RESUMO
Medaillon-like dermal dendrocyte hamartomas are rare congenital cutaneous lesions. They are present at birth as asymptomatic, benign, round, erythematous, well-circumscribed, atrophic patches. Typically, they have characteristic pliable, wrinkled surface; subtle telangiectases may also be appreciated. They are localized on the upper trunk or the neck. They may be misdiagnosed as atrophoderma, cutis aplasia, or anetoderma. Characteristic histologic findings include epidermal atrophy and the presence of CD34-positive spindle cell proliferation in the dermis. Little is known about the pathophysiology of medaillon-like dermal dendrocyte hamartomas. The main diagnosis pitfall is atrophic congenital dermatofibrosarcoma protuberance due to clinical and histological similarities. We emphasize that molecular studies to eliminate the t(17;22)(q22;q13) translocation of dermatofibrosarcomas may provide determinant elements for diagnosis in order to avoid unnecessary mutilating surgery. We present a case of medaillon-like dermal dendrocyte hamartoma with a local recurrence.
Assuntos
Hamartoma/congênito , Hamartoma/diagnóstico , Dermatopatias/congênito , Dermatopatias/diagnóstico , Antígenos CD34/análise , Proliferação de Células , Dermatofibrossarcoma/diagnóstico , Dermatofibrossarcoma/patologia , Dermatofibrossarcoma/cirurgia , Diagnóstico Diferencial , Hamartoma/patologia , Hamartoma/cirurgia , Humanos , Lactente , Masculino , Procedimentos de Cirurgia Plástica , Recidiva , Reoperação , Pele/patologia , Dermatopatias/patologia , Dermatopatias/cirurgiaRESUMO
BACKGROUND: Xeroderma pigmentosum type C (XP-C) is a rare, autosomal, recessive condition characterized by the association of various clinical manifestations mostly involving the skin and eyes. OBJECTIVES: To evaluate the clinical manifestations in a homogeneous, genetically characterized cohort of patients with XP-C. METHODS: All patients with XP-C, which was confirmed genetically or by unscheduled DNA synthesis, from the registry of our department and from the French association of patients 'Les Enfants de la Lune' were contacted. During a planned consultation, clinical information was collected using a standardized case-record form. RESULTS: In total, 31 patients were seen. The mean age at diagnosis was 2.95 years; skin symptoms started at a mean age of 1.49 years. Among the patients, 52% had relatively short stature, with a height-for-weight z-score below -1 SD; 62% showed pyramidal syndrome and 45% had photophobia and/or conjunctivitis. Four patients had several pyogenic granulomas. Twenty-four patients (77%) had skin cancer. The mean age of onset of the first skin cancer was 4.76 years (range 2-14.5 years). Basal-cell carcinoma was the most frequent cancer. Melanomas were rare and mostly desmoplastic. Multinodular thyroid was the most frequent internal tumour. CONCLUSIONS: Our data highlight several new aspects of XP-C. Patients with XP-C are at risk of developing pyogenic granulomas, desmoplastic melanomas and multinodular thyroid. Involvement of the central nervous system is frequent, but its mechanism remains unclear. The relatively short stature of the patients needs further investigation in order to be explained. XP-C is not only a cancer-prone disorder but is also a polysystemic disorder.
Assuntos
Carcinoma Basocelular/diagnóstico , Granuloma Piogênico/diagnóstico , Melanoma/diagnóstico , Neoplasias Cutâneas/diagnóstico , Neoplasias da Glândula Tireoide/diagnóstico , Xeroderma Pigmentoso/diagnóstico , Adolescente , Carcinoma Basocelular/genética , Criança , Pré-Escolar , Estudos de Coortes , Feminino , França , Granuloma Piogênico/genética , Humanos , Masculino , Melanoma/genética , Neoplasias Cutâneas/genética , Neoplasias da Glândula Tireoide/genética , Xeroderma Pigmentoso/genéticaRESUMO
Stevens-Johnson syndrome is an acute, self-limiting disease of the skin and mucous membranes. Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis are all part of a single spectrum illness. We report severe erythema multiform in 4 children aged from 6 to 15 years old. Erythema was mostly related to mycoplasma pneumoniae infection (3/4) and 1 case was attributed to drugs. Two children developed severe sequelae (obliterans bronchiolitis). No patient had recurrent disease. The early use of steroids is still debated, but in our experience it seems to benefit overall. A long term follow-up is necessary with the study of pulmonary function tests and chest X-rays ophtalmologic and dermatologic examination.
