year of follow-up, with only intermittent NSAIDS, her lesion did not progress and there were no signs of systemic involvement or new skin lesions. Figure Figure 1 . (a) A solitary, red to brown plaque with multiple ulcerations and a peripheral elevated yellowish margin on the inner upper thigh Figure 2. (a) A dermal and subcutaneous massive xanthogranulomatous infiltrate with zonal necrobiosis of collagen (× 20). (b) Prominent infiltrate of xanthomatized histiocytes and giant cells with perivascular lymphoplasma cells (H&E, × 100) [source]


The periodontal abscess (I).

JOURNAL OF CLINICAL PERIODONTOLOGY, Issue 6 2000
Clinical, microbiological findings
Background/aims: Little information is available regarding the diagnosis and microbiology of periodontal abscesses. The aim of this descriptive clinical and microbiological study was to provide more information in order to help in the characterisation of the periodontal abscess associated to periodontitis. Method: 29 consecutive patients with a periodontal abscess were studied by the assessment of clinical variables, including both subjective (pain, edema, redness and swelling) and objective (bleeding on probing, suppuration, probing pocket depth, tooth mobility and cervical lymphadenopathy) parameters. Microbiological samples were taken for anaerobic microbiology and processed by means of culture. Systemic involvement was also studied through the analysis of blood and urine samples using conventional laboratory standards. Results: 62% of the abscesses affected untreated periodontitis patients, and 69% were associated with a molar tooth. More than 75% of the abscesses had moderate-severe scores related to edema, redness and swelling, and 90% of the patients reported pain. Bleeding occurred in all abscesses, while suppuration on sampling was detected in 66%. Mean associated pocket depth was 7.28 mm, and 79% of teeth presented some degree of mobility. Cervical lymphadenopathy was seen in 10% of patients, while elevated leucocyte counts were observed in 31.6%. The absolute number of neutrophils was elevated in 42% of the patients. High prevalences of putative periodontal pathogens were found, including Fusobacterium nucleatum, Peptostreptococcus micros, Porphyromonas gingivalis, Prevotella intermedia and Bacteroides forsythus. Conclusions: The periodontal abscess has clear clinical characteristics and is usually associated with severe periodontal destruction. This condition may cause systemic involvement and the lesion generally has a large bacterial mass with a high prevalence of well-recognised periodontal pathogens. [source]


Bilateral Systematized Porokeratotic Eccrine Ostial and Dermal Duct Nevus with Unilateral Breast Hypoplasia

JOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005
A. Jayaraman
Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare hamartomatous malformation of the eccrine duct which typically presents at birth or early in life. Multiple punctate pits and verrucous papules and plaques with nonfollicular hyperkeratotic spines are seen over the palms and soles and may extend to the distal extremities. A linear distribution may be present. A wide range of involvement has been reported in the literature, including bilateral systematized involvement. No systemic involvement has been linked to PEODDN to date. Histologic findings are characteristic and include a dilated eccrine acrosyringium with an overlying parakeratotic column or cornoid lamella. There is slight dyskeratosis and loss of the granular layer under the cornoid lamella. Here, we present a case of bilateral systematized PEODDN in an otherwise healthy, 18 year-old Hispanic female. Physical examination is notable for marked hypoplasia of the left breast with overlying verrucous papules and plaques. Multiple punch biopsies were performed and showed findings diagnostic of PEODDN. To our knowledge, this is the most extensive involvement of PEODDN reported in the literature to date and the only case with associated hypoplasia of the breast. [source]


Fatal Subcutaneous Panniculitis-Like T-Cell Lymphoma (Sptcl) with Interface Change and Dermal Mucin, A Dead-Ringer for Lupus Erythematosus

JOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005
L. Ma
We report a 48-year-old male who presented with ulcerated plaques and nodules of the lower extremities. Skin biopsies revealed a dense lymphocytic infiltrate involving the dermis and the subcutis in a lobular and septal pattern. No overt cytological atypia was present. Notably, several features resembling lupus erythematosus were present, including vacuolar interface change and abundant dermal mucin deposition. The patient developed pulmonary nodules, and a lung biopsy showed a perivascular and interstitial lymphoid infiltrate without overt atypia. The cutaneous and pulmonary lymphoid infiltrates showed similar immunohistochemical profiles: CD3+CD4,CD8+/,CD56+. Monoclonal rearrangements of T-cell receptor gamma gene with similar migration patterns were identified from both locations. The patient developed fatal hemophagocytic syndrome, involving liver, spleen, lymph nodes, and bone marrow. This case is amongst rare reports of subcutaneous panniculitis-like T-cell Lymphoma (SPTCL) with systemic involvement. [source]


Cutaneous pseudolymphoma in association with molluscum contagiosum in an elderly patient

JOURNAL OF CUTANEOUS PATHOLOGY, Issue 7 2003
D. Moreno-Ramírez
Background:, Molluscum contagiosum (MC) is a common cutaneous infection, which has been reported in association with cutaneous pseudolymphoma in few cases. Methods:, A 72-year-old woman with a nodule arising on the external canthus was reviewed. The lesion was surgically removed, and the histopathological study demonstrated an epidermal invagination filled by molluscum bodies and a diffuse infiltrate comprising atypical lymphocytes. Results:, Immunohistochemical stains disclosed predominance of T cells with positive CD30 labeling. Polymerase chain reaction failed to demonstrate clonal rearrangement of the T-cell receptor. Conclusion:, After ruling out systemic involvement, the patient was followed up for 2 years with no evidence of recurrence. We report this case to the best of our knowledge and discuss the literature about atypical clinical and histological presentations of MC. [source]


Sudden Cardiac Death due to Giant Cell Inflammatory Processes,

JOURNAL OF FORENSIC SCIENCES, Issue 4 2007
Rebecca A. Hamilton M.D.
Abstract:, Granulomatous inflammation of the myocardium may occur in a number of systemic disease processes including those with infectious etiologies such as fungal, mycobacterial and parasitic infections, as well as hypersensitivity reactions, and rarely autoimmune disorders. In many of these disorders, giant cells are components of the inflammatory infiltrate. Systemic granulomatous processes of unknown pathogenesis, most notably sarcoidosis, may also be associated with involvement of the myocardium. Occasionally, these disorders are associated with sudden death due to pathologic involvement of the heart. In contrast, giant cell myocarditis, also known as idiopathic myocarditis, a rare, frequently fulminant and fatal disorder of unknown etiology, is isolated to the heart and lacks systemic involvement. This disorder is most commonly diagnosed at autopsy. We present two cases in which sudden death resulted from a giant cell inflammatory process affecting the myocardium. Both individuals lacked antemortem diagnoses and collapsed at their respective places of employment. These cases compare and contrast the clinical and pathologic issues involved in the differential diagnoses of the subgroup of sudden cardiac deaths resulting from giant cell inflammatory processes that affect the myocardium, as well as the value of histologic examination and immunohistochemical studies. [source]


Practical aspects of management of recurrent aphthous stomatitis

JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 8 2007
A Altenburg
Treatment of recurrent aphthous stomatitis (RAS) remains, to date, empirical and non-specific. The main goals of therapy are to minimize pain and functional disabilities as well as decrease inflammatory reactions and frequency of recurrences. Locally, symptomatically acting modalities are the standard treatment in simple cases of RAS. Examples include topical anaesthetics and analgesics, antiseptic and anti-phlogistic preparations, topical steroids as cream, paste or lotions, antacids like sucralfate, chemically stable tetracycline suspension, medicated toothpaste containing the enzymes amyloglucosidase and glucoseoxidase in addition to the well-known silver nitrate application. Dietary management supports the treatment. In more severe cases, topical therapies are again very useful in decreasing the healing time but fail to decrease the interval between attacks. Systemic immunomodulatory agents, like colchicine, pentoxifylline, prednisolone, dapsone, levamisol, thalidomide, azathioprine, methotrexate, cyclosporin A, interferon alpha and tumour necrosis factor (TNF) antagonists, are helpful in resistant cases of major RAS or aphthosis with systemic involvement. [source]


Ichthyosis follicularis, alopecia and photophobia (IFAP) syndrome treated with acitretin

JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 6 2005
S Khandpur
ABSTRACT We describe a 3-year-old male patient with the ichthyosis follicularis, alopecia and photophobia (IFAP) syndrome, who developed cutaneous and ocular involvement in infancy. In addition, he had growth retardation and borderline intelligence; no other systemic involvement was found on detailed investigation. A moderate response to acitretin therapy (1 mg/kg) administered for 6 months was observed, with improvement in cutaneous features and corneal erosions and no change in alopecia or photophobia. [source]


Sequential Plasma Lactate Concentrations as Prognostic Indicators in Adult Equine Emergencies

JOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 1 2010
B.S. Tennent-Brown
Background: Sequential lactate concentration ([LAC]) measurements have prognostic value in that hospitalized humans and neonatal foals that have a delayed return to normolactatemia have greater morbidity and case fatality rate. Hypothesis: Prognosis for survival is decreased in horses with a delayed return to normal [LAC]. Animals: Two hundred and fifty adult horses presented for emergency evaluation excepting horses evaluated because of only ophthalmologic conditions, superficial wounds, and septic synovitis without systemic involvement. Methods: Prospective observational study. [LAC] was measured at admission and then at 6, 12, 24, 48, and 72 hours after admission. The change in [LAC] over time ([LAC],T) was calculated from changes in [LAC] between sampling points. Results: Median [LAC] was significantly (P < .001) higher at admission in nonsurvivors (4.10 mmol/L [range, 0.60,18.20 mmol/L]) when compared with survivors (1.30 mmol/L [range, 0.30,13.90 mmol/L]) and this difference remained at all subsequent time points. The odds ratio for nonsurvival increased from 1.29 (95% confidence interval 1.17,1.43) at admission to 49.90 (6.47,384) at 72 hours after admission for every 1 mmol/L increase in [LAC]. [LAC],T was initially positive in all horses but became negative and significantly lower in nonsurvivors for the time periods between 24,72 hours (, 0.47, P= .001) and 48,72 hours (, 0.07, P= .032) when compared with survivors (0.00 at both time periods) consistent with lactate accumulation in nonsurvivors. Conclusions and Clinical Importance: These results indicate that lactate metabolism is impaired in critically ill horses and [LAC],T can be a useful prognostic indicator in horses. [source]


Characterization of inflammatory cells in oral paracoccidioidomycosis

ORAL DISEASES, Issue 4 2007
E Kaminagakura
Paracoccidioidomycosis (Pmycosis) is one of the most common deep mycoses in many regions of Latin America, particularly in Brazil. Microscopically, it shows granulomatous inflammatory reaction with giant cells, macrophages, lymphocytes, plasma cells, polymorphonuclear neutrophilic leukocytes, and eosinophils. The purpose of this study was to assess the distribution of inflammatory cells in oral Pmycosis. Fifteen cases of oral Pmycosis were studied by immunohistochemistry for the presence of macrophages, CD4+ and CD8+ lymphocytes, CD20+, CD15+, and S100+ cells. Macrophages were the main cells in well-organized granulomas and non-granulomatous areas. The CD4 phenotype was predominant in well-organized granulomas and a balance between CD4+ and CD8+ cells was observed in non-granulomatous areas. Dendritic, S100+ cells were found mainly in the epithelium, in subepithelial connective tissue, and at the periphery of organized granulomas. CD15+ cells were concentrated mainly in areas of intraepithelial microabscess and ulceration. Macrophages and T cells are the predominant cells in oral Pmycosis. Well-organized granulomas contain fewer yeast particles, indicating a more effective host immune response. Better understanding of the histopathological changes in oral Pmycosis might help determine treatment, severity and systemic involvement of the disease. [source]


Cowden's syndrome (multiple hamartoma and neoplasia syndrome): diagnostic dilemmas in three cases

ORAL DISEASES, Issue 4 2000
SI Chaudhry
Cowden's syndrome is a multisystem disease inherited as an autosomal dominant trait with incomplete penetrance and variable expression. The disease has typical oral manifestations which often precede more systemic involvement, and the dental professional is therefore well placed to institute a regime of regular checks to ensure early treatment of any neoplasms which may occur. However, since not all of the classical signs are present in all patients, diagnosis may be difficult. The case report of a patient with most of the features of Cowden's syndrome is presented and features compared with two other possible cases. [source]


