Year : 2006 | Volume
: 51 | Issue : 3 | Page : 178--181
Neonatal and infantile erythroderma: 'The red baby'
Department of Dermatology & Venereology, Maulana Azad Medical College & Associated Lok Nayak Hospital, New Delhi - 110002, India
226, Ashirwad Enclave, Plot No. 104, Indraprastha Extension, Patparganj, New Delhi - 110092
Neonatal and infantile erythroderma or «DQ»the red baby«DQ» is a rare condition. It can be potentially life-threatening and requires urgent attention of the dermatologists. The causes of erythroderma in this age group are different from that in adults and distinctive, but maybe difficult to establish due to poor specificity of clinical and laboratory findings. Careful monitoring and management of the patient may improve the final outcome. In this review, the various causes of «DQ»the red baby«DQ» is discussed along with its prognosis and management.
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Sarkar R. Neonatal and infantile erythroderma: 'The red baby'.Indian J Dermatol 2006;51:178-181
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Sarkar R. Neonatal and infantile erythroderma: 'The red baby'. Indian J Dermatol [serial online] 2006 [cited 2020 Sep 28 ];51:178-181
Available from: http://www.e-ijd.org/text.asp?2006/51/3/178/27979
Erythroderma is an inflammatory skin disorder consisting of erythema and scaling affecting more than 90% of the body surface. Erythroderma in the adult age groups has been studied by various authors, but there is a paucity of studies on erythroderma in pediatric patients. Neonatal and infantile erythroderma or 'the red baby' is a rare condition; its frequency is unknown. In a study done by us at Lady Hardinge Medical College and Associated Kalawati Saran Children's Hospital, New Delhi, India, an incidence of 0.11% of neonatal and infantile erythrodermas was noted out of 19,000 pediatric patients treated over 6 consecutive years. Although, it is a rare entity, it definitely requires the dermatologists, urgent attention, to prevent it from being misdiagnosed and mismanaged.
Adult erythroderma is usually secondary to pre-existing diseases or may be drug induced whereas in neonates, it can be the primary manifestation of several diseases [Figure 1]. The etiology of neonatal and infantile erythrodermas is difficult to establish and is mostly delayed, due to the poor specificity of clinical and histological signs. Moreover, the causes of erythoderma in this early age group are unique and distinctive. The main causes of 'the red baby' are listed in [Table 1].
So far, there are two main studies on neonatal and infantile erythrodermas in the literature., Pruszkowski et al did a retrospective study in France of 51 patients who presented with erythroderma during the first year of their lives in order to determine the frequency of the various underlying causes of this disease, and also to establish which clinical or laboratory findings were relevant for the clinical diagnosis. They found that on an average the cause of erythroderma could be established only 11 months after its onset. The underlying causes which were determined were immunodeficiency (30%), simple or complex ichthyosis (24%), Netherton's syndrome (18%) and eczematous or papulosquamous dermatitis (20%). The erythroderma was of unknown origin in 5 (10%) patients. They found the following parameters valuable in determining the cause of the disease: congenital onset, skin induration, presence of large scaling plaques, alopecia with or without dysplasia, evolution, response to topical corticosteroid therapy, presence of infection and failure to thrive. Histopathology was useful in only 45% of the cases, but the authors felt that it was useful for detecting significant lymphocyte infiltration or keratinocyte necrosis indicating a diagnosis of Omenn syndrome or immunodeficiency. On follow up, the mortality rate was 16%, and severe dematosis continued in 67% of the survivors.
We conducted a retrospective study to delineate the various causes of erythrodermas in neonates and infants over a period of 6 years in one of the largest children's hospital in Asia, having its own dermatology unit. Only 20 (0.11%) neonates and infants had erythroderma, out of 19,000 pediatric patients treated in our unit. In our study we found the following diagnostic parameters useful; as also observed in the previous study: congenital onset, evolution, family history of atopy, pruritus, presence of large scaling plaques, and sites of predilection after clearing. As mentioned earlier by Hoeger and Harper, we included erythrodermas with blister formation in our study unlike the French study, because we felt that their inclusion as causes of neonatal and infantile erythrodermas is important in order to determine their exact frequency. It is well known that bullous diseases can have an initial generalized erythroderma phase which can subsequently develop into a definite clinical entity. The causes identified were infections (40%), ichthyosiform erythrodermas (25%), atopic dermatitis (15%), infantile seborrheic dermatitis (10%) and unidentified (10%). The infections were staphylococcal scalded skin syndrome in 6 (30%) and candidiasis in 2 (10%) while of the ichthyosiform erythrodermas, 3 (15%) had non-bullous ichthyosiform erythrodermas and 2 (10%) were collodion babies. Histopathology was contributory only in 35% cases.
The salient features of the two studies are enlisted in [Table 2]. The major difference was that although immunodeficiency was the leading cause in the study by Pruszkowski et al , we found infections to be the leading cause in our study. According to the French authors, the high frequency of immunodeficiency could be due to the recruitment of neonates and infants to the specialized pediatric department at a tertiary hospital. In the Indian study, we suspected immunodeficiency in 1 unclassified case and 2 cases of erythrodermas due to candidiasis. However, due to unavailability of laboratory facilities, we could not make a conclusive diagnosis. Moreover, systemic features such as lymphadenopathy, hepatosplenomegaly and failure to thrive, which are indicative of immunodeficiency were absent in these children. Although, we referred these patients to specialized centers with better laboratory facilities, they were subsequently lost to follow up. As correctly observed by Pruszkowski et al , an immunodeficiency must be suspected in cases of severe erythroderma with clinical features such as skin induration, severe alopecia, failure to thrive, infectious complications or evocative histological findings.
