Neurosyphilis in HIV-infected patients: clinical manifestations, serum venereal disease research laboratory titers, and associated factors to symptomatic neurosyphilis

GOAL: To describe clinical and laboratory features of human immunodeficiency infection (HIV)-infected patients with neurosyphilis. STUDY DESIGN: Retrospective study of 27 consecutive cases of HIV-infected patients with a positive Venereal Disease Research Laboratory (VDRL) in cerebrospinal fluid (CSF). RESULTS: Median of age was 36 years and 89% were men. Ten (37%) patients had previous nonneurologic syphilis treatment. At the time of neurosyphilis diagnosis, 10 (37%) patients had early syphilis, and 6 of them were neurologically asymptomatic. Nine (33%) patients had symptomatic neurosyphilis. Twenty-six (96%) patients were classified with early neurosyphilis. The medians of serum VDRL and CD4 T cell counts were 1:128 and 182 cell/muL, respectively. Twenty five (93%) patients presented serum VDRL titers > or =1:16. Five of 6 patients with early syphilis and asymptomatic neurosyphilis, presented serum VDRL > or =1:16. Symptomatic patients showed lower CD4 T cell counts (59 cell/muL vs. 208 cell/muL, P = 0.03) and higher protein concentration on CSF (118 mg/dL vs. 39 mg/dL, P <0.001) than asymptomatic patients. CONCLUSIONS: Most patients had early and asymptomatic neurosyphilis, and more than one third had early syphilis. Patients with symptomatic neurosyphilis showed lower CD4 T cell counts and higher protein concentration on CSF than those asymptomatic. Most patients had serum VDRL titers > or =1:16, regardless of syphilis stage.     

Peripheral blood eosinophilia is associated with the presence of skin ulcers in patients with systemic sclerosis

The role of eosinophil in systemic sclerosis (SSc) is still controversial. In the present study, the relationship between skin ulcers and peripheral blood eosinophilia were analyzed in patients with SSc. We retrospectively investigated the clinical records of all patients who were diagnosed with SSc on the basis of American College of Rheumatology/European League Against Rheumatism 2013 criteria, and were followed up for more than 2 years at Wakayama Medical University. As a result, maximum eosinophil counts during the 2‐year follow‐up period were 20–983/mm³ (median, 270), whereas maximum eosinophil percentages were 0.5–14.1% (median, 5.3%) in peripheral blood of 47 SSc patients. On the other hand, patients with skin ulcers during the 2‐year follow up showed significantly increased maximum eosinophil counts compared with those without (median, 520 vs 228/mm³; P = 0.0001). Maximum eosinophil percentage was also significantly higher in patients with skin ulcers (median, 9.7% vs 4.6%; P = 0.00001). To note, in four of the nine patients with skin ulcers, the timing of emerging of the maximum eosinophil counts was associated with the ulcer development during the 2‐year follow up. These results suggest that eosinophils are involved in the pathogenesis of vascular dysfunction of SSc. Larger studies should be performed to clarify the exact mechanism of ulcer formation caused by eosinophilia in SSc patients in the future.     

Staphylococcus aureus virulence factors associated with infected skin lesions: influence on the local immune response

OBJECTIVES: To evaluate Staphylococcus aureus isolates from infected skin lesions for their potential to produce immune system-modulating toxins and to correlate these with white blood cell (WBC) counts associated with these lesions. DESIGN: Specimens were obtained for bacterial culture and gram staining from 105 infected skin lesions, and the number of WBCs per low-power field (LPF) was determined. Chromosomal DNA was prepared from 84 bacterial isolates and subjected to real-time polymerase chain reaction analysis to determine the presence of genes encoding potential immunomodulating toxins. Bacterial populations were divided into 2 groups: those associated with low WBC counts (0-5 WBCs/LPF) and those with high WBC counts (> 5 WBCs/LPF). We applied chi(2) statistical analyses to compare the toxin gene profiles associated with WBC counts on initial swab for culture. PATIENTS: Samples were obtained from patients at a single geographic location. RESULTS: A higher than expected percentage of bacteria capable of producing the exfoliative toxins A and/or B (ETA and/or ETB) and Panton-Valentine leukocidin (PVL) was seen in all skin lesions infected with S aureus without regard to WBC count with initial cultures. Comparison of the toxins associated with the low WBC group vs the high WBC group showed that low WBC counts were associated with ETA and ETB, while high WBC counts were associated with PVL and toxic shock syndrome toxin. There were no differences in the clinical appearance of the lesions between groups. CONCLUSIONS: Staphylococcus aureus virulence factors ETA, ETB, and PVL are associated with WBC counts from infected skin lesions. The exact role they play in affecting the WBC counts remains to be determined.     

