Acroqueratoelastoidosis: reporte de un caso clínico / Acrokeratoelastoidosis: reportof a clinical case

Resumen La acroqueratoelastoidosis es una rara genodermatosis de herencia autosómica dominante, familiar o esporádica, siendo esta última la forma más frecuentemente reportada en la literatura. Fue descripta por el dermatólogo de origen brasileño Oswaldo Costa en el año 1953. Así las acroqueratodermias marginales son un subgrupo de queratodermiaspalmoplantares caracterizadas por la presencia de pápulas y placas queratósicas, con una disposición lineal, que asientan sobre el margen de transición entre la piel dorsal y palmar o plantar.Suelen iniciarse en la infancia, en la adolescencia o en la vida adulta temprana y tienen un curso crónico. Su diagnóstico diferencial con el resto de las acroqueratodermias es un gran desafío, siendo el hallazgo histológico de elastorrexis lo primordial para su correcto diagnóstico. Presentamos el caso de una mujer de 22 años con un cuadro compatible clínica e histopatológicamente con Acroqueratoelastoidosis.

Abstract Acrokeratolastoidosis is a rare genodermatosis of dominant autosomal, familial or sporadic inheritance, the latter being the most frequently reported form in the literature. It was described by the Brazilian dermatologist Oswaldo Costa in 1953. It is characterized by the presence of multiple hyperkeratotic papules, usually asymptomatic, located in the marginal area of the hands, feet or both. It usually begins in childhood, adolescence or early adult life and has a chronic course. Its differential diagnosis with the rest of the acrokeratosis is a great challenge, being the histological finding of elastorrexis the primary for its correct diagnosis. We present the case of a 22-year-old woman with a clinical and histopathology compatible with Acroqueratoelastoidosis.

A baseline evaluation of PAH body burden in sardines from the southern Brazilian shelf.

The concentrations of 37 polycyclic aromatic hydrocarbons (PAHs) and their potential risk to human health were determined in fifty sardine muscle (Sardinella brasiliensis) samples collected along the southern Brazilian shelf. Parental and alkylated PAHs were identified and quantified using a pressurized liquid extraction with in-cell purification method and gas chromatography-mass spectrometry identification and quantification. The concentrations of Σ37 PAHs in muscle ranged between 6.02 and 4074 µg kg-1 wet weight, which are comparable to levels reported for commercially important fish worldwide. The most abundant compounds were pyrene and fluoranthene, which originate from both petrogenic and pyrolytic hydrocarbon inputs. In only 4% of the samples the benzo[a] pyrene equivalent concentration was above the threshold of 6 µg kg-1 suggested for safe fish consumption in Brazil. These findings will serve as baseline data for monitoring the quality of sardines consumed in the country and for studying fish populations.

European S1 guidelines on the management of lichen planus: a cooperation of the European Dermatology Forum with the European Academy of Dermatology and Venereology.

Lichen planus (LP) is a chronic inflammatory and immune-mediated disease that affects the skin, hair, nails and mucous membranes. Although there is a broad clinical spectrum of lichen planus manifestations, the skin and oral cavity remain the major sites of involvement. A group of European dermatologists with a long-standing interest and expertise in lichen planus has sought to define therapeutic guidelines for the management of patients with LP. The clinical features, diagnosis and possible medications that clinicians can use, in order to control the disease, will be reviewed in this manuscript. The revised final version of the lichen planus guideline was passed on to the European Dermatology Forum (EDF) for a final consensus with the European Academy of Dermatology and Venereology (EADV).

Risk of mycosis fungoides in psoriatic patients: a critical review.

Psoriasis has been controversially associated with risk of non-Hodgkin lymphoma (NHL) and mycosis fungoides (MF). Also patients who developed MF after systemic treatment for psoriasis have been reported, and some authors suggested that the association between MF and psoriasis is not infrequent. We performed an extensive literature review in order to examine the risk of developing MF in psoriatic patients with a systematic search of the English-language databases. An increased risk for lymphoma overall in psoriatic patients has been found only by three out of seven studies. The risk of developing MF in psoriatic patients has been investigated by different studies in different populations and with different methodologies presenting bias and limitations, and it seems reasonable that misclassification between psoriasis and MF may explain the association reported. In contrast to the large number of psoriatic patients treated with biologicals, only 27 case reports of MF after biological therapy for psoriasis have been reported, and in 10 cases, the initial psoriasis diagnoses were then revised as MF. A true association between MF and psoriasis is possible, but the real incidence and prevalence are still unknown. The reported higher risk of developing MF in psoriatic patients should be reconsidered in the light of the bias of misclassification and the low magnitude reported in previous studies. There is not enough evidence to support a causal relation among biological therapies and MF in psoriatic patients.

Hymenoptera stings in the head region induce impressive, but not severe sting reactions.

