Patterns of Skin Disease in the Context of a High Prevalence HIV Population in Botswana.

The authors reviewed outpatients in a tertiary dermatology clinic in Botswana to expand knowledge on patterns of skin disease in this population with a high prevalence of human immunodeficiency virus (HIV). Approximately one-third of new and follow-up patients were HIV positive. Common dermatologic conditions included eczematous eruptions, viral and fungal infections, malignant neoplasms, vascular disorders, disorders of pigmentation, and mechanical/physical injury-related disorders. HIV has impacted patterns of dermatologic disease in Botswana, with Kaposi sarcoma being the most frequently biopsied condition. Given the shortage of dermatology specialists, resources should be allocated toward education and management of these most prevalent skin conditions.

Management of cutaneous vasculitis.

Cutaneous vasculitis encompasses cutaneous components of systemic vasculitides, skin-limited variants of systemic vasculitides, such as IgA vasculitis or cutaneous polyarteritis nodosa, and single-organ cutaneous vasculitis, as individualized in 2012 in the Chapel Hill Consensus Conference Nomenclature. In this article, we focus on the management of skin-limited and single-organ vasculitides, often referred to, in clinical practice, as isolated "cutaneous leukocyctoclastic vasculitis", terms which may correspond to histological findings or descriptions, but are imprecise and not specific. Since most cases of isolated cutaneous vasculitis are self-limited and resolve spontaneously over 3 to 4 weeks, most patients require no systemic treatment. For those with severe, intractable, or chronic and recurring vasculitis, systemic therapy can be indicated and should be tailored to the severity of the disease. High-quality literature is lacking to guide management. Oral glucocorticoids may be required for a short period of time for painful, ulcerative, or otherwise severe disease in order to speed resolution. Among drugs which are reasonable longer-term options are colchicine, dapsone, azathioprine or hydroxychloroquine. Additional studies, including an ongoing multicenter randomized trial, are needed to determine the most effective therapies for skin-limited vasculitis.

Clinical presentation of Moyamoya angiopathy in Europeans: experiences from Germany with 200 patients.

INTRODUCTION: Moyamoya angiopathy (MMA) is a rare vasopathy, especially among European Caucasians. Data about demographics, clinical presentation, comorbid conditions, radiological findings as well as laboratory and cerebral spinal fluid (CSF) data are sparse. METHODS: Patients with MMA treated in the Alfried Krupp Hospital, Essen, Germany, between 2010 and 2017 with focus on demographic, clinical, radiological and laboratory as well as CSF data were evaluated retrospectively. Patients with non-Caucasian family background were excluded from this study. RESULTS: Altogether 200 European Caucasian patients with MMA were identified. There was a female predominance of 3.2:1. The mean age at first presentation was 32.9 years and the mean age of diagnosis was 36.0 years. Eleven of 194 index patients (5.7%) showed a familial presentation. In 11.6% posterior cerebral artery was additionally involved, in 4% additionally cerebral aneurysm and in 2.5% dysgenesis of corpus callosum was found. Most patients suffered from transient ischemic attacks (71.5%) and stroke (82%). Cerebral hemorrhage was found in 9.5%. Livedo racemosa was an associated symptom in 12.8% of patients and thyroid diseases were found in 23.8%. CONCLUSIONS: Compared with Asian data, cerebral hemorrhages are infrequent and female predominance is accentuated among European Caucasians. Some former unknown rare features like associated livedo racemosa, dysgenesis of corpus callosum and associated syncope have been discovered systematically for the first time in this huge European Caucasian cohort.

Cutaneous microangiopathy in patients with type 2 diabetes: Impaired vascular endothelial growth factor expression and its correlation with neuropathy, retinopathy and nephropathy.