Plaque and postauricular nodular mucinosis associated with lupus erythematosus

A 31-year-old Korean man first noticed a large plaque-like skin lesion with a cutaneous induration on his back in the middle of June 1994, and visited the Department of Dermatology at Yonsei University on August 18,1994. He had been healthy with no specific illness until the development of the skin lesions. Physical examination showed a weli-developed, moderately nourished man who had a large plaque-like skin lesion with a hard cutaneous induration about 5–10 cm in diameter on his mid-back and several scattered nodular lesions on the upper back. He complained of arthralgia and general weakness. Laboratory investigations disclosed the following results: hemoglobin (Hb), 11.3 g/dL; heinatocrit (Hct), 35.3%; antinuclear antibody (ANA) (1:20 cytoplasmic pattern positive). White blood cell (WBC), Aspartic aminotransferase (SGOT), Alanine aminotransferase (SGPT), blood urea nitrogen (BUN), Creatinine, anti-smooth muscle (anti-SM), n-ribonucleo-protein (n-RNP), Veneral Disease Research Laboratory (VDRL), Rheumatoid arthritis factor (RA), and urinalysis were negative or within the normal range. A biopsy of a plaque on the upper back showed that the collagen was loosened and replaced by homogeneous mucinous materials containing scattered spindle-shaped fibroblasts in the upper and mid-dermis. The mucinous material stained blue with Alcian blue at pH 2.5, but not at pH 1.0, and was periodic acid-Schiff negative. Direct immunofluorescence showed granular and continuous IgG, C3, and fibrinogen deposits along the dermo-epidermal junction. According to these findings, a diagnosis of plaque-like mucinosis was made. The administration of systemic steroid was started at the patient's first visit. During the treatment the previous skin lesions improved, but in March 1995 new skin lesions developed on both postauricular areas. On examination, two slightly erythematous nodules, 10–15 mm in diameter, were seen on both postauricular areas. The patient complained of general weakness, arthralgia, photosensitivity, and transitory facial swelling and erythema. Laboratory tests showed the following results; Hb, 12.2 g/dL; Hct, 38.1%; triglyceride, 253 mg/dL; ANA (1;160 speckled pattern positive); urine protein, 150 mg/dL; urine RBC, 5–9/HPF; urine WBC, 10–29/HPF/ BUN, 22 mg/dL; Creatinine, 1.3 mg/dL. WBC, SGOT, SGPT, chest X-ray, and ds-DNA were normal. Light microscopic examination of a nodule showed marked mucin deposition throughout the entire dermis. A sparse perivascular lymphocyte infiltrate was noted. Direct immunofluorescence of a nodule on the postauricular area showed granular and continuous IgG, C3, and fibrinogen deposition along the dermo-epidermal junction. A lupus band test in uninvolved skin was not carried out at the patient's request. Under a diagnosis of nodular mucinosis associated with systemic lupus erythematosus (SLE), systemic corticosteroid was given for 3 months; subsequently the plaque and nodular skin lesions almost disappeared and the patient's general condition improved. Regular urinalysis showed continued proteinurea despite the treatment, so the patient was referred to the Department of Nephrology for the evaluation of lupus erythematosus (LE) nephritis, which was confirmed after a renal biopsy.     

