RCAAP Repository

Sporotrichosis is the most frequent subcutaneous mycosis worldwide, caused by a geophilic and dimorphic fungus of the species Sporothrix spp. Lymphocutaneous form is the most common clinical presentation and facial involvement is more prevalent in pediatric patients due to low stature and leisure habits of holding cats close to the face. In this case report, a female patient, with previous diagnosis of rosacea, resident in Rio de Janeiro, an epidemic region of the disease, presented ulcerated lesions on dorsal nasal, upper lip and glabella regions, diagnosed lately as sporotrichosis

Neurothekeoma is a rare benign neoplasm of uncertain histogenesis. Progresses in immunohistochemistry have brought new insights into its cellular differentiation and origin, recognizing the possibility of a fibrohystiocitic lineage. Also, it has been subclassified histopathologically as either myxoid, cellular, or mixed type, depending on the amount of myxoid matrix and on immunohistochemical analysis. Few cases of cellular neurothekeoma have been reported. Most of them are found on the head, neck and upper extremities and mostly in young female adults. On review of literature we have not found reports regarding dermoscopic features of neurothekeoma. Herein we report an uncommon case of cellular neurothekeoma on the axilla of a 7–year-old girl, with description of its dermoscopic findings.

Introduction: Hand eczema is a very common disease with a significant social and occupational impact.Objectives: To characterize a Portuguese outpatient population with hand eczema; to evaluate the impairment of the disease in quality of life (QoL); to relate the disease severity with QoL.Methods: The authors designed a prospective study. Information about atopy, psoriasis, occupational exposure and duration of disease, was recorded. The QoL was assessed by the DLQI questionnaire (Dermatology Life Quality Index) and the severity was assessed using the hand eczema severity index (HECSI). All participants were patch tested.Results: Eighty-five patients were included with a female predominance (78.8%). The median DLQI was 9.0. We found statistically significant differences in the variable HECSI between genders with greater impact in males. A significant positive correlation was found between HECSI and DLQI. DLQI and HECSI were independent of atopy, psoriasis or etiology. Increasing age significantly affects DLQI but not HECSI.Conclusions: Hand eczema has a significant impact in QoL. Although the disease is more prevalent among women it was found to be of greater severity among men. The QoL seems to be influenced by disease severity and increasing age but it was independent of the other studied variables.

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Cutaneous adverse drug reactions (CADRs) are common during childhood, although there are still some characteristics that need to be studied. CADRs in children may differ from those in adults in terms of clinical presentation, medications involved, prognosis and treatment. Their prompt diagnosis and suspension of the culprit medication is extremely important to reduce the morbidity and mortality associated with these cutaneous reactions. The aim of this study is to review the characteristics that differentiate CADRs in children from those in adults, in order to help their recognition and understand how its investigation can be improved.

Acne vulgaris is a multifactorial disease that mainly affects adolescents and young adults and can present either with inflammatory or non-inflammatory lesions. This disease is responsible for significant morbidity, both physical and psychological. The importance of both genetic and environmental factors in its pathogenesis has been scientifically proven, and associations with the western diet, excessive body mass index and insulin resistance have been described. In this article we review the current literature on acne vulgaris, focusing on the pathophysiology and influence of these factors on its disregulation. A better knowledge of the physiopathology of acne and a critical evaluation of the influence of diet, body mass index and disruption of the endocrine system will allow a more scientific intervention on exogenous factors that contribute to the initiation and worsening of this interesting and frequent pathology.

Psoriasis is a chronic inflammatory disease that affects 2 to 3% of the population. Several studies have shown that psoriasis is associated with an increased risk of cardiovascular disease due to the increased prevalence of traditional cardiovascular risk factors, and chronic systemic inflammation that promotes the development of endothelial dysfunction and premature atherosclerosis. This literature review aims to evaluate the impact of tumor necrosis factor inhibitors in endothelial function and the risk of cardiovascular disease in patients with moderate to severe psoriasis. The studies conducted to date suggest that treatment of psoriasis with these drugs is associated with improvement in endothelial dysfunction markers and subclinical atherosclerosis and a decreased risk of cardiovascular disease.

Psoriasis is a chronic, common skin disease, which affects the patient’s quality of life to the highest degree. Several exogenous and endogenous factors may act as triggers for psoriasis, as certain hormonal changes. Previous studies have shown that thyroid hormones may be implicated in the physiopathology of psoriasis. This paper reviews the role of thyroid hormones in psoriasis as well as the current scientific evidence of a possible association between psoriasis and thyroid dysfunction.

