Presence Of Cutaneous Lesions Research Articles

Cutaneous lesions and other non-endocrine manifestations of Multiple Endocrine Neoplasia type 1 syndrome.

Multiple Endocrine Neoplasia type 1 is a rare genetic syndrome mainly caused by mutations of MEN1 gene and characterized by a combination of several endocrine and non-endocrine manifestations. The objective of this study was to describe cutaneous lesions and other non-endocrine manifestations of MEN1 in a cohort of patients with familial (F) and sporadic (S) MEN1, compare the prevalence of these manifestations between the two cohorts, and investigate the correlation with MEN1 mutation status. We collected phenotypic and genotypic data of 185 patients with F-MEN1 and S-MEN1 followed from 1997 to 2022. The associations between F-MEN1 and S-MEN1 or MEN1 mutation-positive and mutation-negative patients and non-endocrine manifestations were determined using chi-square or Fisher's exact tests or multivariate exact logistic regression analyses. The prevalence of angiofibromas was significantly higher in F-MEN1 than in S-MEN1 in both the whole (p < 0.001) and index case (p = 0.003) cohorts. The prevalence of lipomas was also significantly higher in F-MEN1 than in S-MEN1 (p = 0.009) and in MEN1 mutation-positive than in MEN1 mutation-negative (p = 0.01) index cases. In the whole cohort, the prevalence of lipomas was significantly higher in MEN1 mutation-positive compared to MEN1 mutation-negative patients (OR = 2.7, p = 0.02) and in F-MEN1 than in S-MEN1 (p = 0.03), only after adjustment for age. No significant differences were observed for the other non-endocrine manifestations between the two cohorts. Hibernoma and collagenoma were each present in one patient (0.5%) and meningioma and neuroblastoma in 2.7% and 0.5%, respectively. Gastric leiomyoma was present in 1.1% of the patients and uterine leiomyoma in 14% of women. Thyroid cancer, breast cancer, lung cancer, basal cell carcinoma, melanoma, and colorectal cancer were present in 4.9%, 2.7%, 1.6%, 1.6%, 2.2%, and 0.5% of the whole series, respectively. We found a significantly higher prevalence of angiofibromas and lipomas in F-MEN1 compared with S-MEN1 and in MEN1 mutation-positive compared to MEN1 mutation-negative patients. In patients with one major endocrine manifestation of MEN1 , the presence of cutaneous lesions might suggest the diagnosis of MEN1 and a possible indication for genetic screening.

Cutaneous manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a distinct group of systemic vasculitis with severe multi-organ involvement. It includes three types: granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Cutaneous manifestations are present at the time of presentation in around 35% of cases and are more frequently encountered in EGPA than in GPA or MPA. They can also occur during the course of the disease or mark a disease relapse. They can be classified into specific or non-specific according to the presence or absence of vasculitis, whether or not associated with granuloma, in skin biopsies. The most common presentation of vasculitic skin lesions in AAV is palpable purpura. Other manifestations include hemorrhagic blisters, tender subcutaneous nodules, livedo reticularis/racemosa, painful ulcers that may mimic pyoderma gangrenosum (PG), and digital gangrenes. A wide range of non-specific cutaneous manifestations can occur in association with AAV including urticarial rash, prurigo, sterile pustules, and oral lesions. The presence of cutaneous lesions is usually associated with severe organ involvement and systemic manifestations. Since skin lesions in AAV can occur in other types of vasculitis and in other disorders, attributing them to AAV requires meticulous clinical, laboratory, and serological correlation. Awareness of cutaneous lesions of AAV is important for all physicians working in the field of systemic vasculitis as a skin biopsy may provide an easy clue to diagnose AAV in such cases.