Lipid levels in patients with lichen planus: a case–control study

Background Cardiovascular risk factors have been assessed with some skin diseases such as alopecia and psoriasis. Recently, a case–control study found that lichen planus (LP) was associated with dyslipidaemia in a large series of patients. However, no data were presented about lipid values in patients and controls. Objective The objective of this case–control study was to evaluate lipid levels in men and women with lichen planus and in healthy controls, excluding lichenoid drug eruption and treatment for LP such as systemic corticosteroids, retinoid acid or methotrexate. Patients and methods This case–control study included 160 patients, 80 with LP (40 men and 40 women) and 80 controls consecutively admitted to the outpatient clinic in Dermatology department of San Cecilio Hospital, Granada, Spain. Results Patients with LP presented higher significant triglycerides values (145.9 vs. 101.5 mg/dL P = 0.0007), total cholesterol values (197.7 vs. 178.4 mg/dL P = 0.001), LDL‐C values (120.8 vs. 100.9 mg/dL P < 0.0001) and lower HDL‐C values (55.3 vs. 61.9 mg/dL P = 0.004) vs. controls. Adjusted OR for dyslipidaemia in patients with LP was 3.03 (95% confidence interval: 1.49–6.17, P = 0.002). Conclusion The results obtained in this study indicate an association between LP and dyslipidaemia. Lipid levels screening in men or women with LP may be useful to detect individuals at risk and start preventive treatment against the development of cardiovascular disease.     

MYCOSIS FUNGOIDES WITH SIGNET-RING CELLS AND MONOCLONAL GAMMOPATHY

A 73-year-old white woman was admitted to our hospital for evaluation of a chronic dermatitis. Personal history was remarkable only for arterial hypertension and noninsulin-dependent6 diabetes mellitus. Clinical and histopathologic findings were consistent with a diagnosis of mycosis fundoides (mf and staging procedures including bolld cell counts, serum biochemistry, urinalysis, bone marrow biopsy, Sé zary cell counts in peripheral bolld, computerized tomography (ct scans, abdominal ultrasonography, chest roentgenograms, serum protein electrophoresis, and immunoelectrophoresis disclosed normal or negative results (T2 N0 M0 B0). After 2 years of standard treatment, the patient's condition had progressively worsened and she presented generalized erythematious plaques and a few, small, red-bluish tumors (Fig. 1). A biopsy specimen of a tumor showed an intense lymphoid infiltrate composed mainly of large and medium-sized cells, many of which exhibited clear interacytoplasmic vacuoles (Fig. 2). These vacuoles were negative for periodic acid-Schiff, alcian blue, and oil-red-O staining. Most of the neoplastic cells stained with uchl1 antibody, and none expressed B-cell markers. Some of the malignant cells reacted wih the Ber-h2 and mb3 monoclonal antibodies. No evidence of immunoglobulin libht chain production was observed. Negative staining with cd68 monoclonal antibody ruled out a monocyte-macrophage lineage of the malignant cells. On physical examination, no palpable lymph nodes were found. The quantitative immunoglobulin study showed an IgG level of 6,950 mg/dL (noral values, 800–1,800 mg/dL), IgA level of 126 mg/dL (normal, 90–450 mg/dL), IgM level of 130 mg/dk (normal, 70–280 gm/dL).Serum immunoelectrophoresis showed an IgG lambda monoclonal protein. Further studies including blood cell count, serum biochemistry, urinalysis, bone marrow biopsy, peripheral blood Sézary cell counts, Bence-Jones proteinuria, ct scans, abdominal ultrasonography, and chest roentgenograms yielded negaive or normal results. Thus, diagnosis of monoclonal gammopathy of unknown significance (mgus ) was made. Despite treatment with systemic chemotherapy, the diesease followed an aggressive course and the patient died from Staphylococcus epidermidis septicemia.     

Sweet's syndrome without granulocytosis

Background Sweet's syndrome (SS), acute febrile neutrophilic dermatosis, has been linked to hematologic malignancies and presents with characteristic edematous dermal plaques. Peripheral blood neutrophilia is frequently seen in association with SS and is one of the diagnostic criteria.
Objective To report the clinical, laboratory, and hematologic data of four patients with myeloid leukemia who developed SS after chemotherapy. Three of these patients were neutropenic.
Methods A retrospective study of four patients with SS and hematologic malignancies was undertaken. Three patients had de novo acute myelogenous leukemia and one was in the acute blast crisis of chronic myelogenous leukemia.
Results Sweet's syndrome was not originally suspected in these patients because of the low peripheral white blood cell counts caused by chemotherapy. All of the patients presented with fevers, arthralgias, and an eruption. They had been treated with antibiotics because of a presumed infection. Once the correct diagnosis was made and oral prednisolone was started, a rapid response followed.
Conclusions Sweet's syndrome should be considered in the differential diagnosis when acute myeloid leukemic patients develop skin lesions and unexplained fevers regardless of the peripheral blood counts.     

