Absence Of Systemic Involvement Research Articles

Recurrent cutaneous eosinophilic vasculitis characterized by annular purpuric lesions: A case report

A 71-year-old woman presented with a persistent, intensely pruritic cutaneous eruption localized on the palmoplantar regions, lips and palate. The histological findings allowed to make the diagnosis of recurrent cutaneous eosinophilic vasculitis, a very rare cutaneous vasculitis characterized clinically by multiple erythematous or purpuric erythematous papules or plaques or angioedema with a relapsing course in the absence of systemic involvement and histologically by a necrotizing vasculitis of the dermal small vessels with a dominant eosinophilic infiltration. The patient was treated with oral methylprednisolone and pentoxifylline which led to a rapid resolution of the cutaneous lesions.

Successful Treatment of Cutaneous Collagenous Vasculopathy with Pulsed Dye Laser: A Case Report

Cutaneous collagenous vasculopathy (CCV) is clinically characterized by symmetrical, progressive telangiectasias and an absence of systemic involvement. Histopathology is distinct and helps in differentiation from other vascular conditions that result in cutaneous telangiectasias. We present a case of biopsy-proven CCV that had localized clearance following biopsy and was successfully treated with pulsed dye laser. A healthy 36-year-old male presented with a 10-year history of progressive, blanching telangiectasias of the legs, thighs, and forearms. A punch biopsy of a representative patch on the patient’s right thigh demonstrated telangiectasias with thickened PAS-positive vessel walls and a sparse perivascular lymphoid infiltrate, features consistent with a diagnosis of CCV. While there was localized clearance at the biopsy site and adjacent skin, the patient opted for treatment with PDL for his lesions elsewhere. After testing various PDL settings, greatest cosmetic improvement and minimal PIH was achieved with a spot size of 7 mm, pulse duration of 3 milliseconds (ms) and a fluence of 8 J/cm2. Continued resolution of CCV was noted at 24 months. We present this case to underscore the potential efficacy of PDL in the treatment of CCV.

Three cases of symmetrical drug-related intertriginous and flexural exanthema (SDRIFE) induced by proton-pump inhibitors.

Symmetrical drug-related intertriginous and flexural exanthema (SDRIFE), previously termed drug-related baboon syndrome, is an uncommon drug eruption. It is characterized by symmetrical erythema involving the gluteal and/or inguinal area in association with one other intertriginous area in the absence of systemic involvement. It typically develops a few hours to days after drug exposure. The diagnosis is based on clinical presentation and drug history. The treatment consists mainly of withdrawal of the causative agent; corticosteroids (topical or systemic) are prescribed to accelerate the resolution. We present three cases that appeared after proton-pump inhibitors (PPIs) intake.

Cutaneous B-cell lymphoma mimicking a keloid: Dermoscopy-assisted diagnosis

Primary cutaneous lymphomas are lymphoproliferative disorders having multiple heterogenous subtypes with a primary cutaneous manifestation in the absence of systemic involvement of the lymph nodes, bone marrow, or visceral organs at the time of diagnosis. Herein, we report the case of a 75-year-old male who presented with a single plaque on the back mimicking a keloid. Clinical and dermoscopic examinations led to the suspicion of a cutaneous malignancy, while histopathology and IHC confirmed a primary cutaneous B-cell non-Hodgkin’s lymphoma. The lesion responded well to a R-CHOP chemotherapy regimen.

Granulomatous Features in Juvenile Idiopathic Arthritis-Associated Uveitis is Not a Rare Occurrence.

