Cutaneous Rosai \u2013 Dorfman disease in a patient with late syphilis and cervical cancer \u2013 case report and a review of literature

BackgroundLatent syphilis refers to the asymptomatic stage in the natural evolution of syphilis in a patient not treated or inadequately/inappropriately treated for syphilis. Latent syphilis has been categorised as early...

BackgroundNeurosyphilis (NS) can lead to acute ischemic stroke (AIS) or transient ischemic attack (TIA). We compared the clinical characteristics and laboratory features among AIS and TIA patients who were syphilis-seronegative (control group) or had latent syphilis (LS) or NS to evaluate their stroke outcome.MethodsThis prospective cohort study was conducted on patients who had recently suffered AIS or TIA. After serological syphilis screening, clinical and laboratory data were collected, and brain imaging and spinal tap (serologically syphilis-positive patients only) were performed. Stroke outcome was re-evaluated approximately three months later.ResultsThe 344 enrolled patients were divided into three groups: control group (83.7%), LS (13.1%), and NS (3.2%). A multivariate analysis revealed: 1) age of ≥ 70 years, generalized brain atrophy via imaging, and alopecia (adjusted odds ratio [AOR] = 2.635, 2.415, and 13.264, respectively) were significantly associated with LS vs controls; 2) age of ≥ 70 years (AOR = 14.633) was significantly associated with NS vs controls; and 3) the proportion of patients with dysarthria was significantly lower (AOR = 0.154) in the NS group than in the LS group. Regarding the NS patient cerebrospinal fluid (CSF) profile, only 2/11 cases had positive CSF-Venereal Disease Research Laboratory (VDRL) test results; the other nine cases were diagnosed from elevated white blood cell counts or protein levels combined with positive CSF fluorescent treponemal antibody absorption (FTA-ABS) test results. Regarding disability, the initial modified Rankin scale (mRS) score was lower in the control group than in the NS group (p = 0.022). At 3 months post-stroke, the mRS score had significantly decreased in the control (p < 0.001) and LS (p = 0.001) groups. Regarding activities of daily living, the 3-month Barthel Index (BI) score was significantly higher in control patients than in LS (p = 0.030) or NS (p = 0.002) patients. Additionally, the 3-month BI score was significantly increased in the control (p < 0.001) and LS (p = 0.001) groups.ConclusionsBecause syphilis was detected in many AIS and TIA patients, especially those aged ≥ 70 years, routine serological syphilis screening may be warranted in this population. Patients with syphilitic infection had worse stroke outcomes compared with NS patients.

We aimed to construct a timeline for nontreponemal titer decline specific to pregnancy and evaluate factors associated with inadequate decline by delivery. This was a retrospective medical records review from September 1984 to June 2011 of women diagnosed with syphilis after 18 weeks of gestation. Women were treated according to stage of syphilis per Centers for Disease Control and Prevention guidelines. Patients with both pretreatment and delivery titers were included for data analysis. Demographics, stage of syphilis, maternal titers, delivery, and infant outcomes were recorded. Standard statistical analyses were performed for categorical and continuous data. The titer decline was analyzed using mixed-effects regression modeling. A total of 166 patients met inclusion criteria. Mean gestational age at treatment was 29.1 ± 5 weeks, and 93 (56%) women were diagnosed with early-stage syphilis. For all stages of syphilis, maternal titers declined after syphilotherapy. Pretreatment titers were higher and declined more rapidly in primary and secondary disease than in latent-stage disease and syphilis of unknown duration. Sixty-three (38%) patients achieved a 4-fold decline by delivery. Patients without a 4-fold decline by delivery were older (24.6 vs 21.5 years; P < .001), treated later in pregnancy (30.3 vs 27.3 weeks; P < .001), diagnosed with latent syphilis or syphilis of unknown duration, and had less time from treatment to delivery (7.8 vs 11.1 weeks; P < .001). Maternal serologic response during pregnancy after adequate syphilotherapy varied by stage of disease. Failure to achieve a 4-fold decline in titers by delivery is more a reflection of treatment timing than of treatment failure.

