The predictive value of quantitative nailfold capillary microscopy in patients with undifferentiated connective tissue disease

The present prospective study was undertaken to follow the natural history of patients with Raynaud's phenomenon and to document in these patients the frequency with which secondary disorders develop. Seventeen patients with primary Raynaud's phenomenon (PRP) and 43 patients with undifferentiated connective tissue disease (UCTD) were examined after 6–8 years to see whether they had developed into UCTD or systemic sclerosis (SSc), respectively. Their nailfold capillary parameters were analysed statistically to ascertain whether they would predict for the development of the diseases into UCTD or SSc. Two patients with PRP(12%) developed into UCTD, and 10 patients with UCTD (23%) into SSc. In PRP, all three parameters of the patients who developed into UCTD showed a tendency to be larger than in those who did not develop UCTD. In UCTD, the apical limb width ( P  < 0.02), capillary width ( P  < 0.01) and capillary length ( P  < 0.01) of the subjects who developed SSc were significantly larger than those who did not. Of the clinicolaboratory findings in patients with UCTD, the occurrence rate of abnormal apical limb width (relative risk 20.7, P  < 0.01), abnormal capillary width (relative risk 10.7, P  < 0.01), abnormal capillary length (relative risk 9.2, P  < 0.02) and antinuclear antibody (relative risk 9.6, P  < 0.05) showed a significant predictive value for the development of UCTD into SSc. These results indicate that quantitative nailfold capillary microscopy, as well as antinuclear antibody, will provide exact predictive information in patients with UCTD in clinical practice.     

Quantitative analysis of nailfold capillary abnormalities in patients with connective tissue diseases

BACKGROUND: Nailfold capillary microscopy has been shown to reflect microvascular disturbances mainly in connective tissue diseases including systemic sclerosis (SSc). METHODS: Nailfold capillary abnormalities were analyzed with a light microscope under immersion oil at magnifications of x60 and x400. RESULTS: Abnormal nailfold capillary pattern in SSc was different from that of systemic lupus erythematosus (SLE) and normal controls, but not from dermatomyositis (DM). Seventy-two per cent of patients with Raynaud's phenomenon showed an abnormal nailfold capillary pattern. In primary Raynaud's phenomenon, 12% of subjects developed undifferentiated connective tissue disease. In undifferentiated connective tissue disease, 23% developed SSc. The apical limb width, capillary width and capillary length of subjects who developed SSc were significantly larger than in those who did not. With regard to the clinicolaboratory findings, the occurrence rate of an abnormal apical limb width, abnormal capillary width, abnormal capillary length, and antinuclear antibody in patients who developed SSc was significantly higher than in those who did not. An abnormal capillary pattern correlated with an elevated pulmonary artery resistance. All the patients with pulmonary arterial hypertension showed an abnormal capillary pattern, decreased diffusion capacity for carbon monoxide, and elevated pulmonary vascular resistance. Nailfold capillary abnormalities show a close relation to pulmonary arterial hypertension. CONCLUSIONS: Nailfold capillary abnormalities are useful for detecting vascular abnormality in clinical practice. These facts stress the importance of nailfold capillary abnormalities in SSc.     

The development of cutaneous lesions during follow-up of patients with primary neuritic leprosy

BACKGROUND: Primary neuritic leprosy (PNL) is a rare form of leprosy where the characteristic skin lesions are absent. Investigations of apparently normal skin from the areas of sensory change have revealed microscopic evidence of nerve involvement. Clinical studies have found that a proportion of patients develop visible skin lesions during follow-up. The aim of the study was to perform a clinical and histological analysis of PNL patients who developed visible skin lesions during treatment and follow-up, to gain insight into the pathogenesis of the disease. METHODS: Twenty-nine individuals in a series of 182 PNL patients developed visible skin lesions during follow-up. Analysis of the number, location, histology and time of onset of the new skin lesions in relation to the type and regularity of the treatment regimen were noted. A biopsy from the new skin lesion when available was compared with the nerve biopsy findings at the time of initial diagnosis. RESULTS: Thirty-eight per cent of patients developed a single patch and 28% developed two patches. Over three-quarters of these were on the lower limb (47%) or the upper limb (29%). Sixty-two per cent of patients developed the lesions within 2 years of the onset of symptoms. Patients on regular treatment developed patches earlier than those on irregular treatment or no treatment. A skin biopsy from the new patch revealed borderline tuberculoid leprosy histology in 47% of the patients. CONCLUSIONS: The findings suggest that leprosy primarily affects the nerve and that a neuritic phase precedes the development of visible cutaneous lesions.     

