|ORIGINAL CONTRIBUTION - CLINICS IN PEDIATRIC DERMATOLOGY
|Year : 2017 | Volume
| Issue : 4 | Page : 184-187
Exfoliative dermatitis in Thai children
Wanee Wisuthsarewong, Rattanavalai Nitiyarom, Theerawat Buddawong
Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkoknoi, Bangkok, Thailand
|Date of Web Publication||7-Jul-2017|
Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkoknoi, Bangkok - 10700
Source of Support: None, Conflict of Interest: None
Background: Exfoliative dermatitis (ED) is an inflammatory skin disorder in which generalized skin erythema and scaling affect at least 90% of the body. Aims: To determine the etiology, clinical presentations, laboratory findings, management, and outcomes of ED. Materials and Methods: This retrospective study was conducted at the Department of Pediatrics, Siriraj Hospital. Data of pediatric patients diagnosed with ED during January 1992 and June 2014 were reviewed. Results: Forty-seven patients were identified. The median age at diagnosis was 4 years and 8 months (range 1–164 months), and 10 cases (21.3%) were infants. Presenting symptoms included itching (76.6%), fever (38.3%), painful sensation (17.0%), and diarrhea (12.8%). Physical findings were dehydration (42.6%), fever (34.0%), failure to thrive (29.8%), tachycardia (29.8%), hepatomegaly (17.0%), edema (12.8%), lymphadenaopathy (8.5%), and splenomegaly (6.4%). Common laboratory abnormalities revealed thrombocytosis (51.4%), eosinophilia (48.6%), elevated erythrocyte sedimentation rate (42.9%), and elevated liver enzymes (30.3%). Hepatosplenomegaly and lymphadenopathy were found to be statistically significantly correlated with immunodeficiency syndrome (P < 0.05). No other clinical or laboratory findings were associated with any specific etiology. Preexisting skin diseases (53.2%), including atopic dermatitis (23.4%), psoriasis (17.0%), pityriasis rubra pilaris (10.6%), and seborrheic dermatitis (2.1%), were the most common causes of ED. Other causes were primary immunodeficiency (12.8%), congenital ichthyosis (10.6%), drugs (10.6%), metabolic disorders (4.3%), and unknown etiology (8.5%). Mortality rate was 8.5%. Limitation: Some data from this retrospective study may have been missing and some investigations may not have been performed in all patients. Conclusion: The most common etiology of ED in children was preexisting skin diseases. Prognosis was poor in immunodeficiency disorders.
Keywords: Children, erythroderma, exfoliative dermatitis
|How to cite this article:|
Wisuthsarewong W, Nitiyarom R, Buddawong T. Exfoliative dermatitis in Thai children. Astrocyte 2017;3:184-7
| Introduction|| |
Exfoliative dermatitis (ED) or erythroderma is an inflammatory skin disorder affecting more than 90% of the body surface area with extensive erythema and variable degree of scaling. The causes in adults include preexisting skin diseases, drug induced, malignancy, and others. ED is an uncommon clinical entity in the pediatric age group. Although the clinical picture in children may appear similar to that of adults, the underlying etiology may be different.
Most studies in ED included only adults, with few having investigated ED in children. Accordingly, the objective of this study was to assess the etiology, clinical features, laboratory parameters, management, and outcomes of this condition in Thai children.
| Materials and Methods|| |
This retrospective study was conducted at the Department of Pediatrics, Siriraj Hospital. The protocol for this study was approved by the Siriraj Institutional Review Board. Data of patients diagnosed with ED during January 1992 and June 2014 were reviewed to determine the demographic data, etiology, clinical presentations, laboratory findings, management, and outcome of the disease. Detailed history was collected and recorded, including personal data, disease onset, drug history, and family history of similar conditions. Clinical symptoms and signs, especially dermatologic findings during the episode, such as itching, fever, alopecia, nail involvement, mucosal involvement, edema, hydration status, lymphadenopathy, and hepatosplenomegaly, were also assessed. Laboratory investigations, including complete blood count (CBC), urinalysis, erythrocyte sedimentation rate (ESR), electrolytes, serum protein, liver function tests, and skin biopsy, were recorded. Treatment and clinical course were also collected during follow-up.
