Conjuntivitis cicatrizal recalcitrante: Tratamiento de nueve pacientes con rituximab / Recalcitrant cicatrizing conjunctivitis. Treatment of nine patients with rituximab

La conjuntivitis cicatrizal es la consecuencia de distintas enfermedades oculares. Entre ellas, las más graves son el penfigoide cicatrizal y el síndrome de Stevens-Johnson crónico. El tratamiento de estas enfermedades con corticoides e inmunosupresores es habitualmente exitoso, pero unos pocos pacientes siguen un curso recalcitrante. En los últimos años se introdujo el uso de rituximab, asociado o no a gammaglobulina endovenosa, en forma abierta, para el control de la inflamación conjuntival. Describimos aquí el tratamiento de siete pacientes con penfigoide y dos con Stevens-Johnson recalcitrante, con rituximab. Ocho recibieron también gammaglobulina y todos alcanzaron la remisión de la actividad. Tres recayeron y recibieron dos o tres nuevos cursos de la medicación con mejoría sintomática. El rituximab probó ser una droga efectiva para el tratamiento de la conjuntivitis cicatrizal crónica recalcitrante.

Cicatrizing conjunctivitis is the final consequence of several diseases. The most severe among them are cicatricial pemphigoid and chronic Stevens-Johnson syndrome. Systemic immunosuppressive drugs and steroids are usually an effective approach to these diseases. However, a few patients follow a recalcitrant course unremitting to usual therapy. We describe the treatment with rituximab of seven patients with cicatricial pemphigoid and two with chronic Stevens-Johnson syndrome. Eight of them also received gammaglobulin and all achieved clinical remission. Three relapsed and required two or three new courses of rituximab with good control of disease activity. Rituximab proved to be an efficacious drug for chronic recalcitrant cicatrizing conjunctivitis.

Bronquiolitis obliterante secundaria a sindrome de stevens-johnson. Presentacion de 2 casos y revision de la literatura / Obliterans bronchiolitis secondary to stevens-johnson syndrome: case reports

Stevens-Johnson syndrome corresponds to a hypersensitivity reaction produced by various etiologies, for example exposure to drugs, microbial agents, or by an idiopathic cause. It is marked by an acute vesicular-bullous eruption, which affects the skin and mucous membranes, with systemic manifestations of variable severity, and it may present a fatal evolution. Stevens-Johnson syndrome can occasionally present chronic pulmonary complications, such as bronchiolitis obliterans; however, other etiologies are more frequent in our environment, for example severe pneumonia due to adenovirus. Our objective is to present two cases of bronchiolitis obliterans post Stevens-Johnson syndrome and to make a literature review.

El síndrome de Stevens-Johnson corresponde a una respuesta de hipersensibilidad producida por diversas etiologías, que incluyen exposición a drogas, agentes microbianos o idiopática. Se manifiesta por una erupción vesículo-bulosa aguda, que afecta la piel y las mucosas, con manifestaciones sistémicas de severidad variable, pudiendo presentar una evolución fatal. El síndrome de Stevens-Johnson puede presentar ocasionalmente complicaciones pulmonares crónicas, como bronquiolitis obliterante, siendo en nuestro medio más frecuente otras etiologías, como la observada luego de una neumonía grave por adenovirus. El objetivo es presentar dos casos de bronquiolitis obliterante post síndrome de Stevens-Johnson y hacer una revisión de la literatura.

Necrolisis epidérmica tóxica: un paradigma de enfermedad crítica / Toxic epidermal necrolysis: a paradigm of critical illness

