Repositório RCAAP

Introduction: In multiple myeloma, the cutaneous lesions are rare and usually occur in late stages of MM as a reflection of increased tumour cell burden. Case report: The authors present the case of a 66 year-old woman with IgG lambda multiple myeloma that, after 16 months and apparently under control haematological, developed a tender and firm erythemato-violaceous plaque, on the back of the right thigh. A diagnosis of cutaneous plasmocytoma was confirmed by skin biopsy. Local radiotherapy induced a complete regression of skin lesion, but the patient died 4 months later, duo to uncontrolled multiple myeloma. Discussion: Secondary cutaneous plasmocytoma usually arise from direct spread from underlying bone, manifest as erythematous to violaceous infiltrated nodules or plaques, are extremely rare and indicate a poor prognosis, as in our case. The presentation as an erysipela-like lesion with no underlying bone disease is even rarer.KEYWORDS – Multiple myeloma; Plasmacytoma; Skin neoplasms/secondary.

Mycobacterium haemophilum skin infection is a rare disease with a difficult diagnosis and a challenging treatment. We report the case of a patient on chronic corticotherapy for myositis with nodules on the lower limbs in which Mycobacterium haemophilum was identified by PCR technique. This case emphasizes the need for a high index of suspicion for the diagnosis. In fact, this infection can currently be underdiagnosed due to the special requirements for culture. It should be considered in patients with chronic granulomatous processes in combination with negative my- cobacteriological examination.KEYWORDS – Mycobacterium haemophilum; Immunocompromised host; Mycobacterium infections; Skin diseases, bacterial.

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Introduction: Skin cancer of the feet require quick surgical management with the less as possible organic impact, especially in elderly patients with multiple comorbidities. The techniques of regional block anesthesia are relatively easy to perform and safe when performed rigorously. Discussion: The sensitive innervation of the feet depends on five nervous branches: posterior tibial nerve, deep peroneal nerve, superficial peroneal nerve, sural nerve and saphenous nerve. The authors discuss the anesthesia techniques and possible complications. Conclusions: The techniques of anesthesia of peripheral nerves of the feet can achieve success rates of 95% and optimize the resources available in dermatologic surgery.KEYWORDS – Nerve Block; Peripheral Nerves; Foot; Skin Diseases.

The systemic vasculitis are a group of diseases, uncommon in childhood, characterized by inflammation and necrosis. Its heterogeneous clinical presentation is conditioned by the type of vessel and organ affected. Recently it was proposed a classification of pediatric vasculitis, which takes into account the size of the vessels involved, and also validated diagnostic criteria for the types most common in children. This review aims to address the most frequent types of vasculitis in childhood, clinical manifestations, diagnosis and therapy.KEYWORDS – Vasculitis; Child.

The development of a melanocytic lesion at the site where it had been previously excised can place clini- cally and histologically some problems because of the differential diagnosis with melanoma, named by some authors as 'pseudomelanoma. In this study, the authors intend to carry out a comparative analysis between the clinical and the pathologic features of recurrent nevi and primary melanocytic lesions.KEYWORDS – Nevus, Pigmented; Melanoma.

Introduction: Tumors of skin appendages represent a heterogeneous group of benign and malignant neoplasms with different morphological differentiation. Material and Methods: A retrospective study of the skin appen- dages tumors diagnosed in the outpatient Department of Dermatology of the Centro Hospitalar do Porto, EPE-HSA from 2000 to 2009 was performed. Results: 404 tumors of skin appendages were diagnosed. Only 3% of were malignant. The majority showed a follicular differentiation (62.6%). Discussion: The review of the literature revealed a paucity of data for retrospective studies of tumors of skin appendages. Further studies are required for a better epidemiological characterization of these entities.KEYWORDS – Carcinoma, Skin Appendage; Skin Neoplasms.

Introduction: Malignant melanoma may present a great variety of histopathological patterns. Besides the classic forms of melanoma, there are a number of variants that have been described, such as polypoid, verrucous, desmoplastic, myxoid, chondroid, balloon-cell, rhabdoid, animal-type, amelanotic, spitzoid, nevoid. The aim of this study was to characterize the rare histopathologic variants of malignant melanoma observed in a Dermatopathology Laboratory in a period of 15 years (1995-2009). Material and Methods: Analized data included: patient age and sex, clinical diagnosis, melanoma location, Breslow and Clark level, presence of ulceration and follow-up. These variants were grouped according to architectural, cytologic and/or stromal changes. Results: Eighty-seven rare histopathologic variants of melanoma were observed, corresponding to 6,5% of all melanomas. We have found predominance of females in spitzoid and of males in animal-type melanoma. There were some preferential locations: face in animal type, trunk in polypoid, limbs in verrucous, and subungual in chondroid melanoma. We identified ulceration in 73% of poly- poid, in 60% of verrucous and 50% of amelanotic melanomas. A higher mortality rate was documented in mixed variant (polypoid/animal-type), desmoplastic, polypoid and animal-type.Conclusions: The recognition of these variants is important, not only by the clinical and histopathological challenge in the differential diagnosis with other skin tumors, but also because of the possible implication of some of these variants, with peculiar biological behavior, in the prognosis.KEYWORDS – Melanoma; Skin Neoplasms.

