Extent Of Cutaneous Involvement Research Articles

Serum pentraxin 3 in systemic lupus erythematosus: A potential indicator of cutaneous disease activity.

Although skin manifestations are common in systemic lupus erythematosus (SLE), there is still a lack of a diagnostic marker for cutaneous involvement. Pentraxin3 (PTX3) has been studied in SLE patients; however, it has not been investigated in relation to cutaneous manifestations. To assess the serum PTX3 level in SLE patients, and to investigate its relationship with disease activity as well as with variable skin manifestations. Thirty-four patients with SLE (17 patients with skin manifestations and 17 without) and 30 healthy subjects were included in the study. Patients were evaluated clinically for systemic and skin manifestations of SLE. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI-2k) and Cutaneous Lupus Erythematosus Activity and Severity Index (CLASI) scores were calculated. Serum level of PTX3 was measured in patients and controls using ELISA. Higher serum PTX3 level was found in SLE patients compared to controls (p < 0.001). Patients with skin manifestations showed higher SLEDAI-2k scores and had higher PTX3 level compared to those without skin manifestations (p = 0.015 and p < 0.001, respectively). PTX3 showed higher levels in association with malar rash (p < 0.001), mucosal ulcers (p < 0.001), alopecia (p < 0.001), and purpuric eruption (p = 0.002). Moreover, PTX3 level positively correlated with CLASI scores (p < 0.001). Our results reinforce the important role of Pentraxin3 in SLE patients with skin manifestations, and it may be considered an interesting biomarker for the pattern and extent of cutaneous involvement in SLE.

Counting Monkeypox Lesions in Patient Photographs: Limits of Agreement of Manual Counts and Artificial Intelligence

The extent of cutaneous involvement is a key aspect for diagnosis and monitoring monkeypox disease, which is considered the most important orthopox virus in humans (Sklenovska et al. 2018). The spread of monkeypox cases in Europe and North America in May 2022 raised global public health concern (Muyembe-Tamfum 2022), leading to the World Health Organization declaring a public health emergency on 23 July 2022 (Ghebreyesus 2022).

Does Disease Activity Influence the Levels of Uric Acid in Psoriatic Arthritis?

Background: Hyperuricemia is not only associated with the development of gout but also with renal and vascular dysfunction. The prevalence of this condition has already been studied in psoriasis, but there are a few studies that have been carried out in psoriatic arthritis (PsA). Some studies have shown an association with metabolic syndrome, while others with the extent of cutaneous involvement, but there are no studies that have evaluated the disease activity with compound indexes. Objective: The aim of the study was to determine if disease activity, measured by different composite scores, influences the levels of uric acid. Method: This was a cross-sectional, observational study, which included 52 PsA patients. Clinical assessments included dactylitis, tender and swollen joint counts, Psoriasis Area and Severity Index, Leeds Enthesis Index, Minimal Disease Activity and Disease Activity for Psoriatic Arthritis. Hyperuricemia was defined as serum uric acid levels ≥ 6mg/dL in females and ≥ 7mg/dL in males. Results: Among the 52 included patients, 55.76% were female. The mean age was 54.9 ± 11.6 years. Hyperuricemia occurred in 26.92%. Demographic data, diet, comorbidities and medication were similar between patients with and without hyperuricemia. Patients with hyperuricemia had higher waist circumference (p &lt;0.0046). There was no difference in disease activity between groups, either in the isolated items or in the composite indexes. There was a significant difference in uric acid levels according to the classification of chronic kidney disease by estimated glomerular filtration rate (p=0.0016). Individuals using leflunomide had significantly lower levels of uric acid than those who were not using (p=0.0071). Conclusion: This study supports the notion that, in PsA, hyperuricemia is more related to metabolic factors than to disease activity.