Stings in the head region are considered to be a risk factor for severe systemic reactions to hymenoptera stings. We supposed that stings in skin areas, which are well supplied with blood, lead to more severe reactions and tested our hypothesis in 847 patients with confirmed hymenoptera venom allergy. However, symptom severity was independent from sting site: only 16.3% of patients with severe reactions were stung on the head (P = 0.017). But we confirmed age > 40 years (P < 0.001) as well as elevated basal tryptase levels (P = 0.001) as risk factors. Taking antihypertensive drugs seemed to have an influence: 41.7% of patients taking antihypertensive drugs experienced a severe reaction compared to 29.5% of patients, not taking such drugs (P = 0.019). However, considering patients' age in regression analysis, taking antihypertensive drugs had no effect on symptom severity (P = 0.342). Importantly, in most patients with severe reactions, cutaneous signs were absent (P < 0.001).

Hydrocarbon concentration and source appraisal in atmospheric particulate matter (PM2.5) of an urban tropical area.

Polycyclic aromatic (46 PAHs) and aliphatic hydrocarbons (C12-C40) were studied in atmospheric particulate matter sampled in Rio de Janeiro Metropolitan Area (RJMA). In total, 236 samples from six different sites were collected simultaneously and weekly over 1 year (January-December 2011) allowing a robust atmospheric characterization of fine particulate matter (PM2.5). Hydrocarbon concentration was in general low compared to previous studies in the area (PAHs range 0.25-19.3 ng m(-3)), possibly due to changes in fuel composition over time. Precipitation is the main meteorological parameter that rules particulate and hydrocarbon concentration, modifying PAH typology by scavenging. Aliphatic and aromatic diagnostic ratios gave indications conflicting with combustion features. However, ratios showed differences among sites. Principal component analysis (PCA) associated to multiple linear regressions (MLR) allowed quantitative estimate of sources and effectively indicated vehicular emission as the main hydrocarbon source in the atmospheric particulate matter.

Lucio Leprosy with Lucio's phenomenon, digital gangrene and anticardiolipin antibodies.

Lucio's phenomenon (LPh) is considered a necrotizing panvasculitis and a variant of leprosy Type 2 reaction, clinically characterised by necrotic-haemorrhagic lesions on the extremities and trunk. LPh is observed in diffuse lepromatous leprosy (DLL or Lucio-Latapí leprosy). This is a distinct form of lepromatous leprosy (LL) reported mainly in Mexico. Anti-phospholipid antibody syndrome (APS) has been rarely described in LPh. We report a case of Lucio-Latapí leprosy with LPh observed in a patient from the province of El Oro in Ecuador, who presented clinical manifestations of long standing DLL (non-nodular infiltration of the skin, collapse of the nasal pyramid, madarosis, atrophy of the earlobes), of LPh (necrotic-haemorrhagic macules with irregular shapes) and of APS (necrosis of the right big and second toe). Histopathology showed perineural and periadnexal foamy macrophages with numerous bacilli (diagnostic of LL) in the subcutis, a mild lobular panniculitis with a large subcutaneous vessel infiltrated by macrophages in the wall (typical of LPh) and vessels of the superficial and mid dermis occluded by thrombi but without signs of vasculitis (typical of occlusive vasculopathy as in APS). Our observations suggest that some cases of LPh may be associated with APS. Anti-cardiolipin antibodies (aCL) and lupus anticoagulant (LA) should be tested in patients with LPh because this may have therapeutic implications.

Analysis of clinical, dermoscopic and histopathological features of primary melanomas of patients with metastatic disease--a retrospective study at the Department of Dermatology, Medical University of Graz, 2000-2010.

BACKGROUND: Incidence rates of malignant melanoma have been increasing worldwide and metastatic melanoma is still a significant problem despite widespread prevention programmes. OBJECTIVES: We made a systemic review of all metastasized melanoma patients treated at the Department of Dermatology, Medical University of Graz in the years 2000-2010 and looked at the kind of melanoma type, e.g. if it has been slowly growing superficial spreading melanoma (SSM) or fast growing nodular melanoma (NM). METHODS: Histological slides and clinical images of patients treated at our department between 2000 and 2010, who received chemotherapy because of proven metastatic disease were analysed with regard to growth type of their primary tumours. RESULTS: A total of 88 patients met the inclusion criteria. Mean age of all patients was 57 years (median 59 years, SD ± 15 years). Of these 88 patients 51 patients (58%) (28 male patients and 23 female patients) had SSM; mean age 58 years (median 58 years, SD ± 14 years) and 37 patients (42%) (18 male patients and 19 female patients) had NM; mean age 56 years (median 61 years, SD ± 17 years). Mean Breslow thickness in the SSM group was 2.26 mm (median: 1.6 mm, SD ± 2.11 mm). In the NM group, mean Breslow thickness was 4.59 mm (median: 3.50 mm, SD ± 4.07 mm). When separated by gender, 46 melanomas were seen in the male group (28 SSM and 18 NM) and 42 melanomas in the female group (23 SSM and 19 NM). CONCLUSIONS: Our results showed that more than half of the patients with metastatic disease had SSMs and not, as suspected, NMs. As SSMs are growing over a longer period to become invasive and potentially metastatic, there might be a chance to focus primary and secondary prevention programmes not only on fast growing tumours but also on slowly changes of tumours.