The diagnosis of ED was based mainly on the clinical features of extensive erythema with scaling affecting near total of the body. Etiology of ED was made by history, clinical, laboratory, and histopathological findings. Most data were analyzed and presented as descriptive statistics. Chi-square test was used to assess correlation between clinical or laboratory tests and etiology. A P-value less than 0.05 was considered to be statistically significant.
| Results|| |
During the study period, 47 patients presenting with ED were identified. The median age at diagnosis was 4 years and 8 months. The youngest patient was 1 month old and the oldest patient was 13 years and 8 months old. There were 10 patients (21.3%) in the infantile age group. Twenty-seven (57.4%) patients were male and 20 patients (42.6%) were female, with a male:female ratio of 1.4:1. Previous history of skin diseases was observed in 24 patients (51.1%). No similar condition was found in any family member of any patient.
Most patients had insidious clinical onset. Presenting symptoms were itching (76.6%), fever (38.3%), painful sensation (17.0%), and diarrhea (12.8%). The systemic signs revealed dehydration (42.6%), fever (34.0%), tachycardia (29.8%), failure to thrive (29.8%), hepatomegaly (17.0%), edema (12.8%), and splenomegaly (6.4%). Skin manifestations involved scalp (83.0%), mucous membrane (38.3%), and nail (36.2%). Most nail abnormalities were observed in patients with psoriasis, pityriasis rubra pilaris (PRP), and ichthyosis. Nail abnormalities included ridging, thickening, dystrophy, and onycholysis. The clinical symptoms and physical findings were shown in [Table 1].
Laboratory investigations demonstrated thrombocytosis, eosinophilia, elevated ESR, anemia, leukocytosis, abnormal liver functions, and hypoalbuminemia [Table 2]. Although eosinophilia was commonly observed in atopic dermatitis (AD) (23.5%), immunodeficiency syndrome (23.5%), and drug-induced ED (17.6%), the associations were not statistically significant. Abnormal liver function tests were found in immunodeficiency syndrome (44.1%) and drug-induced ED (33.3%). All investigations were nonspecific, with no identifiable relationship to any specific etiology. Skin biopsy was performed in 15 cases whose cause of ED was unclear, with confirmed diagnosis being established in 53.3% of the cases. Biopsy-based diagnoses included pustular psoriasis, PRP, and acute generalized exanthematous pustulosis.
Preexisting skin diseases (53.2%) were the most common etiology of ED in children. Those preexisting diseases included AD, psoriasis, PRP, and seborrheic dermatitis in 23.4%, 17.0%, 10.6%, and 2.12% of the cases, respectively. Other causes were primary immunodeficiency (12.8%), congenital ichthyosis (10.6%), drugs (10.6%), and unknown etiology (8.5%). The causes of infantile ED were primary immunodeficiency syndrome (40%), ichthyosis (20%), metabolic diseases (maple syrup urine disease, zinc deficiency) (20%), seborrheic dermatitis (10%), and meropenam (10%). The etiologies of ED in this study are presented in [Table 3].
Lymph node enlargement, splenomegaly, and hepatomegaly were found in 75%, 66.7%, and 62.5% of immunodeficiency syndrome cases, respectively. Lymphadenopathy (P < 0.01), splenomegaly (P = 0.04), and hepatomegaly (P < 0.01) were also statistically significantly correlated with immunodeficiency disease. No other symptoms or signs were found to be significantly associated with any specific etiology.
Topical emollients were used to hydrate the skin in all patients. Wet dressing was applied in 24 cases (51.1%), topical low-potency corticosteroid was prescribed in 36 cases (76.6%), and topical calcineurin inhibitors were given in 11 cases (23.4%). Seven patients (14.9%) with severe clinical symptoms required systemic corticosteroids. Acitretin and methotrexate were administered in 10 cases (21.3%) and 4 cases (8.5%), respectively, to treat the underlying skin diseases. During the follow-up period, 44.7% of the patients showed clinical improvement. The clinical manifestations of ED in 14 patients with AD (9), psoriasis (3), and PRP (2) fluctuated with flare according to the underlying preexisting diseases. Three patients (6.4%), one each with nonbullous congenital ichthyosiform erythroderma, hyper IgM syndrome, and maple syrup urine disease, had persistent lesions despite various treatments.