Cutaneous Type Pemphigus Vulgaris of the Scalp: A Rare Unilesional Presentation

Pemphigus Vulgaris (PV) is the most common subtype of pemphigus, a rare group of autoimmune bullous diseases affecting the skin and mucous membranes. PV can be further subdivided into mucocutaneous and mucosal dominant types, depending on the extent of cutaneous involvement. Almost all cases of PV have mucosal involvement; however, a rare variant of cutaneous-only PV has been reported in the literature. To our knowledge, only two previous accounts of unilesional scalp PV have been reported. We present an unusual case of cutaneous-only PV involving the scalp.

αEβ7 Expression Increases With the Extent of Cutaneous Involvement in Mycosis Fungoides.

Cell adhesion molecules are essential to lymphocyte migration in neoplastic and inflammatory skin diseases. Our aim was to investigate possible differences in cell adhesion molecule expression between mycosis fungoides (MF) and inflammatory skin diseases (drug reactions and allergic contact dermatitis). We selected 33 biopsies from patients with MF and 10 biopsies of patients with inflammatory skin diseases from Department of Pathology-Universidade Federal de São Paulo (UNIFESP) from January 1997 to December 2013. Expression of α4β1 integrin and αEβ7 integrin was assessed by immunohistochemistry in intraepidermal lymphocytes by counting 4 microscopic epidermal fields (×400) and comparing those between the 2 groups. We observed increased expression of integrin αEβ7 in intraepidermal lymphocytes in advanced stages of MF (T3 and T4). αEβ7 expression was detected in intraepidermal dendritic cells of MF and inflammatory diseases samples. The expression of E-cadherin in epidermal cells in MF outlined Pautrier microabscesses, whereas in inflammatory diseases, spongiosis reduced its expression in keratinocytes. The findings presented here support the idea that the lymphocyte migratory mechanism observed in neoplasms is similar to that of inflammatory processes of the skin.

Risk of Cerebrovascular Accidents and Ischemic Heart Disease in Cutaneous Lupus Erythematosus: A Population-Based Cohort Study.

It is unclear whether isolated cutaneous lupus erythematosus (CLE) affects cardiovascular risk. We estimated the cumulative incidence and mortality of cardiovascular diseases in a population-based CLE cohort and compared the risk with a matched non-CLE cohort. All incident cases of CLE in Olmsted County, Minnesota, between 1965 and 2005 were followed until December 2013. The cumulative incidence of cerebrovascular accidents (CVAs [including stroke and transient ischemic attack]), ischemic heart disease (IHD [including coronary artery disease, myocardial infarction, and angina pectoris]), heart failure, and peripheral arterial disease (PAD) was derived and compared to an age-, sex-, and calendar year-matched non-CLE cohort using Cox models. There were 155 patients with CLE (mean ± SD age at diagnosis 48 ± 16 years, 65% female, mean ± SD BMI 26.3 ± 7.1 kg/m2 , 40% smokers, 9% with diabetes mellitus). During a median followup of 14.6 years, 41 CLE patients had cardiovascular events (15 patients with CVAs, 32 patients with IHD), with a 20-year cumulative incidence of 31.6%. As compared to non-CLE subjects, the risk of CVAs (smoking-adjusted hazard ratio [HR] 2.97 [95% confidence interval (95% CI) 1.13-7.78]) and PAD (HR 2.06 [95% CI 0.99-4.32]) was increased in patients with CLE, but the risk of IHD was not increased (HR 0.94 [95% CI 0.57-1.54]). There was no increase in cardiovascular mortality (HR 1.68 [95% CI 0.76-3.75]). The magnitude of risk for any cardiovascular outcome was not significantly influenced by the extent of cutaneous involvement. CLE may be associated with an increased risk of CVAs and PAD, but not IHD. Factors contributing to increased CVA risk in patients with CLE merit evaluation.

Review of Oral Psoriasis

Oral psoriasis is a subset of psoriasis that has many clinical presentations. Among the most common are geographic tongue, fissured tongue, and plaque psoriasis of the lips. Treatment options vary depending on the severity of the oral involvement as well as the extent of cutaneous involvement. Systemic treatments include infliximab, cyclosporine, and methotrexate. In cases with limited skin findings, topical treatments such as tacrolimus ointment, mometasone furoate ointment, fluocinolone acetonide cream, and retinoic acid mouthwash can be used. Direct steroid injections into the oral lesions also have been shown to aid in regression.

