Exfoliative Skin Research Articles

Spectrum of Primary Immunodeficiency Disorders: Single Centre Experience in Capital City of Saudi Arabia

RationaleTo report our local experience with primary immunodeficiency disorders (PID): clinical features, management, and outcome.MethodsWe identified children diagnosed with PID in our centre from 2001 to 2005. Demographic data, time and mode of presentation, family history and outcome were evaluated by chart review.ResultsEleven patients were diagnosed with PID. Seven had severe combined immunodeficiency disease (SCID) (63.6%) (4 males) with a median age of 3 months (Range 0.5 - 4 months). Four had (T-, B-, NK+), 2 with (T-, B+, NK-), and one with omenn's syndrome. All 7 patients are products of consanguineous marriages. Presentation at diagnosis included: diffuse exfoliative erythematous skin rash in 4 patients, recurrent chest infections and diarrhea in 3, oral thrush in one, discharging ears in 2, and necrotic lip and eyelid ulceration in one. Two patients had undergone successful bone marrow transplantation (BMT), and one is under work up for BMT. The remaining 4 patients were not candidates for BMT because of severe disseminated systemic infections that led to death.Two twin brothers were diagnosed with hypogamaglobulimemia [2/11 (18.2%)]. One presented at age 4 years with brain abscess resistant to antibiotics, and the other was diagnosed upon screening.Two brothers were diagnosed with chronic granulomatous disease [2/11 (18.2%]. One presented with pulmonary aspergillosis, and the other was diagnosed upon screening.ConclusionUnlike the international classification, our local experience indicates that SCID is a more predominant form of PID than hypogamaglobulimemia. High consanguineous marriage rate in our community is a likely explanation. RationaleTo report our local experience with primary immunodeficiency disorders (PID): clinical features, management, and outcome. To report our local experience with primary immunodeficiency disorders (PID): clinical features, management, and outcome. MethodsWe identified children diagnosed with PID in our centre from 2001 to 2005. Demographic data, time and mode of presentation, family history and outcome were evaluated by chart review. We identified children diagnosed with PID in our centre from 2001 to 2005. Demographic data, time and mode of presentation, family history and outcome were evaluated by chart review. ResultsEleven patients were diagnosed with PID. Seven had severe combined immunodeficiency disease (SCID) (63.6%) (4 males) with a median age of 3 months (Range 0.5 - 4 months). Four had (T-, B-, NK+), 2 with (T-, B+, NK-), and one with omenn's syndrome. All 7 patients are products of consanguineous marriages. Presentation at diagnosis included: diffuse exfoliative erythematous skin rash in 4 patients, recurrent chest infections and diarrhea in 3, oral thrush in one, discharging ears in 2, and necrotic lip and eyelid ulceration in one. Two patients had undergone successful bone marrow transplantation (BMT), and one is under work up for BMT. The remaining 4 patients were not candidates for BMT because of severe disseminated systemic infections that led to death.Two twin brothers were diagnosed with hypogamaglobulimemia [2/11 (18.2%)]. One presented at age 4 years with brain abscess resistant to antibiotics, and the other was diagnosed upon screening.Two brothers were diagnosed with chronic granulomatous disease [2/11 (18.2%]. One presented with pulmonary aspergillosis, and the other was diagnosed upon screening. Eleven patients were diagnosed with PID. Seven had severe combined immunodeficiency disease (SCID) (63.6%) (4 males) with a median age of 3 months (Range 0.5 - 4 months). Four had (T-, B-, NK+), 2 with (T-, B+, NK-), and one with omenn's syndrome. All 7 patients are products of consanguineous marriages. Presentation at diagnosis included: diffuse exfoliative erythematous skin rash in 4 patients, recurrent chest infections and diarrhea in 3, oral thrush in one, discharging ears in 2, and necrotic lip and eyelid ulceration in one. Two patients had undergone successful bone marrow transplantation (BMT), and one is under work up for BMT. The remaining 4 patients were not candidates for BMT because of severe disseminated systemic infections that led to death. Two twin brothers were diagnosed with hypogamaglobulimemia [2/11 (18.2%)]. One presented at age 4 years with brain abscess resistant to antibiotics, and the other was diagnosed upon screening. Two brothers were diagnosed with chronic granulomatous disease [2/11 (18.2%]. One presented with pulmonary aspergillosis, and the other was diagnosed upon screening. ConclusionUnlike the international classification, our local experience indicates that SCID is a more predominant form of PID than hypogamaglobulimemia. High consanguineous marriage rate in our community is a likely explanation. Unlike the international classification, our local experience indicates that SCID is a more predominant form of PID than hypogamaglobulimemia. High consanguineous marriage rate in our community is a likely explanation.

