Repositório RCAAP

Pachydermodactylyis is a rare form of fibromatosis affecting the lateral aspects of the first phalanges of the hands. Although idiopathic by definition, frequently a repetitive mechanical trauma is identifiable as its cause. The authors describe two case reports of this condition.

The Melkersson-Rosenthal Syndrome is composed of the Triad: linqua plicata, intermittent facial palsy and orofacial swelling. Usually, the dominant and earliest finding of the syndrome is the orofacial swelling. Therefore, it’s frequently the dermatologist the first professional to have contact with the patient. Yet, the condition has been few times described in dermatological literature. We present a classical case of the complete triad.

Due to the development of large areas of permanent alopecia, patients with lichen planopilaris are more likely to evolve with psychosocial disorders and lower quality of life. Lichen planopilaris (LPP) is characterized by an autoimmune response against antigens of the hair follicle. It is known that can coexist with systemic autoimmune diseases, although this relationship is not well understood. Early recognition and prompt therapy are to protect the base of the hair follicle and fibrosis can stop the progression of hair loss. We present a therapy used with excellent results in a case of lichen planus pillar overlap that occurred in dermatomyositis and scleroderma.

Pyoderma gangrenosum (PG) and hidradenitis suppurativa (HS) are inflammatory diseases of unknown aetiology, which association is rare but well established. We describe the case of a 27-year-old female patient, with axillary and inguinal hidradenitis supurativa lesions since four years ago who developed a pyoderma gangrenosum lesion in her right leg. Treatment was instituted with systemic corticosteroids in inpatient setting. Afterwards, clindamycin and rifampicin were chosen followed by low dose isotretinoin with good response. The association of HS and PG can possibly have a common aetiology related to neutrophilic activity dysfunction. The description autossomic dominant syndrome PAPA (PG, acne and pyogenic arthritis) as well as PASH (PG, acne and HS), dermatos included in the spectrum of autoinflammatory diseases, promise a better comprehension of aetiopathogenesis of this association with possible diagnostic and therapeutic improvements.

Pseudotumor cerebri is a syndrome of intracranial hypertension in the absence of enlargement of the ventricles, mass lesions or other structural cause. Drugs commonly used in clinical practice of dermatology are associated with the development of pseudotumor cerebri, including retinoids, corticosteroids and tetracycline. This amendment should be suspected in any patient who develops symptoms of intracranial hypertension while in use of such medications. Therefore, early recognition of the clinical picture is of great importance and allows the immediate suspension of the drug, which is the main conduit in these cases. One case of pseudotumor cerebri associated with the use of isotretinoin, with rapid identification and appropriate treatment of the frame is displayed.

Abacavir hypersensitivity is a potentially fatal systemic adverse reaction that occurs in 2-9% of exposed patients. Symptoms appear during the first 6 weeks of therapy and are similar to DRESS although usually there is no eosinophilia. Studies have confirmed a positive relation with HLA-B*5701, particularly when only cases confirmed by a positive epicutaneous patch test were considered. This HLA allele is now considered an excellent predictor of abacavir hypersensitivity reaction (55% positive predictive value) and it is mandatory to perform a genetic screening before initiating any HIV treatment with abacavir. We report a case of abacavir hypersensitivity reaction, reminding its clinical resemblance with DRESS and pointing out the differences between these two entities, as well as highlighting the importance of HLA phenotyping before beginning treatment with abacavir and the utility of the patch test in confirming this immunologic reaction and studying its effector mechanisms.

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Chronic pruritus is a symptom that has a significant impact on the patient’s quality of life. It can be associated with a wide broad of diseases and in most of the times is difficult to treat. The search for the underlying etiology of pruritus usually requires a meticulous history taking as well as a clinical examination and laboratory investigations. Management of pruritus is often a challenge for the dermatologist and can be achieved by implementing several therapeutic approaches. This review highlights the clinical approach and the treatment of chronic pruritus.

