Life-threatening Dermatoses Research Articles

In-hospital mortality in a dermatology department

Background: Far from popular belief making dermatological conditions benign and harmless, we know that some dermatoses may be life-threatening. The aim of our study was to evaluate the overall mortality resulting from dermatological disorders in our department because of the rarity of similar studies. Materials and Methods: We conducted a retrospective, observational study on all patients who died in the Ibn Sina Hospital of Rabat between January 2017 and December 2021 (a period of five years), after they were initially admitted to the dermatology department. Results: Among 1731 patients hospitalized during this period, 45 died, corresponding to an average of 2.6%. The male-to-female ratio was 1.9 and the mean age at death was 63.2 years. The mean number of comorbidities per patient was 1.2, dominated by diabetes. The reasons for hospitalization were as follows: twenty-three cases of extensive bullous dermatoses (60.5% of the cases), four cases of severe toxidermia (10.5%), three cases of erythroderma (7.8%), one case of acro-lentiginous melanoma, one case of squamous cell carcinoma, and one case of pyoderma gangrenosum. In twenty patients (52.6%), the cause of death was directly related to the dermatosis. In sixteen, the cause of death was septic shock of cutaneous origin. Sixteen patients (42.1%) died of causes unrelated to dermatosis. Conclusion: This study showed that life-threatening dermatoses are not uncommon. Among these conditions, autoimmune bullous dermatoses and severe toxidermia, such as TEN and DRESS syndrome, were found to be the most lethal. An advanced age, a surface area of skin involvement, mucosal involvement, associated comorbidities, and the development of sepsis may be considered poor prognostic factors.

Cutaneous Anomalies of the Critically Ill Patient.

Critically ill patients are at high risk for organ failure, including that of the integumentary system. Nurses working in intensive care are adept at performing comprehensive assessments that include the skin. Although pressure injury is a well-known complication associated with critical illness, patients may also have debilitating and life-threatening dermatoses. Conditions such as skin failure and medical adhesive-related skin damage are commonly seen in the critically ill. Infectious processes, such as Fournier gangrene, invasive candidiasis, mucormycosis, and herpetic lesions, can result in severe or superimposed critical illness and elude detection. Similarly, cutaneous manifestations of COVID-19 may develop prior to commonly recognized symptoms of infection. Nurses and providers caring for critically ill patients should be aware of common, but less widely known, skin conditions to facilitate early detection and treatment.

Clinical significance of umbilical region involvement in pemphigus vulgaris in a series of 81 ethnic Poles: a comparative analysis of the distribution of lesions in two infrequent locations.

IntroductionAutoimmune bullous diseases are potentially life-threatening dermatoses which present with cutaneous and/or mucosal blisters, diagnosed on the basis of clinical manifestations, direct immunofluorescence of perilesional tissue, and serum testing for circulating autoantibodies. Sometimes, lesions in the navel can lead to the diagnosis of a bullous disease.AimTo assess the frequency of occurrence of pemphigus lesions located in the navel area and nail apparatus in pemphigus vulgaris (PV) in ethnic Poles.Material and methodsEighty one patients (31 males and 50 females, mean age 59 years) with dermatoses of the PV group diagnosed in 2002-2020 were retrospectively analysed using their photographic files. Statistical analysis was performed using the difference test between two proportions to check the difference between the percentage of PV patients with navel area involvement and nail apparatus involvement.ResultsThere was no statistically significant difference between PV patients with nail apparatus involvement (12.3%) and navel area involvement (14.8%) (p = 0.4632). Only females had lesions in the navel area in our series of PV patients.ConclusionsIt is speculated that the causal relationship may exist between the female reproductive system and the pattern of expression of PV lesions around the umbilicus. The awareness that PV can infrequently affect the umbilical region and the nail apparatus should help in some cases to establish the diagnosis of PV. The periumbilical involvement can facilitate the performance of DIF in individuals with lesions in less accessible areas.

Accuracy of SCORTEN and ABCD-10 to predict mortality and the influence of renal function in Stevens-Johnson syndrome/toxic epidermal necrolysis.