RESUMEN La necrolisis epidérmica tóxica es una reacción cutánea adversa de tipo inmunológico secundaria en la mayor parte de los casos a la administración de un fármaco. La necrolisis epidérmica tóxica, el síndrome de Steven Johnson y el eritema exudativo multiforme forman parte del mismo espectro de enfermedad. La mortalidad de la necrolisis epidérmica tóxica es alrededor del 30%. La fisiopatología de la necrolisis epidérmica tóxica es semejante en muchos aspectos a la de las quemaduras dérmicas superficiales. La afectación mucosa del epitelio ocular y genital se asocia con secuelas graves si no se trata de forma temprana. Se acepta en general que los pacientes con necrolisis epidérmica tóxica son tratados mejor en unidades de grandes quemados, donde existe experiencia en el manejo de enfermos con pérdida cutánea extensa. El tratamiento es de soporte, eliminación y cobertura con derivados biosintéticos de la piel de las zonas afectadas, tratamiento de la afectación mucosa, y tratamiento inmunosupresor específico. De los tratamientos ensayados sólo se usa actualmente en la mayor parte de los centros la inmunoglobulina G y la ciclosporina A, aun cuando no existe evidencia sólida para recomendar ningún tratamiento específico. Entre los aspectos particulares del tratamiento de esta enfermedad se encuentra la prevención de secuelas relacionadas con la formación de sinequias, los cuidados oculares para prevenir secuelas graves que pueden conducir a la ceguera, y el tratamiento específico inmunosupresor. Un mejor conocimiento de los principios del manejo de la necrolisis epidérmica tóxica llevará a un mejor manejo de la enfermedad, a una mayor supervivencia y una menor prevalencia de las secuelas.

ABSTRACT Toxic epidermal necrolysis is an adverse immunological skin reaction secondary in most cases to the administration of a drug. Toxic epidermal necrolysis, Stevens-Johnson syndrome, and multiform exudative erythema are part of the same disease spectrum. The mortality rate from toxic epidermal necrolysis is approximately 30%. The pathophysiology of toxic epidermal necrolysis is similar in many respects to that of superficial skin burns. Mucosal involvement of the ocular and genital epithelium is associated with serious sequelae if the condition is not treated early. It is generally accepted that patients with toxic epidermal necrolysis are better treated in burn units, which are experienced in the management of patients with extensive skin loss. Treatment includes support, elimination, and coverage with biosynthetic derivatives of the skin in affected areas, treatment of mucosal involvement, and specific immunosuppressive treatment. Of the treatments tested, only immunoglobulin G and cyclosporin A are currently used in most centers, even though there is no solid evidence to recommend any specific treatment. The particular aspects of the treatment of this disease include the prevention of sequelae related to the formation of synechiae, eye care to prevent serious sequelae that can lead to blindness, and specific immunosuppressive treatment. Better knowledge of the management principles of toxic epidermal necrolysis will lead to better disease management, higher survival rates, and lower prevalence of sequelae.

Trimetroprim-sulfametoxazol: Un caso de Necrólisis Epidérmica Tóxica / Trimethoprim-sulfamethoxazole: A Case of Toxic Epidermal Necrolysis

Necrólisis Epidérmica Tóxica (NET) es una rara pero grave emergencia caracterizada por difusa exfoliación de la piel y las membranas mucosas debido a pérdida de la epidermis, mediada por respuesta inmune que puede llevar a sepsis y fallo ventilatorio. El Trimetropin-Sulfametoxazol es un antibiótico ampliamente utilizado que es causa de dicha entidad. El diagnóstico prematuro y el tratamiento agresivo es esencial para la reducción de los elevados niveles de morbilidad y mortalidad asociadas con esta enfermedad. Presentamos un caso que fue precipitado debido al uso de Trimetropin-Sulfametoxazol en un hombre de 22 años de edad...(AU)

Toxic Epidermal Necrolysis (TEN) is a rare but serious emergency characterized by diffuse exfoliation of the skin and mucous membranes due to loss of the epidermis, mediated by immune response that can lead to sepsis and ventilatory failure. Trimetropin-Sulfamethoxazole is a widely used antibiotic that causes this entity. Premature diagnosis and aggressive treatment is essential for reducing the high levels of morbidity and mortality associated with this disease. We present a case that was precipitated due to the use of Trimetropin-Sulfamethoxazole in a man of 22 years of age ... (AU)

Bronchiolitis Obliterans Associated with Stevens-Johnson Syndrome: A Case Report

We report a case of bronchiolitis obliterans associated with Stevens-Johnson syndrome. A 59-year-old man presented with respiratory distress that gradually worsened over 3 months. He had been diagnosed with Stevens-Johnson syndrome 3 months before admission. He had no history of previous airway disease. On physical examination, expiratory breathing sounds were not audible, and a chest X-ray revealed a hyperinflated lung. A pulmonary function test indicated a severe obstructive pattern. Computed tomography scans of inspiratory and expiratory phases of respiration showed oligemia and air trapping, and both were more prominent on expiration view than on inspiration view. The pathogenesis of bronchiolitis obliterans associated with Stevens-Johnson syndrome is largely unknown.