Historical analysis of leishmaniasis cases in the transamazonian region: from 2009 to 2019

Objective: To determine the total number of notified cases, new cases and recurrence of American Tegumentary Leishmaniasis (ATL), to investigate the association between HIV coinfection and the presence of cutaneous lesion, as well to assess the concordance of two different laboratorial procedures: parasitological and histopathological in this region. Methods: This is a cross-sectional study. The research used data from the Information System for Notifiable Diseases, hence forth designated as SINAN (Sistema de Informação de Agravos de Notificação) that contained 6,183 cases of leishmaniasis in 9 municipalities. Results: As results, 5795 were of new cases and 351 recurrence cases. Among the cases 1,146 patients were tested for HIV, 16 presented coinfection from these 15 developed cutaneous lesion. In the overall studied population 5,690 subjects manifested cutaneous lesion. There was no agreement of the results regarding the quality of the parasitological and histopathological exams, demonstrating that they are poorly replicable (p &lt;0.005). Conclusion: The ATL is endemic in the transamazonian region posing as a major public health problem. All patients with leishmaniasis must be tested for HIV and health professionals must register on the notification form. The tests to diagnose leishmaniasis need to be more specific and sensitive.

Characteristic CD86 stain for organisms in cutaneous lesions of Talaromyces marneffei infection

To the Editor: Talaromyces marneffei (previously Penicillium marneffei) infection is endemic to Southeast Asia.1 The change in name reflects the recent taxonomic work in which many members of a subgenus of Penicillium were reassigned to the genus Taloromyces.2 A common sign of the T marneffei infection is the presence of cutaneous lesions, and the diagnosis is often made by skin biopsy. We studied macrophage polarization by performing immunohistochemical staining of cutaneous sections of deep mycosis.

Neuropsychiatric, cognitive and sexual impairment in mastocytosis patients

BackgroundMastocytosis is a rare disease characterised by the accumulation and/or proliferation of abnormal mast cells (MCs) in one or several organs. It may present with a number of different symptoms that involve various organ systems. The current study aims to assess the prevalence of MC mediator-related symptoms in a cohort of mastocytosis patients with a specific focus on neurological, psychiatric, cognitive and sexual symptoms. We also assessed the impact of the disease on patients’ professional lives. Patients were administered a validated multidimensional questionnaire to collect information on patients’ perception of the severity of their symptoms. From the questionnaires we extracted the neurological, cognitive, psychiatric and sexual symptoms and the impact of the disease on patients’ professional lives as well as their grading. The affective status was assessed using the 17-item version of the Hamilton Depression Rating Scale.ResultsWe included 139 patients. Mastocytosis was classified as systemic in 113 patients and cutaneous in 26 patients. The prevalence of MC mediator-related systemic symptoms was as follows: cutaneous (71%), gastro-intestinal (48%), cardio-vascular (36%), musculoskeletal (26.6%), fatigue (24%), urinary (14.4%) and respiratory (10%). Headaches and vertigo were noted in respectively 55% and 32% of patients. Irritability, episodes of memory loss and difficulty concentrating were reported in 54%, 52% and 40% of cases, respectively. Sexual impairment was noted in 24% of patients. No associations were found between neuropsychiatric/cognitive impairment and age, gender, diagnostic delay, disease form, the presence of cutaneous lesions, the level of serum and bone marrow tryptase and the presence of KIT mutation in bone marrow and/or skin. Depression was noted in 49% of patients. One in four patients reported a negative impact of the disease on their professional lives.ConclusionThis current study provides some insights regarding symptoms related to mastocytosis and their impact on patients’ professional lives.