Clinical characteristics and prognosis of primary cutaneous B-cell lymphoma: 26 case reports

[Objective] To assess the predilection age,clinical features,treatment and pognostic factors of primary cutaneous B-cell lymphoma (PCBCL).[Methods] A total of 31 patients with PCBCL were registered in Tianjin Cancer Central Registry from January 1970 to September 2010,and 26 patients had complete medical recortds.A retrospective analysis was conducted on the 26 patients.The following indices were analyzed,including gender,age,body sites of initial involvement,extent of cutaneous involvement,pathological subtypes,metastatic sites,treatment,survival and prognosis.Data were processed with SPSS16.0 software,survival analysis was carried out by using Kaplan-Meier method,univariate analysis of prognostic factors by Log-rank test,multivariate analysis of prognostic faetors by COX proportional hazards model,and variables were selected by Forward LR method.[Results] Clinical manifestafions were atypical in these patients.The ratio of man and women was 1:1.6.Of the 26 cases,12 were primary cutaneous follicle-center lymphoma (PCFCL),8 were primary cutaneous marginal zone lymaphoma (PCMZL),and 4 were PCLBCL,leg type.Follow-up revealed lymph node involvement in 8 patients (4 in cervical nodes and 4 in inguinal nodes),distant metastasis of organs in 3 patients ( 1 in lung and pleura,1 in bone marrow and 1 in central nervous system).Most patients received surgical treatment combined with chemotherapy or chemoradiation.The 5-year overall survival rate was 80.8％(21/26).Statistical analysis showed that the prognosis of PCBCL was associated with histological subtype,lactate dehydrogenase (LDH) level and globulin level,but unrelated to gender,age,site of initial involvement,extent of cutaneous involvement,involvement of lymph nodes and organs,presence of B symptoms,treatment strategies,number of relapses,level of β2-globulin and hemoglobin,or lymphocyte absolute value.[Conclusion]s PCBCL,as a kind of rare extra-nodal lymphoma,usually occurs with atypical clinical mainfestations in persons aged from 39 to 66 years.The 5-year overall survival rate was 80.8％ in these patients.The prognosis of PCBCL seems to be related to histological subtypes,LDH and globulin levels. Key words: Lymphoma; B-cell; Antineoplastic protocols; Prognosis

Plasma cytokine profiles in systemic sclerosis: associations with autoantibody subsets and clinical manifestations

IntroductionSystemic sclerosis (SSc) (scleroderma) is a complex autoimmune disease that clinically manifests as progressive fibrosis of the skin and internal organs. Anti-centromere antibodies (ACAs), anti-topoisomerase antibodies (ATAs), and anti-RNA polymerase III antibodies (ARAs) are three mutually exclusive SSc-associated autoantibodies that correlate with distinct clinical subsets characterized by extent of cutaneous involvement and pattern of organ involvement. The current report sought to determine whether plasma cytokine profiles differ in SSc patients grouped according to these SSc-associated autoantibody subsets.MethodsPlasma from 444 SSc patients and 216 healthy controls was obtained from the Scleroderma Family Registry and University of Texas Rheumatology Division. Patients were classified according to the presence of ACAs, ATAs, ARAs, or none of the above (antibody-negative). Levels of 13 cytokines were determined using multiplex assays.ResultsCompared with females, healthy control males had higher plasma levels of IL-2 (P = 0.008), IL-5 (P = 0.01) and IL-8 (P = 0.01). In addition, in controls, IL-6 (P = 0.02) and IL-17 (P = 0.01) levels increased with advancing age. After adjusting for age and gender, SSc patients had higher circulating levels of TNFα (P < 0.0001), IL-6 (P < 0.0001), and IFNγ (P = 0.05) and lower IL-17 (P = 0.0005) and IL-23 (P = 0.014). Additional analyses demonstrated that disease duration also influenced these cytokine profiles. IL-6 was elevated in ATA-positive and ARA-positive patients, but not in ACA-positive patients. IL-8 was uniquely increased in the ATA-positive subset while both ATA-positive and ACA-positive subsets had elevated IFNγ and IL-10. IL-5 was only significantly increased in the ACA-positive subset. Lastly, patients with interstitial lung disease had elevated IL-6 and patients with pulmonary hypertension had elevated IL-6 and IL-13.ConclusionsPlasma cytokine profiles differ in SSc patients based on the presence of SSc-associated autoantibodies. Plasma cytokine profiles in SSc patients may also be affected by disease duration and the pattern of internal organ involvement.