Prognosis and prognostic factors in adult patients with atopic dermatitis: a long-term follow-up questionnaire study

BACKGROUND: Atopic dermatitis (AD) is a chronic relapsing skin disease. Several investigations concerning the long-term prognosis of AD among children and teenagers have been performed but there are only few data among adults. OBJECTIVES: To investigate the prognosis and prognostic factors in adult patients with AD by a long-term follow-up (25-38 years). The prognostic factors were defined as those factors of importance for the persistence of AD. PATIENTS AND METHODS: A follow-up questionnaire was sent in November/December 1998 to 922 AD patients examined in our outpatient clinic between 1960 and 1973 among 1366 registered patients with AD. The patients were aged 20 years or older when they visited the clinic and 45 years or older when they answered the follow-up questionnaire. RESULTS: The response rate was 90.4%. The age range at the time of follow-up was 45-86 years (mean 55 years). Of the 833 patients who responded, 59% reported AD at some time during the last 12 months, which we defined as persistent AD. The mean value of clearance rate per person-years was 18%. One of the most important factors associated with persistence of AD was a head and neck dermatitis with or without other AD locations at the time of examination according to the old patient records. CONCLUSIONS: This study showed that the majority of adults with AD still had AD when they became older. This applies particularly if negative prognostic factors existed.     

The impact of changes in clinical severity on psychiatric morbidity in patients with psoriasis: a follow-up study

BACKGROUND: Psoriasis has a strong impact on quality of life and is correlated to psychopathological states. It is important to investigate the effect of clinical changes on psychological status. OBJECTIVES: To analyse the extent of clinical change and its effect on the presence of psychiatric morbidity in a group of patients with psoriasis. METHODS: All eligible adults hospitalized with psoriasis in a dermatological hospital (February 2000-February 2002) were given the self-administered Psoriasis Area and Severity Index (SAPASI) to assess clinical severity, the 12-item General Health Questionnaire (GHQ-12) to detect patients with psychological problems (defined as 'cases') and the Skindex-29 to evaluate symptoms. The same questionnaires were completed by the patients a month after hospital discharge. RESULTS: In our population of 414 patients, the incidence of GHQ cases becoming noncases was correlated with the SAPASI percentage improvement, ranging from 17.6% in patients with SAPASI worsened or unchanged at follow-up, to 68.2% in patients with clearance of psoriasis. Also, the proportion of patients who became GHQ noncases was much higher in patients with improvement of >/= 50% in symptoms, compared with patients with no improvement or worsening (70% vs. 32%, respectively). In a multivariate model the possible determinants of the passage from GHQ case to noncase were: SAPASI improvement, symptom improvement, no localization on the face, and gender (i.e. women were less likely to improve psychologically). CONCLUSIONS: The improvement in clinical severity and symptoms was associated with a decreased frequency of psychiatric disturbance. However, dermatologists should be aware that even in the presence of vast clinical improvement patients may still substantially suffer psychologically.     

Imiquimod and lentigo maligna: a search for prognostic features in a clinicopathological study with long-term follow-up

Background  Melanoma in situ /lentigo maligna (LM) is a potential precursor of LM melanoma. It occurs most commonly in elderly individuals on sun-exposed skin of the head and neck. Although surgical excision is the treatment of choice, this may not be desirable or feasible for large lesions at functionally or cosmetically important sites. Imiquimod is a topical immunomodulator which can generate a local cytotoxic response with potentially antiviral and antitumour effects.
Objectives  To present our experience of LM treated with imiquimod.
Methods  A retrospective review was performed of all patients with facial LM treated in our unit with topical imiquimod between January 2001 and December 2006. Pretreatment diagnostic biopsies were also reviewed and histologically graded.
Results  Forty-eight patients were treated with imiquimod. There were 37 responders and 11 treatment failures (of whom two were 'partial responders'). Of the 37 responders, 31 showed a clinical inflammatory response to imiquimod. One patient in whom treatment failed subsequently developed invasive disease. The mean follow-up duration was 49 months. We could not identify histological features of prognostic significance. However, the ability to develop an inflammatory reaction to imiquimod was a strong predictor of therapeutic benefit.
Conclusions  We consider imiquimod to have a role in the treatment of LM in patients in whom surgery may be contraindicated or for those in whom the cosmetic or functional consequences may be considerable. Until better characterized, its use should probably be confined to centres with experience in the detection and treatment of LM and melanoma.     

