Cutaneous manifestations of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. The skin is one of the target organs most variably affected by the disease. The American College of Rheumatology (ACR) established 11 criteria as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. Cutaneous lesions account for four of these 11 revised criteria of SLE. Skin lesions in patients with lupus may be specific or nonspecific. This paper covers the SLE-specific cutaneous changes: malar rash, discoid rash, photosensitivity, and oral mucosal lesions as well as SLE nonspecific skin manifestations, their pathophysiology, and management. A deeper thorough understanding of the cutaneous manifestations of SLE is essential for diagnosis, prognosis, and efficient management. Thus, dermatologists should cooperate with other specialties to provide optimal care of SLE patient.

Clinical and laboratory features in acute generalized pustular psoriasis: a retrospective study of 34 patients.

BACKGROUND: Acute generalized pustular psoriasis (AGPP) is a rare variant of psoriasis that can be lethal without proper treatment. It can be caused by the withdrawal of corticosteroids and, among its extra-cutaneous manifestations, liver abnormalities are frequently under-reported or attributed to drugs. OBJECTIVE: The aim of this study was to assess the clinical and laboratory data, treatment options, and disease outcome in patients with AGPP and to search for significant differences between subgroups of these patients. STUDY DESIGN: This was a retrospective analysis of the clinical files from inpatients with AGPP observed in our department between 1973 and 2008. Statistical tests were performed at a significance level of 5%. SETTING: This was an inpatient, single-center study. MAIN OUTCOME MEASURES: Outcome measures were a previous history of psoriasis, corticosteroid use before admittance, mortality rate, white blood cell count, absolute neutrophil count, and abnormalities in liver enzymes. RESULTS: A total of 34 patients fulfilled the inclusion criteria, of whom 61% were men and 65% had a previous history of psoriasis vulgaris. Topical corticosteroids were applied by 50% of patients before admittance. Skin lesions remitted with methotrexate, etretinate, or acitretin treatment in all but two patients who died of sepsis. Abnormalities in liver enzymes were present in 47% of patients. Patients without a previous history of psoriasis had a significantly younger age at the first episode of AGPP. In the comparison between the groups of patients with and without liver abnormalities, a male preponderance and higher leukocyte counts were found in the former, with a positive correlation between the absolute neutrophil count and total bilirubin also being observed. Previous use of retinoids or methotrexate was not associated with these hepatic alterations. LIMITATIONS: Limitations of the data were that this was a single-center, retrospective study with a small sample size. CONCLUSIONS: Withdrawal of systemic or topical corticosteroids can precipitate or worsen AGPP and these agents should not be used in these patients. Liver abnormalities can be considered an extra-cutaneous manifestation of AGPP. As in other series, no association between the use of drugs and changes in liver tests was found and therefore the deleterious withdrawal of efficient drugs, namely acitretin and methotrexate, should be avoided.

Sweet's syndrome: a retrospective study of 42 admitted patients in a Portuguese hospital.

BACKGROUND: Sweet's syndrome is the most common neutrophilic dermatosis, but its pathophysiology is still largely unknown and different subgroups can be defined. OBJECTIVES: To assess the clinical/laboratory factors in a series of patients with Sweet's syndrome and search for significant differences between defined subgroups. METHODS: A retrospective study of inpatient files with this diagnosis was made, and patients were included if they fulfilled the classical diagnostic criteria. Data were analyzed with the SPSS package, with a significance level of 5%. RESULTS: Forty-two patients fulfilled the inclusion criteria, with a median age of 50 years. Females comprised 88% of patients, and disease onset occurred in autumn or spring in over 70% of cases. A previous oropharyngeal infection was described by 38% of patients, and a previous or concomitantly diagnosed neoplasm by 10%. In contrast with the literature, the lower extremities were also involved in the majority of patients, and arthralgia was the most frequent extracutaneous manifestation. On bivariate analysis, in patients who reported a previous oropharyngeal infection, the lesions were significantly more frequent in autumn, and a shorter inpatient stay as well as a lower temperature on admission and lower prevalence of arthralgia were observed in the same group. CONCLUSIONS: The subgroup of patients with a previous oropharyngeal infection had a less severe form of this syndrome.

Success of prednisone in the treatment of a patient with sarcoidosis and systemic sclerosis: Report of a case.

We report a case of a man with sarcoidosis and diffuse systemic sclerosis who showed improvement of both diseases after prednisone therapy and remains asymptomatic after three years of follow-up. The association of these two diseases is rare and it is controversial whether this is a chance association or if there is a relationship between the two autoimmune diseases. Despite the poor prognosis and response to therapy of the patients with diffuse systemic sclerosis, our patient had a marked improvement of the disease with prednisone.

Erythema multiforme on exposed and non-exposed skin following polymorphic light eruption: a case report.

We report a 35-year-old caucasian female with a history of polymorphic light eruption (PLE) who, after the outbreak of pruritic papules following the first sun exposure of the year, developed target lesions in two different bouts, the first on sun-exposed skin and the second on sun-protected sites. A diagnosis of erythema multiforme (EM) developing as a result of PLE was made. As far as we were able to search in the literature, EM occurring in two bouts has not been described previously. We discuss the differential diagnosis.