Epidermal necrolysis (EN) compromises a spectrum of life-threatening dermatoses (Stevens-Johnson Syndrome [SJS], overlap syndrome and toxic epidermal necrolysis [TEN]). Currently, no active therapeutic regimen with unequivocal benefit exists for SJS/TEN. SCORTEN is the widely-used prognostic scale specific for SJS/TEN. Nevertheless, a new prognostic scale, the ABCD-10, has been recently proposed. In this context, acute renal failure (ARF) seems to be an important comorbidity that could influence prognosis in SJS/TEN patients more than it is assumed by these two scales. Our objectives were to compare the accuracy of the SCORTEN and ABCD-10 scales in predicting the mortality in SJS/TEN, and to investigate the influence of renal failure on prognosis. The prognostic results of 18 patients with EN treated in two referral centers between 2013 and 2018 are presented. SCORTEN, ABCD-10 and renal function values were retrospectively collected for all patients. Out of the 18 patients who were analyzed, nine (50%) received only supportive therapy, four were treated with etanercept 50mg in a single dose (22.2%) and five with corticosteroids (27.8%). Five patients developed ARF. Predicted mortality was 3.48 for SCORTEN and 2.33 for ABCD-10. Eventually, four patients died (22.2%), all had ARF and none of them received active treatment. Despite study limitations and in the absence of active treatment of choice, SCORTEN behaved as a reliable predictor of mortality in patients with EN, outperforming the newer ABCD-10. ARF was an early event associated with a poor prognosis, which could represent a prognostic marker to consider in the future.

Life-threatening dermatoses in neonates and newborns

The differentiation of life-threatening dermatoses in newborn is of utmost importance. Infectious diseases, developmental defects and complicated genodermatoses are among the most important causes. Perinatal history, mother`s general state and epidemiologic data are the other important factors for the best verification and management. Herein, a thorough review of the most common life-threatening dermatoses in newborn, is presented. A diagnostic algorithm and therapeutic clues are provided.

A clinical study of neonatal dermatosis

Background: The neonatal period is the first 4 weeks of extra-uterine life. Neonatal dermatology encompasses a spectrum of cutaneous disorders from benign to life threatening. The present study was undertaken to evaluate the various dermatoses and hence assess the spectrum of dermatological conditions during the neonatal period and to study the relationship of neonatal dermatoses with maturity and birth weight of the neonates.Methods: Three hundred neonates with cutaneous manifestations, attending the OPD of dermatology and Paediatrics during one year period, were studied. Neonates receiving phototherapy and those with lesions secondary to trauma were excluded. Clinical history coupled with complete general physical, systemic and dermatological examination.Results: Three hundred cases of neonatal dermatoses were included in study. Out of 300 cases, 179 (59.7%) were male babies and 121 (140.3%) were female (sex ratio 1:5), 271 (70.3%) were term babies, 27 (9%) were preterm and 2 (0.7%) were post term. Transient/non-infective cutaneous lesions were the commonest 144 (48%) followed by Infectious 68 (22.67%), eczematous disorder 33 (11%) and pigmentary dermatoses 25 (8.33). Vascular lesions and hematomas accounted for 18 (6%) while genodermatoses were seen 7 (2.33%). 5 cases of subcutaneous fat disorders were observed.Conclusions: As the spectrum of neonatal dermatology is vast, its knowledge to dermatologist is very important so as to differentiate the more common benign disorders from less frequent life-threatening dermatoses, so that timely intervention could facilitate a better therapeutic outcome.

Dermatological emergencies: evolution from 2008 to 2014 and perspectives.