Investigating the predictors for lumbar puncture to diagnose the asymptomatic neurosyphilis among HIV and syphilis co-infected patients in Shanghai, China. Respectively, screening the medical records from August 1, 2009 to June 30, 2015. Those HIV-infected patients with concurrent syphilis who had received lumbar puncture were selected and their clinical and demographic data were recorded. Participants comprised symptomatic and asymptomatic patients. The latter ones could be further divided into 3 groups: late syphilis, early syphilis with anti-syphilis treatment failure, and early syphilis with serum toludine red unheated serum test (TRUST) ≥1:32. Both syphilis stage and anti-syphilis treatment effect were defined by common criteria, and syphilis of unknown duration was considered as late syphilis. Asymptomatic neurosyphilis was defined as neurosyphilis without neurological symptoms such as headache, cognitive dysfunction, motor deficits, auditory or ophthalmic abnormalities, and stroke. Neurosyphilis was defined as reactive cerebrospinal fluid (CSF) TRUST and/or CSF white blood cell >20 cells/μL without other reasons. Mann-Whitney test and Fisher's exact test were used for analyzing the difference between neurosyphilis and non-neurosyphilis group. Logistic regression test was performed to analyze the risk factors for neurosyphilis. In total, 170 participants were collected, and the rate of neurosyphilis was 32.35%. Among all the 105 participants without neurological symptoms, 80 patients were with late syphilis and 25 were with early syphilis. Among the early syphilis patients, 23 had a TRUST ≥1:32 and the other 2 experienced an anti-syphilis treatment failure. The differences of clinical and demographic variables between neurosyphilis and non-neurosyphilis group were not statistically significant except the serum TRUST titer (P < 0.01). From HIV/syphilis co-infected patients with or without neurological symptom, those who had neurological symptoms, CD4 <350 per μL and serological TRUST titer ≥1:16 were 4.9-fold (95% confidence interval [CI]: 2.37-10.31), 4.3-fold (95% CI: 1.17-15.78), and 4.1-fold (95% CI: 1.58-10.76), respectively, more likely to be diagnosed with neurosyphilis. Asymptomatic patients whose serum TRUST titer ≥1:16 were 8.48-fold (95% CI: 1.08-66.63) more likely to have asymptomatic neurosyphilis. Among asymptomatic HIV-infected patients with late syphilis or early syphilis experienced an anti-syphilis treatment failure, those who have a serum TRUST titer ≥1:16 are suggested to perform lumbar puncture in order to avoid delayed diagnosis and the occurrence of severe sequelae of syphilis.

To investigate infections of syphilis, neisseria gonorrhoeae, chlamydia trachomatis and the related risk factors in men who have sex with men (MSM) in Jiangsu province. A total of 400 MSM were enrolled by Snowball Sampling Method from August to October in 2010 and then 328 cases were surveyed by a questionnaire and collected serum sample 5 ml per person as well as rectal swab on the spot; all of the serum samples were tested for syphilis by ELISA and TRUST, and all of the rectal swabs were tested for neisseria gonorrhoeae or chlamydia trachomatis. The influencing factors of syphilis, neisseria gonorrhoeae, chlamydia trachomatis were analyzed by logistic regression analysis. The 328 MSM were (32.46 ± 9.72) years old, 59.15% (194/328) were unmarried.75.00% (246/328) MSM had rectal sex with men in the past 3 months, and condom use rate for recent sex was 56.71% (186/328), while 53.05% (174/328) MSM didn't have sex with women in the last 3 months. The syphilis infection rate among MSM was 13.41% (44/328), the neisseria gonorrhoeae infection rate was 3.66% (12/328), and the chlamydia trachomatis rate was 11.59% (38/328). The number of sex partners was the key factor that influenced syphilis infections (OR = 4.213, 95%CI: 1.133 - 15.656). The prevalence of syphilis and chlamydia trachomatis was high in MSM in Jiangsu, while risk behavior rate were high in the MSM and then should be intervened.

