RESUMO
BACKGROUND: Pleomorphic dermal sarcomas (PDS) are frequent UV-induced sarcomas of the skin of intermediate grade malignant potential. Despite the fact that PDS have a noteworthy potential to recur (up to 28%) as well as to metastasize (up to 20%), there are no specific clinical guidelines with respect to follow-up these patients. Moreover, little is known about clinical, histological or molecular prognostic factors in PDS. OBJECTIVE: The aim of the present study was to identify risk factors to predict relapse in a large multicentre sample cohort of PDS which could aid to optimize personalized treatment recommendations regarding surgical safety margins and adjuvant radiotherapy. METHODS: Patients with a diagnosis of PDS were selected from nine European institutions based on the histopathologic criteria described by Fletcher. Clinicopathologic and follow-up data were collected and statistically analysed calculating univariate hazard ratios with 95% confidence intervals by use of the Cox proportional-hazards model and a significance level of P < 0.05. Patients with an incomplete excision of the tumour were excluded. RESULTS: Univariate Cox regression analysis of possible prognostic factors for progression-free survival (PFS) performed in 92 patients revealed that an excision margin of <2 cm is significantly associated with relapse of PDS [hazard ratio 4.478 (95% CI 1.536-13.055), P = 0.006]. Ulceration of the tumour was associated with a significantly better prognosis [0.396 (0.174-0.904), P = 0.028] whereas adjuvant radiotherapy did not reach statistical significance to improve prognosis in patients with PDS [0.775 (0.231-2.593), P = 0.679]. Gender, age, immunosuppression, intratumoural necrosis, tumour location, vertical thickness or horizontal diameter did not significantly influence PFS in PDS. CONCLUSION: We identified surgical safety margins of <2 cm and absence of ulceration as risk factors for relapse in patients with PDS. These findings may be implemented into both the primary treatment as well as the further monitoring of patients with PDS.
Assuntos
Margens de Excisão , Sarcoma/cirurgia , Neoplasias Cutâneas/cirurgia , Úlcera/complicações , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , PrognósticoRESUMO
The rheumatoid neutrophilic dermatitis (RND) is a rare disease associated with severe cases of seropositive rheumatoid arthritis (RA) seen in middle-aged individuals. Mostly this disease presents with erythematous papules, plaques and nodules. In general, RND responds either to corticosteroids or systemic treatment of the RA. We present the case of a 70-year-old man with RND and severe RA that responded to systemic corticosteroids and methotrexate.
Assuntos
Artrite Reumatoide/diagnóstico , Artrite Reumatoide/tratamento farmacológico , Dermatite/diagnóstico , Dermatite/tratamento farmacológico , Neutrófilos/patologia , Corticosteroides/administração & dosagem , Idoso , Humanos , Imunossupressores/administração & dosagem , Masculino , Metotrexato/administração & dosagem , Resultado do TratamentoRESUMO
A 52-year-old man presented with a progressive grey-black pigmentation of facial skin, sclera and teeth. The cause was long-term ingestion of minocycline, as confirmed by history and skin biopsy. Minocycline-induced hyperpigmentation can be divided into four main patterns based on clinical appearance, distribution, light- and electron microscopic characteristics. Some patterns can manifest within weeks of initiating therapy. One must be alert to the early signs and warn the patient about the often cosmetically disturbing and persistent minocycline-induced hyperpigmentation.
Assuntos
Toxidermias/prevenção & controle , Dermatoses Faciais/induzido quimicamente , Hiperpigmentação/induzido quimicamente , Hiperpigmentação/prevenção & controle , Minociclina/efeitos adversos , Doenças da Esclera/induzido quimicamente , Descoloração de Dente/induzido quimicamente , Antibacterianos/efeitos adversos , Diagnóstico Diferencial , Toxidermias/diagnóstico , Toxidermias/etiologia , Dermatoses Faciais/diagnóstico , Dermatoses Faciais/prevenção & controle , Humanos , Hiperpigmentação/diagnóstico , Masculino , Pessoa de Meia-Idade , Doenças da Esclera/prevenção & controle , Descoloração de Dente/diagnóstico , Descoloração de Dente/prevenção & controleRESUMO
A 74-year-old male with granulomatous mycosis fungoides presented with multiple, red-brown macules and plaques up to 8 cm in diameter, just as in classical mycosis fungoides. Dermatohistopathologic findings showed extensive granulomatous infiltrates, in which clonality could be detected in various locations via T cell receptor rearrangement. Granulomatous mycosis fungoides is a very rare form of mycosis fungoides with histological resemblance to granulomatous slack skin. It shows a rather aggressive course and can be challenging to diagnose. In our case, combination treatment with bexarotene and bath PUVA, as recommended in guidelines, resulted in an impressive improvement of the skin lesions within ten weeks.
