Alicia Kowalczuk

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.

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.

Calcinosis Cutis Following Liver Transplantation in a Pediatric Patient

Abstract: We report the occurrence of calcinosis cutis in a 3‐year‐old girl after liver transplantation. The cutaneous lesions consisted of 5 mm white papules on an erythematous base in linear and rosette configurations that developed in the abdominal and lumbar areas 10 days after transplantation. The patient had received calcium chloride solution intravenously during surgery. We excluded other causes of ectopic calcification such as hyperparathyroidism, renal failure, and extravasation of calcium solution. We discuss the etiology of calcinosis cutis after liver transplantation. This sequence of events has not been previously described in pediatric patients.

Lymphocutaneous nocardiosis and cutaneous pheohyphomycosis in a liver transplant recipient.

Background  Infections are the leading cause of morbidity and mortality in transplanted patients. The increasing number of immunocompromised patients has not only augmented infections by specific pathogens, but also by opportunistic microbial agents.

Mohs Micrographic Surgery for the Treatment of Basal Cell Carcinoma

INTRODUCTION Basal cell carcinoma accounts for 75% of all nonmelanoma skin cancer. Although various treatment modalities are available, the most frequently used option is surgical excision. Here, we evaluate the efficacy of Mohs micrographic surgery for the treatment of basal cell carcinoma. MATERIAL AND METHODS A retrospective review of cases of basal cell carcinoma treated with Mohs micrographic surgery between October 2003 and June 2009 was performed using patient records from Hospital Italiano in Buenos Aires, Argentina. RESULTS A total of 2412 basal cell carcinomas treated with Mohs micrographic surgery were identified; 50.5% were in women and 49.5% in men. The mean age of the patients was 70.7 years (range, 8-100 years). The histologic type of the tumor was solid in 65.3% of cases and in 89% of cases the tumor was located on the head or neck. Ten percent of the tumors were recurrent following previous treatment. A mean of 1.74 Mohs stages were used, with a mean of 3.81 sections. The mean size of the initial defect was 0.86 cm² and the mean final defect was 1.88 cm². The ratio of initial tumor size to final defect was estimated at 1.02. Over a mean follow-up of 32 months, there were 9 cases of tumor recurrence (0.37%). CONCLUSIONS In our experience, Mohs micrographic surgery is effective for the treatment of high-risk basal cell carcinoma.

Nephrogenic fibrosing dermopathy

We report a 19-year-old man with end-stage renal disease undergoing haemodialysis. He suffered from familial HUS and had been on haemodialysis since January 2004. After a corrective angioplastia of his left arm arteriovenous fistulae, he developed fever, oedema and limited mobility of extremities as well as myalgias. He had previously undergone magnetic resonance imaging with gadolinium containing contrast agents. An initial laboratory workup showed anaemia, hyperkalemia, hypercalcemia, hyperphosphatemia and increased ESR; bacterial, fungal and viral infections were ruled out. Due to severe and persistent hypercalcemia, pamidronate was administered. Calcium levels remained within normal limits. On physical examination, he presented global muscle atrophy with stiffness and flexion contractures in knees, hips and elbows. He had significant functional limitations and became unable to walk. The skin was thickened and indurate with brownish-grey, 4-mm-diameter, confluent, round papules with slightly desquamate area in lower extremities (fig. 1) and arms. A punch biopsy of the skin was done and showed dermal hyalinization and fibrosis with thickened collagen bundles (fig. 2). The immunohistochemical analysis showed the presence of numerous spindle CD 34 cells in dermis. All these findings were consistent with NFD. He was treated with methylprednisone 40 mg/day, anti–tumour necrosis factor monoclonal antibodies (etanercept) and 2 g/day mycophenolate mofetil. Muscle and joint pain disappeared, and flexion stiffness and skin lesions partially resolved. To our knowledge, this is the first case of familial HUS-associated NFD described in literature. The pathogenesis of NFD is still poorly understood. 1,3,4