Assuntos
Síndrome de Stevens-Johnson/diagnóstico , Adolescente , Criança , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Masculino , Pneumonia por Mycoplasma/complicações , Radiografia Torácica , Síndrome de Stevens-Johnson/diagnóstico por imagemRESUMO
We report on a 3-year-old child with bracelet localisation of chickenpox. Varicella with unusual clinical aspects and course is known as atypical varicella; it is characterized by its unique clinical features, unusual distribution, or a prolonged course. In case of atypical varicella, clinical diagnosis can be difficult. Pre-existing factors have been reported such as immunocompromised status, sun exposition, local injury and pre-existing rash.
Assuntos
Varicela/patologia , Pré-Escolar , Antebraço , Humanos , MasculinoRESUMO
BACKGROUND: Neonatal exanthema of bacterial origin is caused very rarely by Gram-negative bacilli. We report a case of neonatal maculopapular exanthema evocative of Klebsiella pneumoniae septicemia resulting from maternofetal infection. OBSERVATION: On the fourth day of life, a newborn infant presented incipient morbilliform maculopapular exanthema on the face. During delivery, the mother had presented hyperthermia and meconium was found in the amniotic fluid. Clinical examination of the newborn was normal. No clinically obvious site of entry of infection was seen. Laboratory tests revealed major inflammatory syndrome. Blood cultures were positive for K. pneumoniae, which was also found in blood cultures from the mother. Screening for other causes of infection was negative. Parenteral antibiotics for 10 days yielded favorable results with simultaneous resolution of the exanthema, normalization of laboratory values and negative blood cultures. DISCUSSION: Exanthemas of infectious origin are not associated with any specific organism. The most common causative micro-organisms are Listeria monocytogenes, B streptococci, colibacilli, and more rarely, staphylococci. There have been reports of a number of cases of neonatal septicemia due to Gram-negative bacilli responsible for maculopapular exanthemas but the causative organism was not identified. To our knowledge, Klebsiella pneumoniae has never been incriminated in the appearance of this type of rash via maternofetal transmission. Consequently, the presence of neonatal exanthema should prompt screening for sepsis, even in the absence of other evocative signs, and in particular in settings of apyrexia.
Assuntos
Exantema/microbiologia , Transmissão Vertical de Doenças Infecciosas , Infecções por Klebsiella/complicações , Infecções por Klebsiella/transmissão , Klebsiella pneumoniae , Sepse/microbiologia , Humanos , Recém-Nascido , MasculinoRESUMO
BACKGROUND: Fungal agents, chiefly Candida albicans, are the cause of rising morbidity and mortality in newborn infants weighing less than 1500 g. We studied the particular cutaneous effects during the course of these infections. PATIENTS AND METHODS: This was a retrospective 3-year study in premature infants weighing less than 1500 g and hospitalized in the neonatal department of the Lille University Teaching Hospital. The patients included in the study presented sepsis with isolation of Candida in blood and/or urine culture. RESULTS: Twelve infants were included (1.8%). The risk factors seen are those described in literature (broad-spectrum antibiotics, prolonged mechanical ventilation and parenteral nutrition, corticosteroids and central venous catheters). Infection occurred early (mean: D12) and affected extremely premature infants (mean: 25 weeks' amenorrhea) of low birth weight (mean: 758 g) generally born by vaginal delivery (9 of 12 infants). The sole fungal agent isolated was Candida albicans. In 10 of the 12 patients, a characteristic skin disorder was observed (erythema with erosion and desquamation). In 10 of the 12 patients, too, Candida was isolated from skin and/or mucosal samples. DISCUSSION: Although it is now universally accepted that antifungal treatment should be initiated without delay for candidemia in septic newborn infants at risk, diagnosis of systemic candidiasis remains delicate. However, a specific pattern of skin involvement is very commonly seen that is atypical for candidiasis, but which in addition to its diagnostic value indicates early colonization with Candida (first 2 weeks of life). In this setting of immaturity of the skin and immune system, colonization and proliferation in skin and/or mucosa appear to constitute the first stage of systemic infection and we may speak of invasive cutaneous-mucosal candidiasis in extremely premature infants and initiate treatment designed to prevent the disease becoming systemic..