BackgroundJuvenile idiopathic arthritis (JIA)-associated uveitis is the most common form of intraocular inflammation with systemic involvement in childhood. Textbooks tend to describe it as non-granulomatous anterior uveitis. Here, we report the percentage of granulomatous features in JIA-related uveitis among patients at a uveitis referral center.MethodsWe conducted a retrospective study of all patients with JIA-related uveitis who were seen at the Center for Ophthalmic Specialized Care in Lausanne between 2000 and 2020. The sample comprised pediatric patients with bilateral anterior uveitis diagnosed as JIA-related with positive antinuclear antibody (ANA) titers. Exclusion criteria were a positive Mantoux or Quantiferon® test or elevated serum lysozyme and/or angiotensin converting enzyme (ACE) levels. The presence of one of the following features qualified the uveitis as granulomatous: granulomatous keratic precipitates (KPs) of mutton-fat type, smaller granulomatous KPs, or Koeppe nodules. The amount of inflammation was measured using laser flare photometry.ResultsThirty-eight patients were identified (mean age 13.34 ± 2.71 years; all female). In 12 patients (31.5%), the uveitis presented granulomatous features at presentation or during follow-up in at least one eye. Two of the 12 (16%) granulomatous patients had only light or no systemic involvement. The mean flare was 136.3±149 ph/ms, which is much higher than granulomatous uveitis entities of other origin reported in the literature.ConclusionJIA related uveitis can have a granulomatous presentation in about one third of patients with the Oligoarticular type and ANA positive, seen in a uveitis referral center. The presence of granulomatous features in bilateral uveitis in ANA-positive pediatric patients should not exclude the diagnosis of JIA-related uveitis, even in the absence of systemic involvement, as this entity presents a much more severe uveitis than other granulomatous anterior uveitis entities.

Rising trends of symmetrical drug related intertriginous and flexural exanthem due to Itraconazole in patients with superficial dermatophytosis: A case series of 12 patients from eastern part of India.

SDRIFE (symmetrical drug related intertriginous and flexural exanthem) is a benign self-limiting adverse drug reaction (ADR) primarily affecting the intertriginous, gluteal and flexural regions symmetrically in the absence of systemic involvement. It is considered to be a variant of baboon syndrome. Pathogenesis is poorly understood but is thought to be result of a delayed hypersensitivity response after exposure to the drug. Commonly implicated drugs are beta-lactam antibiotics. With the rising incidence of dermatophytosis in the Indian subcontinent, the use of oral anti fungals has become rampant. SDRIFE due to itraconazole has been rarely reported in literature. We hereby present a series of 12 patients presenting with SDRIFE due to itraconazole, which is by far the largest reported in existing literature. This case series highlights the importance of identifying these cutaneous adverse drug reactions in a setting where itraconazole is commonly being used.

Predictive Value of Serum Angiotensin-Converting Enzyme Titers in Sarcoid-Like Dacryoadenitis.

To compare serum angiotensin-converting enzyme (sACE) titers in patients with newly diagnosed sarcoid-like dacryoadenitis and nongranulomatous dacryoadenitis. Retrospective comparative case series. Clinical details and presenting sACE titers were reviewed for patients with newly diagnosed sarcoid-like dacryoadenitis (SLD) and nongranulomatous dacryoadenitis (NGD). All sACE titers were expressed as a percentage of the upper end of normal range ("normalized"). Using Student t test, sACE titers for patients with unilateral SLD were compared with unilateral NGD and the sensitivity and specificity of sACE as a predictor for SLD calculated; this was repeated for patients with bilateral dacryoadenitis. The proportion of patients with raised sACE were compared in each group (Fisher exact test), and the mean sACE titers compared for these selected patients. For unilateral SLD the mean sACE was 97%, compared with 57% in NGD (p=0.0010), and the sensitivity of sACE titer was 42%, specificity 92%, positive predictive value 75%, and negative predictive value 73%. Area under curve from receiver operating characteristic analysis for unilateral dacryoadenitis was 0.78 (p=0.0040). In contrast, there was no significant difference for bilateral disease (SLD 148%, NGD 82%; p=0.33), and somewhat lower positive predictive value (54%), negative predictive value (71%) and area under curve (0.71; p=0.070). Although not significantly so with bilateral disease, the proportion of patients with raised sACE was consistently greater in SLD as compared to NGD-being highest with bilateral SLD (7/12 patients; 58%) and lowest in unilateral NGD (2/26; 8%). Seventeen of the 27 patients with SLD were later found to have systemic involvement: where systemic disease was present, the mean sACE levels were significantly different in unilateral (102%) and bilateral dacryoadenitis (209%; p=0.029) disease, whereas there was no difference in the absence of systemic involvement (sACE with unilateral dacryoadenitis = 86%, bilateral = 75%; p=0.72). Normalized serum titers of ACE are minimally predictive for SLD, whether unilateral or bilateral, with some false negatives and false positives in all groups. However, with unilateral dacryoadenitis, an sACE of >125% maximum range appears absolutely predictive for SLD, and >150% maximum range for bilateral disease.

Childhood Solitary Cutaneous Mastocytoma: Clinical Manifestations, Diagnosis, Evaluation, and Management.