RAPIDLY PROGRESSIVE INTERSTITIAL LUNG DISEASE IN ASSOCIATION WITH MDA5 ANTIBODY DERMATOMYOSITIS

SESSION TITLE: Medical Student/Resident Diffuse Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Clinically Amyopathic Dermatomyositis (CADM) is a subtype of dermatomyositis (DM) characterized by the presence of cutaneous lesions, without clinical evidence of muscle weakness. The presence of autoantibodies against Melanoma Differentiation-Associated protein 5 (MDA5) in this group is highly associated with rapidly progressive interstitial lung disease (RP-ILD) and a worse prognosis. We present a case of anti-MDA5 associated RP-ILD to illustrate the importance of screening for MDA5 in patients with CADM and DM. CASE PRESENTATION: Our patient was a 45-year-old woman with a history of hypothyroidism, seronegative inflammatory polyarthritis treated with hydroxychloroquine, and notably, a history of biopsy-proven DM with a negative myositis-specific antibodies (MSA) panel (which did not include MDA5 antibodies). She had been successfully treated for DM, and maintained in remission with oral steroids. Four years following the diagnosis of DM, she presented with insidious and gradually progressive dyspnea for over a year. Physical examination was unremarkable. Pulmonary function testing showed moderate restrictive lung disease. Chest X-ray was notable for bibasilar interstitial changes. Computed tomography (CT) scan of the chest revealed nonspecific, bilateral ground-glass opacities in the middle and lower lung zones, with associated bibasilar bronchiectasis in the affected areas (Figure 1). Bronchoscopic evaluation, including transbronchial biopsy, was performed, which was negative for malignancy or infection. Open lung biopsy was obtained via robotic-assisted thoracoscopy from the affected lobes, with histopathology revealing interstitial fibrosis with multiple fibrotic foci without clear evidence of honeycombing. The pattern was suggestive of usual interstitial pneumonia (UIP). Repeat testing for MSA revealed an elevated anti-MDA5 antibody level at 22 SI. The patient was consequently diagnosed with RP-ILD in conjunction with CADM, prompting the initiation of oral prednisone and azathioprine. DISCUSSION: Anti-MDA5 antibodies recognize the RNA helicase encoded by MDA5, a protein that is involved in innate immune processes. The presence of anti-MDA5 antibodies in patients with CADM has been linked to the presence of RP-ILD, and portends a poor prognosis. The mechanism of this autoantibody production is not fully understood. One meta-analysis found that the presence of these antibodies was 77% sensitive and 86% specific for the identification of RP-ILD. Additionally, anti-MDA5 antibodies can be utilized to predict clinical outcomes and monitor disease activity in these patients. Due to its high mortality rate from respiratory failure, immediate therapy with high-intensity combination immunosuppression is essential once the diagnosis is made. CONCLUSIONS: This case highlights the importance of screening for anti-MD5 antibodies in patients with CADM to elucidate their risk of developing RP-ILD. Reference #1: Li L, Wang Q, Yang F, et al. Anti-MDA5 antibody as a potential diagnostic and prognostic biomarker in patients with dermatomyositis. Oncotarget. 2017;8(16):26552–26564. doi:10.18632/oncotarget.15716 Reference #2: Chen, Z., Cao, M., Plana, M.N., Liang, J., Cai, H., Kuwana, M. and Sun, L. (2013), Utility of Anti–Melanoma Differentiation–Associated Gene 5 Antibody Measurement in Identifying Patients With Dermatomyositis and a High Risk for Developing Rapidly Progressive Interstitial Lung Disease: A Review of the Literature and a Meta-Analysis. Arthritis Care & Research, 65: 1316-1324. doi:10.1002/acr.21985 DISCLOSURES: No relevant relationships by Komal Ejaz, source=Web Response No relevant relationships by Dan Kazmierski, source=Web Response No relevant relationships by Bassel Noumi, source=Web Response No relevant relationships by Pius Ochieng, source=Web Response No relevant relationships by NISHANT SHARMA, source=Web Response No relevant relationships by Yichen Wang, source=Web Response

