Jesus Rodriguez Santamaria

Fenomeno de Lucio (eritema necrosante) na gestacao

The Lucios phenomenon, a type 2 reactional condition in leprosy probably mediated by immune complexes, is a severe necrotizing skin reaction that occurs mainly in patients with non-nodular lepromatous leprosy. This report presents a 27-year-old woman, in her 32nd week of pregnancy, with a one-week history of painful skin lesions in extremities, reddish-purple, sharply delineated, confluent, with bullae and occasional necrosis and ulceration. The patient also referred fever. Baciloscopy showed acid-fast bacilli and globi, and the histopathologic findings of a skin biopsy were consistent with lepromatous leprosy and Lucios phenomenon. Prednisone and multidrug therapy with rifampin, clofazimine and dapsone were given, with remission. Pregnancy has been associated with a high incidence of first diagnosis of leprosy or with an exacerbation of symptoms in patients with the established disease because hormonal alterations cause immunological imbalance, particularly between the last three months of pregnancy and the first three months of lactation, when immunosuppression is higher. Despite the recommendation not to take drugs during pregnancy, the multidrug therapy regimen must be used, since the benefits achieved with the treatment surpass the risks.

Síndrome de Sweet: estudo de 23 casos

BACKGROUND Sweets syndrome or acute febrile neutrophilic dermatosis is a rare disease characterized by painful violaceous erythematous skin lesions, fever, neutrophilic leukocytosis and dense dermal neutrophilic inflammatory infiltrate. It shows excellent response to corticosteroids. OBJECTIVES To assess cases of Sweets syndrome in a university hospital, identifying its clinical, laboratory and epidemiological characteristics and compare them with the data found on the literature. METHODS We conducted a retrospective epidemiological study by examining the medical records of 23 patients who met the diagnostic criteria for the disease from March 1995 to July 2009. We collected clinical and epidemiological data on the patients, such as lesion location, presence of cutaneous and extracutaneous manifestations, conditions associated with SS and some laboratory data, such as leukocyte count and ESR. RESULTS The age of the patients in the study ranged from 2 to 75 years. There were more females. The lesions mostly affected the trunk and upper limbs. Fever was the most common systemic manifestation, followed by arthralgia and myalgia, conjunctivitis and arthritis. The triggering factors most commonly identified were infections of the respiratory tract. Associated neoplasia occurred in 30% of the patients, especially hematologic neoplasia. CONCLUSION The clinical and epidemiological data found in our study are mostly similar to those found in the literature. Given the high prevalence of malignant diseases in patients with Sweets syndrome, it is necessary to know how to perform the diagnosis, carry out a full investigation as well as do the patients follow up.

Porocarcinoma écrino: relato de quatro casos e revisão da literatura

Eccrine porocarcinoma is an extremely rare skin tumor derived from the acrosyringium. Four cases of eccrine porocarcinoma are described, two males and two females, aged from 49 to 64 years. Tumors grew in an interval of one to 20 years. Lesions ranged from 1.2 x 2 cm to 4 x 5 cm and were on limbs or face. Two patients had basal cell carcinoma in a site different from the eccrine porocarcinoma. Two biopsies were described as eccrine poroma. Malignancy was observed in all cases after wide excision. Therefore, eccrine porocarcinoma should always be considered after a biopsy compatible with eccrine poroma.

Efalizumab in the Treatment of Scalp, Palmoplantar and Nail Psoriasis: Results of a 24‐Week Latin American Study

Introduction Plaque-type psoriasis affecting the nails, scalp, hands or feet can often be difficult to treat; for example, topical treatments and phototherapy may not penetrate the nail plate or scalp. The objective of this large, international, multicentre study was to investigate the efficacy of efalizumab in a Latin American population of adult patients with moderate-to-severe chronic plaque psoriasis who were candidates for systemic therapy or phototherapy. Methods Eligible patients were enrolled in a 24-week, open-label, single-arm, Phase IIIb/IV study of continuous treatment with subcutaneous efalizumab, 1.0 mg/kg/wk. Involvement of the nails, scalp, or hands or feet was assessed using the Nail Psoriasis Severity Index (NAPSI), the Psoriasis Scalp Severity Index (PSSI), or the Palmoplantar Pustulosis Psoriasis Area and Severity Index (PPPASI), respectively. Missing data were handled using a last observation carried forward or nonresponder imputation approach. Results Of the 189 patients who received treatment, 112 patients had nail involvement, 172 had scalp involvement, and 19 had palmoplantar disease at baseline. At Week 24, ≥50% improvement on the NAPSI, PSSI and PPPASI was observed in 31%, 71% and 68% of patients, respectively, whereas ≥75% improvement on these scores was observed in 17%, 52% and 63%, respectively. Descriptive statistics showed lower NAPSI-75 and higher PSSI-75 and -50 response rates among patients with higher baseline scores. Conclusions This open-label, uncontrolled study provides supportive evidence of the potential of efalizumab as a treatment for nail, scalp and palmoplantar psoriasis.