Background: The diagnosis of solitary cutaneous mastocytoma is mainly clinical, based on lesion morphology, the presence of a positive Darier sign, and the absence of systemic involve-ment. Knowledge of this condition is important so that an accurate diagnosis can be made.Objective: To familiarize physicians with the clinical manifestations, diagnosis, evaluation, and man-agement of a solitary cutaneous mastocytoma.Methods: A PubMed search was completed in Clinical Queries using the key term “solitary cutane-ous mastocytoma”. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. Only papers published in English language were included. The information retrieved from the above search was used in the compilation of the present article.Results: Typically, a solitary cutaneous mastocytoma presents as an indurated, erythematous, yellow-brown or reddish-brown macule, papule, plaque or nodule, usually measuring up to 5 cm in diameter. The lesion often has a peau d'orange appearance and a leathery or rubbery consistency. A solitary cu-taneous mastocytoma may urticate spontaneously or when stroked or rubbed (Darier sign). Organo-megaly and lymphadenopathy are characteristically absent. The majority of patients with skin lesions that erupt within the first two years of life have spontaneous resolution of the lesions before puberty. Treatment is mainly symptomatic. Reassurance and avoidance of triggering factors suffice in most cases.Conclusion: The diagnosis is mainly clinical, based on the morphology of the lesion, the presence of a positive Darier sign, and the absence of systemic involvement. A skin biopsy is usually not neces-sary unless the diagnosis is in doubt.

Circulating Myeloid-Derived Suppressor Cells Reflect Mycosis Fungoides/Sezary Syndrome Disease Stage and Response to Treatment

Background: Despite mycosis fungoides (MF) and Sezary syndrome (SS) comprising the most common forms of cutaneous T cell lymphoma, the pathophysiology underlying these disorders remains poorly understood. Consequently, current prognostic guidelines based on disease spread exhibit wide variations in clinical outcome within each stage, underscoring an urgent need for novel approaches to MF/SS disease evaluation. A growing body of research suggests that systemic immune dysregulation represents an early, cardinal feature of MF/SS. We hypothesized that tracking this immune dysfunction in conjunction with disease spread may generate important pathophysiologic and prognostic information for patients. We focused on myeloid-derived suppressor cells (MDSCs), a recently discovered population of immunosuppressive innate immune cells related to neutrophils and monocytes, because their expansion in numerous solid tumor settings have correlated reliably with poor patient outcomes. Whether MF/SS augments circulating MDSC abundance remains unexplored, prompting us to evaluate whether this could serve as a marker for disease progression and treatment response.Methods: We used multiparametric flow cytometry to analyze the frequency and immunophenotype of MDSCs from the peripheral blood of 15 healthy donors and 30 patients with MF/SS. Patients at varying stages of MF/SS disease progression and treatment were included in the study. We defined granulocytic MDSCs (G-MDSCs) as cells positive for CD15, CD11b, and the recently discovered surface marker LOX-1, and negative for CD14. Monocytic MDSCs (M-MDSCs) were defined as cells positive for CD14 and CD11b, negative for CD15, and low/negative for HLA-DR. Each patient sample also underwent flow cytometry evaluating for circulating neoplastic T cells. These results were correlated with each participant's other hematologic parameters and clinical information through manual chart review.Results: We found that healthy donors harbored no quantifiable circulating MDSCs of either monocytic or granulocytic lineage, a result in keeping with previous studies. In contrast, MF/SS patients exhibited robust, statistically significant increases in the frequencies of both G-MDSCs and, to a lesser extent, M-MDSCs. G-MDSCs exceeded 20% of all CD15-positive cells in some patients.When patients were stratified by MF/SS clinical stage, those with more advanced disease displayed significantly higher G-MDSC abundance than early-stage patients. G-MDSC frequency was positively correlated with circulating CD4+ CD26- T cell counts often used in evaluating Sezary syndrome (R2 = 0.498; p < 0.0001). However, patients with early, skin-restricted disease also showed statistically significant increases in circulating G-MDSCs compared to healthy controls. This suggested that G-MDSC expansion may serve as a sensitive, blood-based disease marker even in the absence of systemic involvement by neoplasia.Patients who underwent recent treatment exhibited variable G-MDSC counts in the peripheral blood that were lower than in similar untreated patients on average. Serial measurements for two patients enrolled in a clinical trial for dual phosphoinositide 3-kinase and histone deacetylase inhibition revealed that G-MDSC frequencies markedly decreased over the course of treatment, mirroring the decrements of aberrant T cells circulating in the blood.Conclusion: These findings provide clear evidence of G-MDSC expansion in the peripheral blood of MF/SS patients that begins in early/locally restricted disease, grows with disease progression, and responds to systemic therapy. Such immunometric assays may illuminate a novel source of staging and prognostic information and may permit less invasive disease monitoring than current methods require. DisclosuresNo relevant conflicts of interest to declare.