Acquired Cutis Laxa Type II (Marshall Syndrome) in an 18-Month-Old Child: A Case Report

PEDIATRIC DERMATOLOGY, Issue 1 2010
F.C.P.S. (Dermatology), Mansoor Haider M.B.B.S.
Type II acquired cutis laxa, shows only cutaneous changes without any systemic involvement. We describe an infant with acquired cutis laxa type II following a generalized inflammatory dermatitis. [source]


Primary Cutaneous CD30 Positive Anaplastic Large Cell Lymphoma in an Adolescent

PEDIATRIC DERMATOLOGY, Issue 6 2009
Reena Vaid M.D.
MRI, bone marrow biopsy, and laboratory data demonstrated no other systemic involvement. He was treated with radiation and low-dose oral methotrexate, with improvement of the lesion and lymphadenopathy. Very few cases of primary cutaneous CD30-positive anaplastic large cell lymphoma in the pediatric population have been reported, and our case represents one of the first pediatric patients with local lymph node involvement. [source]


Epidermal Nevus Syndromes: Clinical Findings in 35 Patients

PEDIATRIC DERMATOLOGY, Issue 4 2004
Helena Vidaurri-de la Cruz M.D.
A predisposition to malignant neoplasms in ectodermal and mesodermal structures may also be found. There are six different epidermal nevus syndromes described so far: Proteus, congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome, phakomatosis pigmentokeratotica, sebaceous nevus, Becker nevus, and nevus comedonicus. Thirty-five patients with epidermal nevus syndrome seen at the National Institute of Pediatrics in Mexico City during a 31-year period are described. This syndrome represented 7.9% of 443 patients with epidermal nevi; its relative frequency was 1 case per 11,928 pediatric patients and 1 case per 1080 dermatologic patients. Nine epidermal nevus syndrome patients (26%) had Proteus syndrome. Sebaceous nevus syndrome was found in six patients (17%), while the nevus comedonicus syndrome was found in three (8%). Two patients were diagnosed with phakomatosis pigmentokeratotica and one patient with congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome. This is the first report of phakomatosis pigmentokeratotica and congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome in Mexican patients. One patient had an inflammatory linear verrucous epidermal nevus with systemic involvement. Thirteen patients (37%) had keratinocytic nevi with systemic involvement. We propose the keratinocytic nevus syndrome to be defined as the association of a keratinocytic nevus with neuronal migration and/or musculoskeletal disorders in addition to a higher risk for mesodermal neoplasms. [source]


Successful treatment of cutaneous sarcoid by photodynamic therapy with minimal discomfort using a fractionated dosing regime

PHOTODERMATOLOGY, PHOTOIMMUNOLOGY & PHOTOMEDICINE, Issue 5 2009
Clare Patterson
We report the case of a 42-year-old lady with an 8-year history of a persistent tumid plaque on her forehead. Investigations and presentation were consistent with cutaneous sarcoid with no systemic involvement. Multiple topical and oral treatments had been ineffective. She received seven sessions of 5-aminolaevulinic acid photodynamic therapy (PDT) over the course of 16 months. Each treatment was delivered in discontinuous and fractionated time intervals. Improvement was seen after the first treatment and continued with subsequent treatments. She found the treatment almost painless and was pleased with the cosmetic outcome. We conclude that PDT is a useful therapy in the treatment of facial cutaneous sarcoid. Fractionated exposure may allow the treatment to be less painful and therefore better tolerated. [source]


Cutaneous polyarteritis nodosa: A report of 16 cases with clinical and histopathological analysis and a review of the published work