AIMS/INTRODUCTION: To examine the three-dimensional morphology and vascular endothelial growth factor (VEGF) expression of skin microvasculature in patients with type 2 diabetes in relation to neuropathy, retinopathy and nephropathy. MATERIALS AND METHODS: The present study enrolled 17 individuals with type 2 diabetes and 16 without. Skin sections were double-immunostained for type IV collagen and VEGF-A or protein gene product 9.5. Projected images from confocal microscopy served to quantify the occupancy rate of subepidermal type IV collagen-immunoreactive microvascular basement membrane area (OR-T4MBM), subepidermal VEGF-A-immunoreactive area and the VEGF/T4MBM ratio, as well as the protein gene product 9.5-immunoreactive intraepidermal nerve fiber density. Reduced intraepidermal nerve fiber density was applied for the diagnosis of neuropathy, fundic ophthalmoscopy and fluorescein angiography for retinopathy, and microalbuminuria or persistent proteinuria for nephropathy. RESULTS: A total of 12 patients with diabetes had neuropathy, 10 had retinopathy and eight had nephropathy. Regardless of the presence or absence of neuropathy, retinopathy or nephropathy, OR-T4MBM was significantly increased in patients with diabetes compared with individuals without diabetes. In contrast, VEGF/T4MBM ratio was significantly decreased in those with neuropathy and retinopathy, as well as in those with and without nephropathy, whereas a trend toward a decreased VEGF/T4MBM ratio was seen in patients without retinopathy, as compared with individuals without diabetes. CONCLUSIONS: The present study is the first report to show that cutaneous microangiopathy, as indicated by subepidermal microvascular proliferation and impaired VEGF expression, appears to occur before the development of overt clinical neuropathy, retinopathy or nephropathy in patients with type 2 diabetes.

[Vascular Cutaneous Abnormalities in Togo: a 120-Case Study]. / Anomalies vasculaires cutanées au Togo : étude de 120 cas.

The purpose of this study was to describe the epidemio-clinical profile, and treatment of vascular cutaneous abnormalities in Togo. It was a retrospective study of patients recorded in dermatology for vascular cutaneous abnormality between 1998 and 2017. During the study period, 120 (0.1%) of 88,869 patients received in dermatology have consulted for vascular cutaneous abnormalities. Their mean age was 25.3 months and the sex-ratio (M/F) 0.4. The most recurring vascular cutaneous abnormalities were vascular tumors (97 cases; 80.8%), mainly infantile hemangioma (IH) (93 cases; 77.5%). The mean time of onset of IH after birth was 2.9 months. The IH (54 cases; 50.5%) and port wine stains (8 cases, 53.3%) were predominantly localized at the cephalic region and Klippel-Trenaunay syndrome cases on lower limbs. We opted for a therapeutic abstention in the majority of the patients (50 cases of IH, 16 cases of simple vascular abnormalities, all Klippel-Trenaunay syndrome cases and telegiectasia cases). Of the 43 IH cases treated, the main drugs used were corticosteroids (23 cases of which 17 between 1998 and 2011 and 6 from 2012) followed by propranolol (11 cases from 2012). We had a favorable response in 9 of the 15 patients seen again. Cutaneous vascular abnormalities are very rare in dermatology in Togo and are dominated by IH with female predominance. In IH treatments, propranolol use, started in 2012 in Togo, is increasing when corticotherapy has declined.

L'objectif de cette étude était de décrire le profil épidémioclinique et thérapeutique des anomalies vasculaires cutanées au Togo. Il s'agit d'une étude rétrospective portant sur les dossiers des patients reçus en dermatologie pour une anomalie vasculaire cutanée entre 1998 et 2017. Durant la période d'étude, 120 (0,1 %) des 88 869 patients reçus en dermatologie ont consulté pour une anomalie vasculaire cutanée. L'âge moyen des patients était de 25,3 mois et le sex-ratio (H/F) de 0,4. Les anomalies vasculaires les plus recensées étaient les tumeurs vasculaires (97 cas ; 80,8 %), principalement les hémangiomes infantiles (HI) [93 cas ; 77,5 %]. Le délai moyen d'apparition des HI après la naissance était de 2,9 mois. Les HI (54 cas ; 50,5 %) et les angiomes plans (8 cas, 53,3 %) étaient localisés de façon prépondérante à la région céphalique et les cas de syndrome de Klippel-Trenaunay aux membres inférieurs. Nous avons opté pour une abstention thérapeutique chez la plupart des patients (50 cas d'HI, 16 cas de malformations vasculaires simples, tous les cas de syndrome de Klippel-Trenaunay et le cas de télangiectasies). Sur les 43 cas d'HI traités, les principaux médicaments utilisés étaient les corticoïdes (23 cas dont 17 entre 1998­2011 et 6 à partir de 2012) suivis du propranolol (11 cas à partir de 2012). Nous avions noté une réponse favorable chez 9 des 15 patients revus. Les anomalies vasculaires cutanées sont très rares en dermatologie au Togo et sont dominées par les HI, avec une prédominance féminine. Dans le traitement des HI, l'usage du propranolol, débuté en 2012 au Togo, est en augmentation pendant que celui de la corticothérapie baisse.