Antineutrophil cytoplasmic antibody-positive scleritis: Clinical profile of patients from a tuberculosis-endemic region.

Purpose:To report the clinical profile of a series of antineutrophil cytoplasmic antibody (ANCA)-associated scleritis in Indian population.Methods:We conducted a retrospective review of medical records of 33 eyes of 26 consecutive patients with scleritis, who tested positive for either antibody to proteinase 3 [anti-PR3/cytoplasmic antineutrophil cytoplasmic antibody (cANCA)] or myeloperoxidase [anti-MPO/perinuclear anti-neutrophil cytoplasmic antibody (pANCA)] between 2006 and 2015.Results:The mean age at presentation was 54.1 (11.1) years and 61.5% of the patients were female. Underlying systemic disorder was found in 46.2% of patients and includes granulomatosis with polyangitis (30.8%) and tuberculosis (15.4%). Necrotizing scleritis (48.5%) was the most common scleritis observed, followed by diffuse anterior scleritis (42.4%). Positive cANCA was found in 65.4% of patients and 34.6% was found positive for pANCA. Four of the six patients with positive Mantoux test were started on anti-tuberculosis treatment (ATT) by pulmonologist. Cyclophosphamide was the most common immunosuppressive and 11.5% of the patients required combination of two immunosuppressives. Seventeen eyes developed cataract and four eyes required patch graft. Female gender was more frequently associated with pANCA-associated scleritis than cANCA (P = 0.037). Incidence of necrotizing scleritis was higher in patients with positive cANCA, but this difference was not statistically significant (P = 0.806). cANCA-positive patients had statistically significant higher association with systemic rheumatic diseases (P = 0.021).Conclusion:Necrotizing scleritis is the most common subtype of scleritis in ANCA-positive individuals and even in the absence of systemic involvement. All patients with ANCA positivity should be thoroughly screened to rule out any evidence of tuberculosis, especially in tuberculosis-endemic region before planning aggressive immunomodulatory therapy.

XPO1 Inhibition By Selinexor Synergizes with BCR Inhibition, Blocks Tumor Growth and Prolongs Survival in a Bioluminescent Animal Model of Primary Central Nervous System Lymphoma