Assuntos
Candidíase/diagnóstico , Dermatite/diagnóstico , Dermatite/microbiologia , Doenças do Prematuro/diagnóstico , Doenças do Prematuro/microbiologia , Candidíase/tratamento farmacológico , Dermatite/tratamento farmacológico , Feminino , Humanos , Lactente , Recém-Nascido , Doenças do Prematuro/tratamento farmacológico , Masculino , Estudos RetrospectivosRESUMO
Phacomatosis pigmentovascularis (PPV) is described as the association of cutaneous vascular malformations and different pigmentary disorders. The different associations are classified into four types, with localized and systemic forms. The constant alteration in each type is a nevus flammeus. Associated pigmented abnormalities are nevus pigmentosus and verrucosus in type I, blue spots in type II, nevus spilus in type III, blue spots and nevus spilus in type IV. Type II is the most frequently reported. In this type, half of the patients have visceral involvement, more often Klippel-Trenaunay or/and Sturge-Weber syndrome. We report two cases of phacomatosis pigmentovascularis type II. The particularity of the first case is the association with a cranio-cervical junction malformation (Arnold-Chiari type I), which to our knowledge, has never been described with PPV. The second case presents with a bilateral scleral melanosis without visceral involvement. Definition of visceral involvement and ophthalmologic manifestations are discussed.
Assuntos
Malformação de Arnold-Chiari/diagnóstico , Melanose/diagnóstico , Mancha Vinho do Porto/diagnóstico , Doenças da Esclera/diagnóstico , Adulto , Malformação de Arnold-Chiari/complicações , Diagnóstico Diferencial , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Melanose/complicações , Mancha Vinho do Porto/complicações , Doenças da Esclera/complicaçõesRESUMO
Carcinogenic effects of radiotherapy in breast cancer are well-known. Long-term follow-up of these patients shows a significantly increased risk of leukemia. Cutaneous granulocytic sarcoma is an uncommon leukemia cutis that usually occurs in association with acute myelocytic leukemia or myeloproliferative disorders. We report a case of cutaneous granulocytic sarcoma in a 44-year-old woman who had been treated six months earlier for breast adenocarcinoma. The treatment had associated lumpectomy, axillary lymph node dissection and radiotherapy. Skin lesions appeared firstly and predominantly on the irradiated area. Haematological investigations were normal and the diagnosis of isolated sarcoma was made. The uncommon features of this case were the short interval between radiotherapy and the occurrence of leukemia skin lesions and the fact that, to our knowledge, this is the first report of leukemia cutis localised on the irradiated area. The responsibility of radiation in the distribution of cutaneous lesions of granulocytic sarcoma is discussed.
