Fibroblastic rheumatism: an uncommon arthritis. A case-based review.

Fibroblastic rheumatism (FR) is an uncommon disease of the skin, characterized by the presence of non-tender cutaneous nodules accompanied often by other rheumatic manifestations. This condition shows male predominance, no age preference and unpredictable course, resulting frequently in permanent joint damage. A 60-year-old man came to our department with a 4-year history of multiple non-tender nodules and morning stiffness affecting mainly the upper extremities. Clinical examination revealed arthritis of the hands, confirmed by imaging tests. Laboratory exams were unremarkable. A skin nodule biopsy showed a dermal collagenous lesion with myxoid areas composed of spindle and stellate cells. Immunohistochemical staining demonstrated positivity for CD68 and negativity for CD34, S100, EMA and desmine. FR was diagnosed and the patient started methylprednisolone 16 mg/day. Hydroxychloroquine 400 mg/day and methotrexate 15 mg/weekly were further added as steroid-sparing agents with clinical benefit. Clinicians should be aware of this underreported entity, which can rapidly lead to irreversible deformities.

Illness perceptions and quality of life in families with child with atopic dermatitis.

OBJECTIVE: To assess the Quality of Life (QoL) of children with Atopic Dermatitis (AD) and their families and the impact of the mothers' illness perceptions on the family QoL. MATERIALS AND METHODS: Seventy-five children with AD (54 infants and 21 children) and their mothers participated in the study. The following questionnaires were administrated: 1. Brief Illness Perception Questionnaire (Brief IPQ); 2. Infant's Dermatitis Quality of Life Index (IDQOL); 3. Children's Dermatology Life Quality Index (CDLQI); 4. Dermatitis Family Impact Questionnaire (DFIQ) and 5. The Severity Scoring of Atopic Dermatitis (SCORAD). RESULTS: Atopic dermatitis had a moderate impact on the QoL of the infants (6.67±5.30), children (7.86±7.19) and their families (9.42±7.03). The DFIQ was associated with certain dimensions of the Brief IPQ, specifically, with Illness Identity (greater symptom burden) (r=0.615, p=0.000), beliefs about the Consequences of the illness (r=0.542, p=0.000), the Concerns (r=0.421, p=0.000) and the Emotional Representations (r=0.510, p=0.000). Correlation was demonstrated between IDQOL and DFIQ (r=0.662, p=0.000) and between CDLQI and DFIQ (r=0.832, p=0.000), and a weaker correlation between SCORAD and DFIQ (r=0.255, p=0.035). The chronicity of the AD showed negative association with DFIQ (p<0.001). CONCLUSIONS: The QoL of families with a child with AD is associated with the mother's illness perceptions about AD, the children's QoL and with both the severity and the chronicity of the disease. Therefore, clinicians should pay attention not only to the clinical characteristics of the children, but also to the parents' beliefs and emotions, to improve the family QoL.

Laminin Immunostaining in Biopsies as a Useful Biomarker of Early Invasion in Actinic Cheilitis and Differential Diagnosis Between Actinic Cheilitis and Lip Cancer: New Insights.

BACKGROUND: Squamous cell carcinoma of the lip (LSCC) and oral cavity can be life-threatening if not diagnosed early. Precancerous lesions like actinic cheilitis (AC), can transform into LSCC. Laminin is a fundamental component for basement membrane (BM) and its integrity may prevent neoplastic invasion. Therefore, laminin immunostaining of BM may be useful in identifying early invasion in actinic cheilitis and thus in the differential diagnosis between AC and invasive LSCC or high-grade epithelial dysplasia (ED). MATERIALS AND METHODS: Biopsies from 46 patients with oral lesions were histologically analyzed and immunohistochemically stained for laminin-1. RESULTS: AC was diagnosed in 34 patients and LSCC in 12 patients, including 3 patients with AC and concomitant high-grade ED/in situ carcinoma. Laminin-1 immunostaining revealed intense and linear expression of the BM in AC with low-grade ED. Loss of laminin expression was observed in LSCC. Intracellular laminin expression in parabasal cells was noted in AC with high-grade ED/in situ carcinoma. CONCLUSION: Laminin immunostaining could be useful in identifying AC cases suspected of early invasion. It could also contribute to the histopathological differential diagnosis between AC with low- and high-grade ED and between AC and invasive LSCC. The findings of this study provide new insights into the mechanism involved in the progression process of AC into LSCC, encouraging preclinical studies that may document the stochastic role of laminin in this process.

Evolution and spread of a multidrug-resistant Proteus mirabilis clone with chromosomal AmpC-type cephalosporinases in Europe.

