Filipa Ventura

Indeterminate cell histiocytosis in association with acute myeloid leukemia.

Indeterminate cell histiocytosis (ICH) is a rare proliferative disorder, in which the predominant cells share morphologic and immunophenotypic features from both Langerhans and non-Langerhans cell histiocytosis. We describe a 62-year-old man presenting a 2-month history of firm nodular lesions on the upper lip. Histopathology, immunohistochemical, and ultrastructural analysis showed typical findings of ICH. The patient was treated with thalidomide and almost complete regression of the lesions was reached within 7 months. Nevertheless, one month after remission, he developed an acute myeloid leukemia of the subtype monocytic leukemia (M5). The patients condition rapidly worsened and he died due to a respiratory failure four weeks later. We present this case because apart of being rare it joins the effectiveness of thalidomide and the association with an acute monocytic leukemia. A review of the literature is made.

A proposal for the use of new silver-seaweed-cotton fibers in the treatment of atopic dermatitis

Abstract Background: Atopic dermatitis (AD) is a disease with multifactorial etiology. Staphylococcus aureus is one of the predominant environmental factors acting on the course and intensity of the disease. Objectives: The aims of the study were to evaluate the efficacy and safety of clothing made of cellulose fibers with seaweed enriched with silver ions in the treatment of children with AD. Methods: A prospective, randomized and double-blinded controlled selection was done to recruit 19 children with diagnosis of AD. This sample was divided in two groups with similar demographic and clinical characteristics (the “control” group of seven children who wore placebo clothing and the “trial textile” group of 12 children who wore clothing with the new textile). The severity of AD and clinical response were assessed by the SCORAD index, the intensity of pruritus and the changes in sleep characteristics, at the start of the study and after 7 and 90 d. Results: The SCORAD index improvement in the group with the fiber under study was statistically significant after the first 7 d of treatment (p < 0.001) and was reduced by about 45% after 90 d. There was also a statistically relevant reduction of the intensity of pruritus and an improvement in the sleep quality after the initial 7 d and at day 90. Conclusion: The results showed that the textile clothing with seaweed enriched with silver ions brings a quicker improvement of the patients in the first days in opposition to the use of standard all-cotton clothes. The results also reinforce the importance of non-pharmacological measures, like clothing, in the management of patients with a diagnosis of AD.

Nasal glial heterotopia in a newborn infant

A newborn female infant presented at birth with a congenital, 33 · 25 · 25-mm mass located on the nasal bridge and protruding along the left nasopalpebral region (Fig. 1). The lesion had never bled and there were no problems associated with feeding or breathing. Physical examination revealed a round, solid, nonpulsating, painless tumor covered by erythematous skin with superficial telangiectasias. This mass showed no growth or change in size during crying or jugular vein compression (Furstenberg sign). There were no signs of visual or airway obstruction. The remainder of the physical examination was unremarkable. Magnetic resonance imaging (MRI) was requested, and sagittal MRI reconstruction images showed that the lesion did not exhibit intracranial extension (Fig. 2). Based on the clinical appearance of the lesion and lack of intracranial extension, a presumptive diagnosis of lymphangioma of the nasal bridge was established. Serial ophthalmologic examinations were recommended to assess any visual impairment. During the following months, neither rapid growth nor regression of the lesion was observed, which raised the first clinical suspicion of a misdiagnosis. The infant was referred to the Department of Plastic Surgery, and surgical excision was performed at the age of 18 months (Fig. 3) to prevent further secondary distortion of the nasal bridge and visual developmental sequelae. Pathologic evaluation of the excised mass showed skin overlying glial tissue positive for glial fibrillary acid protein (GFAP) and enlarged neurons positive for synaptophysin (Fig. 4), consistent with neuroglial heterotopia. At the age of 2.5 years, the child is doing well with no evidence of local recurrence (Fig. 5). Discussion