Ocular manifestation, complications and aetiological factors in Stevens Johnson syndrome/toxic epidermal necrolysis

To describe the ocular manifestations of Stevens-Johnson Syndrome/Toxic Epidermal Necrosis among inpatients at a tertiary care hospital. The retrospective observational descriptive study was carried out at the Aga Khan University Hospital, Karachi, and comprised data on age, gender, aetiology and ocular findings related to patients diagnosed with Stevens-Johnson Syndrome/Toxic Epidermal Necrosis between January 2000 and December 2011. SPSS 19 was used for statistical analysis. There were 87 patients; 48[55.2%] of them being males. The overall mean age was 33.2 +/- 22.2 years, ranging from 1 month to 84 years. The most common aetiology was idiopathic 20[23%] followed by non-steroidal inflammatory drugs and anti-epileptics 11[12.6%] each. Besides, 84[96.6%] patients had oral mucosal involvement whereas 45[51.7%] had ocular and 27[31.0%] had genital-mucosal involvement. Glassroding was performed in 16[18.4%] patients due to minor conjunctival adhesions. Ocular manifestations of varying severity were frequent, with drugs being the most common aetiology

Caso 13 / Case 13

Homem de 34 anos de idade foi atendido na Unidade Básica de Saúde comodinofagia, eritema conjuntival, mal-estar geral, mialgia e temperatura corpórea de 39°C, tendo sido tratado empiricamente com dipirona e amoxicilina. Relatou picadas de carrapatos nesse mesmo dia. Poucas horas após o uso da medicação prescrita, evoluiu com erupção eritematoviolácea, placas confluentes e vesículas sero-hemorrágicas em face, tronco e membros, com acometimento de mucosa oral e peniana. Iniciou há dois meses o uso de alopurinol e atenolol para tratamento de, respectivamente, hiperuricemia e hipertensão arterial sistêmica.

Necrólisis epidérmica tóxica asociada a infección aguda por Mycoplasma pneumoniae / Toxic epidermal necrolysis associated with acute infection by Mycoplasma pneumoniae

El eritema multiforme, el síndrome de Stevens-Johnson y la necrólisis epidérmica tóxica representan diferentes manifestaciones de un mismo espectro de graves reacciones cutáneas idiosincrásicas a fármacos y, en menor medida, están asociados a agentes infecciosos. De estos últimos, Mycoplasma pneumoniae es uno de los más frecuentes. Se presenta el caso de una niña de 5 años, con una necrólisis epidérmica tóxica asociada a infección aguda por Mycoplasma pneumoniae, que comenzó con un cuadro febril acompañado de un exantema generalizado y compromiso de todas las mucosas. Se obtuvo serología IgM positiva para Mycoplasma pneumoniae y una biopsia inicial compatible con eritema multiforme mayor. La paciente fue tratada con corticosteroides, gammaglobulina intravenosa, plasmaféresis y estrictos cuidados para la prevención de sobreinfección y posibles secuelas. Después de 31 días de internación fue dada de alta hospitalaria.

Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis represent different manifestations of the same spectrum of severe idiosyncratic cutaneous reactions to drugs and to a lesser extent are associated with infectous agents. Among these, Mycoplasma pneumoniae is one of the most frequent. We report the case of a female patient aged 5 years, with a toxic epidermal necrolysis associated with Mycoplasma pneumoniae infection, which begins with a fever accompanied by a generalized rash with involvement of the mucous membranes. IgM serology for Mycoplasma pneumoniae was positive and initial biopsy was compatible with erythema multiforme major. The patient was treated with corticosteroids, intravenous immunoglobulin, plasmapheresis and strict care to prevent superinfection and sequels. After 31 days of hospitalization the patient was discharged from hospital.

