RCAAP Repository

The authors describe a case of a 7 year old child that developed a cutaneous lichen planus by Epstein-barr. Griseofulvin was used as treatment, with great response.

Paragangliomas or extra-adrenal pheochromocytomas are rare tumors that arise from chromaffin tissues and tend to occur either sporadically or in the context of complex genetic disorders. They are clinically heterogeneous in nature - symptoms deriving either from the secretory profile of the tumor or from the mass effect of the neoplasm. Dermatologic symptoms are quite seldom described in the literature and have been classified in acute or chronic. The case of a 40 YO Caucasian female that had been enduring for the last 2 years recurring self-limited episodes of angioedema, diarrhoea, chest pain, hypertension or hypotension, dyspnoea and anxiety is reported. Biochemical studies and further imagiologic work up and, later on, pathologic exam allowed to identify a paraganglioma that drained to the left renal artery. Upon surgery, during tumor manipulation, the patient developed an additional systemic crisis that required active life support. Recovery was regular, with a quick and sustained normalization of lab results as well as on the clinical side, with no further episode for the last 17 years.

Glomus tumor is an uncommon mesenchymal tumor present in the reticular dermis, frequently located in the hands. We report a case of a woman, 51 years old, with a history of long-lasting changes in the appearance of the left hallux’s nail and intense local pain. After several treatments, being already diagnosed the subungual wart, glomus tumor was suspected, because of the suggestive symptoms and clinical tests. Surgery was performed, with histopathological confirmation of both hypotheses. The possible diagnosis is the sum of suggestive clinical history, clinical tests and imaging tests, but only confirmed by histopathology. The treatment is surgical, treating pain and preventing recurrence. The present case reports associated diagnoses (glomus tumor and subungual wart) culminating with the major changes in the appearance of the nail. This paper emphasizes the need to suspect the diagnostic of glomus tumor on cases of obscure pain in fingers or toes.

Stewart-Treves syndrome is a rare entity with bad prognosis. It has been described as a lymphangiosarcoma in lymphedema context. We present the case of a 79-year-old female patient with a history of left breast invasive ductal carcinoma 10 years ago, treated with mastectomy and axillary lymph node dissection, chemotherapy and radiotherapy. She was observed in the Dermatology department for cutaneous changes in her left arm with two months of evolution, which didn’t improve with antibiotherapy. At the physical examination, we observed a high-grade lymphedema with erythematous-violaceous nodules on the anterior inner face of the left arm. A biopsy was performed and the diagnosis of angiosarcoma was established. The patient died one month after by disease’s progression. The awareness of the dermatologic semiology of this entity could lead to an early detection, with a precocious intervention and a better prognosis.

Prozone phenomenon results from high antibody titers in patients with syphilis, causing a false negative Venereal Disease Research Laboratory (VDRL) results. We report a case of a 50-year-old female patient, with a chief complaint of diffuse, erythematous and asymptomatic lesions, conjunctival hyperemia in her left eye. The physical examination: maculopapular rash on the trunk, back, abdomen and face and right epitrochlear adenopathy. She brought general tests within normal limits and negative VDRL. We requested FTA-ABS IgG:+ and VDRL in diluted serum: 1:512. The optimal ratio of the antigen/antibody yields an insoluble precipitate that is visible, thus rendering the test positive. Thus, phenomenon prozon was confirmed.

The rapidly growing mycobacteria are opportunistic pathogens that are frequently associated with infections related to surgical procedures. These infections have been reported after cosmetic procedures, peritoneal dialysis, hemodialysis, mammoplasty and arthroplasty. The objective of this report is to show the importance and alert clinicians and dermatologists for the occurrence of atypical mycobacterial infections after surgical procedures.

Chronic urticaria (CU) is characterized by an evanescent, itchy wheal and flare reaction that does not resolve completely after 6 weeks. It is a relatively frequent disease that causes significant morbidity. Its therapy with drugs other than H1-antihistamines has not yet been exhaustively studied, but recently some promising treatment options have been described. We describe a case of severe, corticosteroid-dependent CU that was resistant to conventional treatment with high doses of H1-antihistamines. In this case, treatment with omalizumab, an anti-IgE monoclonal antibody, allowed control of urticaria symptoms and permitted corticosteroid interruption. No adverse reactions were reported and the drug remained effective during 18 months of therapy. We present a review of the available literature concerning the use of omalizumab in CU, emphasizing information about its long-term efficacy.