Assuntos
Doença de Hodgkin/tratamento farmacológico , Micose Fungoide/tratamento farmacológico , Terapia PUVA , Neoplasias Cutâneas/tratamento farmacológico , Tetra-Hidronaftalenos/uso terapêutico , Idoso , Anticarcinógenos/uso terapêutico , Bexaroteno , Doença de Hodgkin/diagnóstico , Humanos , Masculino , Micose Fungoide/diagnóstico , Neoplasias Cutâneas/diagnóstico , Resultado do TratamentoRESUMO
Pseudoxanthoma elasticum (PXE) is a rare genetic systemic disease with ectopic mineralization of the connective tissue leading to clinical manifestations in the skin, eyes and cardiovascular system. PXE is caused by mutations in the ABCC6 gene and is transmitted in an autosomal recessive manner. A 9-year-old boy presented with periumbilical skin findings since birth. The detection of typical retinal manifestations ("peau d'orange"-phenomenon) made possible a definite diagnosis, following the new diagnostic criteria for PXE from 2010. Curative treatment options are still unavailable so the interdisciplinary care of PXE patients by dermatologists, ophthalmologists and cardiologists appears to be pivotal to prevent severe ophthalmologic and cardiovascular complications.
Assuntos
Pseudoxantoma Elástico/complicações , Pseudoxantoma Elástico/diagnóstico , Doenças Retinianas/diagnóstico , Doenças Retinianas/etiologia , Criança , Diagnóstico Diferencial , Humanos , Masculino , Pseudoxantoma Elástico/reabilitação , Doenças Retinianas/reabilitaçãoRESUMO
Syphilis is a sexually transmitted disease caused by Treponema pallidum which evolves through three overlapping stages. A 50-year-old woman presented with an expanding painless granulomatous nodule on her lower lip in combination with a maculo-papular exanthem. Both serologic studies and microscopic examination indicated an infection with Treponema pallidum. This case shows an unusual granulomatous nodular presentation of syphilis on the lower lip, emphasizing the variable clinical and histological manifestations syphilis, which shows an increasing number of new infections worldwide in recent years.
Assuntos
Dermatoses Faciais/etiologia , Granuloma/diagnóstico , Granuloma/etiologia , Doenças Labiais/diagnóstico , Doenças Labiais/etiologia , Sífilis/complicações , Sífilis/diagnóstico , Dermatoses Faciais/diagnóstico , Dermatoses Faciais/microbiologia , Feminino , Granuloma/microbiologia , Humanos , Doenças Labiais/microbiologia , Pessoa de Meia-Idade , Sífilis/microbiologia , Treponema pallidum/isolamento & purificaçãoRESUMO
Extranodal NK/T-cell lymphoma, nasal type, is a lymphoproliferative disorder originating from peripheral T-cells or natural killer (NK) cells. While it is a rare disease in Europe, it is more frequent in Asia and South America. It is associated with Epstein-Barr virus (EBV) infection and characterized by an extremely aggressive course and poor prognosis. We report a 46-year-old Caucasian woman who presented with multiple subcutaneous, painful nodules on the trunk first noticed a few weeks earlier. In addition to dermatological findings, the patient reported a 4-months history of necrotizing nasopharyngeal inflammation of unclear origin. Due to nonspecific histological and clinical findings mimicking a chronic inflammatory condition, a diagnosis of Wegener disease was made and immunosuppressive therapy with azathioprine was initiated. However the disease progressed under therapy. Histopathological reevaluation and immunophenotyping revealed a disseminated NK/T-cell lymphoma, nasal type. In the case of an unspecific chronic inflammatory process in the nasopharyngeal space one should always consider the possibility of this rare lymphoma, even in Europe.
Assuntos
Linfoma Extranodal de Células T-NK/patologia , Neoplasias Primárias Múltiplas/patologia , Neoplasias Nasais/patologia , Neoplasias Cutâneas/patologia , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Ciclofosfamida/administração & dosagem , Diagnóstico Diferencial , Doxorrubicina/administração & dosagem , Feminino , Humanos , Linfoma Extranodal de Células T-NK/tratamento farmacológico , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/tratamento farmacológico , Neoplasias Nasais/tratamento farmacológico , Prednisona/administração & dosagem , Neoplasias Cutâneas/tratamento farmacológico , Resultado do Tratamento , Vincristina/administração & dosagemRESUMO
While being treated with azathioprine and dexamethasone, a 21-year old man with myasthenia gravis suddenly developed rapidly progressing brown macules, predominantly on the trunk, palms and soles. We made a diagnosis of eruptive melanocytic nevi (EMN). This rare entity can appear after blistering skin diseases, in immunocompromised patients, and, in particular, during immunosuppressive therapy for autoimmune diseases. Since therapeutic regimens including azathioprine have been frequently reported in association with EMN, we recommended to our patient a treatment switch to mycophenolic acid to prevent the development of more nevi.
