Stevens-Johnson syndrome during an immunosuppressive therapy with cyclophosphamide and prednisone

Corticosteroids are often recommended for the treatment of severe Stevens-Johnson syndrome. We report about a 12 years old boy, who developed this syndrome during an immunosuppressive therapy with prednisone and cyclophosphamide for a frequent relapsing, steroid-dependent nephrotic syndrome. Most of the commonly mentioned etiologic factors for erythema exudativum multiforme could be excluded. The usefulness of corticosteroids for the therapy seems to be doubtful.     

Use of intravenous immunoglobulin in toxic epidermal necrolysis and Stevens-Johnson syndrome

Toxic epidermal necrolysis and Stevens-Johnson syndrome are described as variants of the same disease with distinct severity and constitute the most frequent cutaneous reactions in children, causing considerable morbidity. Several reports support the use of intravenous immunoglobulin therapy in these entities. We report the cases of two patients, one with toxic epidermal necrolysis and the other with Stevens-Johnson syndrome, in whom immunoglobulin treatment was successfully used. We also reviewed the outcomes of 13 patients with toxic epidermal necrolysis and Stevens-Johnson syndrome in the previous 10 years in the Hospital Infantil de Mexico, in whom conventional treatment was used.     

Unusual manifestation of Stevens-Johnson syndrome involving the respiratory and gastrointestinal tract.

There are many recognized acute complications of Stevens-Johnson syndrome: however, long-term sequelae involving the respiratory and gastrointestinal tracts are exceedingly rare. We present an 8-year-old girl who developed esophageal stricture and chronic lung disease while recovering from Stevens-Johnson syndrome and discuss these complications with possible mechanisms for their occurrence.     

Syndrome de Stevens-Johnson sévère chez l''enfant : à propos de 4 observations

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.     

Toxic epidermal necrolysis in children--an update

Toxic epidermal necrolysis (TEN) is a relatively rare disease, characterized by generalized erythema, bulla formation, and exfoliation of the epidermis that resembles scalding. It is usually drug-induced and mediated by immune reactions of several types. TEN has to be differentiated from erythema multiforme and Stevens-Johnson syndrome. TEN bears a life-threatening potential and a risk for significant complications such as dehydration; secondary infections; pulmonary, gastrointestinal, and renal involvement; and late scarring with cosmetic and functional implications. Therapeutic interventions include admission in a burn center unit, systemic antibiotics and corticosteroids, topical therapy with antibacterial agents and biological dressings, and supportive measures.     

Acute vanishing bile duct syndrome after ibuprofen therapy in a child

We report the case of a 10 year-old girl who had Stevens-Johnson syndrome and cholestasis after ibuprofen therapy. Liver histology was compatible with vanishing bile duct syndrome. She received ursodeoxycholic acid, and liver tests normalized within 7 months. This report confirms that ibuprofen may induce acute vanishing bile duct syndrome.     

PFAPA syndrome in siblings. Is there a genetic background?

“PFAPA syndrome” is an autoinflammatory entity composed of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. There have been many reports of children with the disease, but only occasionally have been described in siblings, and no specific genetic mutation has been determined yet. Corticosteroids are the mainstay in the treatment of the acute attacks. The role of surgery in long-term follow-up (tonsillectomy with or without adenoidectomy) is controversial. We report two brothers affected with the syndrome, in whom corticosteroids as the only treatment led to an improvement. A genetic work-up was performed, making very unlikely other possible syndromes of recurrent fever. Conclusion: PFAPA syndrome is the most common recurrent periodic fever disorder described in childhood. Its genetic background has not been elucidated yet. Our contribution with two siblings affected with PFAPA syndrome further support the genetic basis for the entity.     

Mycoplasma pneumoniae and atypical Stevens-Johnson syndrome: a case series

Mycoplasma pneumoniae is a common cause of community-acquired respiratory illness in the adolescent population. Stevens-Johnson syndrome is an extrapulmonary manifestation that has been associated with M. pneumoniae infections. Three adolescent males presented within a 1-month period with M. pneumoniae respiratory illnesses and severe mucositis but without the classic rash typical of Stevens-Johnson. Diagnosis was facilitated by the use of a polymerase chain reaction-based assay. This case series highlights the potential for M. pneumoniae-associated Stevens-Johnson syndrome to occur without rash and supports the use of polymerase chain reaction for early diagnosis.     

Cutaneous hypersensitivity reactions due to thiacetazone in the treatment of tuberculosis in Zambian children infected with HIV-I.

