Repositório RCAAP

Reconstruction of nasal defects must preserve the integrity of complex facial functions and expressions, as well as facial symmetry and a pleasing aesthetic outcome. The localization and size, the anatomical layers involved and the donor tissue availability must be considered in order to establish the indication of the proper surgical procedure.In any nasal reconstruction, it is necessary to take into account three layers: internal lining, cartilaginous framework and external covering.The authors describe the reconstruction of a full-thickness defect of the left lower third of the nose after excision of a recurrent basal cell carcinoma, with ipsilateral septal mucosal flap for reconstruction of the internal lining, free auricular cartilage graft for reconstruction of cartilaginous framework and nasolabial transposition flap for the external coating, in a unique surgical procedure and with acceptable functional and aesthetic final results.

Lichen Nitidus is a chronic idiopathic immunologically-driven dermatosis that is characterized on the clinical domain by shiny, flesh-colored micropapules and, pathologically, by a lymphohistiocytic dermal infiltrate with hydropic degeneration of the basal layer or, on occasions, inflammation with granulomatous features. The infiltrate fulfills and distorts the papillary dermis, compress the overlying atrophic epidermis and is laterally confined by elongated epidermal ridges in a "baseball and glove" appearance. The case of a Common Variable Immunodeficiency patient that developed lichen nitidus in a strictly blaschkolinear distribution is reported. Semiological details foster in this case a discussion on the nosological positioning of lichen nitidus among the lichenoid dermatoses.

Introduction: Pheochromocytomas are tumours arising from chromaffin cells in the sympathetic nervous system. Clinical manifestations are protean, function of the variable secretory profile of several compounds including catecolamines, neuropeptides and other vasoactive molecules. Neurofibromatosis I (von Recklinghausen`s disease), a prevalent autosomal dominant disorder, is the most prevalent type among the neurofibromatoses. Among the diagnostic criteria proposed by the NIH Consensus Conference on Neurofibromatosis (1987), cutaneous, ocular and skeletal alterations are most relevant, although neoplastic disease involving a number of distinct organs cannot be overlooked as the altered NF I - gene product -neurofibromin - is a tumour supressor protein. Pheochromocytoma is a tumour that occurs in less than 1% of NF I patients; only 5% of pheochromocytoma reported cases are related to NF I. Nevertheless, among hypertensive NF I patients, the prevalence of pheochromocytoma may account for about 50%.Case report: The case of a 27 YO Caucasian NF I female patient, with a 4 month history of episodes of colicky abdominal pain, hyperhidrosis, dyspnoea and paroxystic hypertension is reported. Urinary catecholamines and metanephrines were raised and the topographic work up (RMN and I-MIBG) disclosed a right adrenal mass that, upon adrenalectomy, confirmed the diagnosis of Pheochromocytoma.Conclusion:  This case report intends to re-emphasize the relevance of a multisystem, multidisciplinary approach to NF I patients, bearing in mind the systemic, often ominous nature of the associated com plications.

About 30% of adults with morphea have a simultaneous autoimmune disease, which may support the involvement of autoimmunity in the pathogenesis of this fibrosing disorder of the skin and underlying tissues.A 27-year-old man presented with an asymptomatic, erythematous, slightly depressed, indurated oval plaque on the abdominal region that had been present for 1 year. Another dermatological finding was acro-facial vitiligo presented for 15 years. In family history, his mother had been diagnosed with vitiligo and Hashimoto’s thyroiditis.Hystopathological examination of the abdominal plaque supported the clinical diagnosis of morphea. Laboratory findings were unremarkable except for positive anti-glutamic acid decarboxylase antibody, which made the diagnosis of latent autoimmune diabetes of adulthood (LADA), a late-onset type 1-diabetes.Morphea and vitiligo are skin diseases that have only rarely been reported to occur simultaneously, despite the possible autoimmune etiology in both. In this patient the investigation of other associated autoimmune diseases allowed the diagnosis of LADA. This very rare association supports an autoimmune hypothesis.

A greater understanding of the pathophysiology of leg ulcers has allowed the development of new therapeutic modalities. Matrices as collagen and oxidized regenerated cellulose may, by binding to proteases, lower matrix degradation, protection and stabilization of growth factors, promoting the ulcer’s healing. A 72-year-old male patient with metabolic syndrome and venous insufficiency, peripheral arterial disease, presented recalcitrant extensive and long-term ulcers, cured with dressings matrix collagen, oxidized regenerated cellulose and silver.