BackgroundThe diagnosis of neurosyphilis is a challenge, and the criteria for deciding when to perform a lumbar puncture are still controversial, especially in people living with HIV with a late latent syphilis diagnosis.MethodsRetrospective analysis of demographic, clinical, and laboratory data of people with HIV and documented late latent syphilis or syphilis of unknown duration with a cerebrospinal fluid VDRL test.Results122 patients were evaluated, of whom 52 had the diagnosis of neurosyphilis. Patients with and without neurosyphilis presented a similar viral load and lymphocyte CD4+ T-cell count. Neurological symptoms (OR 6.4, 95% CI 2.1–22.4; p < 0.01), serum VDRL titers of 1:32 (p<0.01), 1:64 (p = 0.055), and ≥1:128 (p < 0.001) were associated with neurosyphilis. Furthermore, serum VDRL ≥1:32 were associated with (OR 24.9, 95% CI 5.45–154.9; p < 0.001) or without (OR 6.5, 95% CI 2.0–29.2; p = 0.004) neurological symptoms with neurosyphilis; however, VDRL ≤1:16 with neurological symptoms can be associated with neurosyphilis (OR 7.6, 95% CI 1.03–64.3; p = 0.046).ConclusionNeurological symptoms, particularly headache, were predictors of neurosyphilis in people with HIV irrespective of their viral load and lymphocyte CD4+ T-cell count in late latent syphilis. A serum VDRL ≥1:32 increased the risk of neurosyphilis in patients with or without any symptoms.

Between 1984 and 1988 inclusive 34 patients with syphilis during pregnancy were identified in this unit by routine serological screening. There were 2 stillbirths in this group of patients giving a perinatal mortality rate of 59 per 1,000 total births. Analysis of the patients' history and physical examination findings did not reveal any predictive factors for sexually transmitted diseases in most cases. Despite the dramatic fall in the prevalence of syphilis both during pregnancy and the general population in Hong Kong, routine serological screening for syphilis during pregnancy must continue.

The incidence of positive Chlamydia cultures in females undergoing laparoscopy for tubal patency is high. We advocate routine screening for Chlamydia infection in females with infertility. Of seventy-five patients undergoing laparoscopy for infertility, 49 grew Chlamydia on tissue culture of material from the cervix, an incidence of 65.3%. of these 49 cases, 33 (67.3%) had tubal blockage. In asymptomatic patients, 22 out of 50 had positive cultures from the cervix, an incidence of 44%. Peritoneal fluid culture was negative in all infertile patients. When compared to populations studied elsewhere, the incidence of Chlamydia appears to be high in our population and significantly higher in the infertile patients. In the management of infertility, pelvic inflammatory disease, and neonatal conjunctivitis, Chlamydia should be routinely investigated and treated.

Indications for Lumbar Puncture in Patients With Early Active Syphilis and Human Immunodeficiency Virus Coinfection: Experience in a Tertiary Level Hospital in La Coruña, Spain, 2003-2006

To critically determine the relevance of Venereal Diseases Research Laboratories (VDRL) investigation as a routine serological screening for syphilis among pregnant women who receive antenatal care at the University of Nigeria Teaching Hospital (UNTH). A retrospective chart review of result of serological test for syphilis among pregnant women during a five year period (1st January, 1997 to 31st December 2001) was undertaken. A total of 7469 women booked. 7175 had routine serological test. 294 of the booked women failed to submit themselves for screening. The prevalence rate of syphilis in this study was 0.125%. VDRL seroreactivity had in previous studies in this center declined from 3.06% to 1.30%. It further declined to 0.98% in this study. The results strongly show a continuing very low prevalence rate of syphilis in Enugu. Nevertheless, we support continued screening of pregnant women inspite of this low prevalence rate, since this will eradicated the effects of undiagnosed and untreated syphilis.

