|Year : 2017 | Volume
| Issue : 3 | Page : 248-251
Infantile erythrodermic psoriasis: A case report and review of the literature
Piyush Kumar1, Anupam Das2, Shvetha Jain3
1 Department of Dermatology, Katihar Medical College and Hospital, Bihar, India
2 Department of Dermatology, KPC Medical College and Hospital, Kolkata, West Bengal, India
3 Department of Dermatology, Sri Siddhartha Medical College, Vijayawada, Andhra Pradesh, India
|Date of Web Publication||7-Jun-2017|
Department of Dermatology, KPC Medical College and Hospital, Kolkata, West Bengal
Source of Support: None, Conflict of Interest: None
Erythroderma in infants can be attributed to plenty of causes, the more common ones being nonbullous congenital ichthyosiform erythroderma, lamellar ichthyosis, bullous congenital ichthyosiform erythroderma, severe atopic dermatitis, etc., However, there can be circumstances where infantile psoriasis can present to us with erythroderma; this may be a diagnostic challenge to the clinician. Hereby, we present a case of a young boy with recurrent erythroderma.
Keywords: Infantile erythrodermic variant, psoriasis, rare presentation
|How to cite this article:|
Kumar P, Das A, Jain S. Infantile erythrodermic psoriasis: A case report and review of the literature. Indian J Paediatr Dermatol 2017;18:248-51
|How to cite this URL:|
Kumar P, Das A, Jain S. Infantile erythrodermic psoriasis: A case report and review of the literature. Indian J Paediatr Dermatol [serial online] 2017 [cited 2018 Jun 23];18:248-51. Available from: http://www.ijpd.in/text.asp?2017/18/3/248/193026
| Introduction|| |
The etiological diagnosis of erythroderma in children can be challenging as a wide variety of conditions can present with erythroderma. Pruszkowski et al. found immunodeficiency (30%), simple or complex ichthyosis (24%), Netherton syndrome (18%), and eczematous or papulosquamous dermatitis (20%) as underlying causes. In an Indian study (n = 20), the underlying causes were infections (40%), ichthyosiform erythroderma (25%), atopic dermatitis (15%), infantile seborrheic dermatitis (10%), and unidentified (10%).
Psoriasis is uncommon in neonates and children (only 2% cases of psoriasis start before the age of 2 years) and is a very rare cause of congenital and neonatal erythroderma. Here, we present a case of a young boy with recurrent psoriatic erythroderma which was misdiagnosed as nonbullous congenital ichthyosiform erythroderma (NBCIE).
| Case Report|| |
A 3-year-old boy, born of nonconsanguineous marriage, presented with recurrent erythroderma since 1 month of age. There was no history of constrictive membrane at birth. Further, there was no history of bullous lesions at birth or thereafter. The boy developed generalized erythema and scaling at the age of 1 month. The boy was brought to a dermatologist and was diagnosed as NBCIE clinically. The boy improved with treatment but soon had recurrence. Since then, boy had multiple episodes of generalized erythema, and in between these episodes, skin used to become near normal. No other family member was suffering from similar skin lesion. On examination, boy was lethargic and irritable. His height for age, weight for age, and weight for height were <3 standard deviation indicating chronic undernutrition. There were generalized erythema and scaling involving whole body [Figure 1]a. One notable finding was the presence of pustules on the sole [Figure 1]b. Scalp, mucosa, and nails were unremarkable. Biopsy was done and sent for histopathological examination. Histology showed regular psoriasiform epidermal hyperplasia and perivascular mixed lymphocytic and neutrophilic infiltration. Stratum corneum was thickened and showed broad zones of parakeratosis, harboring numerous pyknotic nuclei of neutrophils. The granular layer was thin and there was prominent suprapapillary thinning [Figure 2] and [Figure 3]. Based on clinicopathological correlation, a diagnosis of infantile erythrodermic psoriasis was done. The child was prescribed isotretinoin (1 mg/kg body weight). Erythroderma subsided within 2 months [Figure 4]a but showed multiple recurrences (within a week) when we tried to reduce the dose of isotretinoin. Hence, the drug was continued for 4 months after the erythroderma subsided completely. The boy was under periodic monitoring for 1 year and subsequently lost to follow-up. On one occasion, boy showed lesions of chronic plaque psoriasis [Figure 4]b.