Proteus mirabilis isolates obtained in 1999 to 2008 from three European countries were analyzed; all carried chromosomal AmpC-type cephalosporinase bla(CMY) genes from a Citrobacter freundii origin (bla(CMY-2)-like genes). Isolates from Poland harbored several bla(CMY) genes (bla(CMY-4), bla(CMY-12), bla(CMY-14), bla(CMY-15), and bla(CMY-38) and the new gene bla(CMY-45)), while isolates from Italy and Greece harbored bla(CMY-16) only. Earlier isolates with bla(CMY-4) or bla(CMY-12), recovered in France from Greek and Algerian patients, were also studied. All isolates showed striking similarities. Their bla(CMY) genes resided within ISEcp1 transposition modules, named Tn6093, characterized by a 110-bp distance between ISEcp1 and bla(CMY), and identical fragments of both C. freundii DNA and a ColE1-type plasmid backbone. Moreover, these modules were inserted into the same chromosomal site, within the pepQ gene. Since ColE1 plasmids carrying ISEcp1 with similar C. freundii DNA fragments (Tn6114) had been identified earlier, it is likely that a similar molecule had mediated at some stage this DNA transfer between C. freundii and P. mirabilis. In addition, isolates with bla(CMY-12), bla(CMY-15), and bla(CMY-38) genes harbored a second bla(CMY) copy within a shorter ISEcp1 module (Tn6113), always inserted downstream of the ppiD gene. Sequence analysis of all mobile bla(CMY-2)-like genes indicated that those integrated in the P. mirabilis chromosome form a distinct cluster that may have evolved by the stepwise accumulation of mutations. All of these observations, coupled to strain typing data, suggest that the bla(CMY) genes studied here may have originated from a single ISEcp1-mediated mobilization-transfer-integration process, followed by the spread and evolution of a P. mirabilis clone over time and a large geographic area.

CMY-31 and CMY-36 cephalosporinases encoded by ColE1-like plasmids.

Two CMY-2 derivatives, CMY-31 (Gln(215)-->Arg) from Salmonella enterica serotype Newport and CMY-36 (Ala(77)-->Cys and Gln(193)-->Glu) from Klebsiella pneumoniae, were characterized. Both cephalosporinases functionally resembled CMY-2. bla(CMY) alleles occurred as parts of a putative transposon comprising ISEcp1B and a Citrobacter freundii-derived sequence carried by ColE1-like plasmids similar to CMY-5-encoding pTKH11 from Klebsiella oxytoca.

Immune-mediated skin lesions in patients treated with anti-tumour necrosis factor alpha inhibitors.

OBJECTIVE: To describe immune-mediated skin lesion (IMSL) development in patients during anti-tumour necrosis factor (TNF) therapy. METHODS: Two hundred and fifty-two patients with rheumatoid arthritis (RA) and 183 with spondyloarthropathies (SpA) treated with anti-TNF inhibitors were analysed to identify IMSLs. RESULTS: Of the 252 patients with RA (146 treated with infliximab, 72 with adalimumab, and 34 with etanercept), 32 developed IMSLs. Eleven patients developed psoriatic skin lesions, 10 presented with granuloma annulare (GA), five had skin vasculitis, two alopecia areata, two discoid lupus erythematosus, one lichenoid eruption (lichen planus), and one vitiligo. Of the 183 patients with SpA (138 treated with infliximab, 37 with etanercept, and eight with adalimumab), 10 cases with IMSLs were identified. All were treated with infliximab. More specifically, six patients with ankylosing spondylitis (AS) developed psoriatic skin lesions, one developed GA, one lichen planus, and one alopecia areata. In addition, one patient with psoriatic arthritis (PsA) developed skin vasculitis. The occurrence of these IMSLs ranged from 3 to 36 months with a median of 20 months. Of all the patients with IMSL development, two with psoriatic skin lesions, two with GA, and one with vasculitis stopped anti-TNF therapy because of the extent and severity of the skin lesions. CONCLUSIONS: Our results on patients treated with TNF antagonists strongly support a link between TNF inhibition and IMSL development. Although these clinical complications are rare, clinicians should be aware of their occurrence and should recognize them.

Induction of psoriatic skin lesions in a patient with rheumatoid arthritis treated with rituximab.

Rituximab is a chimeric monoclonal therapeutic antibody which causes depletion of CD20-positive B cells. Apart from its apparent efficacy in the treatment of non-Hodgkin lymphoma and of several rheumatic diseases, it is associated with adverse events including the induction of autoimmune phenomena. We describe here the development of psoriatic skin lesions in a patient with rheumatoid arthritis after the second course of treatment with rituximab. This report supports the hypothesis that autoimmune phenomena may occur by biologic agents and there is a link between B-cell depletion and the induction of psoriatic skin lesions, which were confirmed histologically. However, further studies are needed in order to identify the underlying mechanism, as well as the risk factors associated with rituximab-induced psoriatic skin lesions.

