Management of Pediatric Dermatological Emergencies

Pediatric skin disorders account for a significant number of cases presenting to emergency departments or pediatric units for evaluation, with around half due to infectious causes.

Skin problems can also occur in the neonatal period and may require prompt treatment.

While many children will have minor conditions, or at least diseases that can be safely treated on an outpatient basis, a minority will present with cutaneous manifestations of diseases that require urgent treatment.

This is a relatively rare presentation in children, accounting for less than 1% of pediatric dermatological clinical presentations, but is potentially life-threatening. Erythroderma or generalized exfoliative dermatitis is defined as an inflammatory disorder with erythema affecting more than 90% of the body surface.

It is often associated with generalized lymphadenopathy , blistering, or peeling. Complications include hypothermia, hypernatremic dehydration, hypoalbuminemia, sepsis, and high-output heart failure.

In infants, hereditary ichthyoses may also present with erythroderma. These are a large group of heterogeneous disorders characterized by scaly, dry, and rough skin.

They include nonbullous ichthyosiform erythroderma, bullous ichthyosiform erythroderma, Netherton Syndrome, and Conradi-Hunermann Syndrome. Children with these conditions have genetically deficient skin barrier function, which predisposes them to inflammatory skin responses.

Immune conditions that present with erythroderma include Omenn syndrome due to severe combined immunodeficiency (SCID). This disorder is characterized by growth retardation, erythroderma, recurrent infections, and lymphadenopathy. Similar presentations occur in graft-versus-host disease and hypogammaglobulinemia.

Regardless of its cause, treatment of erythroderma must be prompt and vigorous. Maintaining adequate oral or parenteral fluid intake and monitoring serum electrolytes is essential for supportive treatment. Attention should also be paid to wound care, nursing management, and thermoregulation.

Topical fatty emollient preparations should be used liberally to hydrate the skin and prevent cracking. Blisters and erosions can be managed with 0.01% potassium permanganate and systemic antibiotics if necessary.

This condition is slightly more common in premature neonates 

A taut yellowish film stretches over the skin. The eyelids and lips may be stuck together and everted (ectropion and eclabion), the nostrils blocked, and the ears flattened. The constriction can lead to digital ischemia and the fingers may appear sausage-shaped.

Typically, the membrane peels off and reforms, drying with cracks and detachments for 1-4 weeks. Common underlying conditions are erythrodermic or lamellar ichthyosis.

Restrictive dermopathy is an important differential diagnosis. This presents as a rigid baby syndrome with tense, inflexible, generalized skin that does not come off from birth.

Harlequin ichthyosis is the most severe form of congenital ichthyosis, characterized by a thickening of the keratin layer of fetal human skin. Thick diamond- or plate-shaped scales are observed, which restrict the baby’s movement and can lead to contractures.

In most cases, mutations are found in the ABCA12 gene. The ABCA12 gene provides instructions for creating a protein that plays a role in lipid transport in the epidermis. Without sufficient amounts of this protein the skin is thick and inflexible.

Movement is restricted and splits develop in the thick scales giving deep red fissures. Respiratory failure may occur due to restricted movement of the chest and also due to prematurity.

Harlequin ichthyosis can generally be distinguished from baby collodion but cases with intermediate features have been seen. For both conditions, complications include hypothermia, dehydration, hypernatremia, and sepsis from erosions (see Table 1).

It is rare and presents as generalized pustules on red, sensitive skin. There may be a history of chronic plaque psoriasis or triggers such as infections and medications, including sudden discontinuation of corticosteroids.

Systemic symptoms such as fever, headache, muscle weakness, diarrhea and vomiting may occur. After a few days, the pustules coalesce to form lakes of sterile pus that dry out and peel off in successive cycles.

The skin should be swabbed to exclude infection and emollients applied. A skin biopsy is required to confirm the diagnosis. A strict fluid balance and control of urea, electrolytes, creatinine and calcium is necessary. A dermatologist should be involved in care as systemic immunosuppressive therapy with methotrexate, cyclosporine, or acitretin is usually required.