Progressive systemic scleroderma. Clinical spectrum and prognostic parameters in 131 patients

The course of progressive systemic scleroderma was analysed from data collected on 131 patients. Classification was according to the extent of cutaneous involvement, type I (28%): sclerosis as far as the wrist-joint; type II (65%): sclerosis beyond the wrist-joint; type III (7%): sclerosis beginning to affect the trunk. In addition, they were subdivided into those with or without signs of systemic inflammation. The sex ratio was 3.5 females to 1 male. In half of the patients the disease manifested itself between the age of 30 and 50 years, with a clear peak about the 40th year. Serological evidence of inflammatory and immunological phenomena was present in nearly 50%, predominantly in those with type II or III. Oesophageal involvement was present in 84%, of the lung in 56% of all patients. ECG and echocardiographic changes were demonstrated in 34% of patients, while liver, intestines and kidneys were only rarely affected. The degree of organ involvement increased from type I to type III. Signs of systemic inflammation were predominantly associated with a clinically severe course.

Cutaneous sarcoidosis: current therapeutic modalities

Cutaneous sarcoidosis is a chronic, inflammatory, granulomatous disorder of unknown etiology that can be recalcitrant to many therapeutic modalities. A number of treatments have been reported to be effective, but none are uniformly successful. Therapy should be tailored to the individual patient and is dependent on the extent of cutaneous involvement and the degree of systemic manifestations. Corticosteroids and antimalarials are the cornerstones of treatment, although cytotoxic agents and numerous anecdotal anti-inflammatory agents have been used. Recently introduced biologics have ushered in a new era of therapeutic options for this potentially serious disorder.

Augmentation de l’hémoglobine A 2 au cours du pseudoxanthome élastique

Pseudoxanthoma elasticum (PXE) is normally associated with mutations in the ABCC6 gene. A PXE phenotype without mutations in ABCC6 has been described in Greek and Italian patients presenting with beta thalassemia. We attempted to determine the incidence of beta thalassemia in a cohort of French patients with PXE.Fifty patients with PXE were included in the study. Laboratory examinations comprised hemoglobin electrophoresis, ABCC6 gene study and in some studies: mutation analysis, beta-globin gene.No cases of beta thalassemia were diagnosed in this cohort of French patients with PXE. However, 20% of the latter exhibited a significant but isolated (i.e. without microcytic anemia) increase of hemoglobin A2 (HbA2). Statistical comparisons showed no difference in terms of geographical origin or severity of PXE between patients with high levels of HbA2 and those with normal levels of HbA2 other than the extent of cutaneous involvement. Study of the beta-globin gene displayed mutations only in the two patients with the highest recorded levels of HbA2. ABCC6 + beta-globin digenism was ruled out of the pathogenesis of PXE.The PXE phenotype seen in some patients with beta thalassemia appears to be associated with epigenetic modification of ABCC6 transcription and depends specifically on the beta globin locus. Isolated increase in HbA2 is probably a laboratory marker for PXE. Here again, a functional epigenetic reaction between ABCC6 and the beta-globin locus was suspected. However, these reciprocal interactions are clearly unequal since the change in ABCC6 transcription occurring during the course of beta thalassaemia is responsible for a PXE phenotype while increased HbA2 during the course of PXE has no clinical consequences.