THE JOURNAL OF DERMATOLOGY, Issue 1 2010
Naoko ISHIGURO
Abstract Sixteen cases of cutaneous polyarteritis nodosa referred to our Department from 1985 to 2003 were studied clinically and histopathologically. Laboratory data, treatments and clinical courses were also evaluated retrospectively. All cases had nodules and/or indurated erythemas on their lower extremities. All cases showed necrotizing vasculitis of small muscular arteries in the subcutaneous tissues and/or occlusion of those arteries histopathologically. Fifteen cases also had accumulation of plasma protein in vessels of the dermis and subcutaneous tissues. Laboratory data showed high activity of platelets and coagulation in some cases. Eleven cases had been effectively treated with non-steroidal anti-inflammatory drugs. Eight cases were observed for at least 5 years (the longest for ,19 years) and had good prognoses and no systemic involvement. Cutaneous polyarteritis nodosa seems to be a benign disease, and differs from systemic polyarteritis nodosa although their histopathological features are common. Cutaneous polyarteritis nodosa might involve local dysfunction of the circulation from the dermis to the subcutaneous area. A review of the published work shows that the cause(s) of most cases of cutaneous polyarteritis nodosa is unknown, that no controlled trials for treatment of cutaneous polyarteritis nodosa compared to polyarteritis nodosa have been reported, and that no definitively effective therapy for cutaneous polyarteritis nodosa has been established. [source]


Subcutaneous sarcoidosis,clinicopathological study of 10 cases

BRITISH JOURNAL OF DERMATOLOGY, Issue 4 2005
J. Marcoval
Summary Background, Subcutaneous sarcoidosis is a specific cutaneous lesion of sarcoidosis that is rarely reported. Objective, Our purpose was to analyse the clinicopathological features of 10 patients with subcutaneous sarcoidosis and its relationship with the systemic features of the disease. Patients and methods, The patients with systemic sarcoidosis, diagnosed from 1974 to 2002 at a university hospital in Barcelona, Spain, who developed subcutaneous involvement, were included in the study. The diagnosis of systemic sarcoidosis was made according to conventional criteria. All the patients were monitored prospectively at the sarcoidosis clinic of the hospital. Skin biopsies were performed when granulomatous cutaneous involvement was suspected clinically. Results, Granulomatous cutaneous involvement was demonstrated in 85 of 480 patients with systemic sarcoidosis. In 10 of these 85 patients subcutaneous sarcoidosis was diagnosed (11·8%). The lesions were most frequently located in the extremities, involving the forearms in nine patients. Indurated linear bands from the elbow to the hand were observed in five patients. In all of our patients the subcutaneous nodules appeared at the beginning of the disease. In six patients, the nodules remitted spontaneously in less than 2 years. In two cases foreign particles were detected under polarized light. Conclusions, Subcutaneous sarcoidosis is a quite uniform clinicopathological entity usually appearing at the beginning of the disease. It usually heralds forms of sarcoidosis with nonsevere systemic involvement and is not associated with chronic fibrotic disease. [source]


Neuro-ophthalmological findings in sarcoidosis

ACTA OPHTHALMOLOGICA, Issue 6 2004
Kjell Heuser
Abstract. Purpose:,This review emphasizes the importance of neuro-ophthalmological signs and symptoms in sarcoidosis. The presence of ophthalmological and neuro-ophthalmological findings may lead to diagnosis of the disease and the initiation of adequate treatment. Material and Methods:,Patients who had been diagnosed with neurosarcoidosis during the period 1990 , 2001 were identified from the departmental diagnostic index. The history, clinical, laboratory and imaging data of patients were analysed. Results:,Fifteen patients were identified, four men and 11 women, with a mean age of 44.1 years (range 26,65 years). In six of the 15 (40%), neurological deficits were the initial symptoms. Nine (60%) had known sarcoidosis at the time of presentation. Ten patients (66%) had ophthalmological/neuro-ophthalmological symptoms and signs. Conclusion:,Neuro-ophthalmological symptoms may develop early in neurosarcoidosis. If neuro-ophthalmological symptoms arise in patients with established biopsy-proven sarcoidosis, the diagnosis is usually easy to make. However, a number of patients with neurosarcoidosis may present with neuro-ophthalmic symptoms before systemic involvement becomes obvious. In this situation the diagnosis is challenging, and the major goal is to establish the presence of systemic sarcoidosis. [source]