Assuntos
Azatioprina/efeitos adversos , Toxidermias/diagnóstico , Miastenia Gravis/complicações , Miastenia Gravis/tratamento farmacológico , Nevo Pigmentado/induzido quimicamente , Neoplasias Cutâneas/induzido quimicamente , Azatioprina/uso terapêutico , Toxidermias/etiologia , Toxidermias/prevenção & controle , Humanos , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Masculino , Nevo Pigmentado/diagnóstico , Nevo Pigmentado/prevenção & controle , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/prevenção & controle , Resultado do Tratamento , Adulto JovemRESUMO
The occurrence of multiple cutaneous leiomyomas can be indicative of hereditary cutaneous leiomyomatosis. This autosomal dominant disorder is due to germline mutations in the fumarate hydratase (FH) gene. Associations with uterine myomas and renal cell carcinomas have been described and are referred to as Multiple Cutaneous and Uterine Leiomyomas (MCUL) or Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC), respectively. A 34-year-old man presented with multiple red-brown papules and nodules. After histopathologic confirmation of piloleiomyomas, we made the diagnosis of hereditary cutaneous leiomyomatosis. Taking into consideration the aforementioned complications, close interdisciplinary management of these patients and regular screening examinations within affected families are mandatory.
Assuntos
Neoplasias Renais/congênito , Neoplasias Renais/patologia , Leiomiomatose/congênito , Leiomiomatose/patologia , Síndromes Neoplásicas Hereditárias/congênito , Síndromes Neoplásicas Hereditárias/patologia , Neoplasias Cutâneas/congênito , Neoplasias Cutâneas/patologia , Adulto , Diagnóstico Diferencial , Humanos , Masculino , Síndrome , Neoplasias UterinasRESUMO
A 45-year old man presented with red-brown hyperkeratotic papules that were distributed in an unilateral segmental fashion on the right thorax. Histopathological examination revealed acanthosis and, in particular, acantholysis and dyskeratosis in the basal and suprabasal epidermal layers. Based on the clinical and histopathologic findings we diagnosed a type 1 segmental Darier disease. Darier disease is an autosomal dominant disorder that is caused by mutations in the ATP2A2 gene and is characterized by dysfunctional adhesion between neighboring keratinocytes. The type 1 segmental manifestation reflects a de novo postzygotic somatic mutation in the heterozygous state and as a rule can be found in all autosomal dominantly inherited genodermatoses.
Assuntos
Transtornos Cromossômicos/diagnóstico , Transtornos Cromossômicos/genética , Doença de Darier/diagnóstico , Doença de Darier/genética , ATPases Transportadoras de Cálcio do Retículo Sarcoplasmático/genética , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade , MosaicismoRESUMO
During the last years, a number of new devices have been developed to improve the dermal and epidermal signs of photo- and chronological skin ageing. There are well-established ablative and non-ablative skin resurfacing options using different lasers and light sources, but side effects have been observed frequently. A recently developed photorejuvenation method using non-thermal stimulation of skin cells with low energy and narrow band light has been termed photomodulation. Light emitting diodes are the ideal source of this kind of light that stimulate mitochondrial cell organelles leading to up-regulation of cytochrome electron transport pathway leading to mitochondrial DNA gene modulation. This paper reviews the most current knowledge of intrinsic and extrinsic changes of ageing and summarizes different systems for skin rejuvenation with focus on non-thermal non-ablative skin rejuvenation modalities.
Assuntos
Epiderme/fisiologia , Rejuvenescimento/fisiologia , Envelhecimento da Pele/fisiologia , Cicatrização/fisiologia , Humanos , Terapia a Laser/métodos , Terapia a Laser/normasRESUMO
Desmoplastic fibroblastoma ("collagenous fibroma") is a rare benign, slowly progressive fibrous soft tissue tumor that ranges 1-20 cm in size. The tumor occurs in all ages, predominantly in the upper part of the body. Children are rarely affected. The tumor often infiltrates the subcutis; less often it invades the fascia and skeletal muscle or causes bone erosions. Histologically, the tumor is composed of spindle-shaped cells embedded in a densely collagenous stroma, sometimes forming a pseudo-capsule. Magnetic resonance imaging and computed tomography as well as ultrasonography are used to define the extensions of the tumor. Because it is clinically extremely difficult to determine if soft tissue tumors are benign or malignant, all should be excised with a margin of safety and examined histologically. We report a 22-year old woman with desmoplastic fibroblastoma located on the upper arm. Clinical and histopathological findings and treatment options are discussed.