Effect of Age and Early Intervention with a Systemic Steroid, Intravenous Immunoglobulin or Amniotic Membrane Transplantation on the Ocular Outcomes of Patients with Stevens-Johnson Syndrome

PURPOSE: This retrospective observational case series of fifty-one consecutive patients referred to the eye clinic with acute-stage Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) from 1995 to 2011 examines the effect of early treatment with a systemic corticosteroid or intravenous immunoglobulin (IVIG) on the ocular outcomes in patients with SJS or TEN. METHODS: All patients were classified by age (18 years) and analyzed by treatment modality and early intervention with systemic corticosteroids (< or =5 days), IVIG (< or =6 days), or amniotic membrane graft transplantation (AMT) (< or =15 days). The main outcomes were best-corrected visual acuity (BCVA) in logarithm of the minimum angle of resolution (logMAR) and ocular involvement scores (OIS, 0-12), which were calculated based on the presence of superficial punctate keratitis, epithelial defect, conjunctivalization, neovascularization, corneal opacity, keratinization, hyperemia, symblepharon, trichiasis, mucocutaneous junction involvement, meibomian gland involvement, and punctal damage. RESULTS: The mean logMAR and OIS scores at the initial visit were not significantly different in the pediatric group (logMAR = 0.44, OIS = 2.76, n = 17) or the adult group (logMAR = 0.60, OIS = 2.21, n = 34). At the final follow-up, the logMAR and OIS had improved significantly in the adult group (p = 0.0002, p = 0.023, respectively), but not in the pediatric group. Early intervention with IVIG or corticosteroids significantly improved the mean BCVA and OIS in the adult group (p = 0.043 and p = 0.024, respectively for IVIG; p = 0.002 and p = 0.034, respectively for corticosteroid). AMT was found to be associated with a significantly improved BCVA or OIS in the late treatment group or the group with a better initial OIS (p = 0.043 and p = 0.043, respectively for BCVA; p = 0.042 and p = 0.041, respectively for OIS). CONCLUSIONS: Our findings suggest that patients with SJS or TEN who are aged 18 years or less have poorer ocular outcomes than older patients and that early treatment with steroid or immunoglobulin therapy improves ocular outcomes.

Síndrome de Stevens-Johnson: presentación de un caso / Stevens-Johnson syndrome: a case presentation

El síndrome de Stevens-Johnson, también llamado eritema multiforme mayor, se caracteriza por una serie de lesiones cutáneas de diversa morfología, es una grave lesión en la cual están afectadas al menos dos membranas mucosas y la piel. La patogenia de esta enfermedad es desconocida aunque generalmente se considera que es provocada por una reacción de hipersensibilidad a drogas, infecciones y exposición a sustancias tóxicas. Caso clínico: esta afección fue diagnosticada en un niño africano de nueve años de edad. El niño desarrolló una severa y florida forma de ulceración mucocutánea, manifestaciones orales y oculares serias, marcados trastornos sistémicos y deshidratación. Se realizó una amplia revisión bibliográfica sobre el tema donde fueron verificadas las manifestaciones clínicas, evolución y el tratamiento de esta enfermedad.

Stevens-Johnson syndrome also called erythema multiforme major, it is characterized by a series of cutaneous lesions with different morphology, and it is a serious lesion in which two mucous membranes and skin are affected at least. The pathogeny of this disease is ignored although it is generally considered that it is caused by a drug hypersensitivity reaction, infections and exposure to toxic substances. Clinical case: this affection was diagnosed in a nine year-old African boy. The boy developed a severe and florid form of mucocutaneous ulceration, serious oral and ocular manifestations, marked systemic disorders and dehydration. A wide bibliographical review on the topic was carried out where clinical manifestations, evolution and treatment of this disease were verified.

Boston ocular surface prosthesis: An Indian experience.