|Figure 1: Generalized erythema and scaling (a) and plantar surface showing multiple superficial pustular lesions (b)|
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|Figure 2: Histopathology of sample from trunk showing parakeratosis, parakeratosis, and Munro's microabscess (H and E, ×400)|
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|Figure 3: Histopathology of sample from pustule on sole showing acanthosis, spongiosis, and neutrophilic collection in upper epidermal layers (H and E, ×100)|
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|Figure 4: Resolution of lesions with systemic isotretinoin therapy (a) and recurrence of disease as papules, pustules, and plaques. It rapidly progressed to erythroderma (b)|
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| Discussion|| |
Erythroderma in adults has been extensively studied, but the condition in pediatric population remains elusive. Sarkar has found infections, ichthyosiform erythroderma, atopic dermatitis, infantile seborrheic dermatitis, and idiopathic cases as the leading conditions responsible for erythroderma in children. Among the infections, Staphylococcal scalded skin syndrome (SSSS) and candidiasis are the important ones. However, nonbullous ichthyosiform erythroderma and collodion babies constitute the major share of causes of ichthyosiform erythroderma.,
In SSSS, the general condition of the child is toxic. Initially, there is a generalized erythematous rash which subsequently develops into erythroderma, tenderness, blistering, and desquamation, with positive Nikolsky sign. Congenital cutaneous candidiasis is acquired during the passage of the baby through the birth canal. Beginning as a maculopapular rash, the lesions rapidly transform into pustules, followed by erythroderma. Paronychia and dystrophic nails help to distinguish from other etiologies of neonatal erythroderma.
Ichthyosiform erythroderma is attributed to NBCIE, bullous ichthyosiform erythroderma, Harlequin ichthyosis, Netherton syndrome, Keratitis-ichthyosis-deafness syndrome, Chanarin-Dorfman syndrome, trichothiodystrophy, and CHILD syndrome. In NBCIE, initially, there is a collodion membrane at birth which subsequently undergoes exfoliation to develop erythroderma. The important features are fine powder white to gray scales, presence of fissures and flexion contractures. Contrary to NBCIE, a child with bullous ichthyosiform erythroderma presents with flaccid blisters, rupturing to form erosions, erythroderma, and progressive hyperkeratosis with gray waxy scales. Netherton syndrome is characterized by a triad of ichthyosis linearis circumflexa, trichorrhexis nodosa, and atopic dermatitis.
Psoriasis is a confusing clinical diagnosis when present in the pediatric population. The diagnosis is even more challenging when it presents in an atypical form and/or the severity is mild. Clinically, the most common types of psoriasis encountered in children are plaque type psoriasis localized to the flexures, guttate psoriasis, and psoriatic arthropathy; followed by pustular psoriasis, erythrodermic psoriasis, and congenital psoriasis. Erythrodermic psoriasis is usually manifested as redness and scaling of more than 90% body surface area, and this variety may not be associated with any of the classical lesions of psoriasis, thus putting the dermatologist as well as the pediatrician in a diagnostic dilemma. It usually begins as recalcitrant diaper dermatitis, developing into generalized pustular psoriasis and arthropathy, infections, and growth retardation. Important diagnostic clues are presence of high fever and the child is toxic. Erythrodermic psoriasis in children is confused with NBCIE, lamellar ichthyosis, bullous congenital ichthyosiform erythroderma, SSSS, Leiner disease, Netherton syndrome, and severe atopic dermatitis. It can be differentiated from NBCIE by proper history taking and clinical examination. NBCIE is usually associated with positive family history and ectropion. As in our case, the clinical diagnosis was NBCIE, but histology confirmed it to be a case of psoriasis. Characteristic histologic features include hyperkeratosis, parakeratosis, hypogranulosis, elongated rete ridges, suprapapillary thinning, papillary dermal edema, dilatation of the capillaries, perivascular lymphocytic infiltrate, and neutrophilic aggregates within the epidermis (Munro's microabscess). However, it should be kept in mind that regular acanthosis and capillary dilatation are the early and consistent of infantile erythrodermic psoriasis, and Munro's microabscess and spongiform pustule of Kogoj are not essential for the diagnosis.