Tuberculosis is one of the most common infections in Zambian adults and children infected with HIV. In Africa, cutaneous hypersensitivity reactions attributed to thiacetazone during treatment of tuberculosis in adults infected with HIV-I have been well documented. This study monitored adverse drug reactions during treatment for tuberculosis over an 18 month period (1 April 1990 to 31 October 1991) in 237 children with a clinical diagnosis of tuberculosis (125 boys and 112 girls; 88/237 (37%) infected with HIV-I) and 242 control children (149 boys and 93 girls; 26/242 (11%) infected with HIV-I). Twenty two (9%) of the 237 children with tuberculosis developed hypersensitivity skin reactions during the course of treatment. Adverse skin reactions were seen more often in children infected with HIV than in those who were not (odds ratio 11.65, 95% confidence interval 3.07 to 34.88). These represented 19 (21%) of 88 children infected with HIV and three (2%) of 149 children not infected with HIV. These skin reactions occurred after a period of treatment ranging between two and four weeks among 14 children receiving the HST (isoniazid, streptomycin, thiacetazone) regimen and eight children receiving the HSTR (isoniazid, streptomycin, thiacetazone, rifampicin) regimen. Twelve (55%) of the 22 children who reacted adversely to treatment developed the Stevens-Johnson syndrome. All 12 of these children with the Stevens-Johnson syndrome were infected with HIV. The mortality among these children who developed the Stevens-Johnson syndrome was 91% (11 of 12 died within three days of the onset of the reaction). No further reactions were observed in the 11 children who recovered from the cutaneous hypersensitivity reactions after thiacetazone was discontinued over a period of six months of further treatment of tuberculosis. The results of this study were in part responsible for the recommendations put forward by the World Health Organization to avoid the use of thiacetazone in the treatment of tuberculosis in children infected with HIV.     

Severe atypical Lemierre syndrome caused by methicillin-sensitive Staphylococcus aureus: Two pediatric case reports

BackgroundLemierre syndrome is typically associated with ear, nose, and throat (ENT) infections caused by Fusobacterium necrophorum. Since 2002, cases of atypical Lemierre-like syndrome secondary to Staphylococcus aureus have been reported.CasesWe report two pediatric cases of atypical Lemierre syndrome with a similar presentation: exophthalmia, absence of pharyngitis, metastatic lung infection, and intracranial venous sinus thrombosis. Both patients had a favorable outcome following treatment with antibiotics, anticoagulation, and corticosteroids.ConclusionRegular therapeutic monitoring of antibiotic levels helped to optimize antimicrobial treatment in both cases.     

THE STEVENS-JOHNSON SYNDROME AND LONG-ACTING SULPHONAMIDES

The authors have summarized the clinical details of 8 children who developed an illness with the characteristics of the ‘Stevens-Johnson’ syndrome, after they had taken long-acting sulphonamides. The detailed records of each case are available by application to the authors.     

Severe Mycoplasma pneumoniae-associated mucositis treated with immunoglobulins

Mycoplasma pneumoniae-associated mucositis (MPAM), previously labelled as atypical Stevens-Johnson syndrome (SJS), SJS with minimal or no skin manifestations, is a rare non-respiratory manifestation of Mycoplasma pneumoniae infection. The nineteen cases described so far in children and young adults were characterized by a high male gender prevalence (16/19) and a good response to appropriate antibiotic treatment and supportive care in the majority of patients. We describe a case of MPAM in a previously healthy girl, who improved after a 0.5 g/kg daily dose of intravenous immunoglobulins (IVIG) for four consecutive days, after traditional therapy had failed. CONCLUSION: The successful treatment with IVIG described in this report suggests that, where appropriate antibiotic and supportive therapy fails to improve the clinical course of severe MPAM, IVIG treatment is worth considering.     

Disseminated vaccinia false alarm

We report the case of a 6-year-old girl who presented in December 2002 with fever and a vesicular rash. Initially she was diagnosed with Stevens-Johnson syndrome. The differential diagnosis was expanded, however, when an exposure to a person who was believed to be vaccinated recently against smallpox was revealed. We describe the sequence of events that ensued and the workup for a possible case of disseminated virus.     

Management of Stevens-Johnson syndrome and toxic epidermal necrolysis in children

A retrospective analysis of 21 consecutive patients hospitalized with either Stevens-Johnson syndrome or toxic epidermal necrolysis was carried out to assess morbidity and mortality rates and to establish the value of a specific management practice. Fourteen children with Stevens-Johnson syndrome and seven with toxic epidermal necrolysis were cared for at the Children's Memorial Hospital, Chicago, between 1978 and 1988. All were managed in a well-staffed medical ward or, when necessary, in the pediatric intensive care unit. Supportive measures included reverse barrier isolation, intravenous fluids and nutritional support, meticulous skin care, early detection and treatment of infection, and daily ophthalmologic examination. No patient was treated with systemic steroids. The mortality rate was zero. Eye complications, consisting of dry eyes or mild chronic symblepharon, were the most significant long-term sequelae.     