Our dermatological department includes a dermatological emergency unit (DEU) whose activity has grown in recent years. An audit to characterize the activity of our DEU and its evolution in terms of medical demographics of the area. We collected the following data from administrative services: number of patients consulting each year in the DEU and in the general emergency unit (GEU) of our hospital between 2008 and 2014; daily and seasonal activity of the DEU; occurrence of a second event in the department and proportion of patients from the DEU who were hospitalized and why. From the medical charts of a random sample of patients consulting in the first 15 days of January and August 2014, we studied the epidemiological profile, time to consultation and diagnoses. Data related to medical demographics (number of general practitioners and dermatologists) between 2007 and 2014 and projections were obtained. The activity in the DEU increased by 67% between 2008 and 2014 but remained stable in the GEU over the same period. The activity was higher on Mondays and in the summer (+30%). More than 15% of the patients were seen a second time in outpatient consultation; 1.2% were hospitalized. Infectious dermatosis was the main reason for consultation; seasonal-disease consultations were more frequent in the summer. Less than 40% of patients consulted in the first week after disease onset. Medical demographics continually decreased since 2007 in Paris and suburbs and will continue to decrease in the next years. The increasing activity of our DEU parallels the decrease in medical demographics in Paris. The proportion of patients hospitalized was low, in part due to specific healthcare networks implemented for some life-threatening dermatoses independent of the DEU. A better coordination between hospital and private practitioners for managing dermatologic emergencies, taking into account the decrease in medical demographics, is warranted.

Apoptosis as a Mechanism for Keratinocyte Death in Canine Toxic Epidermal Necrolysis.

In humans and dogs, toxic epidermal necrolysis (TEN) is a life-threatening dermatosis characterized by sudden epidermal death resulting in extensive skin detachment. There is little information on the pathogenesis of keratinocyte cell death in canine TEN. We studied the occurrence of apoptosis in skin lesions of dogs with TEN to determine if apoptosis contributes to the pathogenesis of this disease. Immunostaining with antibodies to activated caspase-3 and the terminal deoxynucleotidyl-transferase (TdT)-mediated deoxyuridine triphosphate (dUTP) nick-end labeling technique revealed positive apoptotic keratinocytes in basal and suprabasal epidermal compartments in 17 biopsy specimens collected from 3 dogs with TEN and 16 from 3 dogs with erythema multiforme (EM). There was no significant difference in the number of positively stained epidermal cells between TEN and EM. These results suggest that apoptosis of epidermal keratinocytes and lymphocytic satellitosis represent one of the early steps in the pathogenesis of canine TEN, as in the human disease counterpart.

Personal treatment experience for severe generalized pustular psoriasis of pregnancy: two case reports

Generalized pustular psoriasis of pregnancy (GPPP) is a rare and severe variant of pustular psoriasis. It has been called the most dangerous and life-threatening dermatosis in pregnant woman. To explore the treatment of GPPP, we conducted a retrospective study of two cases of GPPP in our clinic and other related reported cases and manuscripts. In 1992, a GPPP patient came to our clinic. We prescribed her antibiotics and dexamethasone. The pregnancy was artificially terminated. After delivery, she took a retinoid and a topical steroid and recovered progressively. In 2012, we treated another GPPP patient. Methylprednisolone and cyclosporine were administered. Maternal and fetal statuses were monitored closely. The treatment was effective and a healthy baby was delivered. We compared our two GPPP cases and found that cyclosporine and a sufficient dosage of steroid were an effective treatment. Antibiotics could be tried in mild cases or the initial stages before excluding sepsis. Based on other reported GPPP cases, TNF-α antagonists are used as rescue therapy in GPPP refractory to steroid and cyclosporine therapy, but careful consideration of the advantages and disadvantages is warranted before using them. Supportive measures are necessary to maintain pregnancy and prevent complications in cases of GPPP.

Intensivpflichtige Hauterkrankungen

Some skin conditions may require treatment in intensive care units. The early diagnosis of life-threatening dermatoses is a considerable challenge. We review skin diseases which may require intensive care. In addition to Stevens-Johnson syndrome and toxic epidermal necrolysis, representing adverse skin reactions, we discuss staphylococcal scalded skin syndrome and necrotizing fasciitis as infection-associated dermatoses, as well as angioedema. We focus on the course of disease describing clinical presentations, diagnostics and therapeutic strategies with respect to critical medical conditions.

