Ana Paula Vieira

Rosacea with extensive extrafacial lesions

Rosacea is a very common skin disorder in the clinical practice that primarily affects the convex areas of the face. Extrafacial rosacea lesions have occasionally been described, but extensive involvement is exceptional. In the absence of its typical clinical or histological features, the diagnosis of extrafacial rosacea may be problematic. We describe an unusual case of rosacea with very exuberant extrafacial lesions, when compared with the limited involvement of the face.

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

Cannabis arteritis: ever more important to consider

Cannabis arteritis (CA) is a major and underdiagnosed cause of peripheral arterial disease in young patients. A 34-year-old man, daily smoker of 20 cigarettes and two cannabis cigarettes for 14 years, presented with a necrotic plaque of left hallux for 3 weeks. The Doppler ultrasound and angiography were compatible with severe Buergers disease. Submitted to a revascularisation procedure and hypocoagulation with rivaroxaban. He had ceased smoking but maintained consumption of cannabis. Owing to the persistence of distal necrosis, amputation of the hallux was performed with good evolution. CA is a subtype of Buergers disease. It is poorly known but increasingly prevalent and manifests in cannabis users regardless of tobacco use. The drug is considered at least a cofactor of the arteriopathy. The most effective treatment is cessation of consumption. Being cannabis one of the most consumed drugs, its mandatory to ask about its use in all young patients with arteriopathy.

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

A Case of IFAP Syndrome with Severe Atopic Dermatitis

Introduction. The IFAP syndrome is a rare X-linked genetic disorder characterized by the triad of follicular ichthyosis, atrichia, and photophobia. Case Report. A three-month-old Caucasian, male patient was observed with noncicatricial universal alopecia and persistent eczema from birth. He had dystrophic nails, spiky follicular hyperkeratosis, and photophobia which became apparent at the first year of life. Short stature and psychomotor developmental delay were also noticed. Histopathological examination of skin biopsy on left thigh showed epidermis with irregular acanthosis, lamellar orthokeratotic hyperkeratosis, and hair follicles fulfilled by parakeratotic hyperkeratosis. The chromosomal study showed a karyotype 46, XY. Total IgE was 374 IU/mL. One missense mutation c.1360G>C (p.Ala454Pro) in hemizygosity was detected on the MBTPS2 gene thus confirming the diagnosis of IFAP syndrome. Conclusions. We describe a boy with a typical clinical presentation of IFAP syndrome and severe atopic manifestations. A novel missense mutation c.1360G>C (p.Ala454Pro) in MBTPS2 gene was observed. The phenotypic expression of disease is quantitatively related to a reduced function of a key cellular regulatory system affecting cholesterol and endoplasmic reticulum homeostasis. It can cause epithelial disturbance with failure in differentiation of epidermal structures and abnormal skin permeability barrier. However, no correlation phenotype/genotype could be established.

Late onset Ito's nevus

Dermal melanocytoses include a variety of congenital and acquired melanocytic lesions characterised by the presence of multiple spindle-shaped dendritic melanocytes in the dermis. These lesions are commonly found in the skin of Asians, but they can also appear in Caucasians. The Mongolian spot, nevi of Ota and Ito are the most common morphological forms. We report a case of a 24-year-old Caucasian woman presented with a 10-months history of progressive darkening of the right side of her upper back. Cutaneous examination revealed a macular blue-grey hyperpigmentation of the right side of her upper back. Biopsy specimen from the macule showed multiple darkly pigmented, spindle-shaped dendritic melanocytes in the superficial dermis, interstitially arranged between collagen bundles. The diagnosis of nevus of Ito was established. Our patient is maintaining vigilance in dermatology consultation.

Sjögren-Larsson syndrome due to a novel mutation in the FALDH gene

ejd.2011.1286 Auteur(s) : Joana Maria BOTELHO GOMES1 gbmjoana@yahoo.com, Ana Paula VIEIRA1, Jorge NAVARRO2, Ricardo MARE3, Purificacao TAVARES4, Celeste BRITO1 1 Dermatology Department Hospital de Braga, Apartado 2242, 4701-965 Braga, Portugal 2 Casa de Saude de Guimaraes, Guimaraes, Portugal 3 Neurology Department, Hospital de Braga, Portugal 4 CGC genetics, Porto, Portugal Reprints.: J. Maria BOTELHO GOMES Sjogren-Larsson syndrome (SLS) is a rare autosomal recessive disorder characterized [...]

Cutis marmorata telangiectatica congenital

Cutis marmorata telangiectatica congenita (CMTC) is a skin vascular malformation of low flow, compromising venous and capillary vessels, of unknown origin, with about 300 cases described in the literature.1 ,2 Clinical manifestations include a purple erythema, which is either generalised or more frequently localised over a limb, with hypotrophy of the affected limb.1 ,3 CMTC can be associated with extracutaneous findings in 20–80%, including ocular and neurological abnormalities.2 ,3 CMTC resembles cutis …

Mycoplasma pneumoniae-induced rash and mucositis: a recently described entity

Mycoplasmapneumoniae is a common cause of respiratory infections. Although most cases are mild, some patients have extrapulmonary complications including mucocutaneous eruptions including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN) and erythemamultiforme (EM). Recently, a new entity, called M. pneumoniae-induced rash and mucositis (MIRM) was described. The authors present a clinical case difficult to classify attending to the classical classification of epidermolytic syndromes that meets the criteria proposed for the diagnosis of MIRM. The mucocutaneous disease associated with M. pneumoniae presents predominant mucositis, with scarce or absent cutaneous involvement. Because of the distinct morphology, pathophysiology and benign clinical course, MIRM should be considered as a new entity, distinct from SJS/TEN and EM.