Is thrombocytosis always an indicator of autosplenectomy in patients with systemic lupus erythematosus?

In systemic lupus erythematosus (SLE), the most commonly encountered finding related to platelets is thrombocytopenia whereas thrombocytosis is rarely reported. Our aim here was to reveal the type and the frequency of thrombocytosis in SLE patients along with its causes. Data of patients were evaluated retrospectively. Patients who had a platelet count of > 450,000/mm3 (> 450 × 109/L) in at least two subsequent counts and lasting more than 6 months during the follow-up were considered to have "persistent thrombocytosis". Peripheral smear results of patients with thrombocytosis were analyzed, and spleen imaging was performed for autosplenectomy/hyposplenism to patients with persistent thrombocytosis. A total of 205 patients with SLE were included in the study [196 (95.6%) female, mean age 41.5 years]. Out of 12 patients (5.9%) with thrombocytosis, 9 (4.3%) had transient thrombocytosis and 3 patients (1.4%) had persistent thrombocytosis. Of those with transient thrombocytosis, 5 were associated with iron deficiency anemia (IDA), 2 to polyarthritis, and the remaining 2 to digital ischemia and/or cutaneous vasculitis. Of three patients with persistent thrombocytosis, one was identified to have had splenectomy due to resistant immune thrombocytopenic purpura, and the other two (0.9%) patients had autosplenectomy. The only independent risk factor for the development of thrombocytosis was the presence of cutaneous vasculitis (OR 10.79 (95% CI 2.14-54.47), p = 0.0004). During the course of SLE, frequency of thrombocytosis is similar to that of the general population and the most common cause is reactive thrombocytosis. If the thrombocytosis was persistent, rheumatologist must consider that the patient may have autosplenectomy/asplenia/hyposplenism.

Vasculitis: Kids are not just little people.

Cutaneous vasculitis, inflammatory destruction of blood vessels, can present with a wide range of clinical and pathologic findings across a number of heterogeneous conditions. Although some vasculitides are present in both children and adults, some important differences exist in clinical presentation, etiology, management, and prognosis in childhood vasculitis versus adult vasculitis. Cutaneous vasculitis is rare in children, and most childhood vasculitides, of which Henoch-Schönlein purpura is the most common, histologically are small vessel leukocytoclastic vasculitis. In children, infectious etiologies are more common than in adults. Childhood cutaneous vasculitis is most often self-limited with a good prognosis, and treatment is mainly supportive. © 2017 Elsevier Inc. All rights reserved.

Wilms tumor screening in diffuse capillary malformation with overgrowth and macrocephaly-capillary malformation: A retrospective study.

BACKGROUND: CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies) syndrome is associated with regional bony and/or soft tissue overgrowth, capillary malformation, and an increased risk for Wilms tumor. OBJECTIVE: To evaluate the frequency of Wilms tumor in patients with 2 similar conditions: diffuse capillary malformation with overgrowth (DCMO) and macrocephaly-capillary malformation (M-CM). METHODS: Culling our Vascular Anomalies Center database, we retrospectively reviewed patients in whom DCMO and M-CM had been diagnosed and who were evaluated between 1998 and 2016 for possible development of Wilms tumor. Patients younger than 8 years of age at their last visit and not seen in more than 2 years were contacted for follow-up. RESULTS: The study comprised 89 patients: 67 with DCMO, 17 with M-CM, and 5 with an indeterminate diagnosis. No case of Wilms tumor was found in these groups. LIMITATIONS: Some patients were younger than 8 years of age at last follow-up visit and the sample size was small. CONCLUSION: Patients with DCMO do not appear to be at increased risk for Wilms tumor. Screening is probably unnecessary in DCMO unless there is associated hemihypertrophy. Although there were no cases in our cohort, there are 2 reports of M-CM associated with Wilms tumor in the literature.

