RCAAP Repository

Introduction: Biologic agents acquired a growing relevance in dermatology, however, adverse events (AE) and reasons to discontinue therapy are not completely known. The objective of this study is to analyse the reasons behind the discontinuation of biologics and characterize the AE in this population of patients. Material and Methods: Descriptive observational study, including patients over 18-years-old under treatment with biologic agents in the Dermatology Department of Hospital de Santarém, Portugal, between January/2007 and December/2017. We analysed reasons for therapeutic discontinuation, defined as the omission of at least 2 consecutive administrations, whatever the reason implicated and whether or not proposed by the dermatologist. Results: A total of 262 cycles of treatment were performed, in 138 patients (59.4% male, 40.6% female). Psoriasis was the most prevalent diagnosis (93.5% of the patients). Cycles of treatment with 8 biologic agents were analysed: etanercept (46.6%), adalimumab (31.3%) and ustekinumab (12.6%) were the most represented. During the study period, 167 suspensions were registered, for which 170 justifications were indicated. The most prevalent causes were: primary or secondary therapeutic failure (35,3%), AE (31.2%), factors related to the patient/noncompliance (17.1%), surgical intervention (7.1%) and excellent clinical response/absence of lesions (6.5%). Among therapeutic discontinuations motivated by AE (n=53), infections were the most frequent cause (35.8%, n=19), followed by malignancies (15.1%, n=8), hematological abnormalities (13.2%, n=7), neurological symptoms (9.4%, n=5) and local reactions at the injection site (5.7%, n=3). Discussion: The main cause for discontinuation of biologics was therapeutic failure, immediately followed by AE. Two different patterns of discontinuation were apparent: definitive suspension, commonly decided by the physician due to primary or secondary therapeutic failure, and temporary discontinuation, frequently without formal medical indication, due to AE, later resuming the same drug. We found a higher percentage of therapeutic cycles discontinued due to AE than reported in the literature. Temporary suspensions of the biologics due to AE, often not sufficiently valued by the dermatologist, are probably under-recognized and contribute to a suboptimal pattern of use of biologics and worse clinical outcomes.

.

With the increasing incidence of frontal fibrosing alopecia topical use of calcineurin inhibitors in the frontal region to control disease activity has increased. We report a rare adverse effect induced by tacrolimus and concomitant alcohol intake.

Odontogenic cutaneous fistulas occur mostly on the mandibula but we report a case due to a periapical infection of a maxillar teeth with a less frequent location: a 32 years-old female patient with an erythematous nodular lesion in the nasogenian sulcus for about 1 year that recurred after surgery. Examination of the oral cavity showed darkening of the upper arch canine tooth, ipsilateral to the skin lesion and imaging examination confirmed a periapical infection responsible for the extra-oral cutaneous fistula.

.

The clinical diagnosis of dermatomyositis can be difficult and challenging. In the right clinical setting and with the use of the recently described myositis specific antibodies, an earlier diagnosis can be made and therapeutic approaches promptly started, minimizing the risk of disease progression. We herein present a case of a patient with mechanic’s hands and a previously documented drug eruption to hydroxychloroquine. Although these clinical findings are not pathognomonic of dermatomyositis, in the right clinical scenario, they are precious diagnostic tools.

The bilobed flap has been extensively used in the reconstruction of distal nasal defects. It is a local flap that recruits skin from areas where there is relative skin mobility to close defects in areas where the skin has less plasticity. Besides its usefulness in the reconstruction of small to moderate cutaneous nasal defects it can also be used to reconstruct large defects located in other anatomical areas. We present a clinical case in which the bilobed skin flap was employed to reconstruct a large surgical defect of the neck resulting from excision of a basal cell carcinoma, with excellent functional and cosmetic results.

Sclerotic fibroma (SF) or storiform collagenoma is a rare and benign skin tumor. Clinically, it usually presents as an asymptomatic, slowly growing papule or nodule on the skin of the head, neck and upper extremities. Other locations have been described including the trunk, oral mucosa and nail bed. SF can present either as a solitary tumor or as multifocal cutaneous lesions and it may occur sporadically or in association with Cowden syndrome. Herein, we report a case of a solitary SF with typical clinical and histologic features, not associated with Cowden disease.