Assuntos
Neoplasias da Mama/radioterapia , Carcinoma Ductal de Mama/radioterapia , Leucemia Mieloide/etiologia , Neoplasias Induzidas por Radiação/etiologia , Segunda Neoplasia Primária/etiologia , Radioterapia Adjuvante/efeitos adversos , Neoplasias Cutâneas/etiologia , Adulto , Neoplasias da Mama/cirurgia , Carcinoma Ductal de Mama/cirurgia , Terapia Combinada , Feminino , Humanos , Leucemia Mieloide/patologia , Mastectomia Segmentar , Neoplasias Induzidas por Radiação/patologia , Segunda Neoplasia Primária/patologia , Neoplasias Cutâneas/patologiaRESUMO
We report two cases of Mycoplasma pneumoniae infections associated with Stevens-Johnson syndrome and antiphospholipid antibodies. Such an association has been noted once in the literature. The relationship between antiphospholipid antibodies and Stevens-Johnson syndrome and others cutaneous manifestations of infections diseases is discussed. Though mainly described in systemic lupus erythematosus and autoimmune diseases, anticardiolipin antibodies and lupus anticoagulant have been found in many infectious disorders. But in the latter conditions, they have been considered by many authors as "non pathogenic" or "non prothrombotic" on epidemiologic and immunologic data. We suggest that antiphospholipid antibodies could possibly play a role in their pathogenesis especially as the mechanisms are not to date clearly understood.
Assuntos
Anticorpos Antifosfolipídeos/fisiologia , Pneumonia por Mycoplasma/complicações , Síndrome de Stevens-Johnson/complicações , Pré-Escolar , Humanos , Masculino , Pessoa de Meia-Idade , Pneumonia por Mycoplasma/imunologia , Pneumonia por Mycoplasma/fisiopatologia , Síndrome de Stevens-Johnson/imunologia , Síndrome de Stevens-Johnson/fisiopatologiaRESUMO
INTRODUCTION: To better individualize drug hypersensitivity reaction, Bocquet et al. have recently called this adverse drug reaction DRESS (Drug Rash with Eosinophilia and Systemic Symptoms). EXEGESIS: We report two cases of DRESS and highlight the main characteristics of this syndrome. Two patients presented severe febrile skin eruption following drug intake (carbamazepine or sulfazalazine), with hypereosinophilia and hepatitis. All symptoms resolved after drug withdrawal and corticosteroid therapy. DRESS syndrome is an idiosyncratic reaction characterised by febrile eruption, occurring 2 to 6 weeks after the beginning of the treatment, accompanied by systemic symptoms and biological abnormalities (hypereosinophilia, hepatitis). Some complications can occur. This syndrome can be fatal. Numerous drugs can be responsible for this reaction to medication. The physiopathology has not yet been elucidated, and the treatment is not codified, but the triggering agent must immediately be stopped. Corticotherapy is sometimes used. CONCLUSION: It is important to recognize this entity recently named DRESS syndrome because it can mimic other pathologies, is potentially serious, and because withdrawal of the incriminating drug is imperative.
Assuntos
Anti-Inflamatórios não Esteroides/efeitos adversos , Anticonvulsivantes/efeitos adversos , Carbamazepina/efeitos adversos , Hipersensibilidade a Drogas/patologia , Eosinofilia/induzido quimicamente , Exantema/induzido quimicamente , Sulfassalazina/efeitos adversos , Adolescente , Corticosteroides/uso terapêutico , Adulto , Doença Hepática Induzida por Substâncias e Drogas , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Prognóstico , SíndromeRESUMO
UNLABELLED: Leukemia cutis (LC) are not rare in acute myeloid leukaemia (AML) in children but exceptionally reveal it. Most authors think that they have poor prognosis. CASE REPORT: We report the case of an infant with isolated cutaneous involvement at the time of diagnosis of leukaemia. Bone marrow aspiration showed AML M5. The child was treated by LAME 91 protocol, arm "infant under one year of age". Complete remission, both in bone marrow and skin, was obtained after induction course. Then the patient received consolidation course and megatherapy followed by autologous bone marrow transplantation. Skin relapse occurred early. The complete remission no. 2 was not obtained by second line treatment: new LC appeared when PMN count increased more than 10(9)/l. Then, the child was treated with oral VP16 but disease progressed with more and more LC, followed by bone marrow relapse. Child's death occurred about one year after diagnosis.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia Monocítica Aguda/diagnóstico , Neoplasias Cutâneas/diagnóstico , Transplante de Medula Óssea , Diagnóstico Diferencial , Evolução Fatal , Feminino , Humanos , Lactente , Leucemia Monocítica Aguda/complicações , Leucemia Monocítica Aguda/tratamento farmacológico , Prognóstico , Recidiva , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/tratamento farmacológicoRESUMO
