RCAAP Repository

The cytomegalovirus infection is prevalent in developed countries. The natural history of disease includes three phases: primoinfection (majority is asymptomatic), latency and reactivation. In certain immunosuppressive states, as in the case of transplantation and HIV infection, the virus can reactivate, occurring cutaneous manifestations, of what stands out the occurrence of genital ulcers. In these, co-infection with other etiologic agents is common, particularly with the herpes simplex virus, leading to more severe manifestations and difficulty in the differential diagnosis. In this context, it is relevant to know the pathogenesis of this virus and the diagnostics means available in order to optimize its therapy.

Introduction: Epicutaneous patch tests are the gold standard for the diagnosis of allergic contact dermatitis. Ideally, the tests should be performed without concomitant systemic imunossupression, which is not possible in some clinical situations. There are no guidelines on how to perform the tests or evaluate the results in such cases. We aimed to review the literature concerning patch testing under iatrogenic immunossuppression.Material and methods: Literature review of Medline indexed publications related to the subject until January 2015.Results: According to published data, patch testing was performed in 77 patients under systemic corticosteroid, 78 under cyclosporine (CyA), 6 under azathioprine, 10 under metothrexate (MTX), 4 under mycophenolate mofetil (MMF), 11 under anti tumoral necrosis factor agents and 7 under IL-12/23 inhibitor. Additionaly, 15 patients under association of immunosuppressors also underwent patch testing. Positive reations were observed in all groups.Conclusion: Ongoing treatment with immunossuppressants should not be an absolute contra-indication for patch testing, as positive reactions can be elicited in patients treated with prednisolone, azathioprine, CyA, MTX, MMF, infliximab, etanercept, adalimumab and ustecinumab. Negative or doubtful results should be interpreted with more cautious.

Porphyria cutanea tarda (PCT) is a disorder of porphyrin metabolism with associated skin photosensitivity, which presents with vesicolobullous lesions, atrophic scars and frequently signs of liver damage. Herditary hemochromathosis (HH) is the most frequent autosomic recessive genetic disease.We present the case of a previous healthy 38 years old patient that came to our office with blisters in face, extensor surfaces of arms and on the dorsum of his hands associated with hypertrichosis in zygomatic area and milia on the dorsum of the hands. Laboratory tests showed serum and urinary porphyrins level, serum iron level and ferritin exceeding the normal range. A diagnosis of porphyria cutanea tarda was reached based on clinical presentation, histopathological examination and laboratory results. Genetic studies on Hereditary Hemochromathosis mutations showed a compound heterozygote patient for the most common mutations: C282Y, H63D. Porphyria cutanea tarda can be an important cutaneous marker for patients with mutations of the HH gene. Skin signs can have a key role in early diagnosis, avoiding future complications, and requesting genetic counseling and genetic testing for the children of the affected patients.

Pityriasis lichenoides is a rare skin disease of unknown etiology, whose spectrum of clinical presentation varies from an acute form, pityriasis lichenoides et varioliformis acuta (PLEVA) to a more insidious and recurrent one, pityriasis lichenoides chronica (PLC). PLEVA, or Mucha-Habermann disease, has an acute onset of papules on the trunk, buttocks and proximal extremities that rapidly progress to vesicles or form hemorrhagic crusts, and may leave depressed scars. PLC, by contrast, has small scaly papules, asymptomatic or pruritic, which can develop over days, on the same distribution. The lesions may continue appearing and disappearing over months, sometimes causing post-inflammatory hypopigmentation. The patient does not usually evolve from a clinical form to another, tending to spontaneous healing or the cure comes after treatment. It is believed that pityriasis lichenoides is a hypersensitivity reaction to various infectious agents and with self-limiting clonal proliferation of T cells, wherein the immune response to the clone leads to clinical and pathological manifestations. It has been detected T-cell receptor gamma gene rearrangement (TCR-gamma) by PCR. Pathological exam shows typical changes of each presentation. This paper presents two very representative cases of this uncommon disease, showing the main features of each form of pityriasis lichenoides. Both had resolution with the use of Doxycycline.

Darier disease is an autosomal dominant dermatosis caused by the mutation of the ATP2A2 gene. There are rarer clinical variants showing atypical skin lesions like the hypertrophic, vesicobollous, hypopigmented, and comedonal subtypes. We present the case of a 28-years-old healthy man with more than hundred pruritic open comedone-like hyperkeratotic dark papules of the upper and lower back. Histological examination showed dilated follicular infundibulum, containing keratotic material, suprabasal acantholysis and dyskeratotic cells compatible with comedonal Darier disease. There are less than 10 published cases of this clinical and histological subtype.

Sertraline is frequently used for treatment of depressive and anxiety syndromes. Side effects are usually transient, and dose-dependent. We describe the case of a 76-year-old woman with depressive syndrome, and a history of recent introduction of sertraline who came to the emergency department with fever, and a cutaneous rash with two days of evolution. On examination, we noticed erythematous target papules, and exulcerations of oral mucosa. A skin biopsy was performed for histopathological examination with diagnosis of major multiforme erythema associated with sertraline. The patient stopped the drug in question, and initiated oral prednisolone with good resolution. Although uncommon, there are reported cases of severe skin reaction consequent to sertraline.

