Aurora Parodi

Small Intestinal Bacterial Overgrowth in Rosacea: Clinical Effectiveness of Its Eradication

BACKGROUND & AIMS To better understand the role of small intestinal bacterial overgrowth (SIBO) in rosacea, we aimed to assess the presence of SIBO in patients with rosacea and the clinical effectiveness of its eradication. METHODS We enrolled 113 consecutive rosacea ambulatory patients (31 M/82 F; mean age, 52 +/- 15 years) and 60 healthy controls who were sex- and age-matched. Patients and controls underwent lactulose and glucose breath tests (BTs) to assess the presence of SIBO. Patients positive for SIBO were randomized to receive rifaximin therapy (1200 mg/day for 10 days) or placebo. A group of patients with negative BTs were also treated with rifaximin. Eradication was assessed 1 month after the end of therapy. Two dermatologists, unblinded on therapy, evaluated rosacea patients before and after treatment on the basis of an objective scale. RESULTS The prevalence of SIBO was higher in patients than controls (52/113 vs 3/60, P < .001). After eradication, cutaneous lesions cleared in 20 of 28 and greatly improved in 6 of 28 patients, whereas patients treated with placebo remained unchanged (18/20) or worsened (2/20) (P < .001). Placebo patients were subsequently switched to rifaximin therapy, and SIBO was eradicated in 17 of 20 cases. Fifteen had a complete resolution of rosacea. After antibiotic therapy, 13 of 16 patients with negative BTs for SIBO remained unchanged, and this result differed from SIBO-positive cases (P < .001). CONCLUSIONS This study demonstrated that rosacea patients have a significantly higher SIBO prevalence than controls. Moreover, eradication of SIBO induced an almost complete regression of their cutaneous lesions and maintained this excellent result for at least 9 months.

Expression of cytokines and chemokine receptors in the cutaneous lesions of erythema multiforme and Stevens-Johnson syndrome/toxic epidermal necrolysis

Background  Erythema multiforme (EM) and Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) are caused by a dysregulation of cellular immunity.

Serology of Lupus Erythematosus: Correlation between Immunopathological Features and Clinical Aspects

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the aberrant production of a broad and heterogenous group of autoantibodies. Even though the presence of autoantibodies in SLE has been known, for more than 60 years, still nowadays a great effort is being made to understand the pathogenetic, diagnostic, and prognostic meaning of such autoantibodies. Antibodies to ds-DNA are useful for the diagnosis of SLE, to monitor the disease activity, and correlate with renal and central nervous involvements. Anti-Sm antibodies are highly specific for SLE. Anti-nucleosome antibodies are an excellent marker for SLE and good predictors of flares in quiescent lupus. Anti-histone antibodies characterize drug-induced lupus, while anti-SSA/Ro and anti-SSB/La antibodies are associated with neonatal lupus erythematosus and photosensitivity. Anti-ribosomal P antibodies play a role in neuropsychiatric lupus, but their association with clinical manifestations is still unclear. Anti-phospholipid antibodies are associated with the anti-phospholipid syndrome, cerebral vascular disease, and neuropsychiatric lupus. Anti-C1q antibodies amplify glomerular injury, and the elevation of their titers may predict renal flares. Anti-RNP antibodies are a marker of Sharps syndrome but can be found in SLE as well. Anti-PCNA antibodies are present in 5–10% of SLE patients especially those with arthritis and hypocomplementemia.

Clinical, Histological and Immunopathological Features of 58 Patients with Subacute Cutaneous Lupus erythematosus

Background: Subacute cutaneous lupus erythematosus (SCLE) is a distinct subset of cutaneous lupus erythematosus clinically characterized by psoriasiform and/or annular lesions and by a mild or absent systemic involvement. Objective: The Italian Group of Immunodermatology of the Italian Society of Dermatology and Venereology reviewed the cases of SCLE seen in 10 years (1987–1996). Patients: Forty-six women and 12 men have been retrospectively studied, 42% had annular lesions, 39% psoriasiform ones and 16% both. Results: Lesions were mainly localized on the neck and face and relapsed in spring and autumn. Seventeen patients had 4 or more American College of Rheumatology criteria and could be classified as having systemic lupus erythematosus. The most frequent histopathological alterations were epidermal atrophy, hydropic degeneration of the basal layer and perivascular lymphocytic infiltrate. Deposits of immunoglobulins and C3 at the dermo-epidermal junction on the clinically involved skin were present in 86% of the patients. Dust-like particles in the epidermis were only found in 3% of cases. Anti-Ro/SSA antibodies were found in 71% of the cases and anti-dsDNA only in 5% of cases. Conclusions: SCLE is a particular subset of cutaneous lupus erythematosus with peculiar clinical and immunopathological features.

Lichen planus, Liver Kidney Microsomal (LKM1) Antibodies and Hepatitis C Virus Antibodies

No anti-liver kidney microsomal (LKM1) antibodies were detected in 46 patients with LP, 16 of whom had also a chronic liver disease (CLD). In contrast, anti-hepatitis C virus (HCV) antibodies were found in 10% of patients with LP and in 50% of those with LP and CLD. Anti-HCV antibodies may be considered as a false-positive reaction in 56% of cases, especially when anti-LKM1 antibodies are present. Our findings do not support such a hypothesis, but suggest that CLD in LP patients is, at least in Italy, mostly a postviral chronic active hepatitis.