Subcutaneous calcification of the lower legs in a patient with mixed connective tissue disease

A 56-year-old woman who had been treated for mixed connective tissue disease (MCTD) noticed a skin ulcer on the lower leg. There was no history of trauma. X-rays of the lower legs showed extensive calcification in the soft tissue. Biopsied tissue from the ulcer showed marked calcium deposition with necrosis. Laboratory findings revealed normal serum calcium and phosphate levels and normal parathyroid function. On the basis of these findings, we diagnosed skin ulcer due to subcutaneous dystrophic calcification associated with MCTD. The ulcer was gradually reduced in size and epithelialized by treatment with local debridement and antibiotics.     

Vessel resistance to blood flow in the nailfold in patients with psoriasis: a prospective case-control echo Doppler-based study

Background Psoriasis is a disease in which endothelial cells seem to play an important pathogenic role. No report published to date has examined nail vascularity in patients with psoriasis. Objectives To evaluate the vascularity in the nails of patients with psoriasis treated with classic and biologic therapies for comparison with disease‐free controls, and to evaluate whether there are differences in nail vascularity among patients with and without nail involvement. Methods We performed a prospective study with two cohorts comprising 23 patients with moderate‐to‐severe psoriasis and 23 controls without psoriasis. We measured the nailfold vessel resistance index (NVRI) in each subgroup. Results We found significant differences in the NVRI between patients and controls (P < 0·001). Among patients with psoriasis, those with nail involvement showed a higher mean ± SD NVRI (0·58 ± 0·10) than those without (0·52 ± 0·45). Conclusions Psoriasis decreases the blood flow in nails, suggesting that this skin disease confers an independent risk for microvascular changes. This outcome may be associated with greater risk of nail‐related signs.     

Acrokeratoelastoidosis of Costa: a primary disease of the elastic tissue?

A 24-year-old black woman from Uganda was seen for treatment of multiple papules on her hands and feet. The lesions corresponded microscopically to foci of hyperkeratosis and acanthosis. Acid orcein stain revealed marked elastic fiber fragmentation. Acrokeratoelastoidosis of Costa (AKE) was diagnosed. The same damage to the elastic fibers was also present in an additional specimen from grossly uninvolved skin. On electron microscopy there were pronounced changes of the elastic fibers with elastolysis in both specimens. This case with generalized damage of the dermal elastic tissue supports the view that elastorrhexis is the key feature of AKE. Accordingly, the disease could be regarded as a primary elastic tissue disorder. The marginal acral keratoderma that is seen in AKE patients could represent epidermal changes secondary to chronic trauma.     

The follow-up of anogenital warts in a specialized consultation: study of patients lost to follow-up

INTRODUCTION: The treatment of condylomata acuminata relies on prolonged management, partly related to the need for repeated applications with regard to destructive therapeutic measures and the clinical supervision because of the high prevalence of relapses. This implies that patients are ready to consult their doctor at regular intervals during a relatively prolonged period of time. We wanted to assess the patients lost to follow-up in a population of patients exhibiting anogenital warts together with the factors associated with the loss to follow-up. PATIENTS AND METHODS: This was a prospective observational study conducted in France among dermatologists and gynecologists between May and December 2000. The physicians were free to prescribe the treatment of their choice. The social data of the patients and the classical characteristics of the condylomata acuminata were assessed. A patient was considered as lost to follow-up when he/she no longer came in for the consultation assessing the treatment. The data of the patients lost to follow-up were compared to those of the other patients. RESULTS: Two hundred ninety-seven cases, with 57 p. 100 men, were included during the study period. The mean age of the patients was of 33.8 +/- 11.4 years, with 43 p. 100 of patients aged under 30. In 40 p. 100 of cases, the lesions had existed for more than 3 months and were symptomatic in 30 p. 100 of cases. At the end of the study, 28.3 p. 100 of patients were considered as lost to follow-up. With multivariate analysis, the loss to follow-up appeared related to the patients' age (more frequent, the younger the patient; p<0.04) and not to the fixing of a precise appointment at the end of treatment (p<0.001). The asymptomatic nature of the lesions, recognized as a loss to follow-up risk factor in univariate analysis, was at the limit of significance in multivariate analysis (p=0.06). DISCUSSION: Clinicians should be particularly attentive with young and asymptomatic patients that they treat for condylomata acuminata, and attempt to reduce the rate of lost to follow-up and improve the quality of their management.     