A child with spider bite and glomerulonephritis: a diagnostic challenge

A previously healthy 7-year-old white boy presented to St. Louis Children's Hospital with a 1-day history of headache, malaise, temperature of 38.7 degrees C, and a progressively erythematous, tender calf with central dusky purpura. On the morning of admission, his mother noticed a 2-mm crust on the patient's right calf with a 3-cm x 3-cm area of surrounding erythema. No history of recent trauma or bite was obtained. He had suffered two episodes of nonbloody, nonbilious emesis during the last day. In addition, over the previous 12 h, he presented brown urine without dysuria. His mother and brother had suffered from gastroenteritis over the previous week without bloody diarrhea. On initial physical examination, there was a 6-cm x 11-cm macular tender purpuric plaque with a central punctum on the right inner calf, which was warm and tender to the touch, with erythematous streaking towards the popliteal fossa (Fig. 1). The inguinal area was also erythematous with tender lymphadenopathy and induration, but without fluctuance. Laboratory studies included an elevated white blood cell count of 20, 800/microL with 6% bands, 86% segs, and 7% lymphocytes, hemoglobin of 12.5 g/dL, hematocrit of 35.1%, and platelets of 282,000/microL. The prothrombin time/activated partial tissue thromboplastin was 10. 4/28.0 s (normal PT, 9.3-12.3 s; normal PTT, 21.3-33.7 s) and fibrinogen was 558 mg/dL (normal, 192-379 mg/dL). Urinalysis showed 1+ protein, 8-10 white blood cells, too numerous to count red blood cells, and no hemoglobinuria. His electrolytes, blood urea nitrogen (BUN), and creatine were normal. The urine culture was negative. Blood culture after 24 h showed one out of two bottles of coagulase negative Staphylococcus epidermidis. The patient's physical examination was highly suggestive of a brown recluse spider bite with surrounding purpura. Over the next 2 days, the surrounding rim of erythema expanded. The skin within the plaque cleared and peeled at the periphery. The coagulase negative staphylococci in the blood culture were considered to be a contaminant. Cefotaxime and oxacillin were given intravenously. His leg was elevated and cooled with ice packs. The patient's fever resolved within 24 h. The lesion became less erythematous and nontender with decreased warmth and lymphadenopathy. The child was discharged on Duricef for 10 days. Because the patient experienced hematuria rather than hemoglobinuria, nephritis was suggested. In this case, poststreptococcal glomerulonephritis was the most likely cause. His anti-streptolysin-O titer was elevated at 400 U (normal, <200 U) and C3 was 21.4 mg/dL (normal, 83-177 mg/dL). His urine lightened to yellow-brown in color. His blood pressure was normal. Renal ultrasound showed severe left hydronephrosis with cortical atrophy, probably secondary to chronic/congenital ureteropelvic junction obstruction. His right kidney was normal.     

Analysis of 303 Ro/SS‐A antibody‐positive patients: is this antibody a possible marker for malignancy?