Context: Boston ocular surface prosthesis (BOSP) is a scleral contact lens used in the management of patients who are rigid gas permeable (RGP) failures as with corneal ectasias such as keratoconus and in those patients who have ocular surface disease such as Stevens–Johnson syndrome (SJS). Aim: To report utilization of BOSP in a tertiary eye care center in India. Materials and Methods: We retrospectively reviewed charts of 32 patients who received BOSP from July 2008 to May 2009. Indications for fitting these lenses, improvement in visual acuity (VA) before and after lens fitting and relief of symptoms of pain and photophobia were noted. Paired t-test was used for statistical analysis using SPSS version 16.0 for Windows. Results: Thirty-two patients (43 eyes) received these lenses. These consisted of 23 eyes of 17 patients who failed RGP trials for irregular astigmatism and corneal ectasia such as keratoconus and post radial keratotomy and scar and 20 eyes of 15 patients with SJS. Mean age of RGP failures was 27.94 years. Pre- and post-BOSP wear mean LogMAR VA was 1.13 and 0.29, respectively, in RGP failures. The P value was statistically significant (P < 0.001). In patients with SJS, LogMAR VA was 0.84 ± 0.92 before and 0.56 ± 0.89 after lens wear. The P value was statistically significant (P < 0.001). VA improved by >2 lines in 7/20 eyes (35%) with SJS, with improvement in symptoms. Conclusion: BOSP improves VA in patients who have irregular astigmatism as in ectasias and RGP failures and improves vision and symptoms in patients with SJS.

Mucous membrane grafting for the post-Steven–Johnson syndrome symblepharon: A case report.

An 18-year-old woman was referred with late sequelae of chloroquine-induced Steven–Johnson syndrome. At the time of presentation, the symblepharon was involving the upper lids to almost the whole of the cornea, and part of the lower bulbar conjunctiva with the lower lid bilaterally. Other ocular examinations were not possible due to the symblepharon. B-scan ultrasonography revealed acoustically clear vitreous, normal chorioretinal thickness, and normal optic nerve head, with an attached retina. Conjunctivo-corneal adhesion released by superficial lamellar dissection of the cornea. Ocular surface reconstruction was carried out with a buccal mucous membrane. A bandage contact lens was placed over the cornea followed by the symblepharon ring to prevent further adhesion. The mucosal graft was well taken up along with corneal re-epithelization. Best corrected visual acuity of 20/120 in both sides after 1 month and 20/80 after 3 months was achieved and maintained till the 2.5-year follow-up.

Lente de contato escleral na reabilitação ocular de pacientes com síndrome de Stevens-Johnson / Scleral contact lens for ocular rehabilitation in patients with Stevens-Johnson syndrome

OBJETIVO: Avaliar a eficácia do uso das lentes de contato esclerais no manejo das sequelas oculares de pacientes portadores de síndrome de Stevens-Johnson. MÉTODOS: Foram avaliados, retrospectivamente, pacientes com sequelas oculares da síndrome de Stevens-Johnson que iniciaram o uso de lente de contato escleral. Os pacientes foram submetidos a avaliação subjetiva dos sintomas através de um questionário; exame oftalmológico (medida da acuidade visual, biomicroscopia, coloração da superfície ocular com colírio de fluoresceína, teste de Schirmer). RESULTADOS: Dez olhos de 7 pacientes foram analisados. A acuidade visual dos pacientes variou de movimentos de mão a 20/25. Todos os pacientes apresentavam algum grau de opacidade corneal e simbléfaro leve. A lente de contato escleral foi adaptada com sucesso em 90 por cento dos olhos. Em todos estes casos os pacientes referiram melhora dos sintomas e da visão. Com relação aos achados biomicroscópicos observou-se melhora da hiperemia conjuntival e da ceratite, e diminuição da secreção mucosa em 90 por cento dos olhos. CONCLUSÕES: Foi possível uma adaptação bem sucedida da lente de contato escleral em grande parte dos pacientes, com melhora dos sintomas e da acuidade visual provavelmente consequentes à melhora da regularização da superfície ocular. As lentes de contato esclerais representam uma importante e acessível alternativa para a redução da limitação ocasionada pelos danos sequelares da síndrome de Stevens-Johnson.