Benign dermoscopic features in melanoma.

BACKGROUND: Various dermoscopic features are usually associated with benign melanocytic lesions. Our objective was to determine frequency and extension of benign dermoscopic features (BDF) in melanoma. METHODS: Retrospective review of dermoscopic images of a consecutive series of 516 histopathologically proven melanomas collected in 6 years in Graz. Correlation of BDF with mean Breslow thickness, with presence/absence of associated benign nevus component and with the pre-operative clinico-dermoscopic diagnosis, as reported on the original histopathologic reports. RESULTS: In addition to melanoma specific criteria, 42% of melanomas showed BDF. In 12.3% cases, the benign features occupied more than the half of the lesion. The BDF typical pigment network, homogeneous pattern and regular globules/cobblestone pattern had the highest frequency. BDF were associated with relatively thinner melanomas (mean Breslow thickness of 0.51 mm). The presence of BDF was observed in 67.1% of histopathologically documented nevus-associated melanoma and in 35.7% of melanoma de novo. A pre-operative clinico-dermoscopic diagnosis of melanoma was achieved in only 54.1% of cases displaying BDF. CONCLUSION: A significant proportion of melanomas may exhibit BDF. Clinicians should be aware of the presence of BDF in melanoma as possible diagnostic pitfall.

UV-induced alterations of the skin evaluated over time by reflectance confocal microscopy.

BACKGROUND: Ultraviolet radiation (UVR) induces various alterations of the skin and plays a decisive part regarding the development of melanoma and non-melanoma skin cancer. For a closer examination of these phenomena in vivo reflectance confocal microscopy (RCM) is one of the most eligible options as it represents a diagnostic tool that allows a non-invasive examination of the skin, showing microanatomical structures and individual cells. OBJECTIVES: The aim of this study was using RCM to observe alterations of the skin induced by UVR and to describe the development of these changes. In addition, the findings were compared with histological examinations of the same area. METHODS: A small area in the gluteal region of 10 healthy subjects was exposed to a threefold individual minimal erythema dose of solar-simulated UVR. The following development of the sunburn reaction was evaluated with RCM 1, 24, 72 h and 1 week after UVR exposure. Furthermore, RCM images of unexposed skin were obtained, serving as a reference. To contrast histological examination with RCM, punch biopsies were performed at each point in time. The obtained data were interpreted regarding histological and RCM-based criteria on sunburn reaction. RESULTS: All important UVR-induced alterations of the skin could be shown in RCM beginning with an inflammatory reaction (inflammatory cells, vasodilatation, oedema), containing the formation of microvesicles, followed by the appearance of apoptotic keratinocytes (sunburn cells), activated melanocytes and at last, loss of the epidermal structure. There was an excellent correlation between RCM and histological features. CONCLUSIONS: Reflectance confocal microscopy is a highly valuable tool for non-invasive monitoring of UVR-induced changes of the skin over time. Furthermore, RCM provides a more detailed visualization of inflammatory cell formation and epidermal blood flow than histological examination can.

Subacute lupus erythematosus during treatment with golimumab for seronegative rheumatoid arthritis.

We report on a 52-year-old woman with a history of severe seronegative rheumatoid arthritis. Several conventional therapies and biological therapy with etanercept and infliximab had been unsuccessful. In 2010 she was given golimumab subcutaneously at a monthly dose of 50 mg. She had a negative ANA titre. After 16 months of uninterrupted therapy and sustained response, she developed skin lesions on the upper trunk, back and upper extremities, which worsened on exposure to the sun. The skin biopsy was compatible with subacute lupus erythematosus. Laboratory findings included an ANA titre 1:640, negative anti-Ro/SSA and anti-DNA antibodies. Topical corticosteroid therapy proved inadequate. The patient's condition improved only after discontinuation of golimumab. The causal relationship between subacute cutaneous lupus erythematosus and golimumab is not dose-related and occurs with some delay (a typical feature of immunological adverse reactions). The association is likely, but not confirmed (because re-challenge was not performed). However, a clear improvement was noted after withdrawal. Based on this case, we hypothesized the aetiological role of golimumab-associated immunogenicity. TNF-α antagonist-induced lupus-like syndrome (TAILS) is a well-known side effect of this class of substances. The British Society of Rheumatology recommends discontinuation of the causal anti-TNF-α treatment in patients with TAILS.