Background The risk of cancer in patients with autoimmune diseases has been investigated in several studies. Ro/SS‐A antibodies are frequent and specific autoantibodies among patients with various autoimmune diseases. Objectives To assess the risk of cancer in individuals with positive Ro/SS‐A antibodies and to analyse their clinical and laboratory characteristics. Methods Consecutive patients (n = 303) with Ro/SS‐A antibody positivity were collected during 11 years in our outpatient clinic for autoimmune diseases and were retrospectively analysed. Standardized incidence ratios (SIRs) and 95% confidence intervals (CIs) for all cancers were calculated. In addition, we identified further clinical and laboratory characteristics of Ro/SS‐A antibody‐positive patients indicating the development or existence of a malignancy. Results Fifty (16·5%) patients were diagnosed with malignancies. Ro/SS‐A antibody was strongly associated with malignant diseases (SIR 2·6, 95% CI 1·9–6·1), particularly melanoma (SIR 33·3, 95% CI 5·2–188·6), T‐cell lymphoma (SIR 16·7, 95% CI 2·9–128·9), non‐Hodgkin lymphoma (SIR 10·6, 95% CI 1·5–78·9) and breast carcinoma (SIR 4·98, 95% CI 1·3–28·3). Logistic regression modelling revealed that Ro/SS‐A antibody‐positive patients aged 55 years or older, presenting with fever, anaemia and cutaneous lupus erythematosus, have a greater probability of developing cancer and are considered high‐risk patients, as compared with Ro/SS‐A antibody‐positive patients with none of the mentioned clinical criteria. Conclusions In our cohort of Ro/SS‐A antibody‐positive patients, an overall increased risk of malignancy was noticed. Regular screening tests including imaging and laboratory values are justified in Ro/SS‐A antibody‐positive patients who exhibit the mentioned clinical criteria.     

Sjögren syndrome and systemic lupus erythematosus are distinct conditions

Sjogren syndrome (SS) and systemic lupus erythematosus (SLE) are both collagen vascular diseases that can be accompanied by Ro antibodies. Clinical evidence suggests that they are wholly distinct diseases. SS is strongly linked to lymphoma while lupus is not. SS patients do not commonly exhibit photosensitivity even though anti-Ro antibodies circulate in their blood; SLE patients generally exhibit photosensitivity. SS does not respond to hydroxychloroquine in a reproducible fashion whereas SLE does. SS has not been linked to parvovirus B19, but SLE has. However, SS and SLE do have similarities. Their autoantibody profiles are similar. They effect women more than men and have similar HLA haplotypes and autoantibodies; this is not likely coincidence but it may not clinically relevant.     

Journey of a patient with scleroderma from renal failure up to kidney transplantation

The increased awareness of systemic sclerosis (SS) and its pathogenetic background made the management of this disease more amenable than previously thought. However, scleroderma renal crisis (SRC) is a rarely seen as an associated disorder that may involve 2%-15% of SS patients. Patients presented with earlier, rapidly progressing, diffuse cutaneous SS disease, mostly in the first 3-5 years after non-Raynaud clinical manifestations, are more vulnerable to develop SRC. SRC comprises a collection of acute, mostly symptomatic rise in blood pressure, elevation in serum creatinine concentrations, oliguria and thrombotic microangiopathy in almost 50% of cases. The advent of the antihypertensive angiotensin converting enzyme inhibitors in 1980 was associated with significant improvement in SRC prognosis. In a scleroderma patient maintained on regular dialysis; every effort should be exerted to declare any possible evidence of renal recovery. A given period of almost two years has been suggested prior to proceeding in a kidney transplant (KTx). Of note, SS patients on dialysis have the highest opportunity of renal recovery and withdrawal from dialysis as compared to other causes of end-stage renal disease (ESRD). KTx that is the best well-known therapeutic option for ESRD patients can also be offered to SS patients. Compared to other primary renal diseases, SS-related ESRD was considered for a long period of poor patient and allograft survivals. Pulmonary involvement in an SS patient is considered a strong post-transplant independent risk factor of death. Recurrence of SRC after transplantation has been observed in some patients. However, an excellent post-transplant patient and graft outcome have been recently reported. Consequently, the absence of extrarenal manifestations in an SS-induced ESRD patient can be accepted as a robust indicator for a successful KTx.     