Multicentric reticulohistiocytosis with lungs and liver involved

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 2 2009
H.-J. Yang
Summary We describe a 56-year-old man with a 2-year history of papulonodules, pruritic and painful on palpation, on the head, trunk, limbs, buttocks and scrotum and a 1.5-year history of rheumatoid arthritis-like joint changes. Biopsies from the nodules on the head and left elbow revealed multinucleated giant cells with eosinophilic ,ground-glass' cytoplasm. Computed tomography revealed that there were scattered nodules in the liver and both lungs. Biopsies taken from nodules in the right lung and liver were consistent with multicentric reticulohistiocytosis. The widely scattered cutaneous papulonodules and the generalized systemic involvement make this patient interesting, and the condition should be differentiated from other diseases in clinicopathological practice. [source]


Benign cutaneous Degos' disease

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 2 2003
R. M. Ojeda Cuchillero
Summary Malignant atrophic papulosis is a rare systemic vaso-occlusive disorder characterized by thrombosis of vessels of the dermis, gastrointestinal tract, central nervous system and, occasionally, other organs. Cutaneous lesions consist of erythematous, dome-shaped papules that develop a central area of necrosis to leave a porcelain-like scar. The most accepted theory of pathogenesis is based on endothelial cell damage. There is no effective treatment of the disease. We describe a 26-year-old man with Degos' disease, a diagnosis based on the clinical and histologic pattern of skin lesions. The good response to antiplatelet therapy and the absence of systemic involvement over 8 years' follow-up is noteworthy. We believe that this case represents the benign form of the disease, typically referred to as benign cutaneous Degos' disease. [source]


Aleukaemic leukaemia cutis presenting as a benign-appearing eruption

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 2 2003
T. P. Millard
Summary A 68-year-old Caucasian male presented with a 5-week history of a widespread pruritic papular eruption. Histology from a papule on the left shoulder showed a dense dermal infiltrate of large mononuclear cells which were positive for leucocyte common antigen, KP1 and PGM1, with an MIB-1 proliferating fraction of 40%, diagnostic of acute monocytic (M5) leukaemia cutis. Full blood count revealed pancytopaenia but no blasts. Bone marrow aspirate showed reduced red cell precursors and 10% blasts, consistent with myelodysplastic syndrome (refractory anaemia with excess blasts). The patient was managed with a 3 unit transfusion of packed red cells, after which his skin eruption resolved within 6 weeks and his peripheral blood counts returned to normal. No chemotherapy was administered. In conclusion, leukaemia can present in the skin, the eruption may be nonspecific and it may precede systemic involvement by either myelodysplastic syndrome or acute leukaemia. [source]


Cardiac complications in Behçet's disease

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 8 2002
Ü. Türsen
Summary Behçet's disease (BD) is a multisystem disease of unknown aetiology characterized by chronic relapsing oro-genital ulcers, uveitis, and systemic involvement including articular, gastrointestinal, cardiopulmonary, neurologic and vascular pathology. Vascular involvement is observed in 30% of cases. Although the pathogenic mechanisms underlying the thrombotic disposition in BD are not well known, prothrombin (PT) gene mutations may be one factor that contributes to the development of vascular involvement in this disorder. We report a case of BD with a PT gene mutation, presenting with cardiovascular involvement. [source]


Acne fulminans ,sine fulminans'

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 4 2000
K. F. Thomson
Acne fulminans is characterized by the sudden onset of a severe, ulcerative acne associated with systemic features. Response to traditional acne therapies is poor. We have recognized a subset of patients with acne of a severity comparable to that of acne fulminans but with the absence of systemic involvement; we suggest modification of the treatment regimes used in this group. [source]


Bartonellosis: light and shadows in diagnostic and therapeutic issues

CLINICAL MICROBIOLOGY AND INFECTION, Issue 3 2005
R. Manfredi
Abstract Cat-scratch disease involves a prolonged and/or complicated course, and lymph node drainage is usually required. Culture and molecular techiques often yield negative results, but immunofluorescence assays may give early information, and elevated antibodies may persist for months. Cat-scratch disease should be suspected in patients with prominent swelling of lymph nodes draining from the upper limbs, limited systemic involvement, and typical epidemiological,clinical features. The temporal antibody response during the sub-acute course remains unknown. Although biomolecular assays are available, the time between onset and investigation is an obstacle to positive results. The role of surgical debridement and the unpredictable activity of antimicrobial agents warrant further investigation. [source]