Diaper rashes can indicate systemic conditions other than diaper dermatitis

BackgroundAlthough the majority of rashes in the diaper area are caused by irritation from urine and feces, irritant diaper dermatitis; IDD, there are some less common but potentially serious cutaneous eruptions associated with systemic diseases that should not be discounted.MethodsThis prospective descriptive study aimed to explore variation in cutaneous disease in the diaper area. It was conducted as a prospective descriptive study between October 2016 and November 2019 in the pediatric department of a tertiary-level hospital.ResultsThree hundred consecutive patients with rashes in the diaper area were enrolled. The most common diagnosed was IDD (125 cases; 41.7%), followed by rashes exacerbated by the diaper (101 cases; 33.67%) and non-diaper-related rashes (74 cases; 24.67%).ConclusionsOur finding suggests that when diagnosing rashes that occur in the diaper area, general pediatricians should consider, in addition to IDD, the possibility of less-common conditions. The simultaneous presence of cutaneous lesions at other sites was linked to diagnoses of systemic diseases other than IDD, (P < 0.001).

ESPOROTRICOSE, O GATO E A COMUNIDADE

Dermatozoonoses have a worldwide occurrence, being more prevalent in tropical and subtropical countries. Epidemiological data indicate that sporotrichosis is the most common subcutaneous mycosis in Latin America. Its importance for public health as an emerging disease is mainly associated with cases involving zoonotic transmission by domestic felines in Brazil. The disease is caused by species of the genus Sporothrix, being the one of greater occurrence in Brazil, Sporothrix brasiliensis. The most common clinical picture in mammals is acute or chronic, with the presence of cutaneous lesions and, in humans, it has a social impact during its active phase with an unpleasant appearance causing discomfort to the affected person. The disease has a cure, however the treatment of this mycosis, in general, is related to the prolonged use of systemic antifungals. The treatment of cats with sporotrichosis is a challenge due to several factors, among them occurrence of therapeutic failures, besides the high cost. Sporotrichosis presents with importance in the environmental health theme, with emphasis in the context of the sociocultural aspects of the disease, which can be observed mainly in hot and humid places, with high population density, poor sanitary conditions or lack of responsible custody of dogs and cats.

CD45+, CD68+ and E-cadherin+ Expressions in Skin Dogs Naturally Infected by Leishmania infantum

Background: In canine leishmaniasis (CanL), infection occurs through phlebotomine vectors that inoculate the protozoan Leishmania infantum into the skin that infected macrophages and activated dendritic cells (CD). Dogs with CanL present variable clinical manifestations, being common the presence of cutaneous lesions. The aim of this study was to evaluate the expression of CD45+, CD68+ and E-cadherin+ associating the skin sentinels cells and to compare the clinical-dermatological manifestations in the skin of dogs naturally infected by L. infantum.Materials, Methods &amp; Results: Dogs infected (n = 22) by L. infantum were divided into asymptomatic group (AD, n = 9), and symptomatic group (SD, n = 13), according criteria based on the presence or absence of skin changes. Dogs non-infected (CD, n = 5) were included as control group. Samples of skin biopsies collected from scapular region were processed by routine histology and labeled by immunohistochemistry with monoclonal antibodies against CD45+, CD68+ and E-cadherin+, and were described as none, mild, moderate and intense. SD presented keratoconjunctivitis, onychogryphose, lichenification, depigmentation, alopecia, hypotrichosis, erythematous dermatitis, exfoliative dermatitis, ulcerative dermatitis and crusted dermatitis, and the frequency these alterations was expressed as percentage. The results of hematological and biochemical parameters were analyzed by Kruskal-Wallis test followed by the Dunn’s test and expressed as mean ± standard deviation, with values P &lt; 0.05. Leukocytosis (not significant), red blood cells, hematocrit and hemoglobin (P &lt; 0.05), total protein serum (P &lt; 0.05), globulins (P &lt; 0.05), albumin and A/G ratio (P &lt; 0.01) were altered in SD in relation to CD. Cutaneous cellular infiltration, composed by macrophages, plasma cells, lymphocytes and neutrophils, was observed in CD. There was an increase of expression of the markers in SD when compared to the other groups, as moderate CD68+ expression and L. infantum, and intense CD45+ and E-cadherin+ expressions.Discussion: Cutaneous involvement is very important in CanL, as it corresponds to where is the first interaction between the parasite and the immune system. Dermatological clinical signs, leukocytosis, anemia, globulins levels have been reported for dogs naturally infected by L. infantum. Inflammatory infiltrate was distributed at superficial and deep dermis, which was composed by mononuclear cells as macrophages, plasma cells, lymphocytes and neutrophils. To characterize the immune sentinels cells in the skin it was evaluated CD45+, CD68+ and E-cadherin+ expressions. In syntomatic dogs, our results revelead an increase of expression of these markers. CD45+ is one of the most abundant molecules expressed on the white blood cell surface in various mammals, while CD68+ is a myelomonocytic marker that seems to be retained during monocyte differentiation. In the skin, increased numbers of CD68+ are related to dendritic epidermal cells, which can be expressed as CD45+/CD1a-/HLA-DR+. DCs of the skin, particularly epidermal Langerhans cells (LCs), form networks anchored to neighboring keratinocytes via E-cadherin. Thus, CD45+, CD68+ and E-cadherin+ expressions may be related to activation of skin sentinels cells in dogs naturally infected by L. infantum. Our results indicated that CanL modify the CD45+, CD68+ and E-cadherin+ expressions, which characterize the immune sentinels cells activation that promove the recruitment the cellular infiltrate, which was composed by macrophages, plasma cells, lymphocytes and neutrophils. Thus, these informations may contribute to the follow-up of CanL progression in skin.