Incidence and prognostic significance of skin disease in patients with HIV/AIDS: a 5-year observational study

Mucocutaneous diseases are common in HIV-infected patients. The aim of the present study was to determine the observed frequency of skin diseases and their prognostic significance for the progression of HIV infection. In a cohort of 150 HIV-infected patients diagnosed from 1986 to 1987 the number of mucocutaneous diseases, the CD4+ cell count, time to development of AIDS and survival time were registered for a period of 5 years. Eight patients were lost for follow-up. In the remaining 142 patients the number of mucocutaneous findings increased markedly during the observation period. The most frequent registered diseases and these with the highest increase were oral candidiasis, hairy leukoplakia, seborrhoeic dermatitis and herpes simplex. On average, 5.2 different diagnoses were registered per patient at the end of the follow-up period. The study showed that the total number of mucocutaneous diseases and the CD4+ cell count were significantly correlated to survival time and time to development of AIDS. The number of mucocutaneous diseases, like the CD4+ count, is an indicator of the immune system and the prognosis of HIV infection.     

Survival data for 299 patients with primary cutaneous lymphomas: a monocentre study

The aim of this study was retrospectively to assess the validity of the 2005 WHO-EORTC classification for primary cutaneous lymphomas (PCL) in a large cohort of patients of a single German skin cancer unit. All patients with PCLs consecutively visiting our hospital between January 1980 and December 2005 were included in a retrospective monocentre study, analysing their histological and clinical data. A total of 312 patients fulfilled the inclusion criteria for PCL. In 299 patients clinical information and paraffin material were sufficient for detailed classification. Of the 299 patients, 63% expressed a T-cell and 37% a B-cell phenotype. Mycosis fungoides was the entity with the highest frequency (30.9%), followed by primary cutaneous follicle centre lymphomas (16.9%) and lymphomatoid papulosis (15.9%). The mean follow-up period was 38.4 months. Five-year disease-specific survival was 80.5% for mycosis fungoides, 92.5% in primary cutaneous anaplastic large cell lymphoma, 100% in lymphomatoid papulosis, 98.1% in primary cutaneous follicle center lymphoma, 100% in primary cutaneous marginal zone lymphoma and 63.2% in diffuse large B-cell lymphoma, leg type. Our data are in line with the data collected by the WHO-EORTC. This is further evidence for the reliability of the WHO-EORTC classification and staging system.     

The wound/burn guidelines – 4: Guidelines for the management of skin ulcers associated with connective tissue disease/vasculitis

The Japanese Dermatological Association prepared guidelines focused on the treatment of skin ulcers associated with connective tissue disease/vasculitis practical in clinical settings of dermatological care. Skin ulcers associated with connective tissue diseases or vasculitis occur on the background of a wide variety of diseases including, typically, systemic sclerosis but also systemic lupus erythematosus (SLE), dermatomyositis, rheumatoid arthritis (RA), various vasculitides and antiphospholipid antibody syndrome (APS). Therefore, in preparing the present guidelines, we considered diagnostic/therapeutic approaches appropriate for each of these disorders to be necessary and developed algorithms and clinical questions for systemic sclerosis, SLE, dermatomyositis, RA, vasculitis and APS.     

The diagnostic value of a culture study in detecting patients with trichomoniasis

The advantages of culture method for the diagnosis of trichomoniasis over microscopy of stained and native preparations are demonstrated. New liquid nutrient media for Trichomonas cultivation reveal vaginal Trichomonas in 99.48% of cases in medium, in 100% of cases in medium, and in 97.78% of cases. These media are universal and may be used at bacteriologic laboratories of treatment-and-prophylaxis institutions engaged in culture diagnosis of trichomoniasis.