Prognostic Factors in Primary Cutaneous B-Cell Lymphoma: The Italian Study Group for Cutaneous Lymphomas

Primary cutaneous B-cell lymphomas (PCBCLs) are a distinct group of primary cutaneous lymphomas with few and conflicting data on their prognostic factors. The study group included 467 patients with PCBCL who were referred, treated, and observed in 11 Italian centers (the Italian Study Group for Cutaneous Lymphomas) during a 24-year period (1980 to 2003). All of the patients were reclassified according to the WHO-European Organisation for Research and Treatment of Cancer (EORTC) classification. Follicle center lymphoma (FCL) accounted for 56.7% of occurrences, followed by marginal-zone B-cell lymphoma (MZL; 31.4%); diffuse large B-cell lymphoma (DLBCL), leg type, was reported in 10.9% of patients. Radiotherapy was the first-line treatment in 52.5% of patients and chemotherapy was the first-line treatment in 24.8% of patients. The complete response rate was 91.9% and the relapse rate was 46.7%. The 5- and 10-year overall survival (OS) rates were 94% and 85%, respectively. Compared with FCL/MZL, DLBCL, leg type, was characterized by statistically significant lower complete response rates, higher incidence of multiple cutaneous relapses and extracutaneous spreading, shorter time to progression, and shorter OS rates. The only variable with independent prognostic significance on the OS was the clinicopathologic diagnosis according to the WHO-EORTC classification (DLBCL, leg-type, showed a significantly worse prognosis v FCL and MZL; P < .001), whereas the only variable with independent prognostic significance on disease-free survival was the presence of a single cutaneous lesion (P = .001). Our study identifies a possible PCBCL subclassification and the extent of cutaneous involvement as the two most relevant prognostic factors in PCBCL. These data can be considered reasonably as the clinical background for an appropriate management strategy.

A4b1+ and a4b7+ CD4+ T cell numbers increase and CLA+ CD4+ T cell numbers decrease in systemic sclerosis

We studied the expression of adhesion molecules affecting recirculation and homing on peripheral blood CD4(+) T cells of patients with systemic sclerosis (SSc), in order to evaluate whether the distribution of tissue targeted subsets could reflect the participation of internal organs or the extent of cutaneous involvement [i.e. limited cutaneous (lc) and diffuse cutaneous (dc)]. Peripheral blood mononuclear cells (PBMC) from 51 patients with SSc and 19 sex- and age-matched controls were investigated by cytofluorimetric analysis for lymphocyte subpopulations carrying the following surface molecules: CD3, CD4, CLA, alpha4beta7 and alpha4beta1. Standard routine biochemistry and clinical examinations were also performed in all patients. We found that both alpha4beta1(+) and alpha4beta7(+) cells within the CD4(+) T cell population were significantly increased, while CLA(+) CD4(+) T cells were significantly reduced in SSc, compared to healthy donors. Significantly lower absolute numbers of alpha4beta7(+) cells were found in lc- compared to dc-SSc. Patients with oesophageal involvement had high numbers of alpha4beta7(+) cells, while those with nephritis also showed low levels of CLA(+) cells. Lung involvement was related directly to alpha4beta1(+) cell numbers and inversely to alpha4beta7(+) CD4 cell numbers. Taken together, our findings demonstrate that distinct CD4(+) T cell populations with selective homing properties show changes from normal distribution in SSc, and such changes are related to clinical expression and organ involvement in the course of the disease.