Morbidity and Mortality of Mucocutaneous Diseases in the Pediatric Population at a Tertiary Care Center

Toxic epidermal necrolysis (TEN) is a life threatening desquamating disease that is often an adverse reaction to drugs. Because mortality is so high, up to 30% nationally, and the morbidity significant, these cases are managed in burn centers. This study was conducted to evaluate what drugs were given to children who developed exfoliating skin disease and to identify the complications that these patients suffered. Thirty-two pediatric cases of erythema multiforme, Stevens-Johnson syndrome (SJS), and TEN were identified during a period of 8 years in which the average number of admissions to the burn center was 200 per year. Age, sex, drug history before admission, drug treatment during hospital stay, and clinical outcomes were noted. Several drugs were identified as probable causative agents. The most common cause of exfoliating disease was a combination of azithromycin and ibuprofen, followed by ibuprofen alone. Notably, the combination of ibuprofen and another drug was responsible for four additional cases, making the total percentage of pediatric cases involving ibuprofen 47%. Although no children died, several children with TEN and SJS suffered severe ocular involvement, sepsis, pneumonia, and genitourinary complications. All of the children who experienced complications had received ibuprofen. Chi-square analysis showed the correlation between ibuprofen and complications to be statistically significant (<0.05). This association was not observed with any other drug administered. Not only is ibuprofen a potential etiologic agent of exfoliating skin disease in children, it also may contribute to the development of complications in pediatric patients with the disease. Although this association does not prove that ibuprofen is the definitive cause of complications in these cases, caution is advised when giving this drug to children with suspected erythema multiforme, SJS, and TEN.

Treatment of a Toxic Epidermal Necrolysis Patient with 100% Skin Involvement-Case Report and Literatures Review

Toxic epidermal necrolysis (TEN) is a rare but life-threatening exfoliative skin disorder. A high mortality rate of 20% to 60% has been reported. Many kinds of dressing materials and immunoreactive agents have been reported to improve the prognosis. We reported a 37-year-old case of TEN involving 100% of the total body surface area (TBSA). We would like to share our experience of treating this patient with a combination of high dose intravenous immunoglobulin (IVIG) and wound dressing with Acticoat(superscript ®). The patient was discharged 22 days after admission without any residual wounds and sequela.

Toxic epidermal necrolysis (TEN) and Stevens–Johnson syndrome (SJS): Experience with high-dose intravenous immunoglobulins and topical conservative approach: A retrospective analysis

Toxic epidermal necrolysis (TEN) and Stevens–Johnson syndrome (SJS) are rare, drug-induced, severe acute exfoliative skin and mucosal disorders. Several treatments previously proposed have produced contradictory results in small series; in 1998 the use of intravenous immunoglobulins (IVIG) was introduced with excellent clinical findings. Our experience (1999–2005) using IVIG in the therapy of TEN/SJS, together with a local conservative approach, is reported and related to our previous treatments (1993–1998). The SCORTEN and the standardized mortality ratio (SMR) was used to evaluate the efficacy of our therapeutic modalities. Eight patients were treated before IVIG era and 23 patients have been treated with IVIG. There was no significant difference in SCORTEN between the two groups. Concerning the local approach, a conservative wound management in IVIG series replaced an extensive epidermal debridment and coverage with artificial skin substitutes of the pre-IVIG series. Overall mortality in patients treated before IVIG was 75% (6/8), in the IVIG group it decreased to 26% (6/23) with a cessation of further epidermal detachment after an average of 5 days (3–10 days) from the onset of the therapy. The SMR showed a trend to lower actual mortality (not significative) with IVIG treatment than the predicted mortality (SMR = 0.728; 95% CI: 0.327–1.620).