OBJECTIVES: Despite the advent of modern dressings, management of leg ulcers remains a long costly process, particularly if no etiological treatment is possible. Autologous skin grafting is more and more widely used in this indication. The aim of this open single center noncomparative study was to analyze the feasibility of ambulatory procedures for skin grafting and the incidence of ambulatory care in a medical nursing clinic as an alternative to traditional hospitalization on total cost in this pathological condition. PATIENTS AND METHODS: Thirty-nine grafts were performed in 34 consecutive patients. No selection was made for etiology or duration of the leg ulcers. Three grafting techniques were used after debridement-cleansing: flap grafts for medium sized ulcers (29 cases), mesh grafts for large ulcers (6 cases) and patch grafts for small ulcers or ulcers with irregular contours (4 cases). The dressing was opened on day 5, nursing care was provided every 2 days and daily in case of infection. Percentage of healing was evaluated clinically on days 5, 15 and 30 then at months 3, 6 and 12. Photographs were taken. RESULTS: Four patients were lost to follow-up and one died. Among the 34 grafts assessed at 6 months, we obtained total healing in 56 p. 100, 75 p. 100 healing in 6 p. 100, 50 p. 100 healing in 9 p. 100 and failure in 29 p. 100. Healing rates were those expected for arterial ulcers and necrotic angiodermas. For venous leg ulcers, the rate of total healing was only 30 p. 100 at 6 months and 43 p. 100 at 1 year. Outcome depended on duration of the lesion and not on the type of skin graft or patient age. DISCUSSION: This prospective study reports outcome of ambulatory skin grafting in a large representative sample of patients with leg ulcers of various etiologies. The less favorable outcome for venous ulcers can be explained by the duration of the ulcerations and infection in these often neglected lesions. The risk of graft displacement, contact eczema, and infection must be recognized for early treatment. There were no cases with general complications. This ambulatory technique has the enormous advantage of limiting the risk of hospital-related problems in this elderly population and of reducing overall cost of care for leg ulcers, and finally of limiting the risk of recurrence by regular post-graft follow-up in a specialized center and by treatment of the causal disease.
Assuntos
Procedimentos Cirúrgicos Ambulatórios , Úlcera da Perna/cirurgia , Transplante de Pele , Retalhos Cirúrgicos , Idoso , Idoso de 80 Anos ou mais , Procedimentos Cirúrgicos Ambulatórios/economia , Custos e Análise de Custo , Feminino , Seguimentos , Humanos , Úlcera da Perna/economia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Transplante de Pele/economia , Retalhos Cirúrgicos/economia , Fatores de TempoRESUMO
BACKGROUND: Granuloma annulare following BCG vaccination or tuberculin skin tests is rarely reported in the literature. We describe three cases occurring in patients under the age of 6 years. CASE REPORTS: In the first two cases, granuloma annulare was initially localized at the vaccinal site, then generalized. In the third case, diagnosis was deep granuloma annulare localized far from the initial vaccination site, with recurrence following tuberculin test. In the three cases, diagnosis was made on the basis of clinical and histological elements. DISCUSSION: The relationship between granuloma annulare and BCG may seem evident when the eruption occurs at the vaccinal site, but remains difficult to prove. Diagnosis essentially relies on the absence of other classical etiologies and a compatible delay. When a vaccination such as BCG appears to be the cause of granuloma annulare, a possible cause would be injection trauma or a cell-mediated-delayed-hypersensitivity reaction to a specific antigen contained in the vaccine, leading to development of skin disorders such as granuloma annulare in predisposed subjects. Reports of granuloma annulare following BCG are uncommon but the incidence may be underestimated. In practice, diagnosis of granuloma annulare following BCG or tuberculin skin test cannot be established until BCG dissemination infection is ruled out, a necessary clinical and paraclinical step required in all cases of eruptions following this vaccination. Granuloma annulare is a benign complication requiring no treatment and does not constitute a contraindication for other usual vaccinations.
