Repositório RCAAP

Allergic contact dermatitis to plants can assume different clinical patterns. We report a 63-year-old female patient with chronic hand dermatitis. Given the clinical suspicion of allergic contact dermatitis, patch tests were performed with the Baseline series of the GPEDC and a plants series which showed positivity to sesquiterpene lactone mix 0.1% pet (++) and parthenolide 0.1% pet (++) at day D2 and D4. These test results allowed the diagnosis of contact allergy to parthenolide that was related to occupational exposure Chamaemelum fuscatum during gardening. We emphasize the importance of a meticulous clinical history to increase the level of suspicion of Allergic contact dermatitis and evaluate the relevance of a positive patch test to sesquiterpene lactone and parthenolide as the etiology in chronic hand dermatitis.

Henoch-Schönlein purpura is a small vessel vasculitis mediated by the deposition of IgA immune complexes. It is characterized by palpable purpura, arthralgia / arthritis, abdominal and renal involvement. Its etiology remains unknown, but various stimuli have been proposed to trigger this pathology, namely infectious agents, drugs and tumor antigens. There is also a possible association with malignancy and auto-immune diseases. Henoch-Schönlein purpura occurs mostly in children and is rare in adults. In the pediatric population the disease is generally self-limited, whereas in adults it is associated with a worse prognosis and a greater number of complications. This article reviews the main aspects of adult Henoch-Schönlein purpura, highlighting its particularities and specificities.

Eosinophilic granulomatosis with polyangiitis is a systemic vasculitis that affects small to medium vessels, belonging to the spectrum of antineutrophil cytoplasm antibody-associated vasculitis. It is a rare disease that presents with vasculitis, asthma and other eosinophilic symptoms. We present the case of a 67-year-old male, with a palpable purpura affecting the superior and inferior limbs, and abdomen, for the past month. The patient complained of chronic cough for the past year, which aggravated in the last month. Complementary studies allowed the diagnosis of eosinophilic granulomatosis with polyangiitis and the patient was treated with prednisolone until remission followed by azathioprine as a maintenance therapy. After one year of follow-up no recurrence was seen.

Lichen aureus is a subtype of a chronic benign pigmented purpuric dermatosis of unknown etiology. It shows predilection for lower limbs and generally affects young adults and, less often, children. We report a case of lichen aureus with a unilateral presentation in a child.

Introduction: Pagetoid reticulosis is a rare variant of mycosis fungoides. The typical lesion is a solitary, slow-growing, well circumscribed and hyperkeratotic plaque or patch. A widespread form was also described, but, according to the last classification, the term is reserved for the localized variant (classically, "Woringer-Kolopp").Case report: We observed a 58-year-old patient, with a solitary keratotic plaque, 15mm x 18mm, on the plantar surface of the left halux. The lesion had been present for years, recently with local pain. The biopsy showed an infiltrate of small atypical lymphocytes with pagetoid invasion of the epidermis. On immunohistochemistry, a predominance of CD4+ T cells was observed. The results confirmed the diagnosis of pagetoid reticulosis. Two months after 0.05% clobetasol propionate applied daily, significant improvement was observed.Conclusions: Although pagetoid reticulosis is considered a variant of mycosis fungoides, it shows characteristic clinical and histopathological features, and a heterogenous immunophenotype. Extracutaneous involvement has not been reported.

Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis with distinctive semiology and histopathology, but that is not free of controversy regarding its individualization as a nosological entity. For some authors, the presence of exclusively telangiectatic macules, along with superficial vascular plexus dilation and low density of mast cell infiltrates in the upper dermis is enough for diagnosis of TMEP. However, some current classifications do not consider this entity separately from other types of cutaneous mastocytosis, because in many cases with telangiectatic lesions, lesions of urticaria pigmentosa can also be observed. We sought to present a case of cutaneous mastocytosis first appearing in the eighth decade of life, with the characteristic clinical, dermoscopic and histopathological findings of TMEP and with no mast cell activation symptoms other than pruritus. However, the complementary workup revealed concurrently the presence of indolent systemic mastocytosis. We additionally revised the most recent evidence favouring the recognition and individualization of this entity.

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Phototoxic and photoallergic reactions consist of sun-induced skin reactions in the presence of photoactive chemicals, applied directly on the skin or of systemic use. They have several distinct clinical presentations, including photocontact urticaria, eczema on sun exposed areas sometimes with erythema multiforme, exaggerated sunburn, phytophotodermatitis, pseudoporphyria, photo-onycholysis, dyschromia and lupus erythematosus. Furthermore, there is mounting evidence that exposure to photoactive chemicals contributes to cutaneous carcinogenesis. Besides their clinical heterogeneity, there is also a great diversity of causative agents, with striking geographical and temporal variations. The authors review the spectrum of clinical patterns and also major photosensitizers, including UV- -filters of sunscreens and cosmetics, vegetal furocoumarins and drugs (NSAIDs, antimicrobials, phenothiazines, amiodarone, among others).