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Pregnancy brings changes in metabolism and immunity in women. Some of these changes have an impact on the skin. Most of these events are so common and benign that constitutes a physiological spectrum. On the other hand there are skin diseases that are specific of pregnancy and should be recognized as they can have implications for maternal and fetal prognosis. This article reviews the physiological changes with cutaneous impact and also the specific dermatoses of pregnancy.

Introduction: Management of antithrombotic therapy is not a consensual topic in the field of dermatologic surgery. Skin surgeons must weigh the risk of bleeding against the thrombotic risk when deciding how to manage those drugs in the perioperative setting. Material and Methods: The authors reviewed the literature in order to provide clinical practice guidelines for the management of these patients.Results: There are few significant studies published in this area, and sometimes presenting different approaches and recommendations. The main objective for the dermatologic surgeon should be to optimize surgical outcomes without forgetting the thrombotic and hemorrhagic risks inherent to the patient and the surgical procedure.Conclusions: We recommend the continuation of antithrombotics during low bleeding risk surgery. In patients with high thromboembolic risk we recommend the continuation of antithrombotic drugs in low bleeding risk surgeries, and its eventual suspension with bridging in high bleeding risk surgeries. However, these global recommendations should not outweigh an individualized approach of each particular patient.

Background: Erythroderma is a diffuse erythema that affects more than 90% of the body surface area that may be caused by a variety of underlying clinical entities.Material and Methods: We reviewed clinical, laboratory and histopathological data of all patients diagnosed with erythroderma admitted to our inpatient clinic over a 5-year period (2008-2012), identified on a digital database.Results: Nineteen cases were identified over the 5-year period, diagnosed on 15 patients, most being male (n = 13; 68.4%). Psoriasis was the most common etiology (n=10; 52,6%), followed by mycosis fungoides/cutaneous T-cell lymphoma (n=4; 21.0%) and drug reaction (n=3; 15.8%).

Introduction: Relapse of Hansen's disease refers to the appearance of new signs and symptoms in patients with a previous diagnosis and who completed the treatment regimen proposed by WHO.Objectives: To determine and characterize relapse cases of Hansen's disease within five years (2008-2012), in Hansen's disease consultation from Hospital Curry Cabral.Material and Methods: We conducted a retrospective and descriptive review of clinical records, identifying cases in a total of 89 patients seen in consultation in that period. The minimum follow-up time was 6 months. In all cases, clinical suspicion of relapse was confirmed by bacteriological and histopathological examination.Results: We identified 4 cases of relapse, which corresponded to 4.5% of patients observed, all with a previous diagnosis of borderline lepromatous clinical form, according to the Ridley-Jopling classification. Therapy has been established for multibacillar forms according to WHO scheme in all cases. Relapses occurred in a follow-up period between 7 and 16 months after treatment discontinuation.Discussion: The frequency of relapse in this population was higher than that observed in most studies. There was poor adherence to therapy in all cases, admitted as the cause of relapse, after excluded other factors. We emphasize the importance of regular monitoring of patients undergoing treatment, to ensure adherence and effectiveness of therapy.

Among the new applications of dermoscopy, five main fields can be distinguished: dermoscopy of skin infections and infestations (entomodermoscopy), inflammatory skin diseases (inflammoscopy), hair disorders (trichoscopy), nailfold cappilaries (capillaroscopy), and dermoscopy for treatment decisions or treatment monitoring. The term entomodermoscopy, from the words dermoscopy and entomology, has been introduced to describe the use of dermoscopy in the diagnosis of infectious skin disorders. Herein, we report several cases concerning its applicability in the diagnosis of molluscum contagiosum, scabies, lice infestations, viral warts and early cutaneous leishmaniasis.

Introduction: The treatment of sacrococcygeal pilonidal sinus is a surgical challenge and no gold standard therapy is defined. Excision and reconstruction with Limberg flap is an excellent choice as an alternative to classical methods with faster recovery and fewer complications and recurrence.Material and Methods: We performed a retrospective analysis of sacrococcygeal pilonidal sinus treated with Limberg flap between 2007 and 2014 in two tertiary-care centers. We analyzed demographic data, surgical procedure, complications, recovery time and recurrence.Results: 55 patients were treated during this period, with a median age of 28 years and male preponderance (82%). Forty-eight patients (87%) were treated with classical Limberg flap and the others (13%) with modified/ lateralized Limberg flap. The final suture was removed on average after 14 days and patients were able to return to work after 15 days. Surgical site infection occurred in 4 patients and recurrence occurred also in 4 patients. The frequency of immediate complications was superior in patients treated with lateralized Limberg flap and occurred in 57% of these patients. The recurrences occurred only in patients treated with classical Limberg flap on average 2.5 years after surgery.Conclusions: The Limberg flap technique has advantages over excision with primary closure or marsupialization, with better complications and recurrence profile. In contrast with published literature, we found more complications in patients treated with modified Limberg flap. Due to its advantages, Limberg flap technique plays an important role in the treatment of pilonidal disease, in a young working population.