Prevalence and CT-Scan Presentations of Brain Malformations in Children at a University-Affiliated Mother and Child Hospital (Cameroon)

Background: Brain malformations (BMs) are congenital abnormalities of the shape or structure of the brain, with corpus callosum agenesis known as the most frequent. Diagnosis of most BMs can be prenatal with ultrasound and fetal magnetic resonance imaging (MRI); post-natal diagnosis is based on transfontanellar ultrasound, CT-scan, and head MRI which is the imaging gold standard technique. MRI has been recently introduced and the CT-scan was previously the reference technique for the diagnosis of BMs in our context. Almost no publication has been made in sub-Saharan Africa on the clinical and CT scan characteristics of Brain malformations in children. Objective: The aim of this study was, in the absence of MRI, to describe the clinical and CT-scan presentations of brain malformations in children at the Yaounde Gynaeco-Obstetric and Pediatric Hospital (YGOPH). Patients and method: This was a cross-sectional descriptive study conducted from February to May 2016 at the YGOPH, including all children of 15-year-old and less with BM diagnosed on CT-scan at YGOPH between April 2006 and March 2016. The studied variables were clinical (age at diagnosis, sex, clinical manifestations) and CT findings (type of cerebral malformation). The data was analyzed using the SPSS 20.0 software with the estimation of hospital prevalence of BMs, frequencies and associations. The chi-square test was used to seek for an association between variables. The threshold of significance was p p = 0.007) with disorders of brain separation (DBS) while the presence of cutaneous lesions was associated (p = 0.013) with anomalies of the cortical development (ACD) especially tuberous sclerosis complex. Conclusion: BMs are infrequent, dominated by tuberous sclerosis complex and agenesis of the corpus callosum. They are mainly revealed by convulsions and psychomotor retardation. Efforts are needed to improve antenatal diagnosis and facilitate access to cerebral MRI.