The mortality rate in ED in this study was 8.5%. However, the cause of death was not directly related to the skin. Four patients died of infectious complications resulting from their underlying primary immunodeficiency disease and biliary atresia. The mortality rate in the infantile group was 30% and all of those had primary immune deficiency. Immunodeficiency status was found to be significantly correlated with death (P = 0.01).
| Discussion|| |
ED is an uncommon condition in the pediatric age group. The overall incidence of ED in children was about 0.11%. Our study confirmed previous reports that ED can be seen in neonates and infants., Age at onset may vary according to etiology. Male preponderance was observed similar to ED in adults.,,,,,,,
The etiology of ED is frequently difficult to establish and is usually delayed due to nonspecific clinical and histological findings. The etiological spectrum ranges from transient harmless disease to potentially lethal disorders. Regarding the cause of ED in children, our series had a high percentage of ED that resulted from preexisting dermatoses, which was the same as another study in children, and similar to the cause of adult ED described in most studies., 5, ,,,,,, Psoriasis was reported as being the most common preexisting dermatosis underlying ED in adults,,,,,,,, however, AD was more common than psoriasis in children. PRP was uncommonly reported as a cause of ED in other series,,, however, it was one of the common underlying skin diseases that caused ED in this study. Drugs that were a common cause of ED in older children and adults in some reports were less common than immunodeficiency syndrome causation in this study. The causative drugs identified in this study were anticonvulsants and antibiotics, which is similar to reports in adult ED.,,,,, There was no ED from malignancy or contact dermatitis in this series. This may be explained by a low incidence of malignancy and less exposure to sensitizers in children, as compared to adults. The causes of ED observed in the neonatal and infantile age group were distinctive from those found in adults. Consistent with many previous reports, the most common cause of ED in neonates and infants was immunodeficiency state.,,,
The clinical features of ED were identical among all our study patients, irrespective of the etiology. Some clinical features may be helpful in identifying the etiology of ED; however, no characteristic features or pathognomonic signs were found. Drug-induced ED had more acute onset and shorter course than other causes. Itching was the most common complaint, as described in many articles.,,,, Pruritus, mucosal, and nail involvement were observed, which is consistent with the nature of underlying diseases. Failure to thrive and alopecia may be related to underlying disease, severity, and duration of ED., In children, hepatosplenomegaly was found mostly in immunodeficiency patients. Nail changes were seen not only in psoriasis but also in PRP and ichthyosis.
Although laboratory investigations were generally not very helpful in diagnosis of the underlying condition, some tests had to be performed to establish the precise diagnosis and to rule out certain diseases. Laboratory tests that were useful in the evaluation of childhood ED were CBC, ESR, skin biopsy, liver function tests, and investigations for infectious organism, immune status, and metabolic disorders. Eosinophil count was increased in drug-induced ED in adults.,,, In childhood ED, eosinophilia developed more often from AD and immunodeficiency syndromes than from drugs. Laboratory findings, such as thrombocytosis, anemia, elevated ESR, and hypoalbuminemia, were likely attributable to the inflammatory process., Disease-specific investigations, including lymph node biopsy, bone marrow examination, and imaging should be performed whenever indicated. Histopathology was a useful investigation and very important for establishing diagnosis in cases with suspected preexisting skin disease or unclear cause. Excellent clinicopathological association was found when performing a biopsy from clinical characteristic lesion in PRP, psoriasis, pemphigus, and mycosis fungoides.,
ED is a potentially life-threatening condition, especially in the neonatal and infantile period. The severe complications that can occur are infection, hypoalbuminemia, hyperpyrexia, and hypernatremic dehydration, which could lead to increased mortality. The principle of management was to treat and avoid precipitating factors. Supportive and symptomatic therapy was the same among all cases regardless of underlying cause. Correction of fluid and electrolyte imbalances, providing adequate caloric intake, and treatment of infection should be prescribed. Appropriate local skin care to maintain barrier function and hydration by wet dressing, topical application of emollients, and topical low-potency corticosteroids were required. Close and long-term follow-up to identify the cause and initiation of earlier treatment are recommended. Prognosis among patients varied according to etiology. Patients with previous dermatologic diseases may develop recurrent ED during disease flare. The outcome was unpredictable in idiopathic cases. Overall prognosis was poor in patients with immunodeficiency. Similar to previous studies, the mortality rate in neonatal and infantile ED was high, as compared to other age groups.,
Due to the retrospective nature of this study, some data may have been missing and some investigations may not have been performed in all patients. The hospital conducting this study is a national pediatric tertiary referral centre that takes care of the advanced and complex cases. As such, the findings described in this report may not be generalizable to the general population. However, this study did highlight and describe important features of pediatric ED in Thai children.
| Conclusion|| |
ED is a diagnostic and therapeutic challenge. Relevant history, clinical assessment, and laboratory investigations are essential for definitive diagnosis. The most common etiology of ED in children was preexisting skin diseases. Prognosis was poor in patients with immunodeficiency disorders.
This research was supported by Siriraj Grant for Research Development and Medical Education, Faculty of Medicine Siriraj Hospital, Mahidol University.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2], [Table 3]