Colloid milium successfully treated with MAL-PDT

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Ecthyma gangrenosum secondary to severe invasive infection caused by Escherichia coli

infection caused by Escherichia coli Ecthyma gangrenosum (EG) is known as a characteristic lesion of Pseudomonas aeruginosa sepsis and is usually seen in immunocompromised patients. We report a case of a previously healthy 48-year-old woman. She was first observed by her primary care physician to have fever and cough and was diagnosed with pharyngotonsillitis and prescribed an oral cephalosporin (cefditoren pivoxil). As the fever persisted, six days later she returned to our hospital. At admission, she was febrile and hemodynamically unstable, with tachycardia and hypotension. Physical examination at this stage found a necrotic ulcer in the tongue. Laboratory investigations documented severe pancytopenia with almost zero neutrophilcs and hypogammaglobulinemia, and C-reactive protein was markedly increased. Chest radiograph revealed pneumonia, and CT confirmed the diagnosis. She gradually deteriorated and was admitted to the intensive care unit with septic shock and a diagnosis of pneumonia, and empiric antibiotic therapy was initiated. On the second day of hospitalization, multiple ecthymatous-like lesions in different stages of evolution were documented, mainly in the gluteal and perineal regions (Figs. 1 and 2). A skin biopsy with culture was performed and was compatible with EG. Cultures were obtained from her blood, bronchoalveolar lavage, bone marrow, and from a skin lesion. All cultures grew Escherichia coli. Urine culture was negative. Bone marrow was hypocellular, with severe decrease of erythroid and myeloid precursors. There were no abnormal cells in the bone marrow biopsy. She continued to deteriorate, with persistent neutropenia despite the use of growth factors and immunoglobulins. There was also progression of the pulmonary infiltrates confirmed by CT. She developed multiple organ dysfunction, and although treated with intensive therapy and appropriate antibiotic therapy, the patient died eight days after the admission. Despite all the laboratory and imaging studies, a cause for pancytopenia or E. coli sepsis, was not found. Ecthyma gangrenosum is a wellrecognized but uncommon cutaneous infection, almost exclusively seen in immunosuppressed patients. There are isolated reports of its occurrence in normal healthy subjects. It was thought to be pathognomonic for Pseudomonas infection until recent years when other organisms were implicated. Our patient was confirmed to have an E. coli sepsis. Although there have been case reports describing occurrences in previously healthy individuals as in our case, history of bacterial or viral infection and the previous antibiotic treatment were found as risk factors for the development of EG in these patients. In our patient, there was not a previous immunosuppressive condition and despite all the laboratory and imaging studies, a cause for neutropenia or E. coli sepsis, was not found. However, she received a cephalosporin (cefditoren

TINHA DO COURO CABELUDO POR TRICHOPHYTON VIOLACEUM EM ADULTO COM LÚPUS DISCÓIDE

A tinha do couro cabeludo e uma infeccao causada por dermatofitos. Os fungos mais frequentemente implicados no Hospital de Braga sao o Microsporum canis e o Trichophyton mentagrophytes var. granulare. Trata-se de uma infeccao frequente em criancas mas rara nos adultos. O Trichophyton violaceum e um dermatofito antropofilico raro nos paises desenvolvidos. Descreve-se o caso clinico de uma mulher adulta, com historia de lupus discoide, com tinha do couro cabeludo por Trichophyton violaceum.

Leg ulcer as a manifestation of eosinophilic vasculitis in a patient with hepatitis C virus infection, medicated with pegylated interferon/ribavirin.

Cryoglobulinaemic vasculitis is a complication of hepatitis C virus (HCV) infection, responding to treatment with pegylated interferon (peg-IFN)/ribavirin (RIB), but vasculitis may first appear after treatment with peg-IFN/RIB. A 35-year-old man with HCV infection presented to our department with a 2-month history of a 3.3×3 cm ulcer localised on the right shin, with a regular border, on a violaceous base. Histopathological examination revealed a leucocytoclastic vasculitis, rich in eosinophils. The patient had been treated with peg-IFN/RIB 10 months prior and treatment was discontinued after 2 months because of the appearance of arthralgias and neuropathy. Laboratory investigations revealed positive cryoglobulins, elevation of rheumatoid factor and reduction of C4 after treatment with peg-IFN/RIB. Dressings with a hydrocellular foam were placed and after 2 months the ulcer resolved. We presented this case because of the rarity of development of a cryoglobulinaemic vasculitis in a patient with HCV infection, previously treated with peg-IFN/RIB.