Diagnostic dilemma: extremely low birth weight baby with staphylococcal scalded-skin syndrome or toxic epidermal necrolysis

Exfoliative skin diseases of the neonate are relatively rare but when present require rapid diagnosis and treatment to decrease morbidity and mortality associated with these entities. We present the case of a 795 g premature male infant who developed exfoliative lesions on day of life 66. Skin and blood cultures were obtained and intravenous antibiotics initiated. Results of a Tzanck smear suggested toxic epidermal necrolysis (TEN), so the infant was emergently transferred to a local children's hospital with burn unit capabilities for further care. Further histopathological evaluation of the skin biopsy confirmed the diagnosis of staphylococcal scalded-skin syndrome (SSSS). The desquamation ceased 24 h after the initiation of antibiotic therapy, and the skin lesions resolved within 10 days. We discuss the pathogenesis of SSSS and TEN and highlight the importance of early diagnosis so appropriate treatment can be initiated.

El síndrome de Lyell. Cuidados de enfermería especializados

Toxic epidermal necrolysis (TEN) is an exfoliative skin disorder that is associated with a high mortality rate. Its pathophysiology is similar to that of superficial burn injuries. Treatment and optimal care of these patients are best performed in Intensive Care Burn Units, with special attention to fluid resuscitation, respiratory support, early identification of sings of sepsis, and immunosuppresive therapy with cyclosporin A. Approach to the treatment of TEN is multidisciplinary, and there is a central and fundamental role of nursing care, particularly in the identification of signs of hypovolemia, respiratory failure, infection and pain. Among the specific care required by these patients is the care of mucosal surfaces, to prevent serious sequelae such as blindness. Specialized nursing care of patients with TEN contributes to an increase in the survival rate and a decrease in the incidence of complications.

Valdecoxib-associated acute generalized exanthematous pustulosis

AGEP (acute generalized exanthematous pustulosis) is a relatively rare exfoliative skin syndrome consisting of generalized eruption of pustules in response to medication or infection. Because AGEP may have other systemic manifestations, such as renal failure, hyperthermia, lab abnormalities, and/or hemodynamic instability, it is important to make the distinction between AGEP and other life-threatening generalized skin diseases, such as toxic epidermal necrolysis (TEN). Here we present a case of AGEP in response to valdecoxib, which has not previously been described in the literature. The patient presented with profound hypotension requiring fluid and vasopressor support and was referred to the burn service for treatment of TEN, but her skin lesions were inconsistent with this diagnosis. A dermatology consult was obtained and suggested a diagnosis of AGEP, which a biopsy confirmed. TEN and AGEP present with similar history, types of associated drugs, and immunology. Both can be associated with antibiotics, non-steroidals, and anticonvulsants, but AGEP is more frequent with aminopenicillins, while TEN is associated more often with sulfonamide antibiotics. Both disorders have a proposed T cell-mediated immune response, but they differ in the mechanism. A description of valdecoxib and its role as a sulfonamide in producing cutaneous reactions is also provided.

Staphylococcal scalded skin syndrome is usually seen in children and must be differentiated from other exfoliative skin conditions

Staphylococcal scalded skin syndrome is usually seen in children and must be differentiated from other exfoliative skin conditions

Single-Dose Oral Tolerance Test with Alternative Compounds for the Management of Adverse Reactions to Drugs