Assuntos
Braço , Fibroma Desmoplásico/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Biomarcadores Tumorais/análise , Biópsia , Feminino , Fibroblastos/patologia , Fibroma Desmoplásico/patologia , Fibroma Desmoplásico/cirurgia , Humanos , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia , Adulto JovemRESUMO
Mid-dermal elastolysis is a rare dermatosis of still unknown etiology with a characteristic mid-dermal loss of elastic tissue on histopatholoy. Papular and plaque-like wrinkling of the skin as well as inflammatory and non-inflammatory variants have been described. We present a 39-year-old patient with extended skin wrinkling of the trunk and upper extremities after extensive UV light exposure and describe the clinical and histopathological findings. Based on our case, differential diagnoses are discussed and the literature is reviewed.
Assuntos
Doenças do Tecido Conjuntivo/etiologia , Doenças do Tecido Conjuntivo/patologia , Radiodermite/etiologia , Radiodermite/patologia , Envelhecimento da Pele/efeitos da radiação , Raios Ultravioleta/efeitos adversos , Adulto , Feminino , HumanosRESUMO
Keratosis follicularis spinulosa decalvans is a rare, X-linked disorder of keratinization of the hair follicle with inflammation and atrophy associated with corneal dystrophy and other symptoms. A family with several affected members is reported. The unaffected parents were related. A 12-year-old girl and her 5-year-old brother had follicular spiny hyperkeratoses on the trunk and extremities. The girl had thinning of the eyelashes and eyebrows as well as scarring alopecia of the scalp as additional features of the disease. Both the girl and her brother had corneal dystrophy and photophobia. Two sisters aged 8 and 10 years did not show similar skin or eye findings.
Assuntos
Doença de Darier/diagnóstico , Doença de Darier/tratamento farmacológico , Doenças Genéticas Ligadas ao Cromossomo X/diagnóstico , Doenças Genéticas Ligadas ao Cromossomo X/tratamento farmacológico , Glucocorticoides/uso terapêutico , Criança , Pré-Escolar , Doença de Darier/genética , Fármacos Dermatológicos/uso terapêutico , Feminino , Doenças Genéticas Ligadas ao Cromossomo X/genética , Humanos , Masculino , Resultado do TratamentoRESUMO
Granuloma fissuratum (Gf) is painful granular tissue caused by constant pressure exerted by glasses. The differential diagnosis includes further granulomatous dermatoses such as cutaneous sarcoidosis, mycobacterioses and autoimmune diseases. It is also important to rule out a basal cell carcinoma. The simplest and most effective treatment of Gf is to correct the glasses frame or, even better, to avoid wearing glasses to correct eyesight. Topical antiseptic/antibiotic treatment is recommended for superinfected lesions. The Gf usually heals within 1-6 months after correction of the glasses. If the lesion does not heal on its own, complete excision is recommended.
Assuntos
Dermatoses Faciais/diagnóstico , Granuloma Letal da Linha Média/diagnóstico , Granuloma/diagnóstico , Diagnóstico Diferencial , Feminino , Humanos , Pessoa de Meia-IdadeRESUMO
OBJECTIVE: To review the literature on menstrual and hormonal changes in women who under go tubal sterilization. DESIGN: A systematic review through MEDLINE and a literature search identified more than 200 articles in the English literature from which the most relevant were selected for this review. RESULT(S): Many authors have investigated the sequelae of female sterilization. Increased premenstrual distress, heavier and more prolonged menstrual bleeding, and increased dysmenorrhea have been reported. However, failure to control for age, parity, obesity, previous contraceptive use, interval since sterilization, or type of sterilization may have affected study results. Most studies that have controlled for these important variables have not reported significant changes, except in women who undergo sterilization between 20 and 29 years of age. CONCLUSION(S): Tubal sterilization is not associated with an increased risk of menstrual dysfunction, dysmenorrhea, or increased premenstrual distress in women who undergo the procedure after age 30 years. There may be some increased risk for younger women, although they do not appear to undergo significant hormonal changes.
PIP: Evidence for a post-tubal sterilization syndrome was sought in a literature review of over 200 English-language articles. This syndrome has been described, variously, as encompassing symptoms such as abnormal bleeding and/or pain, changes in sexual behavior and emotional health, exacerbation of premenstrual symptoms, and menstrual symptoms necessitating hysterectomy or tubal reanastomosis. It has been postulated that the destruction of the fallopian tube and, in some cases, portions of the mesosalpinx, alters the blood supply to the ovary, with consequent impairment of follicular growth and corpus luteum function. Evaluation of the research literature is hindered by the failure to control for age, parity, obesity, previous contraceptive use, interval since sterilization, or type of sterilization. Despite the vast discrepancies in the research findings, it does appear that women 20-29 years of age with pre-existing histories of menstrual dysfunction are at increased risk of some post-tubal sterilization symptoms. After this age, however, there is no consistent evidence that tubal sterilization is associated with an increased risk of menstrual dysfunction, dysmenorrhea, or increased premenstrual distress.