Mycoplasma pneumoniae infection complicated by pneumomediastinum and severe mucositis

We report an 8-year-old boy with Mycoplasma pneumoniae respiratory infection complicated by pneumomediastinum and severe oral and conjunctival mucositis. M. pneumoniae-associated mucositis is distinct from the Stevens-Johnson syndrome. There are no skin lesions and it improves promptly with antibiotics. Spontaneous pneumomediastinum usually only requires rest, analgesia and management of the underlying condition.     

Familial Evans syndrome: a report of an affected sibship.

PURPOSE: This report describes the clinical course of three siblings, all of whom had Evans syndrome in childhood. PATIENTS: The coexistence of autoimmune hemolytic anemia and thrombocytopenia, in the absence of a known underlying cause, led to the diagnosis of Evans syndrome in a 4-month-old girl and subsequently in her two brothers when they were 4 and 13 years old. RESULTS: The 4-month-old girl had a life-threatening relapsing course unresponsive to corticosteroids, intravenous gamma-globulin, thymectomy, and cyclophosphamide. She eventually responded to splenectomy. Her two brothers had milder disease that responded to corticosteroids. Cytogenetic analyses revealed the presence of a familial Y;15 translocation in all three children and their father. CONCLUSION: There are few reported cases of familial Evans syndrome, and they are usually associated with an inherited congenital abnormality. We report the unusual finding of three siblings with the disease and no known congenital abnormality.     

Carbohydrate-Deficient Glycoprotein Syndrome-Associated Pericardial Effusion Treated with Corticosteroids and Salicylic Acid

We describe an infant with a persistent pericardial effusion who was diagnosed with carbohydrate-deficient glycoprotein syndrome (CDGS)-Ia. She was born with mild dysmorphic features and common cardiac abnormalities. However, she re-presented at 2.5 months of age with a pericardial effusion. We decided to embark on a therapeutic trial of corticosteroids and salicylic acid therapy in an attempt to avoid pericardectomy. After 3 weeks of medical treatment the effusion resolved. This experience allows us to propose that medical management with corticosteroids and salicylic acid can be considered as an alternative to surgical therapy for CDGS-I patients with persistent pericardial effusions.     

Cushing syndrome with secondary adrenal insufficiency from concomitant therapy with ritonavir and fluticasone

We present 2 cases of Cushing syndrome with secondary adrenal insufficiency from concomitant use of ritonavir and inhaled corticosteroids in children with human immunodeficiency virus infection. These cases highlight the need for special consideration when treatment with an inhaled/intranasal corticosteroid is indicated in children receiving antiretroviral therapy.     

Obraz kliniczny zespołu Sweeta u 23-miesięcznej dziewczynki – opis przypadku

Sweet's syndrome is a rare acute febrile neutrophilic dermatosis, presenting with tender erythematous skin lesions, fever, leukocytosis with neurtophilia and diffuse neutrophilic infiltrate in the skin. We present the case of a 23-month-old girl with erythematous-nodular skin lesions appearing during febrile infection. We found all diagnostic criteria of Sweet's syndrome and excellent response to the treatment with systemic corticosteroids.     

Double-blind placebo-controlled trial of corticosteroids in children with postpericardiotomy syndrome

Summary The objective of this study was to assess the efficacy of corticosteroids in hastening the recovery of children with postpericardiotomy syndrome, using a randomized double-blind placebo-controlled trial in a tertiary care referral center for pediatric cardiology and cardiac surgery. Twenty-one children, 6 months of age or older (mean age 3.9 years) with postpericardiotomy syndrome following open or closed heart surgery were administered either prednisone 2 mg/kg/day reducing to zero over 14 days (n=12) or placebo (n=9). Progress was monitored by daily clinical assessment and alternate day cross-sectional echocardiograms. The primary measures of efficacy were the number of patients in remission at 72 h and at 1 week. No difference in remission rates were found at 72 h, but at 1 week significantly more children treated with prednisone were in remission (placebo 3/9; prednisone 10/12,p=0.03). A trend to faster resolution of all symptoms and signs was seen in the prednisone-treated group but this was not associated with earlier hospital discharge. Enlargement of pericardial effusion was seen in two children treated with steroids. No complications of treatment were encountered. Prednisone hastens the recovery of children with postopericardiotomy syndrome. Pericardial effusions may increase in size despite the use of corticosteroids.Presented in part at the Society for Pediatric Research, New Orleans, May 1991.