Pain is a major clinical symptom in medicine. In the scope of Dermatology, pain occurs most commonly in the surgical setting, but it is also associated with a wide spectrum of chronic medical entities. Control of acute perioperative pain is an indicator of healthcare quality and a significant determinant of procedure morbidity and patient’s satisfaction. Chronic pain is responsible for excessive suffering, poorer quality of life and important socioeconomic costs. This paper reviews the general principles of acute perioperative and chronic pain management in Dermatology. It aims to raise awareness for its significance and to further reduce the burden of untreated pain and, consequently, improve dermatological patient care. The first part of this revision has a focus on epidemiological aspects, the definition of pain and current conceptual model, and the management of acute perioperative pain.

In hair transplant surgery, the final cosmetic result of donor area has been a growing concern among hair transplant surgeons. In the follicular unit transplant, the closure technique of donor area has been improved in order to produce thin and virtually invisible scars even in males with short hair. Trichophytic closure is a special suture technique that allows hair to grow through the donor scar and make it less visible. This technique has been explored in the follicular unit transplant but is also very useful in treatment of imperfect old scars and closure of wounds in hair-bearing areas such as the scalp, eyebrows and beard. The authors describe this surgical technique, its advantages and applications in other dermatology surgeries.

Granuloma faciale is a rare, benign, idiopathic skin disorder, observed predominantly in caucasian men between the third and fifth decades of life. It is characterized histologically by a mixed inflammatory infiltrate, in which phenomena of vasculitis and fibrosis are associated. It occurs predominantly in the face and has a chronic and slowly progressive course. Its diagnosis is based on clinical and histopathological features, with a recent emphasis on dermoscopy, which reveals aspects that may allow initial differentiation from other diagnoses (sarcoidosis, cutaneous lupus erythematosus, basal cell carcinoma, among others). There are several therapeutic options but with unsatisfactory results. In this article, we report two cases of patients with facial erythematous lesions in which dermoscopy was of great value as a diagnostic tool. Use of this technique will certainly grow in the coming years in relation to this pathology, since, besides providing important disease characteristics it represents a simple and easy way to identify facial granuloma.

Paracoccidioidomycosis (also known as Lutz disease, Lutz-Splendore-Almeida disease, Brazilian blastomycosis or South American blastomycosis) was first described by Lutz nearly a century ago. This is a systemic fungal infection that can appear in two clinical forms: acute / subacute or chronic. Oral lesions of paracoccidioidomycosis are typically characterized by erythematous and finely granular gingival hyperplasia with petechiae. Additionally, shallow ulcers with microgranulations and pinpoint hemorrhages may involve the lips, jugal mucosa, palate, tonsillar pillars and tongue. Lesions have a mulberry-like surface called "moriform stomatitis". Although common, these lesions are under diagnosed and the late recognition of this disease may lead to rapid progression, and death. We report the case of a young patient with generalized lymphadenopathy, moriform stomatitis and constitutional symptoms (fever, weight loss). We emphasize the importance of the dermatologist in the early identification of the cutaneous-mucous lesions typical of paracoccidioidomycosis and in the establishment of an accurate diagnosis.

Lobomycosis or Jorge Lobo's disease is a chronic granulomatous infection, caused by the traumatic implantation of the fungus Lacazia loboi, that affects the skin and subcutis. This dermatosis is typical of tropical and subtropical regions and affects mainly active adult males working in the forest, but it has also been already diagnosed in dolphins. The lesions primarily involve exposed skin areas , such as the auricle, and the upper and lower limbs. Keilodal lesions are the main clinical presentation. Surgical removal is the therapeutic procedure of choice in the initial cases, whereas itraconazole and clofazimine, alone or in combination, may be used in disseminated lesions.

Mongolian spots are common birthmarks in newborns and their incidence and prevalence vary considerably between races, being the lowest in caucasian infants. Although traditionally regarded as benign, some reports suggested that extensive and multiple mongolian spots at aberrant locations and persistent beyond early childhood may co-exist with inborn errors of metabolism. However, this association is not well established. Besides, very few cases in literature reported a particular type of superimposed mongolian spots, whose physiopathological mechanism and clinical significance are unknown. We report a case of a 7-months-old caucasian male infant with multiple, extensive and progressive mongolian spots and also a superimposed mongolian spot located on the right hip.

Permanent chemotherapy-induced alopecia is uncommon and has primarily been reported after high-dose busulfan chemotherapy in bone marrow transplant patients. Nevertheless, cases of PCIA, after taxane chemotherapy protocols for breast cancer, have been recently described. Despite the impact on patients’ quality of life, no consistently effective treatment has been previously described for taxane-associated permanent chemotherapy-induced alopecia. We herein report a case of a 38-year-old woman who presented inaugural and persistent hair loss after completion of adjuvant chemotherapy with taxane (docetaxel) for breast carcinoma, 2 years before the present observation. After 6 months of continuous therapy with topical minoxidil solution 5%, twice daily, significant hair regrowth was noted. We discuss the pathophysiology of permanent chemotherapy-induced alopecia and the effects of minoxidil on hair growth, in order to substantiate topical minoxidil 5% as an option to consider in the therapeutic approach for permanent chemotherapy-induced alopecia.