PURPOSE: To evaluate the efficacy of scleral contact lenses use on the management of ocular sequelae from Stevens-Johnson syndrome patients. METHODS: In a retrospective study, patients who suffered sequelae of Stevens-Johnson syndrome and started the use of scleral contact lenses were followed. Patients were submitted to an evaluation of symptoms through a questionnaire; ophthalmologic exam (visual acuity measurement, biomicroscopy, ocular surface staining with fluorescein drops, Schirmer test). RESULTS: Ten eyes of seven patients were analyzed. Visual acuity varied from hand movements to 20/25. All patients presented some degree of corneal opacity and slight symblepharon. In patients whose adaptation to scleral contact lenses was successful (90 percent), they all refered improvement of symptoms and sight. As for the biomicroscopic findings it was observed an improvement of conjunctival hyperemia and keratitis and a reduction of the mucous secretion in 90 percent the cases. CONCLUSIONS: A successful adaptation to scleral contact lenses was feasible on most patients, with relief of symptoms and better visual acuity, probably due to regularization of the surface. Scleral contact lenses represent an important and accessible alternative to reduce the limitations inferred by the damages from Stevens-Johnson syndrome.

Coin-shaped epithelial lesions following an acute attack of erythema multiforme minor with confocal microscopy findings.

We report an interesting ocular finding of bilateral multiple coin-shaped epithelial lesions along with the confocal microscopy findings in a patient following an acute attack of erythema multiforme (EM) minor. A 30-year-old male presented with a history of watering and irritation in both eyes of three days duration. He was diagnosed to have EM minor and was on oral acyclovir. Slit-lamp examination revealed multiple coin-shaped epithelial lesions. Confocal microscopy showed a corresponding conglomerate of hyper-reflective epithelial lesions. The corneal lesions resolved over six weeks with oral steroids and acyclovir. An immunological mechanism is suspected.

Estudo dos achados oculares na síndrome de Stevens-Johnson em pacientes de centro de referência de atendimento terciário / Study of the ocular findings in Stevens-Johnson syndrome patients from a tertiary ophthalmologic center

OBJETIVO: Avaliar a epidemiologia, os possíveis fatores desencadeantes, complicações encontradas e as variedades de tratamentos administrados a um grupo de pacientes com complicações oculares secundárias à síndrome de Stevens-Johnson. MÉTODOS: Realizado estudo prospectivo, série de casos com 22 pacientes portadores de síndrome de Stevens-Johnson. Os pacientes foram estudados seguindo um protocolo com o objetivo de se buscar uma história detalhada sobre a doença, suas manifestações sistêmicas e oftalmológicas, bem como o tratamento realizado, dando ênfase aos possíveis fatores desencadeantes. RESULTADOS: Foram avaliados 22 pacientes com síndrome de Stevens-Johnson. Quinze pacientes (68 por cento) eram do sexo feminino e 7 (32 por cento) do sexo masculino. Dez pacientes eram brancos (45,4 por cento), 9 pardos (22 por cento), 2 negros (9 por cento) e 1 amarelo (4,5 por cento). A média de idade foi de 27,1 anos (variação entre 8 e 62 anos). A associação com drogas foi o principal fator etiológico. Em 20 pacientes (90,9 por cento) o desenvolvimento da doença esteve associado ao uso de medicações, 1 (4,5 por cento) por infecção herpética cutânea e 1 (4,5 por cento) idiopático. A dipirona (36,3 por cento) foi o agente mais associado à síndrome de Stevens-Johnson seguido por anticonvulsivantes (22,7 por cento), anti-inflamatórios não hormonais (13,6 por cento), sulfonamidas (9,0 por cento), penicilinas (4,5 por cento), espironolactona (4,5 por cento) e anticoncepcional injetável (dihidroprogesterona e estradiol) (4,5 por cento). Vinte e um pacientes (95,4 por cento) desenvolveram complicações oculares e 16 pacientes (72,7 por cento) foram submetidos a procedimentos cirúrgicos oftalmológicos. CONCLUSÃO: Os resultados observados neste estudo mostram aspectos epidemiológicos importantes da síndrome de Stevens-Johnson em nosso meio, principalmente em relação à idade, etiologia e complicações.