Necrotrophic or secondary myiasis is caused by facultative fly larvae by infestating areas in which there is loss of integrity of the skin, causing widespread devastation, with severity of the condition depending on the location of the lesion and the degree of tissue destruction. We demonstrate two exuberant clinical cases of secondary myiasis in lesions with solution of continuity, with therapeutic success, proposing to assist the conventional treatment with ivermec tin the topical use of a healing mixture consisting of honey, albumin powder and propolis.

Introduction: prurigo has not been definitively linked to HTLV. However, there are reports of cases with onset of these lesions years before onset of adult T cell leukemia lymphoma (ATLL), as prodrome of neoplasia. Case Report: A female carrier of HTLV-1 presented pruridermia, xeroderma, hyperchromic papules, with crusted or ceratosic surface on lower limbs. Histopathological examination of the lesion confirmed the impression nodular prurigo. Patient has been treated with hydroxyzine, topical steroids and moisturizers, keeping itching. She continues in regular clinical follow without evidence of hematologic malignancy yet. Discussion: The nodular prurigo is related to some trigger. The literature does not indicate HTLV infection as a risk factor established, but there are cases report of prurigo with years of evolution before the appearance of ATLL, confirming the importance of serology for HTLV, skin biopsy and investigation of peripheral blood in these cases.

The variety of immune reconstitution inflammatory syndrome’s (IRIS) clinical presentations makes this syndrome a challenge, in that it is difficult to manage opportunistic infections and other serious clinical conditions related to the manifestation of this syndrome. The relevance of immune reconstitution inflammatory syndrome – associated with Kaposi sarcoma (IRIS-KS) after initiation of highly active antiretroviral therapy (HAART) is noteworthy, mainly in coun tries that still have high levels of transmission of sexually transmitted diseases and HIV. Clinicians and dermatologists should be aware to identify signs and symptoms of this neoplasm progression and to differentiate them from KS related IRIS according to the recent classification criteria of this disease and antiretroviral therapy should not be discontinued in the most cases.

Bowen's disease is an in situ squamous cell carcinoma which can progress to invasive squamous cell carcinoma. Female, 61 year-old, with a pink, well-demarcated, preauricular annular patch, lasting for 2 years. Two months before, a translucent nodule with telangiectasia appeared in its center. The excisional biopsy of the nodule showed squamous cell carcinoma with Bowen's disease at periphery. The remaining plaque was treated with CO2 laser followed by photodynamic therapy with complete resolution of the lesion. We emphazise this case because of the curiosity of its clinical presentation and the good therapeutic and aesthetic results.

58-year-old male, presenting a circular heterogeneously pigmented lesion, 15mm in greatest diameter, located to the abdominal wall. The patient claimed to have this lesion for "over 30 years," which nevertheless, had "begun to grow" since 3 years. This lesion was submitted to surgical excision under local anesthesia, whose histological examination confirmed the clinical diagnosis of malignant melanoma with 0.65 mm thick. The patient suffered from ulcerative colitis, and had started because of failure of prior therapy (5-ASA, corticosteroids, azathioprine) treatment with infliximab 5mg/kg intravenous 3 years ago. After the diagnosis of malignant melanoma a widening excision was performed and contact with the assistant Gastroenterologist was established, with infliximab therapy being suspended.Conclusion: Our case highlights the need for a complete dermatological observation before the start of anti-TNF-α as well as consultations for regular follow-up.

Cutaneous involvement by myeloid leukemic cells is an unusual phenomenon. The authors analyze the case of a 55-year-old male patient with myelodysplastic syndrome who presented erythematous papules on the face, trunk, arms and legs. Skin biopsy showed a dermal infiltration caused by blast myeloid cells. Immunohistochemical analysis was positive for myeloperoxidase, confirming leukemia cutis. Acute myeloid leukemia was diagnosed and the patient was treated with chemotherapy and then with rescue chemotherapy but without response. The haematologic disease progressed and the patient died after four months. The diagnosis of leukemia cutis in patients with myelodysplastic syndrome is indicative of concomitant or impending acute leukemic transformation. Early recognition is essential with important therapeutic and prognostic implications.