Introduction: Genital infection by Chlamydia trachomatis (CT) and Neisseria gonorrhoeae (NG) is common and may be asymptomatic, particularly for CT. The availability of nucleic acid amplification tests with high diagnostic sensibility and specificity allowed implementing routine screening in Sexually Transmitted Infections (STI) Clinics. We aimed to determine the prevalence of genital infection by these bacteria in asymptomatic men attending an STI Clinic in a Dermatology Department and to characterize this subpopulation.Methods: A retrospective 51-month-period study on male patients attending a STI Clinic in Lisbon and screened for asymptomatic genital infection by CT and NG was conducted. Data on demographics, sexual behavior, CT and NG screening results and other co-existing STIs were retrieved.Results: A total of 199 patients were analyzed. The prevalence of asymptomatic genital infection by CT and NG was 5% (10 patients) and 0.5% (1 patient), respectively. Asymptomatic genital infection by CT was higher in heterosexual men (7.8% vs 1.3%) and median age of the infected patients group was lower, although not statistically significant.Conclusion: Our study showed a prevalence of asymptomatic genital infection of 5.5% in male patients, which is in line with global epidemiologic data, thus further supporting the current recommendations on routine screening of these infections in the STI Clinic.

Contact dermatitis includes any inflammatory skin reaction due to direct or indirect skin contact with noxious agents. The main clinical expression is eczema and other delayed reactions, but immediate reactions, namely contact urticaria, can also occur. Contact urticaria has a broad range of clinical manifestations. The limited form is restricted to the area of contact between the allergen and the skin or mucosa, but more severe presentations include generalized urticaria lesions, angioedema and even anaphylaxis. All topical drugs can theoretically precipitate contact urticaria. The culprit may be either the active compound or the excipients, and the mechanisms of the immediate reactions can be both immune-mediated (IgE-dependent) and non-immune-mediated. The authors present an overview of the different drugs reported to induce contact urticaria, emphasizing the specific contexts of mucosal exposure and contact urticaria induced by drugs in occupational settings.

American cutaneous leishmaniasis is an infectious, not contagious disease, caused by a protozoa from genus Leishmania and affects skin and mucosal tissue. The clinical symptoms is varied and it is related with the Leishmania species and the immune response of the host; Most common symptoms are single or multiples ulcerated lesions, however, we will present an atypical case of the disease. The patient presented mainly verrucous lesions in lower limbs, the polymerase chain reaction confirmed American cutaneous leishmaniasis diagnosis. After meglumine antimoniate treatment, the patient evolved with clinical improvement of the lesions, which supports the diagnosis. This case relevance is the need of knowledge of the disease atypical forms, in order to establish an early diagnosis and also an appropriate therapy.

Leprosy is a chronic granulomatous disease caused by the acid-fast bacillus Mycobacterium leprae, which primarily affects the skin and peripheral nerves. Despite implementation of effective multidrug therapy, the new case detection rate remains high. The type of leprosy that patients develop is determined by their cell-mediated immune response to infection. Immunological reactions can occur throughout the disease course and often rapidly accelerate the nerve damage. Lucio phenomenon is a rare and potentially severe type of immunological reaction. We present a 71-year-old male patient diagnosed with lepromatous leprosy and Lucio phenomenon, presenting skin lesions and peripheral neuropathy during six years, that showed resolution of the lesions with appropriate therapy.

Actinic granuloma of O’Brien, also called annular elastolytic giant cell granuloma is a rare condition. It shows clinically as papules lesions that converge in annular plaques lesions with an atrophic center. Histologically, it evidences elastophagocytosis and elastolysis. The case below describes a female patient, 53 years old, that had classical clinical and histopathological lesions of actinic granuloma that showed a significant improvement upon the introduction of adjunctive griseofulvin therapy.

The Merkel cell is located in the basal layer of the epidermis and its neuroendocrine function is poorly understood. Merkel cell carcinoma is a rare malignant skin cancer that affects more men, caucasians and older ages. Its annual incidence per 100,000 people when adjusted for age is 0.23 for whites and 0.01 for blacks. It inflicts predominantly the cephalic region or extremities and it is rare in mucous regions. This study presents a new case of Merkel cell carcinoma in an atypical location and treated only with adjuvant radiation therapy due to clinical conditions of the patient.

Atypical fibroxanthoma (AFX) is a relatively rare cutaneous tumor, characteristically presenting as a rapidly growing solitary nodule in photo-exposed areas of elderly patients. In spite of worrisome histological features, like pleomorphism and high mitotic rate, prognosis is favorable. Case report: A 62-year-old woman, with cicatricial alopecia on the parietal area, secondary to a thermal burn in her childhood, presented an ulcero-vegetant tumor, on the alopecia area, evolving for 1 year. Histology and immunohistochemistry showed features suggestive of AF. The tumor was completely excised, and a double transposition flap was used for closure, achieving local tumor control. The risk factors for AFX are not completely defined. Nevertheless, chronic exposition to ultraviolet radiation is a primordial factor. Other risk factors are radiotherapy and immunosuppression. Burn scars were only sporadically described in association to AF.