Introduction: Genital aphtosis is the most frequent cause of non-infectious vulvar ulceration, usually associated to oral aphtosis and it is always an exclusion diagnosis. We present three cases of genital ulcer in female adolescents, observed in our Dermatology clinic. Case 1: A 17-year-old-girl, with past history of oral aphtosis, was referred to our clinic with a genital ulcer, fever, asthenia and myalgia. Case 2: A 13-year-old-girl was observed in our emergency unit with a painful genital ulceration and significant edema of the labia minora. Case 3: A 14-years-old-girl was observed in our clinic with a painful genital ulcer, fever and sore throat, without oral aphthosis. In all three cases, sexually transmitted infections, a possible drug reaction and ophthalmologic compromise were excluded. The immunological study and pathergy test were both negative. So far, none of the patients met the criteria of Behçet disease and all of them responded to systemic steroid therapy. Comments: Nonsexually related acute genital ulcers may arise in patients with Crohn or Behçet disease, "reactive non- sexually related acute genital ulcers "- a subset of complex aphtosis, appears to occur in response to an acute illness rather than in a chronic systemic disease. These cases report the challenging differential diagnosis of acute genital ulcers in young patients, without previous sexual life.KEYWORDS – Adolescent; Genital Diseases, Female; Ulcer.

Porokeratosis are the result of a clonal proliferation of keratinocytes. About six clinical variants were des- cribed to date, sharing the histological feature of cornoid lamella in the stratum corneum. The authors report a porokeratosis of Mibelli in a 27-year-old black female appearing in childhood successfully treated with a topical retinoid. Common manifestations of a rare dermatosis are presented with emphasis to the role of histo- pathology in the diagnosis. Porokeratosis of Mibelli is a chronic and progressive dermatosis, rarely with spontaneous remission. Malignant degeneration has been described with a reported incidence of 7%, with highest malignant trans- formation into squamous cell carcinoma, therefore the importance of regular skin checks.KEYWORDS – Porokeratosis; Keratinocytes.

Introduction: Pyoderma gangrenosum (PG) is an idiopathic, recurrent, neutrophilic and ulcerative skin disease. Case report: A 43-year-old man with chronic hepatitis C and drugs addiction in the past presented with a 6-months history of painful, violaceus, suppurative and necrotic ulcers on his lower extremities and any skin area inju- red by needle stick. PG was the main diagnosis proposed. Histological examination of the lesion edge demonstrated microscopic changes compatible with PG. Laboratory and radiological evaluation findings were compatible with his chronic liver disease. The patient was treated, in different moments, with systemic steroids, cyclosporine, dapsone, and thalidomide, with no satisfactory response. A complete remission was achieved with etanercept. Twelve months later, there were no signals of skin lesions, relevant hepatic damages or increase in viral load. Conclusion: Etanercept was a safe and effective therapeutic option.KEYWORDS – Pyoderma Gangrenosum; Hepatitis C, Chronic; Thalidomide; Glucocorticoids; Receptors, Tumor Necro- sis Factor; Immunoglobulin G.

We here report a case of a severe generalized allergic contact dermatitis from contact with the surface of a water bed which happened to be contaminated with the water inside that had been treated with the biocide mixture of methylchloro- and methylisothiazolinone.KEYWORDS – Dermatitis, Allergic Contact; Thiazoles.

Fungal infections represent an important complication for immunosupressed patients and are associated with high morbidity and mortality. Fusarium infections have been reported with increasing frequency and can be locali- zed, locally invasive or disseminated, depending on the immune status of the host. The authors report the case of a four year old boy, with acute lymphocytic leukemia, in chemotherapy, who presented multiple erythematous nodules that evolved into bullae and central necrosis. Cultures of bullae fluid grew Fusarium spp. Despite treatment with intravenous lipid formulation of amphotericin B and voriconazole, the child died 10 days after appearance of skin lesions.KEYWORDS – Fusarium; Fusariosis; Immunocompromised Host; Leukemia, Lymphoid.