[First manifestation of leg ulcers : Analysis of data from 1000 patients]. / Erstmanifestation eines Ulcus cruris : Analyse der Daten von 1000 Patienten.

BACKGROUND: More than 1 million people in Germany suffer from leg ulcers. The diagnosis leg ulcer summarizes many different etiologies. The therapy of leg ulcers is an interdisciplinary and interprofessional challenge. Early identification of the cause of the leg ulcer and initiation of a causal therapy are essential for healing. OBJECTIVES: The aim of this study was to investigate the initial manifestation age of patients with causally associated leg ulcers. Afterwards we calculated the most common etiologies according to decade. PATIENTS AND METHODS: A prospective database at the University Hospital Essen, dermatological wound care center, was used to identify patients with chronic leg ulcers between 2002 and 2014. Clinical data of 1000 patients with chronic leg ulcers were analyzed in this monocentric study. RESULTS: A total of 29 different etiologies were differentiated. Approximately 70% of etiologies were of vascular origin, while 30% were rare causes. The count of different etiologies showed significant differences related to the onset and the occurrence in individual decades of life. In particular, nonvascular etiologies such as pyoderma gangrenosum or necrobiosis lipoidica are relatively more common in younger patients than in the aged. CONCLUSION: Based on the findings of our study, it is possible to limit the underlying etiology on the basis of the age of first manifestation of the leg ulcer in order to make targeted diagnostics more effective. Thus, this information can help to optimize scarce time resources in daily practice and improve the prediction probability of leg ulcers.

[Cutaneous vascular anomalies in children]. / Les anomalies vasculaires cutanées chez l'enfant.

INTRODUCTION: Vascular anomalies, which are erroneously categorized under the term angiomas, are a highly heterogeneous group of lesions that are poorly understood and affect a mean of 5 to 10 % of children. The fortuitous discovery of propranolol's efficacy in one of these entities has made them a topical issue. OBJECTIVES: The paper's main objective is to inform family doctors of the various types of vascular anomalies, clarify their classification, and provide a common terminology. Its secondary objective is to provide a decision tree that enables primary care doctors to avoid diagnostic pitfalls, successfully detect cases, and optimize management. METHODS: Systematic review. CONCLUSIONS: According to a recent study, 71,3 % of publications use the term hemangioma erroneously, regardless of the authors' field. The key for family doctors is to use one international classification only, that of the International Society for the Study of Vascular Anomalies (ISSVA), in order to facilitate management and comprehension between the different healthcare levels. The diagnosis of vascular anomalies is clinical in 90 % of cases, so all family doctors can, whilst using a decision tree, diagnose a vascular anomaly and refer only those that are complex for specialist care. The most common vascular anomaly is infantile hemangioma in infants, which spontaneously regresses around the age of 5-7 years in 90 % of cases. Watchful waiting and regular follow-up suffice, therefore, in such settings.

[Phlebology in German departments of dermatology. An analysis on behalf of the German Society of Phlebology]. / Phlebologie an deutschen Hautkliniken. Eine Bestandsaufnahme im Auftrag der Deutschen Gesellschaft für Phlebologie.

BACKGROUND: Phlebologic diseases have become extremely common and have major socio-economic impact. However, the percentage of dermatologists working in phlebology appears to be decreasing according to the data of the German Society of Phlebology (DGP). METHODS: To investigate the reasons for this development, we--on behalf of the DGP--sent a questionnaire to 120 German Departments of Dermatology in autumn 2012. RESULTS: In 76 returned questionnaires, the number of physicians with additional fellowship training in phlebology averaged 1.5; the average number of those who fulfill the criteria for training fellows in phlebology was 0.9. In 71.1 % of the departments there was a phlebologist. A special phlebologic outpatient clinic existed in 73.7 % of the departments. Sonography with Doppler (89.5 %) and duplex (86.8 %) was used as the most frequent diagnostic tool. For therapy, compression (94.7 %), sclerotherapy (liquid 78.9 %, foam 63.2 %, catheter 18.4 %), endoluminal thermic procedures (radio wave 28.9 %, laser 17.1 %) and surgery (especially crossectomy and stripping 67.1 %, phlebectomy of tributaries 75 %) were used. The average number of treatments was very heterogenous in the different departments. CONCLUSIONS: Phlebology definitely plays an important role in dermatology. Most departments fulfill the formal criteria for the license to conduct advanced training in phlebology. A wide spectrum of phlebological diagnostic and therapeutic procedures is available.