Background: The treatment of leg ulcers is multidisciplinary and varies with extent, duration, factors that triggered the wound and also, patient comorbities. In literature, many studies have focused only on the treatment of venous ulcers in patients without comorbidities or with compensated comorbidities, which, in practice, correspond to a restricted group of patient. There is no study that addresses the treatment of multifactorial ulcers in patients with multiple comorbidities.Methods: Case report about a patient with comorbidities and multifactorial chronic ulcer, who was submitted to 66 applications of trichloroacetic acid (ATA) in a concentration of 90%.Results: The ulcer was complete healed in 10 months with pain control and improvement of quality of life.Conclusion: Our case report discusses the application of trichloroacetic acid (ATA) in a concentration of 90% as an effective therapeutic option, low cost and easy applicability in patients with multiple comorbidities and multifactorial decompensated chronic ulcers.

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Dermoscopy is a noninvasive, in vivo technique that increases the diagnostic accuracy in both melanocytic and nonmelanocytic skin tumors. In nonpigmented tumors it allows the visualization of vascular structures not visible to the naked eye. Part II of this article discusses clinical applications of dermoscopy in non-pigmented tumoral skin lesions.

Introduction: Disability in leprosy may result from peripheral nerve injury. Prevention of disabilities represents one of the major goals of treatment programs.Objectives: Description and comparison of two groups of leprosy patients treated before (“old” patients) and after (“new” patients) multidrug treatment implementation by the World Health Organization, analyzing its potential impact in disability reduction.Materials and methods: Retrospective, descriptive and analytic study of 243 patients distributed in two groups: 164 “old” patient” and 79 “new” patients. Sex, age at diagnosis, time between the onset of symptoms and diagnosis, clinical forms, bacilloscopy, early neurological symptoms, lenght of treatment and occurrence of reaction phenomena were studied. Disabilities were assessed using the World Health Organization disability grading system at least five years after stopping antileprosy treatment. Statistical analysis was performed using software SPSS.18. Chi-square, Mann-Whitney and Kruskal-Wallis tests were used.Results: Disability was more frequent in “old” patients with statistically significant difference in males, BL clinical form, positive basillocopy and neurological symptoms at the onset, and in patients with reaction phenomena. Severe disabilty grades were also superior in “old” patients. There was no relation between the lenght of treatment and disability.Conclusion: Prevention of disabilities is possible by an early diagnosis and proper treatment with multidrug treatment proposed by the World Health Organization.

Introduction: Tinea capitis is the most common fungal infection in children. Recent studies have shown important epidemiological differences in Portugal, particularly with respect to the dermatophytes responsible for tinea capitis.Methods: Our study aimed to characterize the epidemiology of tinea capitis diagnosed at the Dermatology department of Hospital dos Capuchos between 2008 and 2012, through a retrospective analysis of the results of cultures performed in patients with suspected tinea capitis during this period.Results: We studied 935 samples with 556 (59.3%) positive cultures. The study population had an average age of 5.4 years and was predominantly male (63.7%) and black (81.3%). We isolated eight species of dermatophytes, and M. audouinii was the dominant specie (57.7%), followed by T. soudanense (28.8%). Infections by M. audouinii and T. soudanense occurred mostly in black patients (80.1% and 53.8%, respectively). In turn, infection by M. canis was more common in caucasians (76.9%).Conclusion: This study showed a clear predominance of anthropophilic species, with M. audouinii and T.soudanense being responsible for about 87% of tinea capitis. The fact that our patients are mostly of the black race may explain these results, as well as the differences when compared our study to other studies carried out in Portugal.

Introduction: The radiation induced (RI) cutaneous angiosarcoma (CAS) in the context of the treatment of breast cancer is a rare but extremely aggressive tumor.Aim: The aim of this study was to review and present the clinical cases of RI CAS of a Radiotherapy (RT) service of, by analyzing the clinical in the last 12 years.Material and Methods: We retrospectively analyzed the clinical cases of primary atypical vascular lesions (AVL), primary CAS, RI AVL and RI CAS of the breast or chest wall (region previously submitted to mastectomy), diagnosed in our institution between 01.01.2000 e 31.12.2012.Results: We found a total of 14 patients: 2 (14 %) presented primary AVL; 2 (14%) secondary RI AVL, 5 (36%) primary CAS and 5 (36%) secondary RI CAS. The mean age at diagnosis of RI CAS was 62 years (x-y), the mean time after RT was 11.6 years (x-y) and the treatment of secondary cancer was wide excision in 80% of the cases. There were 3 deaths, none of which related to the radiation-induced injury.Discussion and Conclusion: These data are similar to other published series regarding the rarity of the breast RI CAS, age at diagnosis and the average time interval between the RT and the onset of secondary cancer. Differences regarding recurrence, metastasis and prognosis were found. The results highlight the importance of updated data concerning side effects of the treatments applied.