Cutaneous tumor cell load correlates with survival in patients with Sézary syndrome

Background: Sézary syndrome (SS) is defined by the triad of erythroderma, generalized lymphadenopathy and more than 1 000 circulating Sézary cells/μl in the peripheral blood. Patients and Methods: We screened the cutaneous lymphoma registry of our department for SS patients to identify clinical features of SS besides the defining criteria and to correlate them with disease survival. Results: 24 SS patients were analyzed retrospectively. The mean age was 65 years with 62 % male patients. The median follow‐up time was 32.5 months with an estimated 5‐year overall survival rate of 76 %. All patients complained about itching and presented with palmoplantar keratoderma. 62.5 % had nail involvement, 21 % alopecia, 12.5 % ectropion, 4 % prurigo nodularis, 8 % localized and 8 % generalized skin tumors, including leonine facies. In addition, 33 % had infections and also 33 % had venous thromboembolism. We identified cutaneous tumor cell load as a significant prognostic marker for SS. None of the other parameters were associated with disease specific survival. Conclusions: Clinically SS is characterized by various presentations beyond erythroderma. The cutaneous tumor cell load in SS is strongly associated with outcome and survival. We demonstrate a high risk for venous thromboembolism in SS patients who might benefit from anti‐coagulation therapies.     

Acute Monocytic Leukaemia with Cutaneous Manifestation

A 50‐year‐old Caucasian male presented with generalised skin rash. Gingival hyperplasia and hepatosplenomegaly were also noted. His laboratory data showed hemoglobin of 10.3 g/dL, white blood cell count of 203.6 K/uL and platelet count of 72 K/uL. Peripheral blood smears revealed that 67% of the white blood cells was monoblasts, 25% was promonocytes and atypical monocytes. Bone marrow aspirate smears showed that 97% of the nucleated cells were composed of monoblasts and promonocytes. Approximately 80% of the immature cells showed intense non‐specific butyrate esterase activity. The morphologic and cytochemical findings were compatible with a diagnosis of acute monocytic leukaemia. Skin biopsy taken from the left medial thigh revealed a Grenz zone and a dense infiltrate of atypical cells throughout the dermis. The cells were positive for KP1 (CD68) immunohistochemical stain. The findings were consistent with cutaneous manifestation of leukaemia (leukaemia cutis). The patient's condition deteriorated rapidly despite chemotherapy and expired 10 days after initial presentation. Leukaemia cutis is more common in acute monoblastic/monocytic leukaemia (AMoL) than in other subtypes of leukaemia. Leukaemia gingival hyperplasia is another characteristic feature of AMoL. Dissemination to the skin is generally associated with a poor prognosis.     

Interstitial type granuloma annulare associated with Sjögren’s syndrome

We describe a case of granuloma annulare (GA) associated with Sjögren’s syndrome (SS) in a 69‐year‐old woman. She complained of erythematous plaques on the left forearm and neck in addition to dry eyes and mouth. The laboratory and clinical findings also fulfilled the criteria for diagnosis of SS. Histopathological examination revealed the features of interstitial type GA. It is not rare that granulomatous diseases are associated with autoimmune diseases. This case indicated that granulomatous diseases and SS are closely related and that GA should be recognized as a cutaneous manifestation associated with autoimmune diseases, including SS.     

Clonality in the setting of Sweet's syndrome and pyoderma gangrenosum is not limited to underlying myeloproliferative disease

BACKGROUND: The neutrophilic dermatoses encompass, among others, Sweet's syndrome (SS) and pyoderma gangrenosum (PG), which are associated with underlying systemic diseases including myeloid dyscrasias. METHODS: On skin biopsies from 16 patients with biopsy-proven SS and/or PG, we performed an X-inactivation assay to detect clonal restriction of neutrophils. There were two patient categories based on known diseases at the time of diagnosis: patients with myeloproliferative disease and patients without myeloproliferative disease. RESULTS: Among seven patients with acute myelogenous leukemia and two with myelodysplastic syndrome, clonal restriction was found in five; three were homozygous, precluding analysis. Among the seven control patients, infiltrates were clonally restricted in five; one was polyclonal and the other was homozygous for the allele, precluding analysis. Of the five patients with clonally restricted infiltrates, one was subsequently diagnosed with myelodysplasia, one had unexplained neutropenia and an additional patient developed breast cancer. Overall, the incidence of clonality in both groups was the same, averaging 81%. CONCLUSION: These findings suggest that clonality in neutrophilic dermatoses, while characteristic of underlying myeloid dyscrasia, is not observed exclusively in the setting of myeloproliferative diseases. The significance of clonal neutrophilic infiltrates unassociated with myeloproliferative disease is unclear, but it may have some implications regarding the pathogenesis of sterile neutrophilic infiltrates. Clonality is well described in the setting of lymphomatoid hypersensitivity, reflecting an overzealous response to antigenic stimuli. One could speculate a similar mechanism operational in cases of apparently reactive SS/PG associated with monoclonality; a localized form of cutaneous neutrophilic dyscrasia is also possible.     