Early-stage sarcomatoid renal cell carcinoma: multidetector CT findings.

A 64-year-old woman was referred for multidetector CT examination of the abdomen due to an incidental right renal mass, found on routine sonographic examination. A lower pole renal mass was detected on CT, with no signs of invasiveness or metastatic disease. The mass was sharply demarcated and heterogeneously enhanced, the last finding suggestive of malignancy. Pathologically, the tumor was an early-stage sarcomatoid renal cell carcinoma. The patient remains well, with no signs of recurrence or metastases 18 months after surgery.

Granuloma annulare induced by anti-tumour necrosis factor therapy.

OBJECTIVE: To describe granuloma annulare (GA) skin lesion development in patients during anti-tumour necrosis factor (TNF) therapy. METHODS: 199 patients with rheumatoid arthritis and 127 suffering from spondyloarthropathies treated with anti-TNF antagonists were analysed to identify skin lesions suggesting GA. RESULTS: Nine cases of GA during anti-TNF therapy (123 treated with infliximab, 57 with adalimumab and 17 with etanercept) for rheumatoid arthritis were identified. Two have been treated with infliximab, six with adalimumab and one with etanercept, and here the development of GA was 4.5%. No patient with spondyloarthropathies developed such skin lesions. All patients developed the generalised form of GA. None had or developed diseases, or conditions known to be associated with GA. In seven patients the skin eruptions developed during the first year of anti-TNF treatment, while they developed in two patients during the second year. Two patients had to stop anti-TNF therapy due to the extent of skin lesions. All patients responded well to the local corticosteroid therapy. CONCLUSIONS: Our series strongly supports a link between TNF inhibition and the development of GA in some patients. When dealing with patients on these agents physicians should be aware of possible adverse events and the potential development of such complications.

Eruptive pseudoangiomatosis: report of an adult case and unifying hypothesis of the pathogenesis of paediatric and adult cases.

One month after the onset of immunosuppressive treatment with corticosteroids and mycophenolate mofetil for a newly diagnosed pemphigus vulgaris, a 50-year-old female patient developed a new eruption clinically and histomorphologically consistent with eruptive pseudoangiomatosis (EP). Its self-limited course further confirmed this diagnosis. Although initially described as a paediatric eruption, meanwhile more adult cases of EP (30 out of a total of 53 cases identified by a Medline search) are reported in the literature. The review of adult cases of EP disclosed some common clinical and epidemiological characteristics: adult EP cases tend to cluster in the Mediterranean region of Europe, develop during the summer months, sometimes in the form of limited micro-epidemics, affect immunocompromised individuals and have lesions confined to the exposed skin sites. These characteristics, together with the exanthematic nature of the disease in children, point to some vector-transmitted infectious agent as the cause of this probably underdiagnosed disease.

A single pulmonary rheumatoid nodule masquerading as malignancy.

We present a 54-year-old man, a heavy smoker, with clinical and laboratory evidence of familial hypercholesterolemia and an asymptomatic solitary pulmonary nodule (SPN) increasing in size on follow-up chest X-ray. Laboratory work-up revealed high titers of rheumatoid factor and the presence of acute phase reactants. Because of the patient's age and history of smoking, open lung biopsy was performed to rule out malignancy. The biopsy showed histological features compatible with a rheumatoid nodule. Identical features were noted in the histological examination of the subcutaneous nodule. Usually, rheumatoid nodules occur in patients with active, long-standing rheumatoid arthritis (RA) with other extra-articular manifestations. The presence of a single pulmonary rheumatoid nodule as the first manifestation of RA is extremely rare. Histologic proof is often required since pulmonary carcinoma can present an identical clinical and radiological pattern. The diagnostic work-up and the differential diagnosis of an SPN, particularly in a heavy smoker, is a common clinical request and remains a diagnostic challenge.

Diagnostic accuracy of pericardial fluid cytology: an analysis of 53 specimens from 44 consecutive patients.