Mastocytosis comprises a group of rare disorders with diverse clinical presentations, characterized by an abnormal increase number of mast cells in various organs. All patients with mastocytosis are at risk for anaphylaxis due to excessive mast cell mediator release that can occur during anesthesia, administration of radiographic contrast media or after hymenoptera stings. Beside the symptomatic treatment of acute and chronic mast cell-mediator release, and the reduction of the local or systemic mast cell burden, a rational management approach in mastocytosis must include a careful counseling of patients (parents in pediatric cases) and care providers, and avoidance of factors triggering acute mediator release. This protocol forms a pratical guideline for the management of patients with mastocytosis. It includes global preventive measures and measures in specific contexts: local and general anesthesia, administration of radiographic contrast media, and hymenoptera stings. As there are many anaphylaxis triggers in patients with mastocytosis, it is important to have a protocol with guidelines for physicians who are involved in these patients care.

Introduction: The majority of melanomas appear to arise de novo, however in about one third of the cases it can arise in association with a preexisting nevus. The histopathological diagnosis in these situations can be difficult, particularly in the separation of the two cellular populations and, as a consequence, establishing the tumoral thickness. Immunohistochemical staining can help in this distinction.Objectives: To evaluate the immunohistochemical staining patterns of HMB-45 and Ki-67 in selected cases of malignant melanoma associated with melanocytic nevi.Methods: Immunoperoxidase stains for HMB-45 and Ki-67 were done on 5 dermal melanocytic nevi, 5 compound melanocytic nevi, 5 invasive melanomas and 16 melanomas arising in a nevus.Results: The dermal melanocytes of nevi were consistently negative for HMB-45 and Ki-67. The melanomas showed diffuse moderate to intense staining for HMB-45 and Ki-65. In melanomas associated with nevus the HMB-45 was consistently positive in the melanoma component and negative in the nevus, allowing the separation of the two cellular populations. We find a considerable variation in the Ki-67 staining in this group.Conclusion: Although the histopathological examination with hematoxilin and eosin remains the main component in the diagnosis of melanocytic lesions; the immunohistochemical staining with HMB-45 and Ki-67 can be helpful in the diagnosis of melanoma associated with nevi.

Psoriasis is a chronic inflammatory disorder, multisystem and immunologically mediated. Approximately one third of adults refer the age of onset before 16, representing about 4% of all dermatoses seen in pediatric age. The majority of cases are mild and adequately managed with topical medications. A minor subset of children present with severe, rapidly evolving disease that requires systemic therapy. The use of these therapies is limited by the risk of toxicity, the low acceptance and lack of studies to validate and guide the use of most of these drugs in children. This is the second of two articles which aim to review the topical (Part I) and systemic (PartII2) treatment options available, as well as its safety and efficacy in children.

Introduction: Chlamydia trachomatis genital infection (Ct) is now the most common sexually transmitted bacterial infection (STI) diagnosed in United Kingdom and in United States and its prevalence is highest in persons younger than 25 years. Asymptomatic infection is also common among both men and women.Objectives: To assess epidemiological characteristics and prevalence of Ct in a population of patients attending the STI Clinic of Centro de Saúde da Lapa, Lisbon. Methods: Every patient attending this STI Clinic in the first six months of 2010 and tested to Ct (urine, urethral or vaginal swab) was included in our study. Data was analyzed with an exact significance level of 5%.Results: A total of 968 patients were included (643 men and 326 women). Ct was diagnosed in 56 patients, of whom 75% were asymptomatic. The diagnosis of CT was significantly associated to being heterosexual (p=0,026). For women significant association was also found between Ct diagnosis and the presence of genital warts or having more than one sexual partner during the last six months.Comments: These results were similar to other European studies. Although the Ct screening is recommended by STI guidelines (CDC/ IUSTI), in Portugal it is not routinely performed outside the few existing STI clinics. Knowing the serious consequences of untreated disease, implementation of regular screening in our country should be considered.

Reactive arthritis, previously known as Reiter’s Syndrome, is the classic triad of arthritis, urethritis and conjunctivitis, which occurs after an acute episode of genito-urinary or gastrointestinal infection. In adults, the most frequent cause is infection by Chlamydia trahomatis. It may also present as an incomplete triad or be associated with dermatological or visceral symptoms. It is classified as a seronegative spondyloarthropathy, and is strongly associated with HLA-B27. Reactive arthritis may occur as an acute disease or progress to a chronic form, and treatment depends on the eliciting infection.

The reconstruction of surgical defects of eyelids is challenging due to the anatomic complexity of eyelids allied with its aesthetic and functional significance. Since skin cancer is frequent on this region, the dermatologic surgeon have to be aware of the anatomical particularities of eyelids and the reconstructive options available in order to achieve the better aesthetic and functional results. The authors review the surgical anatomy of eyelids, the local anesthesia techniques and the main reconstructive options according to the type and localization of the surgical defect.