Introduction: Teledermatology (store and forward) has been used to improve priority screening for hospital consultations and to respond, in a minority of cases, to simple situations. The Dermatology consultation request via Consulta Tempo e Horas must now be accompanied by clinical photo(s), according to Despacho 6280/2018 of 28 June 2018. We intend to evaluate the satisfaction of patients and family doctors regarding teledermatology consultation. Material and Methods: A survey was carried out on patients subjected to teledermatology consultation during the 1st quarter of 2018, with a retrospective analysis of the clinical record. An online survey was also carried out on family doctors belonging to the Agrupamentos de Centros de SaúdeACeS of the Centro Hospitalar Vila Nova de Gaia/Espinho reference area. Results: Of the 153 patients surveyed, response was obtained in 27.5% (n = 42). Pigmented lesions corresponded to 54.8% (n=23) of the total consultations. Treatment was proposed in 42.9% (n=18). Patients would prefer in-person consultation in 90.2% (n=38) of cases. Eighty-two responses were obtained from family doctors. Half of the responders agree with the mandatory attachment of clinical photos to the consultation request, even if this leads to an increase of 11.21 ± 8.84 minutes to the request time. When a teledermatology consultation is made, 47.6% (n=39) of family doctors report that the patient's problem is only partially treated. Conclusion: Teledermatology consultation (store and forward) only solves a minority of clinical situations. Furthermore, it is not considered a satisfactory solution for patients.

Pediatric melanoma is the most common skin cancer in children. However, it is extremely rare this population, being even rarer in younger than 10 years of age. Its diagnosis is often difficult, due to its rarity and atypical presentations. There are three main subtypes of pediatric melanoma: Spitzoid melanoma, melanoma arising in a congenital melanocytic nevus and conventional melanoma. Congenital melanomas exist and are exceptionally rare, although they do not constitute a different subtype of melanoma. Spitzoid melanoma is the most common subtype affecting children younger than 11 years. Despite presenting with local aggressive features and frequent nodal involvement, it encompasses an excellent prognosis. The risk of malignant transformation of congenital melanocytic nevi varies widely accordingly to the projected adult size, number, and concomitant abnormalities found in the central nervous system. The surveillance and treatment of melanoma arising in a congenital melanocytic nevus is challenging, enclosing poor outcomes. In adolescents, the most common subtype is the conventional (adult-type). Contrary to the adult population, the majority of conventional pediatric melanoma arises from previous nevi but follows the general adult epidemiology and risk factors. Specific guidelines for management of pediatric melanoma do not exist and it is treated similarly to melanoma in the adult.

We report a case of early syphilis, presenting as balanitis and papular syphilides in an HIV-infected patient, with a previous history of syphilis infection, which demonstrated a false negative VDRL testing due to a prozone phenomenon. This false negative response results from overwhelming antibody titers, which interfere with the proper formation of the antigen-antibody lattice network, necessary to visualize a positive flocculation test.

Volumizing the dorsal hand is one of the anatomical regions most frequently requested during the last decade. Calcium hydroxyapatite is the material used for this procedure since its beginning, due to its several benefits. However, despite the consensus among doctors of the dermal filler to be applied, there is little consensus on the most adequate injection technique. This variation in methods is due to the divergence between anatomical studies in this region. With the presentation of two cases treated with the calcium hidroxiapatite injection under ultrasonography, and a review of the literature we intend to show the advantages of this injection method with confirmation of the correct plan to inject this material through ultrasound.

Introduction: Mohs micrographic surgery (MMS) is a surgical method of treating skin tumors that consists of histological control of the tumor margins using horizontal slices in thin, freshly frozen layers. This technique is a preferred indication for cutaneous facial tumors (basal and squamous cell carcinoma, among others) associated with higher risk of recurrence, recurrent tumors, tumors with undefined clinical margins, when there is perivascular and perineural involvement or with aggressive histological subtypes. Methods: The authors carried out a retrospective study of a 6-year period (from July 2012 until June 2018) at a Portuguese hospital – Egas Moniz hospital, to define the characteristics of patients undergoing Mohs micrographic surgery and to evaluate the advantages of this technique. The following factors were analyzed: age, gender, provenience, diagnosis, tumor location, number of stages of MMS, type of reconstruction of the surgical defect, follow-up and recurrences. Results: A total of 835 tumors were excised, 459 in male patients and 376 in female patients. The mean age at surgery was 71 years old (range 23- 95 years). Most of the lesions submitted to MMS were basal cell carcinomas (87%) and the most common location was the nasal pyramid (43%). In 44% of the cases, it was necessary to perform more than one micrographic stage. Half of the surgical defect closures were performed by graft or skin flap. Considering patients with a follow up equal or superior to 3 years, the recurrence rate was 4.9%. Discussion: The most frequent diagnosis was basal cell carcinoma, reflecting the importance of MMS in this type of skin malignancy, in both primary and persistent/recurrent lesions. Compared to other European surgery reviews, our recurrence rate is within normal range. This fact is particularly relevant, if we consider that our institution receives patients sent from hospitals and dermatologists from all over the country, specially selected for tumor aggressiveness or previous relapse. More than half of these patients were referred from other centers and consisted of persistent/recurrent basal cell carcinomas. The follow-up at recurrence for MMS was 27.9 months, supporting that a long follow-up of these patients is necessary. Conclusion: MMS allows lower rates of recurrence compared to simple surgical excision, so its practice should be encouraged in selected tumors. The surgical control of the margins instead of the “blind margins” is an advantage in saving healthy tissue and allows the complete excision of the tumor in the same surgical time.