Primary central nervous system lymphoma (PCNSL) is a very aggressive non-Hodgkin lymphoma localized in the cerebral parenchyma, eyes, leptomeninges or spinal cord in the absence of systemic involvement. Approximately 95% of PCNSL are classified as diffuse large B-cell lymphoma (DLBCL), being most of them more closely related to activated B-cell type (ABC-DLBCL). PCNSL is associated with poor prognosis, particularly because of the difficulty for drugs to cross the blood brain barrier. High dose methotrexate is the most effective treatment, but relapse is very common and salvage therapies are scarce. Therefore, the development of effective drugs with ability to penetrate the CNS is highly needed.Selinexor (KTP-330) is a Selective Inhibitor of Nuclear Export (SINE) compound that binds and inactivates XPO-1 protein. This inactivation induces anti-tumor effects by forcing nuclear retention and activation of tumor suppressor proteins. Selinexor has shown excellent brain penetration, exhibits promising results in pre-clinical models of glioblastoma and can inhibit both BCR and NF-kB signaling in malignant B-cells. Therefore, in order to provide a pre-clinical rationale for the use of selinexor for patients with PCNSL, we tested the role of XPO-1 inhibition in PCNSL using intracerebral xenograft murine models.We then determined the inhibitory concentration 50 (IC50) in 4 ABC and 5 GCB DLBCL cell lines. Cell survival and proliferation were tested 96 hours after incubation with selinexor. Analysis showed that the DLBCL cell lines have equivalent sensitivity to selinexor, regardless cell of origin (COO). In detail, survival assessed by AnnexinV-PI exclusion showed that mean ID50 for ABC cell lines was 4.98 µM +/- 3.6 and 6.3 µM +/- 3.8 for GCB (p=0.9). Proliferation assessed by MTS was also blocked by selinexor (mean ID50 for ABC-DLBCL was 1.35 µM +/- 0.7 vs. 16.16 µM +/- 11.17 for GCB-DLBCL (p=0.41)).Since SINE compounds have been shown to inhibit BCR signaling, we next tested the potential synergy between ibrutinib and selinexor. All three ABC-DLBCL cell lines tested were sensitive to ibrutinib and in two of them the combination with selinexor was strongly synergic. In contrast, none of the 3 GCB-DLBCL cell lines analyzed were sensitive to up to 100 µM single agent ibrutinib; interestingly, however, treatment with selinexor sensitized SUDHL4 cells to ibrutinib and the combination index values indicated strong synergism between the two drugs.Finally, we established an orthotopic xenograft model of PCNSL by stereotactic injection of the OCI-Ly10 (ABC) cell line stably transfected with firefly luciferase into the cerebral parenchyma of nude athymic mice. This allows the non-invasive longitudinal quantification of intracerebral tumor growth (bioluminescence detection, IVIS¨ Spectrum). Eleven days after the injection of cells all animals had developed detectable tumors confined to the CNS. Tumor size was measured and animals were randomly distributed into drug or vehicle experimental group (vehicle: n=8, mean radiance= 1.33á107 p/sec/cm2/sr ± 0.68á107; treatment: n=9, mean radiance=2.99á107 p/sec/cm2/sr ± 2.3á107). At this time point mice were treated with 5mg/kg of selinexor or vehicle via oral gavage three times a week. Treatment with selinexor significantly increased mice survival, with a median survival of 48 days in the treatment group compared to 34 days in the vehicle group (p< 0.0001; figure 1A). Mice in the treatment group showed a significantly slower increase in bioluminescence signal (two-way ANOVA: p< 0.0001; figure 1B). Specific time-point analysis showed that differences were significant as soon as 8 days after treatment. At final point, histopathological analysis showed diffuse infiltration in meninges and cerebral parenquima of highly proliferative CD20-positive B-cells (virtually 100% Ki-67-positive malignant B cells)In conclusion, selinexor inhibits proliferation and survival of DLBCL cell lines, regardless of the cell of origin and it can synergize with ibrutinib. Moreover, treatment of mice with CNS confined ABC-DLBCL with selinexor significantly reduces tumor growth and increases survival. Therefore, our results provide pre-clinical evidence for the development of selinexor as new therapeutic option for PCNSL or DLBCL with CNS involvement. [Display omitted] DisclosuresBosch:Hoffman La Roche: Consultancy, Honoraria, Research Funding; Millennium: Research Funding.

Cardiac Arrhythmias as Initial Presentation of Sarcoidosis

SESSION TITLE: Student/Resident Case Report Poster - Cardiovascular Disease I SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Cardiac sarcoidosis is an infiltrative disease which is usually clinically silent but occasionally may be the first manifestation of sarcoidosis even in the absence of overt systemic involvement. It frequently presents with structural and conduction abnormalities including supraventricular and ventricular arrhythmias, myocardial damage simulating infarction, heart failure, cardiomyopathy, valvular heart disease, and sudden death. CASE PRESENTATION: A 33 year old African American female presented to emergency room with episodes of palpations. Her exam and laboratory work up were unremarkable. Electrocardiogram showed sinus arrhythmia with pre-ventricular contractions and she was started on Metoprolol. She was having non-sustained ventricular tachycardia (NSVT) which subsequently became sustained requiring Lidocaine with conversion to sinus tachycardia. Echocardiography was normal. However she continued to have NSVT which raised a concern for an infiltrative process. She had cardiovascular magnetic resonance imaging (CMRI) which showed a large area of delayed mid myocardial enhancement at the inferolateral, basal, mid portion and apical wall with 25% viability and hypokinesis. A high resolution computed tomography of her chest was unrevealing. Endomyocardial biopsy (EMB) was considered as she had no other obvious manifestation of sarcoidosis but was not done as her lesions were patchy. She eventually got an Implantable Cardioverter-Defibrillator and was discharged on Metoprolol. DISCUSSION: The diagnosis and management of cardiac sarcoidosis is a major challenge especially in the absence of systemic manifestations. Findings on electrocardiogram and echocardiogram are nonspecific; CMRI and even positron emission tomography (PET) features may be suggestive but are not diagnostic. EMB which demonstrates noncaseating epithelioid granulomas lacks sensitivity as it may miss the lesion and is usually reserved for patients without histologic confirmation from another source. The ability to diagnose this promptly, predict progression and prognosis, and intervene before a fatal cardiac event, deserves further investigation. The use of steroids lack confirmatory randomized clinical trials. A low threshold for invasive procedures is suggested due to the risk of sudden death. CONCLUSIONS: In young patients presenting with cardiac arrhythmias, sarcoidosis should be considered even in the absence of systemic involvement as this might be a herald sign for an opportunity to intervene and prevent impending sudden cardiac death. Reference #1: Schulte W, Kirsten D, Drent M, Costabel U.Cardiac involvement in sarcoidosis. Eur Respir Mon. 2005;32:130 Reference #2: Patel MR, Cawley PJ, Heitner JF, Klem I, Parker MA, Jaroudi WA, et al. Detection of myocardial damage in patients with sarcoidosis. Circulation. 2009;120(20):1969. Reference #3: Nery PB, Leung E, Birnie DH. Arrhythmias in cardiac sarcoidosis: diagnosis and treatment. Curr Opin Cardiol. 2012;27(2):181. DISCLOSURE: The following authors have nothing to disclose: Chukwunweike Nwosu, Deepika Nandiraju, Danila Deliana, Joseph Neri No Product/Research Disclosure Information