Background: Adverse reactions to drugs are common in the clinical practice. Many outpatients are frequently referred to allergists in order to determine which drugs they can safely take in the future. Objective: We set up an oral single-dose tolerance test procedure to find out for each patient one or more alternative drugs that can be taken when needed. Methods: 452 outpatients (130 male, 322 female) with well-documented reactions (urticaria/angioedema, respiratory symptoms, laryngeal edema, anaphylaxis, exfoliative skin diseases) underwent the challenge. All tests were preceded by a single-blind placebo: if a reaction occurred, a second placebo was administered. Otherwise, a single dose (1/10 of the therapeutic one) of an alternative drug was given blindly and the patient was then observed for 6 h. The drugs used were different in structure from those suspected of having caused the adverse reaction. The patients were followed up at 4- to 6-month intervals, in order to detect any reaction that may have occurred with the tested drugs. Results: 98 patients (89 women) had untoward reactions after the first placebo and 34 out of them reacted to the second placebo, too. During challenges the reaction rate ranged between 4.6 and 9.0%; these reactions were easily managed and none of them was severe. We followed up 407 patients: 87.2% of them were able to use one or more of the suggested drugs without reactions, 9.3% did not take the drugs and only 3.5% reported reactions to the previously tested drugs. Conclusion: The challenge procedure proved to be a simple tool for managing patients with adverse reactions to drugs. Its safety and reliability were validated by a long-term follow-up.

Smallpox and Pregnancy

In Brief Health care personnel must be prepared for the threat of bioterrorism. Our objective is to educate primary care providers, obstetricians in particular, in the prevention, diagnosis, and treatment of smallpox. Smallpox poses a particularly serious threat because of its high case-fatality rate in unvaccinated populations (no one younger than 25 years has been vaccinated, and older persons have little remaining residual immunity). Routine nonemergency smallpox vaccination is restricted to laboratory staff working with smallpox-related viruses. Under these circumstances, contraindications to vaccination are pregnancy, immunodeficiency, exfoliative skin conditions (eczema), and allergy to vaccine components. In case of an intentional release of the smallpox virus, those directly exposed and their close contacts must be vaccinated and isolated. Under such emergency circumstances, pregnant women exposed to the variola virus should be vaccinated because of the lethality of the disease during pregnancy. Currently, there is a limited supply of vaccine available. The threat of terrorists to use smallpox mandates health care providers to learn to prevent, diagnose, and manage this eradicated disease, given its potential catastrophic consequences.

Smallpox and pregnancy: from eradicated disease to bioterrorist threat

Health care personnel must be prepared for the threat of bioterrorism. Our objective is to educate primary care providers, obstetricians in particular, in the prevention, diagnosis, and treatment of smallpox. Smallpox poses a particularly serious threat because of its high case-fatality rate in unvaccinated populations (no one younger than 25 years has been vaccinated, and older persons have little remaining residual immunity). Routine nonemergency smallpox vaccination is restricted to laboratory staff working with smallpox-related viruses. Under these circumstances, contraindications to vaccination are pregnancy, immunodeficiency, exfoliative skin conditions (eczema), and allergy to vaccine components. In case of an intentional release of the smallpox virus, those directly exposed and their close contacts must be vaccinated and isolated. Under such emergency circumstances, pregnant women exposed to the variola virus should be vaccinated because of the lethality of the disease during pregnancy. Currently, there is a limited supply of vaccine available.

Wound healing enhancement in leg ulcers: A case report.

Wound healing in the skin is a complex biological process in which numerous types of cells, cytokines, growth factors, proteases and extracellular matrix components act in concert to restore the integrity of injured tissue. Cultivated allogenic human keratinocytes have been used for the treatment of various skin defects like burnwounds, surgical wounds, in exfoliative skin diseases and chronic wounds. A new method for wound healing enhancement in leg ulcers using cultured allogenic keratinocytes suspended in fibrin glue and used in spray technique is introduced. Allogenic keratinocytes are supposed to enhance granulation tissue production and to stimulate reepithelisation due to their release of growth factors and thus are able to recreate an active wound.