A 50-year-old man presented with a broad ulcerated lesion on the glans penis, persisting for 3 weeks since a spa stay some weeks before. He had been seen by a dermatologist in the preceding weeks, who prescribed various topicals, including dexpanthenol, gentamicin, resin salve, clotrimazole, and octenidinine dihydrochloride 2-phenoxyethanol. Serology for syphilis was negative. As the ulcer persisted, the patient was sent to our department for a diagnostic re-evaluation. The patient did not take any medication on a regular basis and denied application of any topicals, except those that were prescribed. Moreover, the patient denied any local trauma, previous oral aphthae or genital lesions, holidays abroad, or any previous pathologies at that site. The patient was systemically well and denied any sexual intercourse. Physical examination showed an otherwise unsuspicious integument, despite a right inguinal lymphadenopathy. However, almost the entire superior part of the glans was involved by a sharply demarcated ulcer coated with fibrinous debris, clinically being highly suspicious for squamous cell carcinoma (Fig. 1). To exclude any malignancy, a punch biopsy of the Glans penis was performed on the day of the patients first presentation at our clinic. Histological evaluation revealed an ulcer with a dense lymphoplasmacellular infiltrate and showed no hints for malignancy. Staining for Treponema pallidum (TP) otherwise, showed several micro-organisms. Lesional PCR for TP was positive and repeated serology confirmed the diagnosis of syphilis (Venereal Disease Research Laboratory [VDRL] 1:16, TP Particle Agglutination [TPPA] 1:1280, treponemal membrane protein A [TMPA] reactive, TMPA immunoglobulin M [IgM] reactive). The patient was admitted for a thorough discussion of the diagnostic results and all possible treatment options in such a case. With this, he admitted having had a previous sexual intercourse with a random male acquaintance at the spa stay. Treatment of the present primary syphilis was performed with a single intramuscular (gluteal) injection of benzathine penicillin 2.4 IU, leading to a complete re-epithelisation of the ulcer within some weeks (Fig. 2). According to the International Union Against Sexually Transmitted Infections (IUSTI) Europe guidelines, the patient was screened for all relevant sexually transmittable infections (STIs). At the anorectal region, Neisseria gonorrheae (NG) was verified via PCR, following treatment with a single intravenous injection of ceftriaxon 1 g. The oropharyngeal site was negative for relevant STIs. Hepatitis B virus (HBV), hepatitis C virus (HCV), and HIV were excluded via repeated serology. The patient was informed to abstain from any sexual contact until the complete healing of the ulcer. A control visit for a test of cure (TOC) according to the NG infection after 1 month was performed and was negative. Up until today, the patient presented himself to control visits at 1 and 3 months after the injection of the penicillin. At the latest laboratory results, the Rapid Plasma Reagin (RPR)/VDRL declined to negative results. Genital ulcers’ aetiopathogenesis is broad, including infectious (STIs/non-STIs) and non-infectious diseases [1]. However, most genital ulcers are caused by an STI, and therefore TP, herpes simplex virus I and II, Chlamydia trachomatis serovars L1, L2, L3 and more, seldomly Haemophilus ducreyi must be kept in mind at first sight [1]. Syphilis, known as the ‘great imitator’ of various diseases, represents a systemic but curable disease [1, 2]. Its incidence in a global context is very much on the rise, with >130 000 cases in 2020 according to the Centers for Disease Control and Prevention (CDC), and >35 000 cases in 2019, according to the European Centre for Disease Prevention and Control (ECDC) [3, 4]. It is caused by the obligate human pathogenic bacterium TP with a long incubation period of up to 90 days [1, 2]. Transmission occurs via sexual intercourse, diaplacental, or via contaminated blood [2]. The natural course of the disease classically runs in three stages, with specific clinical signs in each clinically apparent stage; however, an observable overlap of those stages is not uncommon [2]. Early syphilis, which is defined as an infection taking place within the very last year, includes primary, secondary, and early latent syphilis [2]. Latent syphilis represents a stage without clinical signs, but serological positivity [2]. Primary and secondary syphilis are indeed highly infectious stages [2]. After a potentially long incubation period (of up to 90 days), the chancre, a typically solitaire, ulcerous, painless lesion at the gate of entry and regional lymphadenopathy form the primary syphilitic complex does appear [1, 2]. However, its clinical behaviour is extremely broad, resulting in sometimes delayed diagnosis. In early syphilis, serology might initially be negative and should therefore be repeated after 1, 2 and 6 weeks after the onset of an ulcerous lesion [2]. Secondary syphilis, resulting from a bacteraemia, is a highly infectious and clinically broad stage. It may follow an asymptomatic course, but in many cases presents with a mild maculous exanthema and generalised lymphadenopathy, less frequently as an angina specifica or palmoplantar clavi syphilitici, and seldomly as an alopecia specifica, amongst many others [1, 2]. Late syphilis (exact time-point of infection unknown or >1 year) is divided into late latent and tertiary syphilis. Tertiary syphilis again is characterised by organ involvement [1, 2]. The diagnosis is based on typical clinical presentation of the patient, serology, or proof of TP via PCR, and, in the case of the presence of ulcerous lesions, dark-field microscopy [1, 2]. Irrespective of the stage of syphilis, penicillin G is still regarded as the preferred treatment option of choice [1, 2]. Early syphilis should be treated with a single injection of benzathine penicillin G (BPG) 2.4 × 106 IU intramuscularly and late syphilis must be treated with three injections of BPG 2.4 × 106 IU weekly on days 1, 8, and 15 [1, 2]. After treatment, clinical and serological control visits have to be performed at 1, 3, 6, and 12 months, respectively [1, 2]. A STI seldom develops alone and therefore all patients in whom one STI is verified, should be sent to a venereologist, as screening for HBV and HCV, HIV, and syphilis should be performed via serology, keeping the various incubation periods in mind [1]. Moreover, testing for (at least) Chlamydia trachomatis and NG at all relevant sites of exposure (oral, anal, genital), is strongly recommended. In our patient, we additionally identified an anorectal gonorrhoea. Gonorrhoea represents the second most common bacterial STI, being caused by the gram-negative bacterium NG [1, 5]. Similarly to syphilis, its incidence is very much on the rise, and above all, the antimicrobial resistance profile of this bacterium fuels concern in experts [1, 5]. Transmission occurs via sexual intercourse or perinatally [5]. After a very short incubation period of some days, the typical clinical symptom is a (muco)purulent urethral discharge and dysuria, resulting in symptoms in almost each affected male [5]. However, in females, and mainly in affected extragenital sites (including anorectal or pharyngeal site), most patients present completely asymptomatically [5]. Diagnosis is performed via PCR, gram staining (due to the detection of intracellular gram-negative diplococci within polymorphonuclear leucocytes [Fig. 3]), or culture [1, 5]. The latter is recommended in any case for the highly relevant antimicrobial resistance profiling [1, 5]. In all patients who present with discharge, ideally a gram staining should be performed. Performing a gram staining, the diagnosis of gonorrhoea can be undertaken within some minutes, if gram-negative diplococci can be verified intracellularly within polymorphonuclear leucocytes. The treatment options depend on differing regional recommendations, whereby a TOC is recommended not earlier than 2 weeks after treatment [5]. Globally STIs are on the rise, and any genital ulcer, especially those of sudden onset, should be considered suspicious for syphilis, keeping in mind that serological tests may initially be negative shortly after infection. None of the contributing authors have any conflicts of interest, including specific financial interests, relationships, and affiliations relevant to the subject matter or materials discussed in the manuscript.