Tinea capitis, a dermatophytic infection of the scalp, is rare in adults, even in geographic areas with high prevalence rates. The source of the infection is usually a straight contact with cohabitants, and in adults it is mainly, but not always, associated with immunosuppression. A careful anamnesis and a complete physical examination are crucial to establish tinea capitis as a clinical diagnostic hypothesis in order to promptly collect samples for mycological studies, avoiding unnecessary additional studies or incorrect prescriptions. The authors report three cases of tinea capitis in adults – two cases caused by Microsporum audouinii and the other one caused by Trichophyton soudanense. The cases are iconographically depicted and a brief review of the relevant literature is presented.KEYWORDS – Adulto;Tinea Capitis; Microsporum; Trichophyton; Mycological Typing Techniques.

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In modern societies, as beauty and youth are paired with success, patients driven by aesthetic concerns involving skin and body imperfections are raisingly common in the daily practice of Dermatology. Body Dismorphic Disorder (BDD), also known as dismorphophobia, is a frequent psychiatric condition characterized by an excessive dis- tress from minimal or inexistent physical defects, resulting in severe emotional, social and professional disabilities and compulsive behaviors aimed to correct or hide these defects (excessive acquisition of hats or scarves, abusive and un- satisfactory application of makeup products, obsessive verification of the mirror image, skin picking, and consecutive requests of cosmetic surgical procedures). Being unaware of the true nature of their condition, BDD patients commonly seek for Dermatology, Plastic Surgery or Dentistry clinics. In fact, the prevalence of BDD within dermatological patients is estimated at 2,9-29,4%, being significantly higher than in general population (1-2%). Considering that cosmetic procedures are rarely beneficial in this setting, dermatologists should be aware of this frequent condition and be able to identify BDD patients, as referral to psychiatric care is of extreme importance.KEYWORDS – Body Dysmorphic Disorders; Body Image; Plastic Surgery.

It is estimated that 2 billion people worldwide have been infected with the hepatitis B virus (HBV) and over 350 million are chronic carriers. Patients who present antibodies to hepatitis B core antigen (anti-HBc) with con- current HBsAg negativity do not have chronic hepatitis, but only experienced HBV infection and were able to clear it. Nevertheless, some of these patients may be occult carriers, in whom intrahepatic HBV replication can be detected. HBV reactivation is a commonly reported complication in patients undergoing chemotherapy for malignancies and in patients after bone marrow transplantation. Reactivation largely occurs in patients with chronic hepatitis B who are positive for HBsAg, but it can also affect previously infected patients who have apparently cleared the virus. The HBV reactivation risk in patients with chronic hepatitis B (HBsAg +) undergoing anti-TNF-α therapy is also well established, however, information regarding the use of these drugs in patients Ac anti-HBc + / HBsAg- is more scarce. Recently, it has been reported a 5% HBV reactivation rate in patients Ac anti-HBc + / HBsAg- treated with anti-TNF-α agents, showing that this risk is real. Since 2010, all patients of the Department of Dermatology at the Centro Hospitalar do Porto – Hospital de Santo António (CHP-HSA) that initiate biological treatment for psoriasis / psoriatic arthritis and are positive for anti-HBc are also consulted in the Hepatology Clinic in order to establish the existence of chronic liver disease, and if there is an indication for early anti-viral prophylactic therapy. The authors aim to highlight the need for mandatory screening of HBV in all patients who will initiate biological therapy with TNF-α inhibitors, relatively consensual in the scientific community, but especially the need for vigilance and close monitoring of patients with potential occult HBV infection due to the possible risk of HBV reactivation.KEYWORDS – Hepatitis B; Hepatitis B Surface Antigens; Tumor Necrosis Factor-alpha; Hepatitis B Core Antigens.

Introduction: The authors describe the process of translation, cultural adaption and validation of the instrument for measuring quality of life of patients with psoriasis PDI (Psoriasis Disability Index). Methods: This questionnaire comprises 15 questions concerning the last four weeks, divided into five chapters; daily activities, work or school, personal relationships, leisure activities and treatment. The questionnaire was translated according to the standards for cross-cultural validation of health outcomes instruments. Results: In a sample of 171 patients aged between 16 and 82 years, 61,9% were male. Content validity was assured by a cognitive debriefing and the reliability was verified by a reproducibility test (r=0,888) and by internal consistency (α=0,857). Criterion validity was tested by comparing PDI results with those obtai- ned by PASI, and there has been a positive correlation (p<0,05) in all dimensions of the questionnaire. Conclusions: We can defend the performance quality of the Portuguese version of the PDI. The study of psychometric characteristics of PDI allows the use of this instrument for the Portuguese population, either in research or in clinical practice. There remains, however, the need to obtain values of clinical significance and national reference standards.KEYWORDS – Quality of life; Psoriasis; Questionnaires.