Treatment of toxic epidermal necrolysis with intravenous immunoglobulin: a series of three cases

Stevens-Johnson's syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening dermatoses, that lead to keratinocyte apoptosis induced by interactions between Fas (cell death receptor) and soluble Fas-ligand, present in serum of Stevens-Johnson's syndrome / toxic epidermal necrolysis patients. Anti-Fas antibodies in intravenous immunoglobulin (IVIG) would block the apoptosis cascade. Three cases of toxic epidermal necrolysis occurred in one male and two female patients, after use of allopurinol, leprosy multidrug therapy concomitant with dipyrone, and diclofenac. The cases were treated with intravenous immunoglobulin 2-3 mg/kg and prednisone 20-50 mg/day. The interruption of new lesions outbreak and reepithelization were extremely fast after the use of intravenous immunoglobulin, without adverse effects. Controlled studies are needed to confirm the efficacy of intravenous immunoglobulin in Stevens-Johnson's syndrome / toxic epidermal necrolysis, but the results seem promising.

Laser Phototherapy for Stevens–Johnson Syndrome: A Case Report

Stevens-Johnson syndrome (SJS) is a life-threatening dermatosis characterized by epidermal sloughing and stomatitis. We report the case of a 7-year-old boy in whom laser phototherapy (LPT) was highly effective in reversing the effects of an initial episode of SJS that had apparently developed in association with treatment with phenobarbital for a seizure disorder. The patient was first seen in the intensive care unit (ICU) of our institution with fever, cutaneous lesions on his extremities, trunk, face, and neck; mucosal involvement of his genitalia and eyes (conjunctivitis); ulcerative intraoral lesions; and swollen, crusted, and bleeding lips. He reported severe pain at the sites of his intraoral and skin lesions and was unable to eat, speak, swallow, or open his mouth. Trying to prevent and minimize secondary infections, gastric problems, pain, and other complications, the patient was given clindamycin, ranitidine, dipyrone, diphenhydramine (Benadryl) drops, and morphine. In addition, he was instructed to use bicarbonate solution and Ketoconazole (Xylogel) in the oral cavity. Because of the lack of progress of the patient, the LPT was selected. At 5 days after the initial session of LPT, the patient was able to eat gelatin, and on the following day, the number and severity of his intraoral lesions and his labial crusting and swelling had diminished. By 6 days after his initial session of LPT, most of the patient's intraoral lesions had disappeared, and the few that remained were painless; the patient was able to eat solid food by himself and was removed from the ICU. Ten sessions of LPT were conducted in the hospital. The patient underwent three further and consecutive sessions at the School of Dentistry, when complete healing of his oral lesions was observed. The outcome in this case suggests that LPT may be a new adjuvant modality for SJS complications.

Erythroderma: review of a potentially life-threatening dermatosis.

Erythroderma, or generalized exfoliative dermatitis, is a disease characterized by erythema and scaling of greater than 90% of the body's surface. The resultant dysmetabolism is potentially life threatening. A detailed history is to identify and treat the underlying cause of this dermatitis. We present two cases of erythroderma in African patients and review this important disease.

Practical guidelines for the management of toxic epidermal necrolysis and Stevens–Johnson syndrome

Toxic epidermal necrolysis and Stevens-Johnson syndrome are acute life-threatening dermatoses characterized by extensive sloughing and mucositis. At the University of Florida, we use practical guidelines for the management of these gravely ill patients. These can be of help to other practitioners.

Erupções vesicopustulosas benignas no neonato

As erupções vesicopustulosas neonatais compreendem um grupo de desordens que surgem nas primeiras quatro semanas de vida. Apresentam extensa relação de diagnósticos diferenciais e, na maioria das vezes, podem ser identificadas clinicamente ou mediante recursos laboratoriais simples. Os dermatologistas devem reconhecer esses quadros cutâneos e, sobretudo, saber diferenciá-los de outras dermatoses graves e potencialmente fatais.