Dear Editor: Nodular hidradenoma (NH) is a benign, rare adnexal neoplasm. NH usually occurs on the scalp, trunk, and proximal extremities, and rarely on the hands and feet1. The subdivision of NH into two groups was recently suggested, as follows: those with eccrine differentiation (known as poroid hidradenoma [PH]) and those with apocrine differentiation (known as clear cell hidradenoma [CCH])2,3. To our knowledge, there have been no previous reports of CCH on the volar surface of the hands and feet. Herein, we report a case of CCH on the palm. A 51-year-old Korean woman without any underlying diseases presented with a brownish nodule on the thenar area of her left palm (Fig. 1). Histological examination revealed a circumscribed dermal neoplasm comprising several aggregations of neoplastic cells and cystic areas (Fig. 2A). Epidermal connection was observed. Most of the tumor cells were polygonal cells with eosinophilic cytoplasm, and few clusters of clear cells were observed (Fig. 2B). Apocrine differentiation was not observed. Immunohistochemical analysis revealed positive expression of cytokeratin (CK) 5/6 in the neoplastic cells. Carcinoembryonic antigen and epithelial membrane antigen (EMA) were partially expressed in the ductal structures and polygonal cells. The clear cells were negative for both EMA and androgen receptor (AR). Histologically, NH mostly is a dermal neoplasm presenting as a well-circumscribed lobulated mass. Occasionally, an epidermal connection can be identified4. Tubular or ductal structures with luminal and cystic spaces can be observed. The intervening stroma of NH shows various patterns including sclerotic, vascularized, and myxoid patterns. CCH is composed of polygonal cells and clear cells. Clear cells usually account for 5% to 30% of the total tumor cell mass3. PH is composed of poroid cells and cuticular cells. In this case, some clear cells were observed between the predominant polygonal cells, and thus, we diagnosed this case as CCH (Fig. 2B). Although the origin of CCH is controversial, it has been considered that it usually arises from folliculo-sebaceous-apocrine units; however, it can also originate from eccrine units2. To determine the origin of this tumor, we immunostained the specimens with monoclonal AR antibodies; however, the clear cells stained negative for AR antibodies. The positive staining for CK5/6 and the connection with the acrosyringium in this case suggest that this tumor originated from the basal layer5. Fig. 1 A brownish nodule on the thenar area of the palm. Fig. 2 (A) A circumscribed dermal neoplasm with mostly solid areas and some cystic areas with myxoid stroma. An epidermal connection was observed (black arrow; H&E, ×40, scanning view). (B) Clusters of clear cells were observed (H&E, ... Owing to the site of the tumor in the area without folliculo-sebaceous-apocrine units and the connection with the acrosyringium, we believe that this case of CCH originated from the acrosyringium of the eccrine gland.