Merkel cell carcinoma is an aggressive skin neoplasm affecting predominantly older or immunosuppressed patients. The progression to metastatic disease is frequent while spontaneous regression is quite uncommon. Chronic sun exposure and infection by Merkel cell polyomavirus are known etiopathogenic factors. An 89-year-old female was observed with a locally advanced Merkel cell carcinoma of the left nasal ala. Lymph node metastases in left submandibular region were clinically apparent and confirmed by fine-needle aspiration. A solitary metastasis in the liver was identified by positron-emission tomography/computed tomography with (68)Ga-DOTATOC. While she was waiting for palliative radiation therapy, the patient experienced a complete clinical remission of the primary tumor and regional lymph nodes metastases occurring after 6 weeks. A liver ultrasonography disclosed remission of the liver metastasis as well. The spontaneous remission is rarely reported in medical literature. As far as we know, this is the first report in Portugal. The underlying mechanism of the spontaneous remission remains unknown, although theoretically it can be explained by the development of an efficient immune response against the tumor.

A spectrum of skin conditions – inflammatory, preneoplastic and neoplastic in nature – have been reported to occur as late side effects of radiation therapy. Postiradiation multiple minute digitate porokeratosis (PIMMDP), although seldom reported, has emerged as a distinctively unique variant among porokeratoses. The case of a 47-year-old Caucasianwoman who developed multiple filiform spiny papules mainly over the right chest wall, 12 months after receiving post-operative cobalt therapy for infiltrating ductal carcinoma of the right breast, is reported. The papules, mostly confined to the irradiation field, were pathologically found to consist of cornoid lamellae overlying a somewhat atrophic epidermis devoid of granular layer. PIMMDP is a rare, peculiar type of a late-onset radiation-induced porokeratotic skin reaction pattern that should be differentiated on pathological grounds from other clinical mimickers such as those included in the heterogenous group of multiple minute digitate hyperkeratoses. This case is ichnographically depicted and the relevant literature is discussed.

A 43-year-old man presented to our department with generalized erythroderma since early childhood, previously treated with topical and systemic corticosteroids with only partial improvement. The physical examination revealed polycyclic erythematous scaly plaques on the trunk and extremities and lichenification of the body folds (Fig. 1). In addition to skin lesions, we observed sparse hair of both eyebrows and eyelashes, however hair of the scalp was normal. The patient had a history of asthma in childhood. The family history was non- -contributory and the patient didn’t have any children. Analytically, routine blood tests were normal, however an elevated serum level of IgE 29650 UI/mL (N < 165) was detected. We performed trichoscopy of the eyebrows that showed nodules along the hair shaft and distal fractures (Fig.s 2a and 2b). The trichoscopic examination of the hair of the scalp did not identify any changes.

Major advances have been made in the understanding of melanoma in the past decade. The pathophysiology of genetic aberrations in melanoma oncogenesis as well as the evidence of family risk associated, have relevant implications in thetreatment and prognosis of patients, namely in patients with advanced disease.In this paper the authors describe, in a schematic and precise way, the different molecular pathways implicated in oncogenetic events in melanoma, which are the molecular targets of new on-going and newly developed targeted therapies.

D-penicillamine induced degenerative dermatosis include, among others, elastosis perforans serpiginosa, and pseudo- -pseudoxanthoma elasticum. Elastosis perforans serpiginosa is a rare perforating disease characterized by transepidermal elimination of abnormal elastic fibers. This condition can be idiopathic, reactive or induced by D-penicillamine, commonly used for the treatment of Wilson disease, cystinuria, rheumatoid arthritis or systemic sclerosis. Cutaneous manifestations resembling pseudoxanthoma elasticum but lacking familiar history and ABCC6 mutations have been identified as a D-penicillamine induced dermopathy and called pseudo-pseudoxanthoma elasticum. The authors present a 17-year-old caucasian female treated for several years with D-penicillamine for Wilson disease who developed asymptomatic papules, some hyperkeratotic skin-colored and other soft and yellowish, on the cervical region and face. Histopathology showed transepidermal elimination of numerous, branched, sawtooth-like elastic fibers. These findings suggested a D-penicillamine induced dermopathy and the authors considered the diagnosis of both elastosis perforans serpiginosa and pseudo- -pseudoxanthoma elasticum in the same patient. The drug was switched to zinc acetate. No newer lesions appeared thereafter but previous lesions persisted at the 1 year follow-up.