Ricardo Galimberti

Chronic Benign Familial Pemphigus

ABSTRACT Ten patients with benign familial chronic pemphigus (BFCP) (Hailey‐Hailey disease) were evaluated; semio‐logic and localization differences were described, and special attention was given to solitary or atypical forms. In all the cases, the diagnosis was confirmed by histopathology; some histopathologic differences and the results achieved by direct irnmunofluorescence of skin are discussed. Most of the patients responded to the treatment with corticosteroids and antibiotics.

Characterization of the lipase activity of Malassezia furfur

Enzymes capable of metabolizing lipids are essential for the growth of Malassezia furfur in vitro and in vivo. We designed a series of experiments to characterize the lipolytic system in this yeast. The optimal pH of the lipase system was 7.5 Lipase activity was detected in soluble and insoluble saline cell extracts and in supernatant from the cultures. Esterase activity screened in samples separated by native polyacrylamide gels showed that it was restricted to one band of low mobility. An FPLC analysis of the soluble saline extract demonstrated that the lipase activity was present in three major peaks with different protein composition as revealed by SDS-PAGE. The enzymatic activity and cell growth were first induced and later inhibited by increasing concentrations of polyethylene-sorbitan-monooleate (Tween-80). The characterization of the lipolytic system (e.g. its induction by substrate and the effect of pH and/or different cations) could help to explain the increment in the number of M. furfur infections related to alterations of surface lipids in the skin such as seborrheic dermatitis.

Emerging systemic fungal infections

Systemic mycoses are a heterogeneous group of infections caused by different species of fungi that mainly affect individuals with primary or secondary alterations of immunity. In recent years, there has been an increase in the incidence of infections related to migration, AIDS, and other causes of immunosuppression, such as solid organ and bone marrow transplantation; oncological, hematological, and autoimmune diseases; and the use of new drugs. In this paper, we outline the microbiological and epidemiological characteristics of 3 fungi: Aspergillus spp, Fusarium spp, and Penicillium spp. We describe the clinical manifestations of disease with emphasis on those that should alert the dermatologist to make an early diagnosis. We detail the various tools for early diagnosis, prevention, and the epidemiology of different antifungals available for treatment and their mechanism of action and therapeutic efficacy.

Antibodies to proteins from Pityrosporum ovale in the sera from patients with psoriasis

In order to analyse the humoral immune response to the commensal yeast Pityrosporum ovale, we developed a western immunoblot technique with a salt soluble extract of P. ovale cytoplasm. In the present study, we tested sera from patients with psoriasis (n= 15), seborrhoeic dermatitis (n= 10), pityriasis versicolor (n= 8), and normal controls (n = 10). Seventy‐three per cent (11/15) of the patients with psoriasis showed specific reactivity with a protein derived from P. ovale of estimated molecular mass 120 kDa, and 46% (7/15) of the cases recognized a 100‐kDa protein. Sera from pityriasis versicolor and normal donors showed nonspecific reactivity with several bands of lower molecular weight.

Cutaneous necrosis by calcific uremic arteriolopathy.

Background  Calcific uremic arteriolopathy is a rare and serious disorder characterized by systemic medial calcification of the arteries and tissue ischemia. Most often it is found in patients with chronic renal failure on dialysis and in renal transplant recipients with secondary hyperparathyroidism.

Disseminated strongyloidiasis in immunocompromised patients – report of three cases

Background  Strongyloides stercoralis is an intestinal nematode of humans. The characteristic cutaneous manifestation of strongyloidiasis is larva currens. Patients with suppressed immunity can develop a severe disseminated strongyloidiasis involving wide spread of the larvae to extraintestinal organs, outside the usual migration pattern. Patients with cell‐mediated immunodeficiency and on corticosteroid therapy appear to be at highest risk for the development of this highly fatal entity.