Dermatomyositis is an idiopathic inflammatory myopathy with cutaneous involvement. In patients over 60 years may be associated with underlying neoplasms. The most frequently involved are the lung, ovary and digestive system. The Struma ovarii is a rare ovarian tumor, which is defined by the presence of thyroid tissue, associated to a teratoma or a mucinous cystadenoma. There are benign and malignant histological variants. We present the case of a 56-years-old, Caucasian woman, without previous relevant history, with a dermatosis of three months of evolution, consisting of dispersed scaly erythematous plaques, distributed by the face, both thighs and back, with sizes ranging from 20 to 10 cm. The patient was initially treated with corticosteroids and antimalarial drugs, without improvement.Skin biopsy revealed lupus panniculitis. Muscle biopsy was normal. CT body scan had thickening of the endometrium.Transvaginal ultrasound showed atypical morphology of the right ovary. Thyroid ultrasonography and thyroid function were normal. The patient underwent laparoscopic hysterectomy with bilateral adnexectomy. The result of the surgical specimen revealed Struma ovarii in the right ovary and small serous adenofibroma in the surface. Currently the patient is treated with oral prednisone and azathioprine with significant clinical improvement. In this case the importance of screening for underlying malignancy revealed a benign tumor of ovary (struma ovarii), which is a very rare histological variant.

Dermatitis artefacta is a disease characterized by self-inflicted skin lesions as the result or manifestation of psychiatric disorders or specific stress situations.Clinical manifestations range from superficial erosions to deep wounds. Because of its rarity and the polymorphism of the lesions, dermatitis artefacta is often a challenge for the clinicians. This report presents the case of a 62-year-old woman who had an ulcer of the nose lasting for three years caused by digital manipulation. Early recognition of dermatitis artefacta is difficult but avoids unnecessary treatments. A multidisciplinary approach to this entity is necessary to obtain the best results.

Urticaria is a skin condition characterized by lesions of papules and / or erythematous and edematous plaques fleeting, that persist for minutes or hours, and which appear and disappear in different locations of tegument.Intense itching it´s usually associated. We report the case of a girl preschool, 2 years and 10 months, who presented erythematous, pruritic lesions on the extensive right hemifacial, forearms, chest and abdomen after ingestion of coconut shells and juicebox. The diagnosis was established only after the evidence of elimination of Ascaris lumbricoides through the mouth and anus was then treated with albendazole with complete resolution.

Leprosy is an endemic disease in Brazil and constitutes a serious public health problem. The delay in diagnosis and treatment can lead to irreversible sequelae. In children, the diagnosis requires a careful scrutiny, given the difficulty of implementation and interpretation of sensibility testing. The treatment in this age group demands attention, with adequate doses of anti-leprosy drugs to the patient's weight, a factor that contributes to the reduction of adverse effects and promotes adherence to treatment. The authors present a case of tuberculoid leprosy in a 3 year old child and discuss the multidrug therapy recommended by Brazil’s Health Ministry.

Lichen sclerosis is a chronic inflammatory disease of multifactorial origin involving genetic, physiological and environmental. Usually affects women between 50-60 years old, in the genital form. The pathology is uncommon in children in the extragenital form. It´s accepted as na autoimune disorder, but the mechanism remains unclear. The case report presented the flame extragenital attention as the variant form, displaying an unusual location to be affected.We report the case of a child with extragenital form with an unusual location, femoral region. Patient made use of other medications, but had clinical improvement after the use of topical colchicine. This drug has good absorption topically and has the capacity to interrupt the mitoses of the cell. Besides that have an anti-inflammatory effect reducing mobility, adhesion and chemotaxis of polimorphonuclear cells, on the desgranulacion of lysossomes.

The case of a 6 year old Caucasian male bearing, for the last 12 months, asymptomatic nondescript, flesh colored papules on his anterior thorax and arms is reported. Upon pathological examination, the diagnosis of Eruptive Vellus Hair Cysts was confirmed. A brief revision of this entity, as well as its nosographic framing within the cyst-like developmental skin abnormalities is done.

Recent reports showed promising results with topical beta-blocker therapy for ulcerated infantile hemangiomas.The authors report the case of a 6-month-old preterm girl with an ulcerated infantile hemangioma of the intergluteal cleft successfully treated with topical timolol.

Hyperandrogenism is common among teenagers, causing great psychological and social impact and may have underlying conditions that require specific medical intervention often multidisciplinary.The authors review the pathophysiology, clinical manifestations, differential diagnosis and therapeutic alternatives.We also propose a model of diagnostic approach according to their clinical presentation and therapy.

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