Although no clinical feature is characteristic of the cause of erythroderma in neonates and infants, some features may help in the diagnosis. In fact, sometimes there can be a long delay in reaching the diagnosis after the erythroderma clears after several months and the characteristic lesions can be observed. A congenital onset would be indicative of ichthyosis, infections or immunodeficiency. In the case of a collodion baby, the diagnosis of hereditary ichthyoses can be made. Once the collodion membrane is shed, it can evolve into non-bullous ichthyosiform erythroderma or some form of ichthyosis. A skin biopsy would be helpful in making the diagnosis.
Clinical features such as fever, irritability and increased skin tenderness would point towards infectious causes. Vesiculobullous lesions would be indicative of either bullous ichthyosiform erythroderma, staphylococcal scalded skin syndrome (SSSS) and mastocytosis. The Nikolsky sign would be positive in SSSS whereas mastocytosis would require a positive Darier's sign as well as a characteristic biopsy to establish the diagnosis. Alopecia, can be observed in Netherton's syndrome, immunodeficiency syndromes, and disorders of biotin metabolism. Systemic features, such as lymphadenopathy, hepatosplenomegaly and failure to thrive would again point towards immunodeficiency or a metabolic disorder.
Although atopic dermatitis can present with skin symptom in neonates, erythroderma is a rare manifestation of the disease in this age group. Vesicular and exudative lesions may occur. There is considerable clinical overlap between clinical features of atopic dermatitis and infantile seborrheic dermatitis at birth and infantile seborrheic dermatitis is actually considered to be a variant of atopic dermatitis. A positive family history and presence of dermatitis on the cheeks, flexural creases of the limbs and itching which is apparent after 3 months of age would point to the diagnosis. Infantile seborrheic dermatitis may have clinical presentation of cradle cap, psoriasiform appearance and involvement of the skin folds of the neck, axillae and groins. Congenital erythrodermic psoriasis is rare and may be indistinguishable from congenital ichthyosiform erythroderma. A positive family history, evolution into typical psoriasis lesions after a few months and histopathological features would indicate a diagnosis of psoriasis.
There are some rare causes of the "red baby". Neonatal erythroderma has also been attributed to ceftriaxone and vancomycin which may be obvious from the history. Holocarboxylase deficiency would have features of neonatal erythroderma and alopecia besides ketoacidosis, dehydration and coma with eventual death. There would be sharply marginated dermatitis on the scalp, eyebrows and eyelashes that spreads to the perioral, perianal and other flexural areas. Biotinidase deficiency presents after 3 months of life with hypotonia, lethargy and seizures with patchy alopecia, both these enzyme deficiencies come under "multiple carboxylase deficiency".
There is an entity known as "Leiner's disease" which is an umbrella phenotye rather than a specific entity and applied to neonatal erythroderma when other causes have been ruled out. It was initially described by Carl Leiner in 1988 to describe young infants with desquamative erythroderma, sparse hair, diarrhoea and failure to thrive. Actually, its aetiology is multifactorial. Glover et al reported five infants with Leiner phenotype, all of whom had immunological abnormalities; one had hypogammaglobulinemia, one had combined immunodeficiency which was later revealed to be Omenn's syndrome and three were diagnosed as Netherton's syndrome with elevated IgE levels.
Although laboratory investigations contribute minimally to the diagnosis, a few useful laboratory tests which can be done, if atopic or seborrheic dermatitis, ichthyosis and nutritional causes have been ruled out are: potassium hydroxide preparation, swabs from skin or eyes, nose, umbilicus or high vaginal swab from mother to show growth of Staph-aureus or yeasts, blood culture, Gram stain, complete blood counts, hair mount, total IgE levels and quantitative immunoglobulins, eosinophil count, sweat chloride, zinc and alkaline phosphatase levels, biotinidase and holocarboxylase assays and essential fatty acid levels. Of course, these would have to be correlated with the diagnosis the dermatologist has in mind.
Skin biopsy is important and it is advisable to take two or three simultaneous biopsies from different sites. Histopathology may sometimes show only dermatitic or psoriasiform changes as observed in the two main studies of neonatal and infantile erythroderma where the histopathology contributed to the diagnosis only in 45% and 35% cases, respectively., It may reveal changes of significant lymphocytic infiltration and keratinocyte necrosis with satellite lymphocytes in cases of erythroderma due to GVHD or Omenn's syndrome.
Prognosis and treatment
In the study done by Pruszkowski et al , the prognosis was poor with a mortality of 16%. Patients died either due to complications arising from their erythroderma or their underlying disease at a mean age of 7 months. In 67% of the survivors, severe dermatoses persisted at a mean follow up of 5.5 years. The main complication observed were hypernatremic dehydration, infections and failure to thrive, due to severity of skin disease and the underlying disease.
Neonatal and infantile erythroderma is a potentially life-threatening condition with risks of septicemic infections, hypoalbuminemia, hyperpyrexia and hypernatremic dehydration. Careful management would include monitoring of vital signs and electrolyte levels, adequate oral or parenteral fluid intake, prevention and treatment of infection, condition of caloric and protein intake, topical application of emollients or antifungals, wet dressings, or steroids. Recognition and proper diagnosis by a dermatologist is necessary to prevent treating the condition wrongly with topical corticosteroids.
Neonatal and infantile erythroderma is a rare condition, but can be potentially life threatening irrespective of the cause. It is mostly difficult to establish the diagnosis of the condition due to poor specificity of clinical and histological signs. However, certain parameters may be helpful in making the diagnosis. Careful monitoring and management of the patients may improve the final outcome. However, most neonatal and infantile erythrodermas progress to severe dermatoses except those attributable to infection or drug related causes.
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