Disfunção erétil em homens com esclerose sistêmica

O objetivo deste trabalho é analisar a prevalência da disfunção erétil (DE) e a utilidade do Índice Internacional de Função Erétil (IIFE) na investigação da DE em pacientes com esclerose sistêmica (ES). Quinze homens com ES foram questionados acerca dos sintomas da DE e submetidos ao questionário de IIFE. Seus prontuários foram revisados para identificar outras possíveis causas da DE. Os pacientes estudados eram predominantemente caucasóides e tinham ES difusa. DE foi referida por 67% dos pacientes, iniciando-se, em média, 29 meses após o início da ES; quatro pacientes relataram início concomitante da DE e da ES. O diagnóstico da DE pelo IIFE e por interrogação direta foi concordante em todos os pacientes. Não foi encontrada associação nítida entre DE e extensão do envolvimento cutâneo, envolvimento visceral, drogas, escore cutâneo total e duração da doença. Cinco pacientes com DE também tinham outras causas de impotência. A DE afeta um grande porcentual de homens acometidos por esclerose sistêmica e o IIFE pode ser um instrumento válido em sua avaliação. A alta incidência da DE em jovens com ES e a eventual concomitância do início da ES e da DE enfatizam a necessidade de estudos posteriores sobre uma provável relação causal.

Primary cutaneous non-Hodgkin’s lymphoma with aggressive histology: inferior outcome is associated with peripheral T-cell type and elevated lactate dehydrogenase, but not extent of cutaneous involvement

BackgroundThe aim of this study was to explore the association between extent of cutaneous involvement, presenting features and progression-free survival (PFS) in patients with primary cutaneous non-Hodgkin’s lymphoma (PCNHL) of aggressive histology. MethodsPreviously untreated patients with localized or extensive PCNHL of aggressive histology, treated with combination chemotherapy, but excluding lymphoblastic lymphoma and mycosis fungoides and its variants, were reviewed retrospectively. ResultsWe identified 53 patients, of whom 52 (35 males, 17 females) were treated with doxorubicin-based regimens. Median age was 52 years (range 25–81 years), and disease was localized and extensive in 37 and 16 patients, respectively. Twenty-four patients had diffuse large B-cell lymphoma, nine had grade 3 follicular lymphoma, 13 had peripheral T-cell lymphoma (PTCL; not otherwise specified) and seven had anaplastic large cell lymphoma (WHO classification). With a median follow-up of 101 months (range 2–237 months) for survivors, the 10-year PFS was 65 ± 7% and overall survival was 72 ± 8%. The first failure involved the skin in 33% of B-cell and 91% of relapsing T-cell lymphomas. Univariate analysis revealed that PTCL (P = 0.005), lymphopenia (P = 0.01) and high serum levelsof β2-microglobulin (P = 0.0006) and LDH (P = 0.002), but not extent of skin involvement, were associated with inferior PFS. Multivariate analysis revealed that only PTCL and high serum lactate dehydrogenase (LDH) were independently associated with inferior PFS. ConclusionsPTCL and elevated serum LDH level, but not extent of cutaneous involvement are associated with inferior PFS in aggressive PCNHL treated with combination chemotherapy.

Cutaneous lupus erythematoses

The clinical features of the major types of cutaneous lupus arewell known. The three broad categories of discoid lupus, subcutaneous lupus and acute cutaneous lupus describe particular entities with generally discreet clinical features. However there is considerable overlap between these forms and the initial focus of management of patients presenting with cutaneous signs of lupus erythematosus consists of establishing the degree of systemic involvement present. In those patients with systemic disease the over-riding therapeutic need is for control of the systemic features of the condition. Cutaneous disease will normally respond at the same time. However there are many patients who have cutaneous disease as their major disease presentation. In these, choice of therapeutic option depends on the extent of cutaneous involvement, the time course of disease and the presence or absence of scarring. Whilst there is general agreement that first-line therapy with sunscreen, topical steroids and then if necessary anti-malarials is effective in the majority of patients (around two-thirds), subsequent therapy is based more on anecdote and experience than hard evidence. Being female, smoking and having extensive disease are features that often go together and lead to relative resistance to therapy. Once a single anti-malarial has failed in treatment, combination anti-malarials, dapsone, oral gold, methotrexate, thalidomide and many other agents have all been used though clinical evidence in support of their use is noticeable by its absence. Short courses of oral steroids are also often necessary in patients with severe and extensive disease as well as those with scarring. This talk will review the spectrum of disease, and the available treatment options.