An important differential diagnosis is acute generalized exanthematous pustulosis (AGEP). PEAG is an acute febrile reaction to medications that results in numerous small sterile pustules on erythematous skin.

Unlike Stevens Johnson syndrome, there is no mucosal involvement

Treatment of PEAG is supportive and the offending drug should be withdrawn.

SEPE is a rare disease of early childhood. It typically affects children under 5 years of age. It is important to differentiate PES (synonyms Ritter’s disease, neonatal pemphigus) from toxic epidermal necrolysis (TEN), which is usually a clinical diagnosis.

The characteristic feature of skin sloughing off with light pressure (Nikolsky’s sign) is usually present, but skin biopsy may be useful in cases where there is diagnostic doubt. In SEPE, there is a superficial separation within the epidermis, just below the stratum corneum, while in TEN there is dermo-epidermal separation and total epidermal necrosis.

The superficial nature of the skin that is shed in SEPE ensures that healing occurs without scarring. However, infants are vulnerable to fluid loss and infection and without prompt treatment can be fatal. Although often considered a specific feature of SEPE, Nikolsky’s sign can also be seen in Stevens-Johnson syndrome.

SEPE-like TSS is caused by exotoxins produced by Staphylococcus aureus or Streptococcus Pyogenes (Group A Streptococcus). It is an acute multisystem disease that causes tissue damage, disseminated intravascular coagulation, and organ dysfunction. Even with optimal care it still has a high mortality (5-15%) and is a diagnostic and therapeutic challenge.

TSS can present at all ages with fever, hypotension, and shock. Shortly after birth it is most commonly due to transmission of toxigenic S. aureus from an intrauterine infection or acquired at the time of delivery.

The extensive skin rash initially resembles scarlet fever and may develop into erythroderma. Rapid multi-organ failure can occur within 8-12 hours after the onset of symptoms. There may be hyperemia of the mucous membranes and peeling of the skin of the hands and feet 10-21 days after the onset of the disease.

Biochemical disorders often include elevated creatinine, serum transaminases, and bilirubin. S. aureus is cultured in less than 5% of cases. Therefore, the diagnosis is mainly clinical.

Treatment includes prompt and aggressive management of intravascular volume loss, oxygen, and intravenous antibiotics.

Antimicrobials suitable for suppression of toxin production include clindamycin and linezolid . The use of intravenous corticosteroids and immunoglobulins in TSS is still controversial and should be consulted with a pediatric infectious disease specialist.

Eczema herpeticum is an acute disseminated herpes simplex virus (HSV) infection in a patient with atopic dermatitis, often associated with systemic symptoms. Generalized cutaneous HSV infection also occurs with other underlying dermatoses, including congenital burns and ichthyosis, and in immunocompromised children.

It typically presents as a sudden deterioration of childhood eczema. Lesions include vesicles, papules, scabs and punched lesions that tend to appear in outbreaks and are therefore seen at different stages. Any skin site may be involved. Associated symptoms include fever, severe itching, malaise, vomiting, anorexia, diarrhea, and lymphadenopathy.

Prompt recognition is essential, since multi-organ involvement is involved and multi-organ failure can occur. Signs of eczema herpeticum can be subtle and difficult to distinguish from bacterial infection, especially since secondary bacterial infection usually with S. aureus and S. pyogenes can occur in eczema herpeticum.

Treatment is supportive and with antiviral therapy. Most children will need intravenous acyclovir (500 mg/m2 three times a day between 3 months and 11 years; 10 mg/kg between 12 and 17 years). Children who are systemically well and have more localized lesions can be treated with oral acyclovir or valacyclovir (although the latter is not licensed in children under 12 years of age).

Supportive treatment includes antipyretics and analgesics, antibiotics for secondary bacterial infection, intravenous fluids to correct dehydration and electrolyte imbalance, and blood components if necessary.

Topical steroid ointments/creams as well as calcineurin inhibitors (pimecrolimus and tacrolimus) should be discontinued in the acute phase of eczema herpeticum as they are thought to promote the spread of HSV.

ADRs can mimic other childhood skin diseases, especially viral exanthems. Serious, life-threatening drug reactions in children are fortunately rare.