A Rare Case of Chin Cutaneous Metastases from Transitional Cell Carcinoma of Bladder in a Patient Who Underwent Radical Cystectomy

Facial cutaneous metastases from primary bladder cancers are very rare and usually they are associated with poor prognosis. In literature, very few cases of cutaneous metastases from urothelial malignancies are reported but we did not find any case of bladder cancer metastases localised to chin; usually cutaneous metastases from bladder are localised in chest or scrotal skin. We report a 66 year-old patient affected by muscle-invasive transitional cell carcinoma of bladder who underwent radical cystectomy and who developed in two months after operation a skin lesion localised to chin. Clinical stage of bladder cancer was cT2 N0 M0; the patient was studied before operation through total body CT-scan, TB bone-scan negative for metastases. The patient was totally asymptomatic and he had a good performance status. We discuss the need to have suspicion in presence of cutaneous lesions, apparently benign, in a patient affected by malignancy in order to start quickly a proper treatment.

Skin Findings in Newborns and Their Relationship with Maternal Factors: Observational Research

BackgroundCutaneous lesions are commonly seen in the newborn period and exhibit inconsistency from the skin lesions of an adult.ObjectiveThe present study was carried out with an aim to determine the frequency of physiologic and pathologic cutaneous findings in newborns.MethodsTypically, 1234 newborns were included in this study. A questionnaire about maternal gestational history, maternal and family history was issued to the parents of each newborn. The presence of cutaneous lesions was recorded.ResultsOverall, 642 (52%) of the newborns were male and 592 (48%) were female. Typically, 831 newborns (67.3%) had at least one cutaneous lesion. The prevalence of genital hyperpigmentation and milia was significantly higher in males. In premature newborns, the pervasiveness of cutis marmorata and genital hyperpigmentation was found to be significantly higher. Caput succedaneum, transient neonatal pustular melanosis and cyanosis appeared predominantly in vaginally born infants. Erythema toxicum neonatorum was seen in infants, who were born by cesarean section. The predominance of Mongolian spots and erythema toxicum neonatorum were significantly higher in the newborns of the multiparous mothers; however, caput succedaneum was significantly higher in newborns of the primiparous mothers.ConclusionA number of studies about neonatal dermatoses have been carried out involving different methods in various countries. We consider that our study may be useful in literature, as it has been carried out involving large number of maternal parameters.

Epidemiological Findings and Laboratory Evaluation of Sporotrichosis: A Description of 103 Cases in Cats and Dogs in Southern Brazil

Sporotrichosis is a subcutaneous mycosis, which affects mainly small animals, and is considered an important public health disease. This paper describes the epidemiological and laboratory characteristics of 103 clinical cases of sporotrichosis diagnosed over a 10-year period in southern Brazil. The 92 cats and 11 dogs from eight municipalities in Rio Grande do Sul State developed especially the disseminated cutaneous and fixed cutaneous forms of the disease. Respiratory signs such as sneezing, serous nasal discharge and dyspnea were found in about 57% of the animals. The detection of Sporothrix schenckii in different clinical samples showed highest isolation in testicles (46.6%), oral cavity (45.2%) and conjunctival mucosa (38.1%). A differentiated histological pattern was found between the fixed cutaneous and disseminated cutaneous (DC) manifestations of the disease; well-organized granulomas of nodular distribution and various fungal structures prevailed in the DC form in cats. Melanin detection in S. schenckii cells by the Fontana-Masson technique was positive in 45.4% of the samples. The study revealed that the State of Rio Grande do Sul is an endemic sporotrichosis area and demonstrated the possibility of involvement of other pathways in the infection and spread of the disease. In addition, it emphasized the importance of laboratory tests for mycosis confirmation, especially in dogs that develop clinical manifestations without the presence of cutaneous lesions.