Natural killer/natural killer-like T-cell lymphoma, CD56+, presenting in the skin: an increasingly recognized entity with an aggressive course.

To describe and identify the clinical and pathologic features of prognostic significance for natural killer (NK) and NK-like T-cell (NK/T-cell) lymphoma presenting in the skin. This study was a retrospective review of 30 patients with CD56+ lymphomas initially presenting with cutaneous lesions, with analysis of clinical and histopathologic parameters. The median survival for all patients was 15 months. Those with extracutaneous manifestations at presentation (11 patients) had a shorter median survival of 7.6 months as compared with those without extracutaneous involvement (17 patients), who had a more favorable median survival of 44.9 months (P =.0001). Age, gender, extent of cutaneous involvement, and initial response to therapy had no statistically significant effect on survival. Seven patients (24%) had detectable Epstein-Barr virus (EBV) within neoplastic cells. The patients with tumor cells that coexpress CD30 (seven patients) have not yet reached a median survival after 35 months of follow-up as compared with those with CD30- tumor cells (20 patients), who had a median survival of 9.6 months (P <.02). Routine histopathologic characteristics had no prognostic significance nor did the presence of CD3epsilon, EBV, or multidrug resistance. NK/T-cell lymphoma is an aggressive neoplasm; however, a subset with a more favorable outcome is identified in this study. The presence of extracutaneous disease at presentation is the most important clinical variable and portends a poor prognosis. The extent of initial skin involvement does not reliably predict outcome. Patients from the United States with NK/T-cell lymphoma presenting in the skin have a low incidence of demonstrable EBV in their tumor cells. Patients with coexpression of CD30 in CD56 lymphomas tend to have a more favorable outcome.

Elevated levels of eosinophil major basic protein in the sera of patients with systemic sclerosis.

To examine eosinophil activation, as reflected by evidence of eosinophil degranulation in the blood and affected tissues, in patients with diffuse and limited cutaneous forms of systemic sclerosis (SSc). Levels of the eosinophil-derived major basic protein (MBP), a marker of eosinophil degranulation, were determined in sera from 46 SSc patients, from patients with rheumatoid arthritis and giant cell arteritis, and from healthy volunteers, and in bronchoalveolar lavage fluid from 4 SSc patients. Extracellular tissue deposition of MBP was evaluated in biopsy specimens from affected skin or lung of 11 SSc patients. Patients with diffuse cutaneous SSc (dcSSc) had elevated serum MBP levels compared with normal individuals (mean +/- SD 762 +/- 271 ng/ml versus 534 +/- 144 ng/ml; P = 0.0004). MBP levels were positively correlated with the extent of cutaneous involvement, and negatively correlated with pulmonary function and duration of disease (r = -0.20). By immunohistochemical analysis, modest extracellular MBP deposition could be demonstrated in involved skin in 7 of 10 biopsy specimens, and MBP staining was prominent in affected lung tissues in 2 patients. Eosinophil degranulation appears to be increased in some patients with dcSSc, as indicated by elevated serum levels of MBP and extracellular accumulation of MBP in the lung. Eosinophil granule proteins may contribute to the development of cutaneous and pulmonary fibrosis in SSc.

Mastocytosis in infants and children: recognition of patterns of skin disease.

Mastocytosis is a disease characterized by an increase in the number of tissue mast cells and a concomitant increase in mast cell-derived mediators. To demonstrate the spectrum of skin disease in mastocytosis in the pediatric population, five children with mastocytosis and complaints of urticaria (4/5), bullae/vesicles (3/5), abdominal pain (3/5), flushing (2/5), headache (1/5), and bone pain (1/5) are reviewed. Confirmation of the diagnosis of cutaneous mastocytosis was obtained by histologic examination of a biopsy of lesional skin; however, mast cell numbers in lesional skin did not correlate with plasma histamine levels or the extent of cutaneous involvement. Mastocytosis is a diagnosis that must be recognized in the differential diagnosis of pediatric urticarial diseases.