Assuntos
Adjuvantes Imunológicos/efeitos adversos , Vacina BCG/efeitos adversos , Granuloma Anular/etiologia , Granuloma Anular/patologia , Biópsia , Pré-Escolar , Diagnóstico Diferencial , Feminino , Granuloma Anular/epidemiologia , Humanos , Incidência , Masculino , Recidiva , Teste TuberculínicoRESUMO
INTRODUCTION: Major trauma was the only aetiologic factor found in a male patient presenting sclerosing lipogranuloma. This condition is well-known in women but is rarely described in men. CASE REPORT: A 31-year-old non-obese man with no sign of gynaecomasty presented with well-delimited plaques of pigmented and sclerosous tissue located at the pubis and both breasts as well as hard nodules of the scrotum and the penis. The lesion developed progressively after a major trauma involving the anterior aspect of the thorax and the public area. An inflammatory border persisted eight years after the initial trauma. Histological examination of a biopsy specimen showed evidence of adipocyte necrosis with lipophagia and lipogranuloma. No foreign body was found. DISCUSSION: The diagnosis of post-trauma cytosteatonecrosis was retained on the basis of the clinical history and the clinical, histological and radiographic evidence. This condition is a well-identified clinical entity which is sometimes confounded with false panniculitis resulting from self-injection of oily products. The endogenous origin of the fatty acids found within the cytosteatonecrosis tissue has recently been proven. Usual localizations are the breast in women and genital organs in men. Favouring factors in women include obesity, age, anticoagulants, frequency and intensity of trauma. The exceptional localization in our patient was strongly related to the earlier trauma.
Assuntos
Tecido Adiposo , Granuloma/diagnóstico , Pele/patologia , Traumatismos Torácicos/complicações , Adulto , Diagnóstico Diferencial , Genitália Masculina/lesões , Granuloma/etiologia , Humanos , Masculino , Paniculite/diagnóstico , Radiografia Torácica , Esclerose , Traumatismos Torácicos/diagnóstico por imagemRESUMO
INTRODUCTION: Low-molecular weight heparin-induced cutaneous necrosis is exceptional. Pathogenesis remains unclear. We report an exceptional case with elective localization of the necrotic areas in insulin lipodystrophic tissue. CASE REPORT: A 69-year old patient developed areas of skin necrosis after starting enoxaparin therapy. These areas were located far from the points of injection and focalized on skin areas where the patient had been injecting insulin daily for the last four years. These areas had an aspect of insulin lipodystrophy. Biopsy specimens showed leukocytoclastic vasculitis. There were no associated biological anomalies. One month later, prick-tests were made with different low-molecular weight heparins and calcium heparinate in a lipodystrophic area together with an enoxaparin control test in healthy skin. The only positive test was for enoxaparin in an insulin lipodystrophic area (hard erythema at 24 hours). Histology at 72 hours demonstrated leukocytoclastic vasculitis. DISCUSSION: Six cases of cutaneous necrosis induced by low-molecular weight heparin have been reported, including three cases with enoxaparin. Two pathophysiological mechanisms could be involved: (i) localized heparin-dependent platelet aggregation, or (ii) vasculitis induced by type III hypersensitivity reaction. In our case, the leukocytoclastic aspect of the vasculitis was compatible with an immune complex hypersensitivity reaction. The localization of the necrotic areas would be explained by enoxaparin-induced preferential deposit of immune complexes in the vascular turbulences present in lipodystrophic areas.