Measuring the activity of the disease in patients with cutaneous lupus erythematosus.

The Systemic Lupus Activity Measure (SLAM) is a system proposed by rheumatologists to measure disease activity in their patients with systemic lupus erythematosus (LE). It involves scoring a group of clinical symptoms and laboratory findings, the maximum possible score being 84. In systemic LE, the mid‐point is between 9 and 12. We applied SLAM to 176 patients with cutaneous LE. Ninety‐seven had localized discoid LE (L‐DLE), 59 had disseminated discoid LE (D‐DLE) and 20 had subacute cutaneous LE (SCLE). Eighty‐five patients had low activity disease (0–4 points), 72 mildly active disease (5–9 points), 15 moderately active disease (10–14 points) and only four had very active disease (≥ 15 points). The most frequent lesions in patients who scored more than 10 points were photosensitivity, cicatricial alopecia, Raynaud’s phenomenon and oral ulcers. Fifty patients were followed up for more than 5 years (mean follow‐up 9 years). Nine of these had an increased SLAM score. Seven had L‐DLE, one D‐DLE and one SCLE. Seven of the 50 patients had photosensitivity, five cicatricial alopecia, five non‐cicatricial alopecia, two Raynaud’s phenomenon and two oral ulcers. Three patients who started with L‐DLE evolved to D‐DLE. The SLAM system is useful in the monitoring of disease activity in patients with cutaneous LE. Over time, even L‐DLE patients may develop active disease. Photosensitivity, alopecia, oral ulcers and Raynaud’s phenomenon seem to herald a worse prognosis.

Scleromyxedema: A multicenter study of characteristics, comorbidities, course, and therapy in 30 patients

BACKGROUND Scleromyxedema is associated with a monoclonal gammopathy and other comorbidities. Its prognostic and therapeutic features are poorly documented because most reports deal with single cases or small series. OBJECTIVE We sought to describe the characteristics of patients with scleromyxedema regarding demographics, clinical characteristics, comorbidities, therapeutic interventions, and course. METHODS We conducted a retrospective and prospective multicenter study. RESULTS We identified 30 patients with scleromyxedema (17 men and 13 women). The mean age at diagnosis was 59 years. The mean delay between disease onset and diagnosis was 9 months. Monoclonal gammopathy was detected in 27 patients. Extracutaneous manifestations were present in 19 patients including neurologic (30%), rheumatologic (23.3%), and cardiac (20%) manifestations. Two patients developed hematologic malignancies. The most common therapies included oral steroids and intravenous immunoglobulins. Although corticosteroids were ineffective, intravenous immunoglobulins (alone or in combination with other drugs) induced complete remission in 4 and partial remission in 9 patients with a mean treatment duration of 2 years. In all, 21 patients were followed up for a mean period of 33.5 months, at which time 16 patients were alive, 12 with and 4 without skin disease. Five patients died: 2 with dermatoneuro syndrome and 1 each with myeloid leukemia, Hodgkin lymphoma, and myocardial insufficiency. LIMITATIONS This is mainly a retrospective study. CONCLUSIONS Our study confirms that scleromyxedema is a chronic and unpredictable disease with severe systemic manifestations leading to a guarded prognosis. There is no specific definitive treatment. Our data support the contention that intravenous immunoglobulin is a relatively effective and safe treatment. The response is not permanent and maintenance infusions are required.

The CD40/CD40 ligand system is expressed in the cutaneous lesions of erythema multiforme and Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum.

Background  Erythema multiforme (EM) and Stevens–Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) are determined by a dysregulation of cellular immunity.

Dermatomyositis in 132 patients with different clinical subtypes: cutaneous signs, constitutional symptoms and circulating antibodies.

We retrospectively studied 132 patients with dermatomyositis; 84 had idiopathic, 30 paraneoplastic, 5 juvenile and 13 amyopathic forms of the disease. The commonest features were macular erythema, heliotropic erythema and Gottrons papules. Flagellate erythema occurred in 5% of patients with idiopathic dermatomyositis and correlated with the disease activity. Necrotic lesions were also found in this group of patients but did not always signal malignancy. The prevalence of malignancy was high (23%). Raynauds phenomenon occurred in 10.6% of patients, also in those with malignancy. Dysphagia, interstitial lung disease and arthralgias affected 20%, 8% and 40% of patients, respectively. Anti-Jo-1 antibodies were found in 5% of patients with idiopathic dermatomyositis and low titre ANA in 1/3 of patients. ANA did not correlate with the disease activity. We confirmed the data from the literature, but no cutaneous sign, constitutional symptom or circulating antibody was found marking a particular subtype of the disease.

Scar sarcoidosis after hyaluronic acid injection

A 54‐year‐old woman presented with a 5‐month history of tender nodules in both nasolabial folds that had developed 4 months after the injection of hyaluronic acid (HA) (Restylane®) for wrinkles. The patient was treated with 1.5 mg/day betamethasone for 6 days and her lesions disappeared within 1 week. About 8 days after stopping therapy, however, new nodules developed at the same site, on previously healthy buttocks, and on old scars.