Cutaneous metastases of visceral neoplasms are rare conditions, and urothelial or transitional cell carcinomas are responsible for less than 1% of cases. We report the case of a 50-year-old male patient who progressively developed multiple cutaneous nodules on his head, abdomen, axilla, and groin, some with ulceration. Skin biopsy and immunohistochemistry were consistent with cutaneous metastasis of urothelial carcinoma. Computed tomography scan revealed generalized metastasis in internal organs. In this rare case, cutaneous metastases were the presenting sign of the disease and, as reported, a sign of bad prognosis.

The selection of fungal isolates resistant to available therapy associated with an increase in the number of immunosuppressed patients has contributed to the incidence of infections caused by dematiaceous fungi. Thus, this study evaluated the therapeutic efficacy of the main antifungal agents currently used in clinical practice in relation to Curvularia spp. and Hortaea werneckii from cases of superficial phaeohyphomycosis from southern Brazil. The susceptibility profile of amphotericin B, fluconazole, itraconazole, terbinafine and voriconazole against dematiaceous fungi (Curvularia lunata, C. pallescens and H. werneckii) was evaluated by microdilution in broth. Terbinafine showed greater efficacy against C. lunata - gemometric mean (GM = 0.38 μg/mL), C. pallescens (MIC = 0.125 μg/mL) and H. werneckii (GM = 0.031 μg/mL) when compared to the other antifungals tested. Most of species showed sensitivity to itraconazole and voriconazole, with a minimum inhibitory concentration (MIC) range from 1 - 8.0 μg/mL and 0.5 - 2.0 μg/mL, respectively. All isolates tested show low sensitivity to fluconazole (MIC range 4 - 16 μg/mL). Although itraconazole is considered gold standard, terbinafine has been showed to be a good alternative for the treatment of superficial phaeohyphomycosis. Lastly, antifungal susceptibility testing is essential to indicate the ideal therapy against these infections.

Skin biopsies remain an indispensable tool for aiding dermatologists in accurate diagnosis and treatment. However, some clinically evident skin diseases show histological picture resembling normal skin when examined after preparation with hematoxylin and eosin (H&E). In order to establish the correct diagnosis, clinicopathological correlation is essential, together with further investigations such as special stains and immunohistochemistry techniques. Hereby, we discuss the most relevant of these “invisible” dermatoses on H&E, and include strategy for approaching such cases.

Photosensitivity is characterized by an abnormal response to non-ionizing radiation, disclosing a variety of morphological patterns, which reflect their different etiologies. We describe the case of a man presenting with a photosensitive dermatosis with no apparent exogenous cause and refractory to multiple treatments. Phototests revealed a reduction in minimal erythema dose to UVB. Laboratory tests in order to start azathioprine, showed a positive test for HIV and a diagnosis of AIDS (high viral load and low TCD4+ cells). A few months after starting antiretroviral therapy photosensitivity resolved completely. Photosensitivity affects up to 5% of HIV-positive patients, mostly due to drug photosensitivity, but it has also been reported as the presenting sign. The authors emphasize the clinical association between HIV infection and photosensitivity which has been increasingly recognized, although it is still poorly characterized from a clinical and photobiological point of view.

Dermoscopy is a noninvasive technique that increases diagnostic accuracy of several skin lesions. Being painless, dermoscopy is particularly useful in children, sparing them from unnecessary biopsies and treatments. In part II of this article, we highlight the importance of dermoscopy for the diagnosis and follow-up of infectious and inflammatory skin disorders in pediatric patients.

.

.