Atopic dermatitis leading to erythroderma can be a troublesome diagnosis for the clinician. Important diagnostic pointers include positive family history of atopy (hay fever, asthma, rhinitis, atopic dermatitis), sparing of the napkin area and axilla, and presence of itchy lesions on the cheeks and flexural creases of the limbs. However, the latter is usually prominent at the age of 3 months and this adds to the diagnostic confusion until frank lesions develop. Infantile seborrheic dermatitis, another cause of erythroderma, starts as an inflammatory, yellowish, scaling on the scalp gradually progressing to involve the intertriginous areas. Rare causes of erythroderma in children include immunodeficiency syndromes (Omenn syndrome), metabolic and nutritional disorders such as multiple carboxylase deficiency, essential fatty acid deficiency, protein energy malnutrition, and diffuse cutaneous mastocytosis. An underlying immunodeficiency should be ruled out in the presence of skin induration, severe alopecia, failure to thrive, alopecia of hair, eyelashes, and eyebrows, generalized lymphadenopathy, and infectious complications. Erythroderma with alopecia is also a diagnostic pointer toward metabolic causes including biotin deficiency and citrullinemia.
Although erythroderma is a clinical diagnosis, some cases require extensive workup. Investigations such as complete blood count; serum IgE levels; zinc levels; renal profile; hepatic profile; potassium hydroxide preparations; swabs from eyes, skin, and umbilicus; swabs from the vagina of mother; blood culture; sweat chloride levels; and various enzymatic assays to rule out metabolic disorders might be needed. Skin biopsy serves as an important diagnostic tool in ascertaining the cause of erythroderma in children. The three important histological patterns include eczematous, psoriasiform, and ichthyosiform. In case of an eczematous pattern, the presence of keratinocyte necrosis, satellite cell lymphocytes, along with lymphocytic or eosinophilic infiltration, point toward immunodeficiency syndromes. However, an absence of necrosis of keratinocytes does not rule out immunodeficiency, and it goes more in favor of atopic dermatitis. Psoriasiform hyperplasia, laminated parakeratosis, spongiform pustules of Kogoj, Munro's microabscess, and minimal dermal lymphocytic infiltrate go with psoriasis, consistent with our case. Compact orthokeratosis, ostial plugs, normal to thickened stratum granulosum with mild dermal infiltrate is consistent with ichthyosis. Psoriasiform hyperplasia with parakeratosis in the absence of neutrophils is a feature of Netherton syndrome.
The treatment of erythroderma in children consists of supportive management in the form of maintenance of fluid and electrolyte balance, ambient temperature, and strict aseptic environment. Besides, the role of antibiotics, antifungals, emollients, and topical corticosteroids cannot be overemphasized. Therapeutic options in pediatric psoriasis are plenty, but the choice should be governed by the type and extent of the disease; otherwise, it can lead to significant problems. According to a recent update, calcipotriene (±topical steroids) has emerged as the first-line drugs followed by dithranol. Indications for using systemic therapy in childhood psoriasis are refractory disease, erythrodermic, pustular, and psoriatic arthropathy. Methotrexate is the first-line therapy for childhood psoriasis. However, pustular and erythrodermic psoriasis respond better to systemic retinoids. We had prescribed isotretinoin to our patient. The response was good, but unfortunately, there were multiple episodes of recurrence.
Since the severe forms of the disease are rare in pediatric age group, there is a dearth of consensus and guidelines regarding the role of systemic drugs. Besides, lower tolerability and cumulative toxicities are important limiting factors in the use of these drugs in children.
This case demonstrates the difficulty in diagnosis of infantile erythrodermic psoriasis, and the subsequent hurdles in the management of the condition.
Declaration of Patient Consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial Support and Sponsorship
Conflicts of Interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]