DLE Progressing to Squamous Cell Carcinoma: A Case Report

A 90 years female diagnosed as a case of Discoid lupus erythematosus (DLE) developed Squamous cell carcinoma from the lesions over a period of 5 years due to treatment gap and late follow up. Diagnosis was based on clinical aspects (erythematous, nodular and scanty bleeding), dermoscopic features and histopathological examination, the absence of systemic involvement and routine laboratory parameters, which registered all within normal range.SCC in a patient with DLE is rare in Nepalese patients. It is every essential to counsel the diagnosed cases of DLE and warn all patients about all the possible outcomes and compliance with medications should be ensured.Nepal Journal of Dermatology, Venereology &amp; Leprology, Vol.14(1) 2016, pp.51-55

Intérêt de la TEP/TDM au 18fluorodésoxyglucose dans le bilan initial des lymphomes du système nerveux central des patients immunocompétents

IntroductionPrimary central nervous system lymphoma (PCNSL) is a rare malignant lymphoma where disease is limited to the central nervous system. The diagnosis is suspected on brain MRI and confirmed after histological examination, in the absence of systemic involvement. Conventional systemic assessment includes CT, bone marrow biopsy and testicular examination. The primary objective of this study was to evaluate the use of FDG-PET in the initial systemic assessment of suspected PCNSL. Materials and methodsExamination of 21 immunocompetent patients, who have had an FDG-PET for the diagnosis of a cerebral lymphoma before any chemotherapy treatment, was reviewed. All the conventional systemic assessments were negative. ResultsSystemic involvement was detected in three patients (14%). Diagnostic was confirmed histologically in one patient, and based on combined pieces of evidence in two others. The brain lesions’ metabolism was high (mean SUVmax: 15.5±5.4). Thirty-five of the 45 (78%) lesions visible on contrast-enhanced MRI resulted in a pathological cerebral uptake. The infracentimetric enhancements were only visible in 50% of cases (8/16). One-year PFS was weaker in the group of patients with the most FDG avid lesions (SUVmax≥15; P=0.015). This relation was also found and persisted was confirmed in Kaplan-Meier analysis on the whole follow-up by using a ratio of the tumoral uptake by average hepatic uptake with a threshold of 7 (P=0.001). ConclusionsThe results of this work suggest that the FDG-PET could be useful in the systemic initial assessment of PCNSL.