Cutaneous manifestations in patients with POEMS syndrome.

BACKGROUND: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome is a rare multisystem paraneoplastic condition associated with plasma cell dyscrasia. METHODS: From our institution's dysproteinemia database, 107 patients met criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome between January 1, 2000, and October 1, 2009. Medical records were reviewed for documented syndrome features at diagnosis. We assessed prevalence of skin findings and associations between dermatologic and other characteristic disease findings. RESULTS: Of the 107 patients, 96 (90%) had a recognized cutaneous manifestation. Hyperpigmentation and hemangioma were most common (47%), followed by hypertrichosis (38%). Vascular skin changes--acrocyanosis (34%), Raynaud phenomenon (20%), hyperemia/erythema (20%), flushing (16%), or rubor (11%)--occurred in 62%; white nails, sclerodermoid changes, and clubbing occurred in 30%, 26%, and 6%, respectively. Mean number of skin findings per patient was 2.9 (median, 3.0; range, 0-7). Presence of cutaneous manifestation was associated with abnormal pulmonary function tests (P < 0.001); immunoglobulin G gammopathy was associated with hyperpigmentation and hypertrichosis. No other significant associations were seen. CONCLUSIONS: The high prevalence of skin findings (90%) shows the value of dermatologic evaluation in diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. Our data indicate new associations between skin findings and other disease characteristics.

[Skin vessel lesions in aluminum potroom workers].

The features of development of the skin vessels lesions in 550 aluminum production workers have been investigated. The high prevalence of these disorders have been revealed in anode-operators and cell-operators, 49, 3 and 26.0% of workers, respectively. The regularity and staging of the development of this abnormity have been established, etiology, pathogenesis and clinical significance of those remain unknown.

Medial fronto-facial capillary malformations.

OBJECTIVE: To evaluate the characteristics of facial medial capillary malformations (CM), which differ from salmon patches by their wider extent, darker color, and incomplete resolution. STUDY DESIGN: Children were prospectively recruited from pediatric dermatology clinics and retrospectively from clinical and photographic databases. RESULTS: From June 2006 to June 2008, 84 children (56 girls; 66.6%) were included. The medial fronto-FCM (FFCM) involved the forehead and glabella (100%), upper eyelids (57.1%), nose (66.6%), philtrum (50.0%), and upper lip (22.6%). Extended forms were observed in 26.2%. A similar FFCM was observed within the family in 27.3% of cases. Outcome data showed complete regression in 10%, incomplete in 71.1%, and unchanging in 18%. An association with an extra facial CM was found 67.8%. Nape and/or occipital CM were associated in 63.8%. A median dorsal CM, mostly lumbosacral, was observed in 13.4%. An associated disease was seen in 33.3%. Neurological anomalies were observed in 9.5% (two cases of developmental delay, two of epilepsy, one of macrocephaly, one of cerebral arteriovenous malformation, one of cutis marmorata telangiectatica congenita, one of "macrocephaly- cutis marmorata telangiectatica congenita," and one of Rubinstein Taybi syndrome). No correlation between the site or the extent of the FFCM and extrafacial vascular or neurological anomaly was found. CONCLUSIONS: This study identifies a specific type of congenital medial FFCM that looks like salmon patch but has a wider median topography, a darker color, with slower and often incomplete resolution. Family cases are often observed. Despite their slow and incomplete regression, the aesthetic consequences are mild.

Frequency of thrombophilia determinant factors in patients with livedoid vasculopathy and treatment with anticoagulant drugs--a prospective study.