Nasal reconstruction after the excision of a malignancy can be accomplished in several ways. Treatment options include full-thickness skin grafts, various loco-regional flaps and the paramedian forehead flap. It is always a challenge for the surgeon, mainly because of the complexity of the nasal surface lines and the diversity of the colour, texture and appearance of its lining skin. The principles of an ideal nasal reconstruction must accomplish an adequate balance between surgery with curative intention, functional preservation and acceptable cosmetic result. Herein the authors describe, with an illustrative case report, a bilateral triangular cheek advancement flap, complemented with a full thickness skin graft, for the reconstruction of medium-large defects of the nose, in a single staged procedure.

Squamous cell carcinoma is the most common malignant neoplasm of the lips, and in about 90% of cases it’s located in the lower lip due to the higher cumulative exposure to ultraviolet radiation. The authors present two surgical techniques for reconstruction of large lower lip defects, resulting from tumor surgical excision, exemplifying and comparing them with two clinical cases.

Erythropoietic protoporphyria (EPP) is a rare inherited disorder of heme biosynthesis caused in most patients by a deficient activity of the enzyme ferrochelatase (FECH), with a consequent accumulation of protoporphyrin (PP) in various tissues. We report a 16-year-old male (index case) with EPP characterized by acute episodes of painful photosensitivity in the photoexposed areas since his early infancy, permanent changes, microcytic anemia, thrombocytopenia and mild hepatic dysfunction. His 18-year-old sister presented slighter acute symptoms and had no chronic changes. Lesional biopsy performed in index case disclosed perivascular deposition of PAS positive hyaline material. Rimington-Cripps test was positive in both cases and PP erythrocyte levels were markedly elevated, but normal in their asymptomatic parents and younger brother. Genetic study in both patients and their mother (father’s sample not available) revealed heterozygosity for a novel mutation (c.1052delA) in FECH gene of both children, and heterozygosity for the hypomorphic allele IVS3-48T>C in all of them. This report confirms the “pseudodominant” inheritance pattern observed in most studied families, explained by the combined presence of a disabling FECH mutation and a common intronic polymorphism affecting the counterpart allele (IVS3-48T>C). The mutant allele was probably inherited from the father. This is the first description of a Portuguese family with EPP characterized at the molecular level.

Nail-Patella Syndrome (OMIN 161200) (Hereditary Onychodysplasia), first reported by Chatelain in 1820, is a rare polymalformative syndrome that affects tissues of both ecto and mesodermal origin. It is an inherited disorder, transmitted as an autosomal dominant trait, having the responsible gene been mapped to 9q34 and further characterized as encoding for a LIM-homeodomain protein that plays a pivotal role in the dorso-ventral patterning of tissues of the developing vertebrate limbs. Clinically, NPS classic features include hypoplastic or absent nails and patellae, joint abnormalities, iliac horns, pigmentary anomalies of the irides and nephropathy. Nail anomalies, usually present at birth, are highly evocative – hypoplastic or even absent nails on the first or second fingers and triangular-shaped lunulae – and allow, upon recognition, to prevent a delayed diagnosis and, as such, joint crippling deformities and renal failure that eventually develop, as patients age. The case of a 4 YO Caucasian boy bearing unstable gait, absent patellae, a PDD (Pervasive Developmental Disorder) meeting some Asperger`s Syndrome criteria and fingernail anomalies is reported. In this case it is noteworthy the sharp contrast between the extent of the dermatological findings and the absence or scarcity of both renal and orthopaedic alterations. In fact, not only no renal and hearing alterations were detected but, apart from the bilaterally absent patellae, a 5th finger clinodactily and a mild iris dyschromia, no further anomalies were found in this underdeveloped child, whose weight and height were in the 10th centile.

We report a case of herpes zoster in a 10-month old male infant. This otherwise healthy boy had no prior history of varicella and parents denied any exposure to varicella-zoster virus (VZV) during pregnancy. A household exposure to varicella occurred at 1 month of age. A diagnosis of herpes zoster was established on clinical grounds and confirmed by serological assays. He was treated with oral acyclovir, with complete resolution of lesions without sequelae. Clinical and laboratorial evaluation did not uncover any underlying illness. In this setting, we discuss some peculiar aspects of VZV infection in this age group and review current recommendations for the management of herpes zoster in pediatric patients.

We present the cases of two young females with severe atopic dermatitis, treated with anti-histamines, topical corticosteroids, high-dose systemic corticosteroids bursts and/or oral cyclosporine, without significant improvement and worsening with tapering of corticosteroids and/or cyclosporine. Several therapeutical adjustments were made without success before starting treatment with subcutaneous omalizumab. The clinical evolution of these patients undergoing treatment with omalizumab is described and its role in the treatment of atopic dermatitis is discussed.