Presence of cutaneous lesion is a poor prognostic factor in patients with smoldering-type adult T-cell leukemia-lymphoma

Background Prognosis of indolent types of adult T-cell leukemialymphoma (ATL) including the smoldering type was recently reported to be poorer than that shown in previous studies. Prognostic factors of smoldering-type ATL have not been defined. Cutaneous-type, which is defined as cases of smoldering type predominantly involve skin with or without peripheral blood involvement has been proposed as a distinct clinical subtype. Prognosis of cutaneous-type ATL was reported to be poorer than that of smoldering-type without cutaneous involvement. Aim To determine prognostic factors for survival and disease progression of smoldering-type ATL. Patients Thirty-one patients with smoldering-type ATL including 21 with cutaneous lesion. Methods Multivariate Cox proportional hazards model was used to identify variables associated with survival and disease progression. Peripheral blood abnormal lymphocytes (<5% vs ≥5%), serum lactate dehydrogenase (normal vs high), albumin level (<4 vs ≥4 g/dl), and cutaneous lesion (none vs present) were used as variables. Overall survival (OS) and progression-free survival (PFS) were estimated using the Kaplan-Meier method and compared using the log-rank test. Results In the multivariate analysis, presence of cutaneous lesion was the significant prognostic factor for PFS (hazard ratio, 8.69; 95% confidence interval, 1.4-54.0; P = 0.02). Peripheral blood abnormal lymphocytes, serum lactate dehydrogenase, or albumin level was not significant. None of the variables was significantly associated with OS in the multivariate analysis. OS (P = 0.117) and PFS (P = 0.089) of the patients with cutaneous lesion were worse as compared to those of the patients without cutaneous lesion, though statistically not significant. Conclusions In this study we confirmed that presence of cutaneous lesion is an independent prognostic factor in patients with smoldering-type ATL. Cases of smoldering-type with cutaneous lesion should be classified as cutaneous-type ATL irrespective of the peripheral blood involvement.

Acinar cell carcinoma of the pancreas: A rare tumor with a particular clinical and paraclinical presentation

Acinar cell carcinoma (ACC) of the pancreas is a rare tumor with an extremely low incidence rate. While the number of reported patients with ACC is relatively small, a long-term survival rate has been noted in patients with neuroendocrine differentiation. A 39-year-old woman visited our emergency department for upper gastrointestinal bleeding. Endoscopy indicated extrinsic compression of the posterior body of the stomach, together with a large, 10-cm, central ulcer covered with necrotic tissue. Abdominal computed tomography (CT) indicated a lesion that involved the whole of the pancreas, with a fistula in the stomach, which was suspected of being a degenerative intraductal papillary mucinous tumor of the pancreas. Magnetic resonance imaging (MRI) of the pancreas was performed, and the results further strengthened our suspicions by demonstrating the presence of cystic lesions and tumor buds. A total duodenopancreatectomy, including total splenectomy and gastrectomy, was performed, along with two independent Roux-en-Y anastomoses (one esojejunal and one hepaticojejunal). The tumor also had a wide opening in the stomach. The patient's postoperative course was marked by partial thrombosis of the portal vein, which was treated medically. Histopathological examination provided evidence of pancreatic ACC. The diagnosis of ACC should be considered in the presence of cutaneous lesions, which were absent in the case of our patient, and colonoscopy is also highly desirable because of the various forms associated with familial adenomatous polyposis. The prognosis, which includes a 5-year survival rate of 45%, in the population with an R0 resection is better than that for ductular adenocarcinoma, thus prompting the more aggressive management of this type of tumor.

Cutaneous angiolymphoid hyperplasia with high endothelial venules is characterized by endothelial expression of cutaneous lymphocyte antigen

Two cases in which the presence of cutaneous lesions with a characteristic admixture of lymphoid hyperplasia and vascular proliferation lead to the diagnosis of so-called acral pseudolymphomatous angiokeratoma (APA) of children are reported. Owing to the prominence of its blood vessels, so striking as to be reminiscent of high endothelial venules (HEVs), APA was initially interpreted as a vascular lesion rather than a pseudolymphoma. This resemblance is further compounded by our finding that cutaneous lymphocyte antigen (CLA), an HEV marker, is also expressed in APA epithelioid blood vessels. Consequently, we believe that “cutaneous angiolymphoid hyperplasia with high endothelial venules” (ALH-HEV), which alludes to the dual nature of proliferating elements and the HEV-like phenotype of the vascular component, would be a better denomination for this entity. Additionally, we speculate that ALH-HEV lymphoid hyperplasia self-perpetuates through the transformation of dermal capillaries into HEVs, which would bind non-skin homing lymphocytes expressing L-selectin and promote their local recruitment and recirculation. HUM PATHOL 32:227-229. Copyright © 2001 by W.B. Saunders Company

Acute respiratory failure associated with cryptococcosis in patients with AIDS: analysis of predictive factors.