Introduction: Herpes zoster (HZ) is caused by the varicella zoster virus, with an estimated incidence in the general population of 3.64 cases per 1000 person-years. Its incidence increases in older age and immunosuppression states such as infection with the human immunodeficiency virus (HIV) and malignancy.Material and methods: The authors reviewed the literature in order to study the correlation between the diagnosis of HZ and occult malignancy or HIV infection.Results: In most of the studies, the evidence clearly points to the existence of an association between HZ and occult malignancy, however, the flaws of the study designs preclude the extrapolation of guidelines for clinical practice. In developed countries, data suggests that HZ has a predictive value for HIV infection only in individuals belonging to high risk groups. In countries with limited resources for HIV screening, HZ may serve as a sentinel disease to help in the decision of taking the exam, especially in endemic areas.Conclusion: There are few practical recommendations for what complementary investigation should be offered to patients with HZ. It is necessary to conduct more studies to ascertain if the implementation of sometimes complex or invasive complementary examinations is cost-effective.

The authors describe the case of a healthy 3-year-old boy with temporal triangular alopecia, and discuss the main clinical and trichoscopic aspects of this condition, comparing it, from a trichoscopic point of view, with other causes of focal alopecia.

The surgical reconstruction of the lower eyelid is conditioned by functional concerns. We report the case of a patient submitted to the excision of a basal cell carcinoma in the left lower eyelid, resulting in a full-thickness surgical defect affecting approximately one-half of its width. The execution of the initial surgical plan, which consisted in a cantothomy combined with a V-Y advancement flap, proved insufficient in order to maintain the lower eyelid rigidity and consequently its functionality. It was decided to reinforce the flap with a chondromucous graft harvested in the nasal septum.

Introduction: Digital mucous cyst is a relatively common lesion with controversial etiopathogenesis. Earlier it was considered a primary cutaneous mucinosis, as a result of a degenerative process, unrelated to the adjacent joint. Currently, it is believed that digital mucous cyst arise as a result of a communication with the distal interphalangeal joint. There are reported several treatments such as puncture, aspiration, drainage, cryotherapy, laser and excision with variable results and potential complications. The objective of this study was to evaluate the efficacy and safety of surgery that includes the identification and suture of the pedicle(s) that connects the digital mucous cyst to the distal interphalangeal joint.Material and Methods: A 1-year retrospective study (August 2012 - August 2013), including patients with digital mucous cyst treated with identification and suture of the pedicle(s) at the Department of Dermatology and Venereology of Hospital Garcia de Orta was performed.Results: During this period, 11 patients were submitted to surgery. The mean age was 62 years. The cysts were more often located on the right hand (7). In 2 cases, 2 pedicles were identified simultaneously. In a median follow-up of 16 months, there weren’t complications neither relapses.Conclusions: The results are similar to the reported in the literature, revealing superior cure rates with fewer complications. As disadvantages of this technique, it is pointed the duration of the procedure and the necessary practical experience.

Introduction: Dermatitis herpetiformis is a chronic, pruritic, polymorphous dermatosis, considered the cutaneous equivalent of coeliac disease. In pediatric patients, differential diagnosis with other more common dermatosis makes this a challenging diagnosis.Case Report: We present the case of an 8-year old male patient, with polymorphous, pruritic lesions on the extensor surfaces of limbs, evolving for 2 years. Lesional skin biopsy showed neutrophils on the tips of the dermal papillae, and direct immunofluorescence of perilesional skin demonstrated granular deposits of IgA also on the tips of the dermal papillae, establishing the diagnosis of dermatitis herpetiformis. Subsequent studies confirmed the association to celiac disease and the adoption of a gluten-free diet led to the clinical resolution of both cutaneous lesions and enteropathy.

Dermatofibrosarcoma protuberans is a rare fibrohistiocytic tumor with frequent locoregional recurrences and a low metastatic ability. Only 6% of dermatofibrosarcoma protuberans are diagnosed in children. The gold standard treatment is the surgical excision and the lower recurrence rates are observed with wide surgical margins (2,5cm) or with margin control by Mohs micrographic surgery. The authors report the case of a female 8-year-old child with a dermatofibrosarcoma protuberans of the abdominal wall. The tumor was excised with wide margins with a good outcome after a 12-month follow-up. The typical features of this tumor in children and the main differential diagnoses were pointed out.

Eccrine angiomatous hamartoma is a rare, benign malformation, of unknown aetiology, histologically characterized by capillary and eccrine glandular proliferation. It begins more frequently at birth or during childhood, presenting with a patch, plaque or nodule growing proportionally to the child’s growth. It can present with hyperhidrosis, hypertrichosis and pain. We report the case of a 3-year-old boy who was referred by 3 lightly brownish hyperpigmented patches, with more than 20cm long each, irregular borders, hypertrichosis, at the left thoracolumbar region. At friction, lesions became more vivid, with an evolution from 6 month-old with hyperhidrosis. The biopsy confirmed eccrine angiomatous hamartoma diagnosis. Due to the lesion’s benign nature and subtle symptomatology it was decided not to perform any treatment. Eccrine angiomatous hamartoma is a rare entity and this case, to the best of our knowledge, is the biggest one described until now.