Clinical analysis of skin lesions in 796 Chinese HIV- positive patients

Skin lesions are often associated with human immunodeficiency virus (HIV) infection, reflecting the immunocompromised status of the individual. We investigated the relationship between skin lesions and immune function in a retrospective study of 796 Chinese HIV patients with and without highly active antiretroviral therapy (HAART). Of the 651 patients who had not received HAART, we found that 531 (81.6%) had apparent skin lesions. The incidence of infectious skin diseases (fungi, viruses, bacteria, spirochetes and parasites) and non-infectious skin diseases (excluding skin cancer) was 68.8% and 34.9%, respectively. Mean CD4(+) T-cell counts and CD4(+)/CD8(+) ratios were lower in patients with skin lesions than in patients without lesions (178 ± 96/μl vs. 306 ± 189/μl (p < 0.05) and 0.22 vs. 0.34 (p < 0.01), respectively). Candidiasis (25.8%), eczema (19.2%), nodular prurigo (13.8%), dermatophyte infections (10.6%) and herpes zoster (9.4%) were most common in Chinese patients with HIV. Among the 145 patients who had started HAART, there was a significantly lower prevalence of skin diseases (29.0%), although drug eruptions (12.4%) were more commonly observed. These findings indicate that HAART often reduces the incidence of infectious and non-infectious skin lesions in patients with HIV, but can itself be the cause of drug eruptions.     

Detection of clonal T-cell receptor gene rearrangements in the peripheral blood of patients with mycosis fungoides/Sezary syndrome

Involvement of the peripheral blood in mycosis fungoides/Sezary syndrome (MF/SS) has a significant impact upon prognosis, but it is often difficult to distinguish circulating cells of MF/SS from atypical reactive lymphocytes. We compared the standard morphologic method of identifying leukemic cells, the Sezary preparation, to a genotypic method using Southern blot analysis of T-cell receptor gene rearrangements in concurrent blood samples. We studied 26 MF/SS patients, five of them in remission, together with five controls from cases of various non-MF/SS skin diseases. Six of 26 MF/SS patients had morphologically atypical circulating leukocytes (3%, 4%, 5%, 14%, 16%, 19%). Seven of 26 MF/SS patients had clonal T-cell receptor gene rearrangements, including the four patients with the greatest percentages of atypical cells and three patients lacking atypical cells. Six of seven patients had skin disease at the time of sampling, including three with erythroderma, two with generalized thick plaques, and one with generalized patches, while one patient was in clinical remission. All five controls lacked morphologic and genotypic evidence of atypical or clonal T-cells. Relative to genotyping, in our series the Sezary preparation was less sensitive and less specific. There were three apparent false negative results in the Sezary preparations, and two potential false positive (patients with 3% and 4% atypical leukocytes); however, there was agreement between the two techniques in most cases. We conclude that gene rearrangement studies may provide an effective test with which to assess the peripheral blood of MF/SS patients.     

Assessment of nailfold capillaroscopy by x 30 digital epiluminescence (dermoscopy) in patients with Raynaud phenomenon