The incidence of acute respiratory failure (ARF) associated with cryptococcal disease in patients with AIDS is underestimated in the literature. We performed a retrospective, case-control (referent) study to determine the prevalence of ARF associated with cryptococcal disease and analyzed associated factors. Potential cases of ARF were identified at four university-affiliated teaching hospitals from a cohort of 210 patients with AIDS who had positive cryptococcal antigen tests and/or Cryptococcus neoformans isolated from any body site. Twenty-nine of the 210 (13.8%) had ARF associated with cryptococcal disease. Nineteen were thought to have respiratory failure due solely to C. neoformans. The demographic, clinical, laboratory, treatment, and outcome data of 19 cases of respiratory failure were compared with data for 20 patients without respiratory failure. In-hospital mortality was 100% and median survival was 2 days for cases, vs. 25% and > 365 days, respectively, for referents. The clinical presentation was identical to that of Pneumocystis carinii pneumonia. In multivariate analysis, variables independently predictive of ARF in patients with cryptococcal disease were black race, a lactate dehydrogenase level of > or = 500 IU/L, the presence of interstitial infiltrates, and the presence of cutaneous lesions. ARF with cryptococcosis in patients with AIDS is associated with disseminated disease and high mortality. The diagnosis frequently is not considered before death. Serum cryptococcal antigen testing is a sensitive and rapid screening method.

IgA Deposition and Pathogenesis of Dermatitis Herpetiformis

These authors examined the relationship between granular deposits of IgA in the papillary dermis and the presence of cutaneous lesions of dermatitis

Herpes Zoster Radiculopathy

Herpes zoster-related radiculopathy usually can be easily diagnosed in the presence of cutaneous lesions. Before development of the skin rash, the diagnosis may be in doubt, particularly if motor symptoms and signs are a major clinical feature. We report a patient with herpes zoster-related radiculopathy whose clinical features mimicked other spinal disorders.

Delusions of parasitosis: a review.

Patients who are erroneously convinced that they harbor dermatologic parasites present a diagnostic and therapeutic challenge to the practitioner. Delusions of parasitosis can exist as an isolated psychosis or as part of a much more complicated medical and/or psychiatric illness. To diagnose these patients, physicians must be familiar with the patterns of presentation usually seen in patients with delusions of parasitosis and must be confident that other psychiatric and/or medical conditions that could be causing the symptoms--including true parasitosis--have been ruled out. Clinical signs, such as the presence of cutaneous lesions on exposed and accessible parts of the body (wounds self-inflicted in efforts to extricate parasites with needles, knives, or fingernails), and behavioral signs, such as the collection of bottles and jars purported to contain samples of the parasites, are characteristic. These patients can be rigid, antagonistic, distant, and demanding; they may write long letters to physicians describing their parasites in detail, enclosing drawings or samples. Psychotherapy, with or without confrontational denial, has a reported efficacy of approximately 10%; placebo therapy is usually not recommended. The recent application of a new neuroleptic agent, pimozide, to the treatment of this disorder is controversial and a new neuroleptic agent, pimozide, to the treatment of this disorder is controversial and needs further study. Guidelines for an appropriate approach to patients with delusions of parasitosis include the necessity of identifying medical or psychiatric disease, if present, and of listening carefully to the patient's history.(ABSTRACT TRUNCATED AT 250 WORDS)