Toxic Epidermal Necrolysis Treated with Intravenous High-Dose Immunoglobulins: Our Experience

Background: Toxic epidermal necrolysis (TEN) is a rare severe acute exfoliative drug-induced skin disorder which has recently been ascribed to alterations in the control of keratinocyte apoptosis, mediated by an interaction between the cell surface death receptor Fas and its respective ligand. A therapeutic approach with intravenous immunoglobulins (IVIG) associated with pulse methylprednisolone, based on the inhibition of Fas-mediated keratinocyte death by naturally occurring Fas-blocking antibodies included in human immunoglobulin preparations, has produced good preliminary results. Objective: To analyse the efficacy of IVIG in the treatment of TEN. Patients: Nine patients with erythematous body surface area ranging from 38 to 85% and dermo-epidermal detachment from 4 to 37% were treated. Results: Eight patients were healed and 1 died of septic shock and multiple organ failure. Interruption of further epidermal detachment occurred after an average of 4.8 days from the onset of IVIG therapy. Complete wound healing occurred after an average of 12 days. Concerning complications, 3 out of 8 surviving patients had acute respiratory failure requiring mechanical ventilation and 1 acute renal failure was treated with dialysis. Late sequelae were limited to dyschromia and nail dystrophies. No hypertrophic scars were observed. Conclusion: IVIG therapy represents a safe and valid approach for TEN.

Clinical considerations on canine visceral leishmaniasis in Greece: a retrospective study of 158 cases (1989-1996).

The medical records of 158 dogs with visceral leishmaniasis confirmed cytologically and/or serologically were reviewed. Ages of affected dogs varied from nine months to 15 years, with a male-to-female ratio of 1.3. The most common clinical manifestations of the disease were variable cutaneous lesions such as exfoliative dermatitis and skin ulcerations, chronic renal failure, peripheral lymphadenopathy or lymph node hypoplasia, masticatory muscle atrophy (i.e., chronic myositis), ocular lesions (i.e., conjunctivitis, keratoconjunctivitis sicca, blepharitis, and uveitis), and poor body condition. Ascites, nephrotic syndrome, epistaxis, polyarthritis, and ulcerative stomatitis were seen only in a small number of cases. Clinical splenomegaly was not a common finding. The clinicopathological abnormalities were nonregenerative anemia, hyperproteinemia, glomerular proteinuria, and symptomatic or asymptomatic azotemia. In this study, an indirect immunofluorescence assay's diagnostic sensitivity was found to be higher than that of lymph node aspiration cytology.

Skin coverage with Biobrane biomaterial for the treatment of patients with toxic epidermal necrolysis.

Toxic epidermal necrolysis (TEN) is an exfoliative skin disorder that may involve a large body surface area and mucosal surfaces. The microscopic changes that occur with this condition are similar to those that occur with superficial dermal burns, such as dermal detachment from the underlying dermis. Complications of TEN are related to the loss of the epithelial skin barrier and include pain, fluid and electrolyte loss, and an increased risk of sepsis. The treatment of a patient with TEN is best accomplished in a burn unit, where expert treatment of these complications can be provided. Medical treatment includes the administration of immunosuppressive therapy and the discontinuation of any previous corticosteroid treatment. Surgical management includes the debridement of necrotic areas. In this article, the surgical management of 8 consecutive patients with TEN who were admitted to the intensive care burn unit at the Hospital Universitario de Getafe in Madrid, Spain, from 1996 to 1998 is described. These patients were treated with extensive early debridement of necrotic skin areas followed by wound coverage with Biobrane (Dow B. Hickam, Inc, Sugarland, Tex), a temporary semisynthetic skin substitute. Skin coverage with this material decreases pain and fluid loss, and it possibly facilitates epithelization and decreases the risk of sepsis, without adverse side effects. This semisynthetic material meets some standards of an ideal skin substitute: it is easy to use, provides several beneficial physiologic effects, and improves patients' comfort. In the 8 cases of patients with TEN that were studied, the use of Biobrane skin substitute for the coverage of massive areas of detached skin was found to be an important aspect of treatment.