Reconstructive Surgical Repair of a Forth Degree Iatrogenic Burn in a Dog

Background: Burns are uncommon in small animal surgery routine and represents a real therapeutic challenge. Skin can be affect in different degrees of deepness as superfcial, partial thickness, full thickness and full thickness affecting underlying tissues. Each degree has individual features. Progression through degrees can occur in the frst 24 h or if treatment is inadequate. This paper aims to describe therapeutic approach and surgical technique to treat a huge dorsal iatrogenic thermal burn injury. Case: A 4-year-old female German Shepard was referred to veterinary hospital after 11 days of elective ovariosalpingohisterectomy. The patient presented a severe skin injury with crusts, eschars, purulent discharge and myiasis on dorsal cervical, thoracic and lumbar region. Beside severity of the wound, animal present good general status and normal vital signs. Complete blood count and biochemical analysis were within normal ranges. Histopathologic analysis of a wound tissue sample revealed the presence of coagulation necrosis and inflammatory response. The absence of traumatic events, histopathologic result and wound features increased suspicious of an iatrogenic burn injury from a poorly regulated thermal mattress used during surgical intervention. “Rule of Nines” was estimated as 25% of total body surface area. Systemic antibiotics and topical treatment with 0.05% chlorhexidine digluconate and 2.5% silver sulfadiazine ointment followed by wet-wet bandage was performed after initial surgical debridement. Muscle and vertebral bone explosion were evident. Topical treatment with Silver Sulfadiazine and chlorhexidine persisted twice daily until granulation tissue formation. Pain killers and sedation were often necessary during bandage changes. At 65th day proceeded to en bloc excision of scar tissue and surgical reconstruction with skin advancement and recruitment technique. Tie-over dressing was applied to cover the fnal wound. After 14 days animal received hospital discharge, and was assessed bi-weekly for 60 days. Discussion: Besides great extension of the burn injury, patient’s age, absence of systemic involvement and fluid therapy or critical care the prognosis was good. Modifed “rule of nine” are used to estimate wound size in dogs. The wound was classifed as forth degree or full thickness injury due to exposed muscular layer and bone. Early debridement, standard topical and systemic treatment protocol applied granted good recovery of wound bed. Due to be uncommon in small animal routine, main diagnosis and therapeutic recommendations for veterinary patients are based on human data. Systemic antimicrobial drugs are usually not recommended due to low concentration in burned tissues, however, prophylactic use was necessary due to highly infected tissues and possible bacterial absorption from devitalized tissues. Delayed primary closure technique was necessary due to highly infection site and extension, which turns surgical approach hard to perform. After formation of a granulation tissue bed for wound reconstruction, surgical treatment was performed. Surgical reconstruction is indicated in large full thickness defects and for better cosmetic appearance. The correct use and regulation of thermal mattress can avoid burns that can be life threatening. This case report rises the important concept that even low contact temperatures when persistent for long time can cause burn injuries. Keywords: thermal, mattress, injury, dogs, plastic.

Isoniazid - induced chronic cutaneous Lupus erythematosus

A 23-year-old man developed drug-induced chronic cutaneous lupus erythematosus 8 months after isoniazid (INH) therapy for pulmonary tuberculosis. Diagnosis was based on clinical aspects (discoid lesions on the face, erythema, photosensitivity, hyperpigmentation), histopathological examination, along with direct immunofluorescence examination (DIF), the absence of systemic involvement and the routine laboratory parameters, which registered all within normal range. Hydroxychloroquine therapy associated to photo protection and emollients determined the clear up of the facial eruption within six months. Transient residual hyperpigmentation could be noticed 2 months after discontinuation of the treatment. This case illustrates a rare form of drug-induced chronic cutaneous lupus erythematosus developed 2 months after withdrawal of antituberculosis therapy, with excellent results with hydroxychloroquine.NJDVL Vol. 13, No. 1, 2015 Page: 52-56

A case of symmetrical drug-related intertriginous and flexural exanthema caused by valacyclovir

Introduction Symmetrical drug-related intertriginous and flexural exanthema (SDRIFE) has recently been separated from baboon syndrome and proposed as a unique type of drug eruption [1], which appears only on intertriginous or flexural folds and in gluteal areas in the absence of systemic involvement. Antibiotics including amoxicillin and cephalosporins are the most common drugs causing SDRIFE [2]. We report herein a case of SDRIFE showing symmetrical erythema predominantly on major flexural areas, rapidly developed after taking valacyclovir, which was suggested as a causative drug by skin patch test.