BACKGROUND: Livedoid vasculopathy (LV) is a chronic idiopathic disease characterized by painful purpuric macules on lower extremities. Its exact aetiology remains uncertain, but thrombotic and microcirculatory phenomena have been implicated as possible pathogenic factors. OBJECTIVES: To assess prospectively the frequency of thrombophilia and to verify the effectiveness of anticoagulant therapy among LV patients. METHODS: Thirty-four LV patients were tested for prothrombin time, activated partial thromboplastin time, antithrombin activity, protein C and S activity, anticardiolipin antibodies, lupus anticoagulant, prothrombin gene mutation, factor V Leiden mutation, methylenetetrahydrofolate reductase mutation, plasma homocysteine and fibrinogen. Thirteen of these patients were treated with anticoagulant drugs (either warfarin or heparin). RESULTS: Of 34 patients, 18 (52%) presented laboratory abnormalities of procoagulant conditions. Positive treatment response to anticoagulant therapy was observed in 11 patients. Improvement of pain was obtained in 1-3 weeks, an average of 1.8 week. Complete healing of the lesions was observed in about 2.3 months. Remission was sustained even after treatment interruption and lasted an average 7.8 months. No severe adverse effects were noticed. CONCLUSION: The authors suggest all patients with diagnosis of LV to be investigated for thrombophilic status. Anticoagulant drugs were well tolerated and seemed to be effective in treating not only LV symptoms but also its ulcerations.

Frequency and phenotypes of cutaneous vascular malformations in a consecutive series of 417 patients with familial cerebral cavernous malformations.

BACKGROUND: Familial cerebral cavernous malformations (FCCM) are vascular malformations inherited as an autosomal-dominant condition. Three genes (KRIT1/CCM1, MGC4607/CCM2, PDCD10/CCM3) have been identified so far. Extra-neurological manifestations include retinal and cutaneous vascular malformations. The cutaneous vascular malformation, which had been more specifically associated with FCCM, is hyperkeratotic cutaneous capillary venous malformation (HCCVM). OBJECTIVES: To define the frequency of cutaneous vascular malformations in patients with FCCM, to precise their different phenotypes, and to study the association of each cutaneous vascular malformation subtype with the different three mutated CCM genes. METHODS: Dermatological inquiry was systematically performed in a large series of consecutive FCCM patients. Cutaneous biopsies were reviewed when available. Cutaneous vascular malformations classification was based on predominant anomalous channels, using the current International Society for the Study of Vascular Anomalies classification. Molecular screening of CCM genes was performed. Results Four hundred seventeen consecutive FCCM patients from 182 unrelated families were included. 38 patients (9%) from 25 different families had cutaneous vascular malformations. In these 38 patients, cutaneous vascular malformations were classified as follows: 13 capillary malformations (CM), 15 HCCVM, 8 venous malformations (VM) and 2 unclassified lesions. All patients (92%), but one with CM had a KRIT1/CCM1 mutation. The last patient had no detectable mutation. All of the 15 patients with HCCVM had a KRIT1/CCM1 mutation; 86.7% of cutaneous vascular malformation patients (33 of 38) had a KRIT1/CCM1 mutation. CONCLUSION: Cutaneous vascular malformations are seen in 9% of FCCM patients. Three distinct major cutaneous vascular malformations phenotypes were identified: HCCVM (39%), CM (34%) and VM (21%). CCM1 is the most frequently mutated gene in cutaneous vascular malformations-FCCM patients.

Anti-Ro antibody and cutaneous vasculitis in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a complex systemic autoimmune disease with various clinical and serological manifestations. Previous studies have shown the association of SLE and anti-Ro antibody with a series of clinical manifestations. We investigated this association in Brazilian patients with SLE. Five hundred and nine consecutive patients who fulfilled the revised American College of Rheumatology criteria for the SLE were enrolled in the study from June to December 2007. All patients were from our Service of Rheumatology, School of Medicine, University of São Paulo, Brazil. Frequencies of a series of laboratorial and clinical manifestations were calculated. Anti-Ro antibody was associated to anti-La antibody, female, and cutaneous vasculitis. In multivariate analysis, patients with anti-Ro antibody has 1.63 (95% CI 1.07-2.50) more risk to develop cutaneous vasculitis than patients without this antibody. Our data have demonstrated that anti-Ro antibody is an independent useful serologic marker for cutaneous vasculitis.