Assuntos
Anticoagulantes/efeitos adversos , Enoxaparina/efeitos adversos , Lipodistrofia , Necrose , Pele/patologia , Anticoagulantes/administração & dosagem , Enoxaparina/administração & dosagem , Feminino , Humanos , Injeções Subcutâneas , Insulina/efeitos adversos , Lipodistrofia/induzido quimicamente , Lipodistrofia/patologia , Pessoa de Meia-Idade , Testes Cutâneos , Vasculite Leucocitoclástica Cutânea/induzido quimicamente , Vasculite Leucocitoclástica Cutânea/patologiaRESUMO
BACKGROUND: Paraneoplastic pemphigus is a bullous skin disease with characteristic polymorphous clinical presentation and precise histological and immunological features. We report a case of paraneoplastic pemphigus associated with chronic lymphoid leukemia involving the tracheobronchial epithelium. CASE REPORT: A patient with chronic lymphoid leukemia developed pluriorificial lesions. There were several conjunctival, buccal and genital erosions associated with erosive plaques on the trunk, Nikolski's sign and bullous lesions suggestive of paraneoplastic pemphigus. Histology examination of a bulla showed intraepidermal blistering and suprabasal acantholysis. Direct immunofluorescence evidenced intercellular IgG and C3 deposits. Search for anti-intercellular substance antibodies was positive with fluorescence on specific paraneoplastic pemphigus substrates. At immunotransfer, the serum recognized several bands corresponding to 250, 230, 210 and 190 kD antigens, confirming the diagnosis of paraneoplastic pemphigus. Several days later, the patient's general condition deteriorated with bronchorrhea. Bronchial endoscopy visualized ulceronecrotic plaques. Tracheal biopsy evidenced acantholytic cells and intraepithelial cleavage. General corticosteroid therapy was initiated and led to improvement of the skin lesions but the patient died rapidly from pneumonia. Autopsy confirmed the presence of epithelial cleavage and acantholysis involving the trachea and bronchi. DISCUSSION: This case illustrates the difficulty of diagnosing paraneoplastic pemphigus in the early stages. The pluriorificial lesions were suggestive of a Stevens-Johnson syndrome. Besides the genital, conjunctival and buccal mucosa, other mucosa can be involved. In our case, despite the absence of an immunological element, histology was highly suggestive of specific tracheobronchial localizations. The presence of such lesions, which should be searched for in all cases with bronchopulmonary manifestations, worsens the prognosis.
Assuntos
Broncopatias/etiologia , Leucemia Linfocítica Crônica de Células B/complicações , Síndromes Paraneoplásicas , Pênfigo/complicações , Doenças da Traqueia/etiologia , Acantólise/etiologia , Acantólise/patologia , Broncopatias/patologia , Evolução Fatal , Humanos , Leucemia Linfocítica Crônica de Células B/patologia , Masculino , Pessoa de Meia-Idade , Pênfigo/patologia , Doenças da Traqueia/patologiaRESUMO
INTRODUCTION: Several skin diseases can be seen in patients with trisomy 21. We report a case of miliary calcinosis of the extremities. CASE REPORT: A 15-year old adolescent with Down's syndrome presented small papular miliary lesions which had developed over 18 months and tended to discharge a chalk-like substance via the epidermis. Approximately 15 lesions were present on the hands and feet. Histologically, there was a well-delimited calcium deposit in the superficial dermis. There was no alteration in phosphorus/calcium metabolism. Brain CT-scan and cardiac echography did not reveal any calcifications. DISCUSSION: Miliary calcinosis cutis may not be exceptional in Down's syndrome, although only 9 observations have been reported. Preferential localizations include the hands, wrists and feet. Association with syringoma has been noted but would appear to be fortuitous. Transepidermal elimination of the calcium deposits is frequent. Pathogenic hypotheses include precipitation of calcium salts in sudation products and/or increased synthesis by fibroblasts. The association with trisomy 21 appears to be significant since only three cases have been reported in patients with normal karyotypes. This entity should be individualized as perforating milia-like idiopathic calcinosis cutis of the extremities.