Topical application of ankaferd hemostat in a patient with gastroduodenal amyloidosis complicated with gastrointestinal bleeding

Amyloidosis rarely manifests itself as gastrointestinal hemorrhage, especially in the absence of systemic involvement. Despite urgent endoscopic and/or pharmacological therapy, bleeding due to gastric amyloidosis usually recurs after a short period and has considerable morbidity and mortality rates, even in patients undergoing gastrointestinal surgery. For this reason, there is a need for a therapeutic armamentarium for such cases that is effective, easily applicable and has minimal side effects. In this respect, ankaferd blood stopper (ABS) offers a well tolerated and effective alternative approach for these patients. Herein we would like to report a 77-year-old man who had massive bleeding from a gastric ulcer complicating primary gastroduodenal amyloidosis, in whom topical ABS was successfully applied.

MASTOCITOSE CUTÂNEA NA INFÂNCIA – ESTUDO RETROSPETIVO DE 32 DOENTES

Introdução: A designação de mastocitose engloba várias entidades clinicamente distintas caracterizadas pela acumulação tissular de mastócitos. A pele é o órgão mais frequentemente envolvido. Consideram-se 4 padrões clínicos de mastocitose cutânea: urticária pigmentosa (UP), mastocitose cutânea difusa, mastocitoma e telangiec- tasia macularis eruptiva perstans. Na infância, a doença é habitualmente autolimitada e exclusivamente cutânea. Material e Métodos: Apresenta-se um estudo retrospetivo dos doentes com mastocitose cutânea observados na Con- sulta de Dermatologia Pediátrica do Hospital de Curry Cabral entre 2001 e 2010. Resultados: Foram englobados 32 doentes (20 do sexo masculino e 12 do sexo feminino). Em 90.6% dos casos, as manifestações surgiram antes dos 2 anos. Apenas foram observadas UP (53,1%) e mastocitomas (46,9%). O tronco e a raíz dos membros foram as locali- zações preferenciais. O sinal de Darier estava presente em 87,5% dos casos (94,1% das UP e 80% dos mastocitomas). As manifestações associadas foram: prurido (40,6%), formação de bolha (28,1%), flushing (18,8%) e dermografismo (12,5%). Não foram documentados casos de mastocitose sistémica. Dois doentes tinham história familiar de mas- tocitose cutânea. Não foram detetadas alterações laboratoriais significativas. Nos 16 casos determinados, os níveis séricos de Triptase-alfa foram normais. Discussão: Estes resultados estão de acordo com a literatura, nomeadamente em relação às formas clínicas mais frequentes, localizações habituais, elevada frequência do sinal de Darier e idade precoce de início. Quando determinados, os níveis séricos de Triptase-alfa foram normais, o que está de acordo com a ausência de envolvimento sistémico. A maior prevalência no sexo masculino tem sido relatada em alguns estudos. PALAVRAS-CHAVE – Mastocitose cutânea; Criança.

Erythematous, firm, papular lesions over the face

A 63-year-old female presented with a history of multiple asymptomatic, erythematous, non-indurated papules and plaques on the upper lip, below the nose, above the medial end of both eyebrows, and in front of both the ears, of 5 years duration [Figures 1 and 2]. The patient denied of having any other symptoms of itching, photosensitivity, chest pain, weight loss, and was otherwise in good health. Her baseline investigations were within normal limits and chest X-ray showed bilateral hilar lymphadenopathy. A skin biopsy was done from the face, which showed epitheloid cell granulomas in the dermis [Figures 3 and 4].Figure 1: Multiple erythematous firm papular lesionsFigure 2: Closer view demonstrates the lesional morphologyFigure 3: Histopathologic photograph in low powerFigure 4: Better visualization in 100×DISCUSSION Sarcoidosis is a multi-system disease characterized by epitheloid cell tubercles without caseation although fibrinoid necrosis may be present at the center of few, proceeding either to resolution or to conversion of epitheloid tubercles into hyaline fibrous tissue.[1] Between 20 and 35% of patients with systemic sarcoidosis have skin lesions, but cutaneous sarcoidosis can also occur in the absence of systemic involvement.[23] Recognition of cutaneous lesions is important because they provide a visible clue to the diagnosis and are an easily accessible source of tissue for histologic examination.[4] Because lesions can exhibit many different morphologies, cutaneous sarcoidosis is known as one of the “great imitators” in dermatology.[3] Specific manifestations include papules, plaques, lupus pernio, scar sarcoidosis, and rare morphologies suchas alopecia, ulcers, hypopigmented patches, and ichthyosis.[56] Treatment of cutaneous lesions can be frustrating. The most effective treatment for patients with severe lesions or widespread is systemic corticosteroids.