Over a 7-yr period, a total of 53 pericardial fluid specimens from 44 patients was examined. A correlation between cytological and histological diagnosis was made in 19 of these cases. In the remaining 25 cases, where a biopsy was not performed, the cytological diagnosis was correlated with the final clinical diagnosis and the patients' clinical outcome. Finally, in 9 out of 14 cases of malignancy where both cytological and a histological diagnosis was made, the cytologic prediction of the histologic type of cancer was evaluated. The overall sensitivity was 100%, the overall specificity was 93.3%, and the overall cytological accuracy was 95.4%. The predictive value of the correct histologic type of cancer by cytology was 77.7%. Our findings show that the careful cytomorphological examination of pericardial fluid aspirates is a valuable, reliable, and diagnostically highly accurate method, which could be performed on a routine basis in a busy cytopathology department. Judiciously chosen ancillary procedures, as well as clinicopathological correlation, are of great value for an accurate diagnosis in problematic cases.

A case report on two primary malignancies of the ovary and kidney.

Multiple primary malignant tumors in the same patient are very rare. Moreover, cases with primary ovarian and renal cell carcinoma in the same patient is an extremely rare phenomenon. In this report, a case with these two primaries is described. Concomitantly, some data suggesting a relationship of these primaries with the hypothesis of hormone dependency of renal cell carcinoma is presented.

Appendiceal diverticulitis in a femoral hernia causing necrotizing fasciitis of the right inguinal region: report of a unique case.

De Garengeot's hernia--a rare finding occurring mostly in women--is defined by the presence of the vermiform appendix within the sac of a femoral hernia. The incidence of appendicitis is rarer still, with less than a 100 cases reported to date. We present a unique case of an 84-year-old male patient with perforated appendiceal diverticulitis within a De Garengeot's hernia causing abscess and necrotizing infection of the overlying soft tissues.

Illness perceptions and quality of life in families with child with atopic dermatitis

OBJECTIVE: To assess the Quality of Life (QoL) of children with Atopic Dermatitis (AD) and their families and the impact of the mothers' illness perceptions on the family QoL. MATERIALS AND METHODS: Seventy-five children with AD (54 infants and 21 children) and their mothers participated in the study. The following questionnaires were administrated: 1. Brief Illness Perception Questionnaire (Brief IPQ); 2. Infant's Dermatitis Quality of Life Index (IDQOL); 3. Children's Dermatology Life Quality Index (CDLQI); 4. Dermatitis Family Impact Questionnaire (DFIQ) and 5. The Severity Scoring of Atopic Dermatitis (SCORAD). RESULTS: Atopic dermatitis had a moderate impact on the QoL of the infants (6.67 ± 5.30), children (7.86 ± 7.19) and their families (9.42 ± 7.03). The DFIQ was associated with certain dimensions of the Brief IPQ, specifically, with Illness Identity (greater symptom burden) (r = 0.615, p = 0.000), beliefs about the Consequences of the illness (r = 0.542, p = 0.000), the Concerns (r = 0.421, p = 0.000) and the Emotional Representations (r= 0.510, p = 0.000). Correlation was demonstrated between IDQOL and DFIQ (r = 0.662, p = 0.000) and between CDLQI and DFIQ (r = 0.832, p = 0.000), and a weaker correlation between SCORAD and DFIQ (r = 0.255, p = 0.035). The chronicity of the AD showed negative association with DFIQ (p < 0.001). CONCLUSIONS: The QoL of families with a child with AD is associated with the mother's illness perceptions about AD, the children's QoL and with both the severity and the chronicity of the disease. Therefore, clinicians should pay attention not only to the clinical characteristics of the children, but also to the parents' beliefs and emotions, to improve the family QoL

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Cryosurgery during topical imiquimod: a successful combination modality for lentigo maligna.

BACKGROUND: Either cryosurgery or topical imiquimod have been used to treat patients with lentigo maligna in cases where surgery is not feasible. METHODS: We report a patient with lentigo maligna, who was treated with the combination of topical imiquimod and cryosurgery, and review the rationale, which led us to design the present combined cryo-immunological treatment modality. RESULTS: Sustained clearance of lentigo maligna to date (26 months after treatment). The successful treatment of this patient was based on the following rationale: A cryosurgery session during continuing imiquimod application may: (i) reinforce apoptosis of tumor cells; (ii) strengthen antiangiogenesis in the treated tumor; and (iii) build-up a potent tumor-destructive immune response by a cascade of events starting with imiquimod-promoted attraction of immature dendritic antigen-presenting cells (DCs) into the tumor. DCs further mature within the tumor-antigen-rich environment of subsequently cryo-destructed tumor and upon imiquimod-driven migration into the peripheral lymph nodes can stimulate a specific antineoplastic cell-mediated immunity. Finally, continuing imiquimod application after cryosurgery may increase recruitment of activated effector cells into the tumor tissue leading to its destruction. CONCLUSION: Cryosurgery during continued topical imiquimod seems to be a promising treatment for lentigo maligna.