Indicaciones de la punción lumbar en pacientes con sífilis precoz activa coinfectados por el VIH. Casuística en un hospital terciario de La Coruña (España) 2003-2006

Patients with latent syphilis or syphilis of unknown duration should be evaluated for tertiary disease and neurosyphilis. The aim of this retrospective study was to determine relevant serological parameters for the identification of those individuals with syphilis who are most likely to have neurosyphilis and who therefore require lumbar puncture. After excluding repeated estimates and patients whose blood syphilis serology had either been negative or not been determined within 3 months of lumbar puncture, 265 out of 710 cerebrospinal fluids from 1988 to 2004 were analysed. In each of those patients the earliest available pairs of serum and cerebrospinal fluid samples were evaluated. The diagnosis of neurosyphilis was based on criteria according to established guidelines. Forty-three of 265 patients (16.2%; 5 women, 38 men; mean age 47+/-16 years) had neurosyphilis. Seven of 72 (9.7%) of those testing HIV-positive, fulfilled the criteria of neurosyphilis. Not a single patient with neurosyphilis tested Venereal Disease Research Laboratory test (VDRL)-negative in peripheral blood, an effect which was highly significant (p < 0.01, chi2-test). The median blood-VDRL titre was significantly higher in patients with neurosyphilis than in those without (1:32 vs. 1:0; p < 0.01, t-test, two-sided). Hence, neurosyphilis is very unlikely in patients with a negative blood-VDRL. Therefore, lumbar puncture is not recommended in these patients.

Background/Objectives: Secondary syphilis typically presents with a non-pruritic maculopapular rash. However, vesicular and bullous manifestations are exceedingly rare in adults and may mimic autoimmune blistering diseases. The objective of this report is to describe atypical presentation of secondary syphilis with predominant vesiculobullous lesions and to emphasize the importance of including syphilis in the differential diagnosis of blistering skin diseases. Methods: We describe the case of a 46-year-old bisexual man with syphilis of unknown duration who presented with recurrent polymorphic skin eruptions, predominantly bullous and vesicular in nature. Clinical examination, serologic testing, and histopathologic evaluation were performed to establish the diagnosis. Results: Serologic tests confirmed active syphilis infection. A brief review of similar reported cases was conducted to highlight the clinical variability of vesiculobullous syphilis. Conclusions: Atypical vesiculobullous presentations of secondary syphilis pose significant diagnostic challenges and may be mistaken for autoimmune blistering disorders. Clinicians should maintain a high index of suspicion for syphilis in patients with polymorphic or blistering eruptions, particularly in those with risk factors for sexually transmitted infections. Awareness of these uncommon manifestations can facilitate timely diagnosis and appropriate treatment.