ONYCHOMYCOSIS TREATED WITH A SHORT COURSE OF ORAL TERBINAFINE

Materials and Methods The trial was directed to test efficacy of and tolerance to terbinafine in the treatment of dermatophyte infections of the nails for a short period of 12 weeks. The study was designed as an open, nonrandomized, uncontrolled clinical trial. The patients, with a confirmed clinical and mycologic diagnosis of onychomycosis, were referred from the dermatology outpatient clinic. All of the patients were evaluated mycologically before treatment, at 12 weeks (end of treatment), and after 6 months of follow-up after the treatment was concluded. The mycologic survey included KOH preparations and cultures in Sabourauds and lactrimel media. The safety controls performed at the beginning and during the treatment period included measurements of serum aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase, and total bilirubin. The flowchart of the treatment schedule is depicted in Figure 1.

Outcomes in 11 patients with dermatofibrosarcoma protuberans treated with Mohs micrographic surgery

Background  Dermatofibrosarcoma protuberans (DFSP) is an uncommon intermediate‐grade fibrohistiocytic sarcoma. It occurs most often in adults aged 20–50 years and is associated with local invasion and a high recurrence rate. Uncontrolled local disease or metastases may result in death. Treatment has involved wide excision, Mohs micrographic surgery (MMS) and other approaches. The purpose of the current study was to review our experience with MMS in the treatment of patients with DFSP over the past six years.

Síndrome de Sweet asociado a neoplasias

Sweets syndrome was described in 1964 by Robert Douglas Sweet, as an entity he named acute febrile neutrophilic dermatosis. It is characterized by five main features: 1) sudden appearance of erythematous and tender plaques on the face, neck and extremities; 2) fever; 3) polymorphonuclear leukocytes; 4) predominantly neutrophilic dense infiltrate in the dermis, and 5) rapid response to steroid therapy. Sweets syndrome can be classified into five groups: idiopathic, parainflammatory, paraneoplastic, drug-induced, and pregnancy-related. Twenty percent of cases are associated with malignancies; 85% out of them involve hematologic alignancies and the remaining 15%, solid tumors. A series of seven cases of Sweets syndrome associated with neoplasms which were diagnosed from 2002 to 2006 is presented. Six cases were related to oncohematologic diseases and one to solid tumors. These results highlight the importance of the diagnosis of the syndrome, since it may predict tumor relapse or underlying disease progression. The timely use of diagnostic and treatment methods may improve the quality of life of these patients. The fact that oncology patients take multiple medications (a colony-stimulating factor) which may be associated with the onset of this entity must also be considered in excluding possible causes.

Validation in Spanish of a screening questionnaire for the detection of psoriatic arthritis in patients with psoriasis

OBJECTIVE A patient self-administered questionnaire [PsA Screening and Evaluation (PASE)] has been developed and validated in English, but has not been tried in Spanish speaking populations. This study aimed to adapt and validate PASE in Spanish to screen Spanish speaking psoriasis patients for signs and symptoms of inflammatory arthritis. METHODS Initial translation from English to Spanish (forward translation) was performed by two independent translators and the resulting versions were synthesized during a consensus meeting. The questionnaire was tried in a pilot study and resulted in a change in the agreement scale for a frequency scale with wording adaptation [Spanish PASE (PASE-S)]. RESULTS One hundred and eleven patients were screened with PASE-S; 25 with PsA (without previous treatments), 23 with psoriasis, 22 with psoriasis and OA and 41 with OA without psoriasis. The diagnosis of psoriasis was performed by a dermatologist, and a rheumatologist determined the diagnosis of PsA or OA. Patients with PsA had statistically significant higher symptoms, function and total PASE-S scores compared with those without PsA. Receiver operator curves showed an area under the curve of 0.79 (95% CI 0.69, 0.89) for the total score. A cut-off value ≥34 showed sensitivity of 76%, and specificity of 74.4% for the diagnosis of PsA. CONCLUSION The validated PASE questionnaire is a self-administered tool that can be used to screen for PsA among patients with psoriasis in a Spanish speaking population. PASE was able to distinguish between symptoms of PsA and OA.