Sarcoma de Kaposi primário do pênis

Kaposis sarcoma is a vascular tumor involving the wall of lymphatic vessels. There are four types: classic, endemic, iatrogenic and HIV-associated. It is a systemic, malignant and multifactorial disease and has a variable course. The primary presentation on the penis is uncommon and is mainly observed in HIV-positive patients. In HIV-negative individuals, asymptomatic papules, nodules, plaques and verrucous lesions are found. The treatment for the classic form involves surgery, cryotherapy, electrosurgery, laser and radiation therapy. The authors present a rare case of a patient with the classic form on the penis, who was successfully treated by radiation therapy.

Recurrence rate of basal cell carcinoma with positive histopathological margins and related risk factors

BACKGROUND The best way to approach surgically removed basal cell carcinoma with positive histopathological margins is a controversial issue. Some authors believe that the more appropriate treatment is an immediate reoperation while others prefer a periodic follow up. The rates of recurrence are variable in literature, between 10% and 67%. OBJECTIVE To define the recurrence rate of basal cell carcinoma with positive margins after surgery. Secondarily, identify morphological aspects that can suggest a more frequent tumoral recurrence. METHODS This was a retrospective and observational study made by analysis of medical records of 487 patients between January 2003 and December 2009 in Hospital de Clínicas da Universidade Federal do Paraná (HC-UFPR). From 402 basal cell carcinomas surgically treated, 41 fulfilled inclusion criteria and were evaluated for five years or more. Recurrence rate of these tumors was analyzed in all patients and clinical characteristics such as sex, age, tumor size, tumor site, ulceration, and histological type were evaluated in order to find if they were related to more common tumoral recurrence. RESULTS The rate of positive margins after surgery was 12.18%. There were five cases of tumoral recurrence in the observation group and three cases in the re-excision group. Tumor size, site, histological type, ulceration and type of positive margin did not differ statistically between groups. It was not possible to consider if these factors were important in recurrence rates. STUDY LIMITATIONS Ideally, a prospective study with a larger sample would be more accurate. CONCLUSION The treatment of choice in basal cell carcinoma with positive margins must be individualized to reduce recurrence rates.

Vulvovaginal gingival syndrome and coeliac disease.

Editor In 1982, Pelisse et al . described the vulvovaginal gingival syndrome (VVGS), a special form of erosive lichen planus (LP) of mucous membranes. 1 The association of chronic erosive gingivitis, chronic vulvitis and erythematouserosive or desquamative vaginitis are the main clinical manifestations. 1 There are very few cases in medical literature reporting the association of the syndrome with coeliac disease. 2 Herein, we report a case of this association. A 27-year-old-woman searched a dermatological clinic complaining of gingival, genital and anal problems. She had a 3-year history of gingival lesions, accompanied by anal fissures, lesions on the genital mucosa and severe dyspareunia. She was in treatment for celiac disease, diagnosed by a small bowel biopsy, with a gluten-free diet, for the last 3 years. The intestinal disease was controlled, and she remained asymptomatic at the time. On physical exam, she had no skin lesions. Oral cavity examination showed a diffuse gingivitis along the superior gingival mucosa and on the central part of the inferior gingival mucosa (fig. 1). Genital examination revealed erythematous mucosa around the anus, vulval vestibule, vaginal orifice, labia majora and labia minora (fig. 2). Two biopsies (oral and genital) revealed a squamous mucosa presented a band-like liquenoid inflammatory infiltrate, with blurring of epithelial–chorium junction, intense exocytosis of lymphocytes and rare epithelial necrotic cells. The diagnosis of VVGS was made, and she started treatment with topical corticosteroids, with improvement. LP is an idiopathic inflammatory disorder. 3 It is believed that an autoimmune mechanism, involving activation of T cells against basal keratinocytes is responsible for the development of the disease. 3 About 25% of women with oral LP may also have vulvovaginal lesions, 4 and the presentation is synchronous in one half of the cases. 5,6 On the other half, the appearance of a lesion in one site is followed by lesions in other mucosal sites within 2 years. 6

Connective Tissue Diseases

Connective tissue diseases share certain common traits such as skin inflammation, and joint and other structures rich in connective tissue, along with immune defects. They represent a heterogeneous group of diseases characterized by a wide variety of signs and symptoms. The autoimmune connective tissue diseases include lupus erythematous, dermatomyositis, and morphea, and are considered classic connective tissue diseases. In this chapter The Epidemiology, etiopathogenesis, clinical presentation, differential diagnosis, complementary examinations, and therapeutic approaches for connective tissue diseases are discussed.