Introduction: Kaposi's sarcoma (KS) is a systemic disease, whose neoplastic or reactive nature is controversial and its etiologic agent is human herpes virus type 8 (HHV-8). There are 4 clinical subtypes: classical, epidemic, endemic and iatrogenic.Material and methods: A retrospective review of clinical records and histological preparations of KS cases, diagnosed between January 2001 and December 2013 at the Dermatopathology Department of Hospital Egas Moniz and Centro de Dermatologia Médico-Cirúrgica de Lisboa was made and we assessed differences between the various types.Results: Ninety-one cases were diagnosed, 67.0% of classic KS, 30.8% of epidemic KS; 1.1% of endemic KS and 1.1% of iatrogenic KS. It was found that 85.7% of patients were male and 14.3% were female, and this dominance occurred in all variants. The average age of patients with epidemic KS was lower than the classic variant (37.3 vs 70.0 years, p< 0.001). The lower limbs were the preferred location for all variants (74.2%). Histologically, in patients with classic KS, there was a predominance of the nodule stage (62.3%), while in epidemic KS, plaque was the most common stage (46.4 %) and this difference being almost statistically significant ( p = 0.057). No differences were observed in the various histological stages between classic and epidemic SK.Conclusions: The observed differences in epidemic KS seem to relate more with subtle changes inherent to early lesions rather than only differences of this variant , which is in agreement with the literature.

Introduction: Actinic keratosis is the most common premalignant lesion. The therapeutic approach to patients with multiple lesions involves field-directed treatment.Objective: To evaluate the efficacy and safety of ingenol mebutato, a new drug treatment for topical field-directed treatment of actinic keratosis.Design: Systematic review and meta-analysis of randomized controlled trials (RCTs) trials.Data sources: Medline and Cochrane Library (June 2014).Study selection: Two reviewers independently searched for studies and retrieved their characteristics and data estimates.Data synthesis: Random-effects meta-analysis. Heterogeneity was assessed with the I2 test.Results: Six trials (n = 1,492) versus placebo were included. The odds of a patient experiencing complete removal of the lesions was 17 (95%CI: 9 to 31, I2 = 0%) and 8.5 (95% CI: 5 to 15, I2 = 0%) times higher, compared to placebo, in the face/scalp and trunk/extremities, respectively. The incidence of adverse events related to treatment was higher in the group of mebutato ingenol (+23%, 95%CI: 11 to 35%), with no differences between groups in the discontinuation rate due to adverse events.Conclusions: Mebutato of ingenol is efficacious in the treatment of actinic keratosis. Compared to other field-directed treatments available in Portugal, its therapeutic value comes from the favourable safety profile and tolerability, simplicity and short duration of the treatment regimen and the possibility of different treatment to injuries according to anatomical location (individualization of therapy). Future studies should directly compare the different therapeutic options and evaluate the effectiveness of the same in the real world.

Background: Lupus Erythematosus (LE) represents a multiorganic, autoimmune disease that affects mainly women of child-bearing age. The skin is the second most frequently affected organ, after the joints, and may precede the clinical onset of systemic symptoms.Objectives: To present the clinical findings which evoke the possibility of LE-specific skin lesions and the histopathological findings that may co-substantiate the diagnosis.Methods: Case studies of LE-specific skin lesions, in which the clinical-pathological correlation enabled the diagnosis of different subtypes of LE.Results: The correlation of clinical and histopathological findings, in a consistent serological setting, enabled the recognition of actual cases of LE-specific skin lesions: 1) acute; 2) subacute (annular and papulosquamous forms); 2.1) drug-induced subacute LE; 3) chronic, namely 3.1) classic discoid (face and scalp); 3.2) tumidus; 3.3) panniculitis.Moreover, the clinical and histopathological findings expected in other LE-specific skin lesions are described.Conclusions: Lupus Erythematosus exhibits a variability of cutaneous manifestations. The histopathology is critical for the diagnosis requiring a correct preservation and handling of biopsy material. Direct immunofluorescence remains useful to improve the diagnostic specificity. The accurate diagnosis, based in a systematic clinical-pathological correlation and integrated with the clinical and serological status, is crucial for the prognosis and treatment of the patients.

Acne vulgaris is a common disease in society, particularly affecting teenagers and young adults. Acne often results in secondary damage in the form of scarring that can generate negative psychological effects. Currently there are several treatments for the different types of scars leading to a complex therapeutic challenge. For these reasons the article seeks, through a literature review, describing one of the classifications for different types of scars from acne vulgaris with their best treatments. The research serves as an aid to qualified professionals for such cosmetic procedures, developing a plan of treatment for scars, facilitating the proper operation thereof.