Exfoliative skin manifestations in acute lymphatic filariasis

Exfoliative skin manifestations in acute lymphatic filariasis

Staphylococcal Endophthalmitis Following Cataract Extraction in a Patient with Darierʼs Disease

To report staphylococcal endophthalmitis following cataract extraction in a patient with Darier' s disease. A 67-year-old man presented with decreased visual acuity OD and hypopyon 3 days status post-cataract extraction with intraocular lens placement. The patient was hospitalized and placed on topical and intravenous antibiotics. A diagnostic vitreous tap, pars plana vitrectomy, and intravitreal antibiotic installation were performed. Vitreal tap cultures indicated Staphylococcus epidermidis. His clinical status improved after vitrectomy and antibiotic therapy. The same bacteria was cultured from the patient's eyelids. The source of the S. epidermidis was the skin lesions on the patient's face and eyelids. Darier's disease is an exfoliative hyperkeratotic skin disease that affects all areas on the body except the buttocks. Although clean, sterile surgical techniques were followed, the risk of endophthalmitis following intraocular surgery in a patient with Darier's disease may be increased due to his or her dermatologic condition.

Experience with toxic epidermal necrolysis treated in a burn center.

Toxic epidermal necrolysis syndrome is one of several clinically similar, severe acute, exfoliative skin disorders that have become of increasing interest to burn surgeons in recent years. Recognition of a clinical course similar to extensive second-degree burns has resulted in the development of treatment protocols that are best carried out in a burn unit by personnel experienced in critical care techniques, the management of extensive cutaneous injuries, fluid and electrolyte derangements, and intensive nutritional support of critically ill patients. Current evidence suggests that in most instances toxic epidermal necrolysis syndrome is a CD8 lymphocyte-mediated reaction triggered by exposure to certain drugs. The target organs of the immune reaction are skin and mucous membranes. Appropriate management of the extensive skin wounds and the nutritional and critical care support afforded by treatment in burn units appears to have contributed significantly to the increasing survival of patients with this devastating and potentially lethal illness.

Fluconazole

Fluconazole is a triazole antifungal agent which is now an established part of therapy in patients with immune deficiencies. It is effective against oropharyngeal/oesophageal candidiasis (candidosis) when used orally once daily either as treatment or secondary prophylaxis in patients with AIDS or as treatment or primary prophylaxis in neutropenia associated with cancer therapy. Fluconazole also resolves symptoms in up to 60% of patients with cryptococcal meningitis and AIDS. However, in this infection its efficacy as treatment relative to that of amphotericin B is equivocal, and its major role is as the drug of choice for maintenance therapy following amphotericin B induction. In this regard, fluconazole has been proven superior to amphotericin B and to itraconazole 200 mg/day. Comparisons with other drugs used for the treatment of mucosal candidiasis in patients with AIDS show fluconazole to be superior to nystatin, similar to itraconazole and at least as effective as clotrimazole and ketoconazole; it was more so than the latter azole in 1 study. In patients undergoing chemotherapy or bone marrow transplantation, fluconazole as primary prophylaxis has produced greater clinical benefit than a clotrimazole regimen. The incidence of adverse events appears to be somewhat higher in patients with AIDS compared with HIV-negative cohorts, but the qualitative pattern of events is similar. The most frequent events are gastrointestinal complaints, headache and skin rash: rare exfoliative skin reactions and isolated instances of clinically overt hepatic dysfunction have occurred in patients with AIDS. Issues yet to be clarified include: the use of fluconazole in children with AIDS, in whom results have been promising; its efficacy against other fungal infections encountered in immunocompromised patients; whether the drug influences mortality, as has been suggested by one placebo-controlled trial in patients undergoing bone marrow transplant; and the appropriateness of its potential for use as primary prophylaxis against cryptococcal meningitis in patients with AIDS, where it shows efficacy but there is concern over increasing risk of development of secondary resistance. Notwithstanding these undefined aspects of its clinical profile, fluconazole is now confirmed as an important antifungal drug in the management of fungal infections in patients with immune deficiencies. In patients with AIDS it is the present drug of choice as maintenance therapy against cryptococcal meningitis and is a preferred agent for secondary prophylaxis against candidal infections; it is also a favoured agent for primary prophylaxis in patients at risk because of neutropenia associated with chemotherapy or bone marrow transplantation .

Preventing complications in severe exfoliative skin diseases.

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Syndrome Type II are both exfoliative skin diseases with complications similar to burn patients. The critical care nurse's responsibility is to recognize the disease processes early and assure aggressive nursing care is provided to prevent the serious respiratory, gastrointestinal, sepsis, renal, and pain complications.