Background Dermoscopy is a useful tool for dermatologists to study melanocytic lesions. Its possible usefulness in the assessment of capillary nailfold morphological changes (capillaroscopy) has recently been advocated. Objectives To assess the practical utility of digital epiluminescence microscopy as a capillaroscopic instrument in patients with Raynaud phenomenon (RP). To compare the sensitivity and specificity rates obtained by epiluminescence microscopy with those previously reported with conventional capillaroscopic devices. Methods Fifty‐six consecutive patients with primary RP (PRP; n = 5) or secondary RP (SRP; n = 51) (11 men and 45 women in total) were included in the study. A control group of 10 healthy subjects was also evaluated. Twenty‐six patients (46%) had systemic sclerosis (SS), 12 (21%) presystemic sclerosis (pre‐SS), one (2%) dermatopolymyositis–SS, one (2%) mixed connective tissue disease, two (4%) Sjögren syndrome, two (4%) an overlap syndrome, one (2%) rheumatoid arthritis and six (11%) other connective tissue diseases. Capillary nailfold changes were studied using a nonportable digital epiluminescence device (magnification × 30). Following a systematized protocol, capillary nailfold morphology, density and distribution were evaluated. Several capillaroscopic patterns were identified (normal, sclerodermic, nonspecific, nondiagnostic) as previously defined. A possible relationship between capillary nailfold changes and the intensity of RP or the presence of associated autoimmune diseases was assessed. Results The sclerodermic pattern showed a sensitivity of 76·9% and a specificity of 90·9% in SS. A typical capillaroscopic SS pattern was observed in 73% of cases of limited SS and in 82% of cases of diffuse SS. Patients with Sjögren syndrome and dermatopolymyositis–SS showed a nonspecific capillaroscopic pattern. All patients with PRP presented a normal capillaroscopic pattern. A normal capillaroscopic pattern was also observed in 11 of 12 patients with pre‐SS. In one of two patients presenting severe sclerodactyly and in all patients showing hand oedema (three of 56), capillaroscopic changes could not be evaluated. Avascular areas correlated significantly with severe RP (P < 0·002), bone resorption (P < 0·007) and diffuse SS (P < 0·008). Conclusions Digital epiluminescence seems to be a useful and reliable technique in the evaluation of capillary nailfold morphological changes. This technical variation allows the identification of specific capillaroscopic patterns associated with connective tissue diseases. It also permits us to differentiate PRP from SRP. The results obtained with this technique are similar to those previously reported using standard capillaroscopy devices.     

Treatment of advanced mycosis fungoides/Sézary syndrome with fludarabine and potential adjunctive benefit to subsequent extracorporeal photochemotherapy

BACKGROUND: Purine analogues [fludarabine monophosphate (FAMP); deoxycoformycin and 2-chlorodeoxyadenosine) and extracorporeal photochemotherapy (ECP) have been suggested to be active agents in advanced cutaneous T-cell lymphoma (CTCL) patients. OBJECTIVES: To explore further the clinical efficacy and safety of FAMP monochemotherapy in advanced CTCL and to evaluate if the sequential association of ECP to FAMP in selected patients may improve the response rate (RR) and/or lengthen the remission duration. PATIENTS AND METHODS: Forty-four CTCL patients [17 Sézary syndrome (SS); 26 mycosis fungoides (MF), stage IIB-IV or with peripheral blood involvement; one MF associated with lymphomatoid papulosis (LyP)] were enrolled in this pilot cohort study. All the patients received FAMP 25 mg m(-2) 5 days monthly; 19 patients (43.2%) underwent ECP after FAMP was discontinued. The majority of patients with erythrodermic CTCL or peripheral blood involvement underwent the combined FAMP-ECP schedule. RESULTS: After a median follow-up of 4.2 years, the overall FAMP RR was 29.5% (13/44); a higher RR was obtained in SS (35.3%) than in MF patients (25.9%). According to the treatment group, the RR of the FAMP-ECP group (63.2%) was significantly higher than that of the FAMP monotherapy group (24%; P=0.021). No statistically significant difference was found in time-to-progression (TTP) or survival by therapy group, even if the TTP of the patients treated with the FAMP-ECP combination therapy was higher (median 13 vs. 7 months). A decrease or a normalization in the CD4+CD26- circulating subset was observed in responding patients, paralleling the reduction in the circulating Sézary cells. CONCLUSIONS: FAMP confirms its clinical activity as a single agent in SS; conversely, FAMP results do not compare favourably with other therapeutic approaches for advanced stage MF patients. The sequential association of ECP after FAMP seems to increase the RR, even if future randomized studies are needed to confirm these results.