Assuntos
Calcinose/etiologia , Síndrome de Down/complicações , Dermatoses do Pé/etiologia , Dermatoses da Mão/etiologia , Adolescente , Calcinose/patologia , Feminino , Dermatoses do Pé/patologia , Dermatoses da Mão/patologia , HumanosRESUMO
BACKGROUND: We report the first case of eosinophilic pustular folliculitis (Ofuji's disease) which was successfully treated with acitretin. CASE REPORT: A 50-year old women (HIV negative) had developed over 3 months an erythematopapulous plaque under the left orbit. The clinical and histological diagnosis was eosinophilic pustular folliculitis. Successive treatment with cetirizine then indomethacin was ineffective. Acitretin (0.5 mg/kg/d) was then started and led to cure within 1 month. Six weeks after the patient spontaneously stopped the treatment, the lesion recurred at the same localization. Further treatment with isotretinoin (0.5 mg/kg/d) was then given but did not alter progression of the lesion. Acitretin was then reintroduced at the same dose and again produced rapid cure. Acitretin was then tapered off to 10 mg/d then maintained at this dose as lesions reappeared with further dose reduction. DISCUSSION: It is difficult to treat eosinophilic pustular folliculitis because of the random nature of response to different drugs. General corticosteroids, dapsone and indomethacin are classically proposed but with variable success. Isotretinoin is proposed on the hypothesis of a link with sebaceous secretion, but results have been contradictory. This drug was ineffective in our case. Acitretin did however provide very rapid improvement with an efficacy confirmed at reintroduction. This retinoid which does not have the specific action of isotretinoin could affect follicular keratinocytes which have been shown to be activated in this chronic skin disease.
Assuntos
Acitretina/uso terapêutico , Eosinófilos , Dermatoses Faciais/tratamento farmacológico , Foliculite/tratamento farmacológico , Ceratolíticos/uso terapêutico , Dermatopatias Vesiculobolhosas/tratamento farmacológico , Dapsona/uso terapêutico , Dermatoses Faciais/patologia , Feminino , Foliculite/patologia , Humanos , Isotretinoína/uso terapêutico , Pessoa de Meia-Idade , Recidiva , Dermatopatias Vesiculobolhosas/patologia , Resultado do TratamentoRESUMO
BACKGROUND: Pyostomatitis-pyodermatitis vegetans is an uncommon condition associated with chronic inflammatory bowel disease in 75% of the cases, usually hemorrhagic rectocolitis. CASE REPORT: A 48-year-old man was referred for recent development of pustulous lesions of the lips and buccal mucosa and weight loss. He complained of abdominal pain and intermittent diarrhea which had persisted for more than one year. During the last three months, a pseudotumoral plaque with a pustulous rim had developed over the two distal phalanxes of the right middle finger in association with ungueal lysis and nodular, vegetating and crusted lesions on the lateral aspect of the left arm. Small pustules covered the entire buccal mucosa excepting the tongue and the glans forming a typical snail trace aspect. Bacterial and mycological samples were negative. The histology reports for skin and mucosa were similar: epithelial hyperplasia, intra- and subepithelial granulocyte micro-abscesses and polymorphous infiltration of the superficial derma with numerous neutrophils and eosinophils. There was a discrete interkeratinocytic fluorescence at direct immunofluorescence but indirect immunofluorescence was negative. Anti-desmogleine 1 immunolabeling showed typical normal skin uptake and immunotransfer was negative. Digestive tract endoscopy and histopathology examination of the bowel specimens confirmed the diagnosis of Crohn's disease. Clinical manifestations improved dramatically with prednisone. DISCUSSION: This case of pyostomatitis-pyodermatitis vegetans involved several aspects rarely reported in the literature: a) the cutaneomucosal signs were inaugural; b) the association with Crohn's disease; c) the presence of lesions to the genital mucosa; d) the unusual localization of the inaugural skin manifestations. This clinical entity has now been clearly distinguished from pemphigus vegetans. There was however a long debate on the similar clinical, histological and even immunological expressions. We suggest that pyostomatitis-pyodermatitis vegetans belongs to the spectrum of neutrophilic dermatoses and other authors even propose it is a clinical form of pyoderma gangrenosum.