The nasolabial transposition flap, when performed with a superiorly placed pedicle, has multiple indications in mid-face surgical reconstruction. Apart from its primary indication in nasal ala reconstruction, the nasolabial transposition flap is an alternative for all the nasal subunits, except for the upper third ones. The nasal tip and full-thickness defects of the nasal ala are not exceptions. Moreover, it can also be employed in infraorbitary surgical defects. It is a extremely safe an easy-to-perform flap, with a low incidence of complications, as long as some basic principles are observed.

The V-Y advancement flap is perfectly adaptable to the nasolabial fold and is extremely useful in that location. Apart from its classic application as vertical advancement flap, multiple variants have been described. The authors describe two case reports with two different applications of this flap, which represent two different degrees of technical complexity. In the first case, a 64-year-old male has a left paranasal basal cell carcinoma that was excised, resulting in a 2 cm width surgical defect. The second case mentions a 61-year-old male with a basal cell carcinoma of the nasal ala, which excision resulted in a defect affecting the full-thickness of part of the nasal ala and the perialar portion of the nasolabial fold.The details of the surgical reconstructions by means of a nasolabial fold- centered V-Y advancement flap, in the first case, and a turnover advancement flap centered in the nasolabial fold, in the second case, will be discussed.

Microstomia is the medical term to define the reduction of the oral aperture. It is a common complication after lip tumors excision. It can be severe enough to compromise cosmesis, nutrition, and quality of life. Microstomia correction is a challenge, with many reconstructive techniques described in the literature. A 83-year-old male patient is observed with a verrucous lesion that occupied the left inferior half of the lower lip. Biopsy revealed a squamous cell carcinoma. He was submitted to a radical excision but developed an iatrogenic microstomia, with an important compromise of the nutrition. Microstomia was corrected using comissurotomy and orbicularis oris muscle repositioning. Vermillion was reconstructed with 2 romboidal mucosal flaps.

The herpes simplex virus is a common cause of genital ulcers. In the immunocompromised, it may have an atypical presentation, with exuberant lesions, frequently resulting in resistance to the mainstream therapeutic options, and a consequent prolonged course.A 25-year-old male with a previous history of common variable immunodeficiency, presented with herpetic extensive genital ulcerations on the inguinoscrotal region. Despite a long period of valacyclovir therapy, the patient remained symptomatic. We decided to add topical 5% imiquimod as an adjunctive therapy, and observed a completed resolution of the ulcerations.Imiquimod, working as a local immunomodulator, stimulates the anti-viral immunologic response. Therefore, it is an alternative in recalcitrant herpetic lesions, especially in the setting of immunosuppression.

Introduction: Allergic contact dermatitis to mercaptobenzothiazole and its derivatives has been increasingly recognized in industrialized countries because of their widespread use in polymerization of synthetic rubber. The authors describe two clinical cases with relevant occupational exposure.Case reports: A 46 year-old male patient, with an eight month pruritic dermatitis, limited to the wrists, refractory to high potency topical corticosteroid and oral antihistamine. Physical examination showed erythematous-violaceous infiltrated plaques and excoriated erythematous papules on both wrists. Prick tests and RAST to latex were negative. Epicutaneous tests were performed with basic series from Portuguese Contact Dermatitis Group, rubber and glue series and fragments of the sleeve and it was found positivity for mercaptobenzothiazole (++), mercapto mix (++) and elastic band of gloves in reading at 72h and at 7 days. A 61 years old non atopic man, woodworker, with a 12- month history of itching, erythema, infiltration of genius region of face and extension to posterior cervical region.Epicutaneous tests were performed with basic series from Portuguese Contact Dermatitis Group, rubber and glue series, cosmetics series and fragments of the protection mask work and it was found positivity for mercaptobenzothiazole (++), mercapto mix (++) and elastic band of mask (++) in reading at 72h and at 7 days.Discussion: Despite being well known allergens, mercaptobenzothiazole and its derivatives may be present in other sources of exposure to rubber like elastic fabric in clothing. Once the worker sensitized, it can be an important cause of disability in an occupational and personnal setting.