Life-threatening dermatoses due to connective tissue disorders

Abstract Connective tissue disorders such as scleroderma, dermatomyositis and lupus erythematosus are autoimmune, multi-system disorders whose clinical manifestations can be restricted to the skin or may involve many organs. The degree and rate of organ system involvement defer, as does the prognosis and rapidity of disease progression. In this article, scleroderma, dermatomyositis and lupus erythematosus will be reviewed in respect to their life-threatening potential.

Life-threatening dermatoses in travelers

Life-threatening dermatoses in travelers relates to infections, or allergic or drug reactions. Of the infectious dermatoses, most are parasitic or viral- because of the short time frame. Bacterial infections usually have a longer incubation. More significant are allergic reactions from environmental or dietary causes, and drug reactions, especially those drugs taken for prophylaxis from tropical diseases. All of these can be fulminant and therefore an awareness and early therapeutic intervention is mandatory.

Life-threatening dermatoses due to metabolic and endocrine disorders

Cardiovascular heart disease due to atherosclerosis is the commonest cause of mortality and morbidity in the Western world. Atherosclerosis is directly related to disorders of lipid metabolism, diabetes mellitus (DM), and insulin resistance. Disorders of thyroid function and porphyrin metabolism occur less frequently but may cause life-threatening situations if unrecognized. All these disorders are associated with characteristic dermatoses, which should permit early diagnosis and meaningful intervention.

What???s new in pediatric dermatology: update for the pediatrician

Common pediatric skin conditions such as infantile atopic dermatitis, vitiligo, hemangiomas of infancy, warts, and molluscum contagiosum do not always respond to standard therapy. In some settings pediatricians will use "off-label" medications if the benefit-to-risk ratio is favorable. This article reviews important literature from the past year related to "off-label" immune-based treatment of skin disease, using the topical immunomodulators tacrolimus, pimecrolimus, and imiquimod, as well as intravenous Ig. The topical immunomodulators tacrolimus and pimecrolimus have been embraced by pediatricians as long awaited alternatives for treating atopic dermatitis in children 2 years of age and older. Their unique appeal as nonsteroidal topical agents with good safety profiles has led to their frequent use for unapproved indications. A number of recent publications detail their use in infantile atopic dermatitis in children as young as 3 months of age, as well as use in other conditions such as vitiligo. Imiquimod, another topical immunomodulator, approved for genital wart treatment in adults, has also been examined for "off-label" pediatric use in nongenital warts, molluscum contagiosum, hemangiomas of infancy, and basal cell carcinoma. Finally, "off-label" use of intravenous Ig has been evaluated for the life-threatening dermatoses Stevens-Johnson syndrome and toxic epidermal necrolysis. In the absence of larger controlled trials, pediatricians must consider the cumulative weight of smaller studies with their personal experience when assessing any role for "off label" therapy. The recent literature reviewed herein will facilitate such assessments of the non-steroid topical immune modifiers tacrolimus, pimecrolimus, and imiquimod as well as intravenous immunoglobulin.

Die Haut als Spiegel der Seele: Psychosomatische Dermatologie - aktueller Forschungsstand/ Psychosomatic dermatology - State of the art

Based on the current literature, the paper gives an overview of the state of knowledge in psychosomatic dermatology. The authors propose the following classification: (1.) Dermatologic problems as a consequence of psychic disorders (e.g. factitious disorder, trichotillomania, dysmorphophobia, delusions of parasitosis etc.); (2.) Multifactorial dermatologic disorders, which can be substantially influenced by psychological factors (e.g. atopic dermatitis, psoriasis, chronic urticaria etc.); (3.) Psychic disorders developing as a consequence of disfiguring or life-threatening dermatoses (e.g. adjustment disorder, depressive disorder, anxiety disorder); (4.) Comorbidity with other psychiatric disorders. Psychosomatic dermatology developed from an anecdotal initial stage to more systematic research with larger numbers of patients, employing control groups and psychometric instruments. During the last decade, research focused on psychophysiological and psychoneuroimmunological mechanisms which influence dermatologic disorders. This course of development and the current state of knowledge are presented for atopic dermatitis and psoriasis.