PURPOSE: To evaluate the epidemiology, possible etiologic factors, complications and treatment of a group of patients with ocular complications secondary to Stevens-Johnson syndrome. METHODS: Twenty-two consecutive patients with Stevens-Johnson syndrome were studied. The patients were examined according to the following protocol: identification, previous clinical history, systemic and ophthalmologic manifestations and possible etiologic factors. RESULTS: A total of 22 patients with Stevens-Johnson syndrome were identified. Fifteen patients (68 percent) were female, 7 (32 percent) male. Ten patients were caucasian (45.4 percent), 9 brownish (22 percent), 2 black (9 percent) and 1 yellow (4.5 percent). Mean age was 27.1 (8 to 62). Medications were the most commonly identified etiologic factor (90.9 percent), followed by skin herpetic infection (4.5 percent) and idiopathic (4.5 percent). Dipirone (36.3 percent) was the most frequently identified agent, followed by seizure medications (22.7 percent), non-steroidal anti-inflammatory drugs (13.6 percent), sulfonamides (9.0 percent), penicillin (4.5 percent), spironolactone (4.5 percent) and dihydroprogestagen and stradiol (4.5 percent). Twenty-one patients (95.4 percent) had ophthalmologic complications and sixteen patients (72.7 percent) underwent ophthalmologic surgical procedures. CONCLUSIONS: The results of this study show important epidemiological aspects of Stevens-Johnson syndrome in our environment, specially related to age, etiology and ocular complications.

Bilateral conjunctival retention cysts in the aftermath of Stevens-Johnson syndrome.

In this case report, we describe the rare occurrence of bilateral conjunctival retention cysts in a child with Stevens-Johnson syndrome. The case was managed conservatively as there were no functional or cosmetic problems.

The Effect of In Vivo Grown Corneal Epithelium Transplantation on Persistent Epithelial Defects with Limbal Stem Cell Deficiency

We report our experience with corneal epithelium, grown in vivo, transplantation in three patients with persistent epithelial defect (PED). The three patients had ocular surface disease unresponsive to standard treatments and were therefore chosen for transplantation. They underwent transplantation of epithelial sheets, grown in vivo, to the most affected eye. In vivo cultivation was carried out in the cornea of a living related donor. After epithelialization was completed, the epithelium grown on an amniotic membrane was harvested gently; it was then transplanted into the patient's eye after debridement of fibrovascular tissue. The cultivated epithelium was completely epithelialized by 2 weeks; it was well-differentiated with well-formed hemidesmosome. On immunohistochemical staining, p63, connexin 43, and Integrin beta4 were expressed in the cells on the epithelial sheet. The PED was covered completely and maintained for 4 weeks in all cases. However, corneal erosion recurred after 5 weeks in two cases. This novel technique demonstrates the corneal epithelial cells can be expanded in vivo successfully on denuded amniotic membrane of a healthy cornea and harvested safely. A corneal epithelial sheet, grown in vivo, can be transplanted to treat eye with a severe ocular surface disease, such as total limbal deficiency.

Síndrome de Stevens-Johnson: relato de caso e revisão da literatura / Stevens-Johnson syndrome: case report and literature review

As reações cutâneas às drogas são as que geralmente ne­cessitam de internação hospitalar, por vezes em unidade de terapia intensiva ou de queimados, com observação minuciosa dos sinais vitais e função de órgãos internos. A síndrome de Stevens-Johnson é uma rara e grave reação cutânea e muco­sa, causada comumente por drogas, como as sulfonamidas, AINES, agentes anticonvulsivantes e alopurinol. Neste artigo, nós abordamos as características clínicas da síndrome de Ste­vens-Johnson, com o objetivo de facilitar o seu reconhecimen­to e tratamento de forma precoce.