SJS and TEN are delayed-type (Type IV) hypersensitivity reactions and are part of the same spectrum of disease entities. The main difference lies in the percentage of body surface area (BS) that presents skin detachment.

In SJS, epidermal detachment affects less than 10% of the SC, SJS/TEN overlap is when the detachment varies between 10% and 30%, and NET when it affects more than 30%.

SJS/TEN is an acute inflammatory disease, characterized by 1-7 days of nonspecific prodromal symptoms such as headache, malaise, fever, and upper respiratory tract symptoms, followed by skin and mucosal involvement.

It is common to have at least two mucous membrane sites involved. Common symptoms include purulent conjunctivitis, erosions, ulcers and crusts affecting the mouth, nose, pharynx, respiratory and digestive tracts. The skin lesions are characteristically targetoid and progress to blisters and sheet-like epidermal detachment.

As in SEPE, the Nikolsky sign can be positive. Visceral involvement of the kidneys, gastrointestinal tract, or liver may occur. Recovery may be slow, with the acute episode lasting 4 to 6 weeks.

The causative agents for SJS include mainly medications but also infectious agents such as Mycoplasma pneumoniae . Commonly the drugs involved are sulfonamides and anticonvulsants such as phenobarbital, lamotrigine and carbamazepine.

Supportive treatment is the only universally accepted treatment for SJS. While some studies have shown that short courses of high-dose corticosteroids have improved outcomes in SJS, others have been inconclusive, showed no benefit, or even showed worse outcomes with corticosteroid use.

A small number of studies and case series have explored the use of intravenous immunoglobulin (IVIg) in SJS and TEN. Most show favorable results, but others show no benefit and more high-quality studies are needed.

Complications include nail loss, scarring of mucosal surfaces, including the respiratory and gastrointestinal tract, joint contractures, and a number of eye problems .

SJS must be distinguished from erythema multiforme (EM), which is a hypersensitivity reaction generally caused by infections, most commonly HSV. It presents as a polymorphic skin rash characterized by macules, papules with an abnormal center (the typical target lesion), and plaques. Injuries can be in different stages.

In minor EM, there is very little mucosal involvement while in major EM one or more mucosal sites may be affected, including the eyes, mouth and genitals.

The involvement of an ophthalmologist is necessary since ocular complications can be serious. It is acute and self-limiting, generally resolving in 2-3 weeks for minor MS and 6 weeks for major MS, without complications, and without progression to SJS/TEN.

TEN is almost exclusively caused by a drug rash. Sensitive, painful skin with toxicity is the hallmark of the disease. The epidermis peels off in large sheets revealing diffuse erythroderma.

Complications of TEN can be very severe, affecting the respiratory, gastrointestinal, hepatic, and renal systems. Mortality is high, even with optimal care, ranging in the literature between 10% and 70% of cases (see Table 2).

Because of the potential for serious complications, patients with NET are best managed in pediatric intensive care or burn units. Discontinuation of the offending drug is essential, and supportive treatment includes aggressive wound care, mechanical ventilation, and hemodynamic support.

Fluid intake and nutritional care are vital due to extensive epidermal loss. Patients should be isolated and appropriate measures taken to prevent infection. Ocular involvement must be anticipated and controlled by ophthalmology.

Some studies have suggested that silver sulfadiazine dressings should be avoided due to the association of sulfonamides with TEN.

Specific treatments for NET are controversial but have included cyclosporine, plasmapheresis, and TNF-α inhibitors. Patients treated with steroids and IVIg seem to have a better outcome. Where possible a consultant specialist in pediatric immunology should be involved in management.

Although the SCORTEN criteria work best in adults, they give an indication of severity (Table 2). One point is scored for each of the seven criteria present at the time of admission. Mortality increases with increasing score, rising from less than 12% for two points to less than 90% for ≥ 5 points.

DRESS syndrome (drug rash/reaction with eosinophilia and systemic symptoms) is a severe drug reaction that presents with rash, fever, lymphadenopathy, and visceral involvement, and tends to occur 2 to 6 weeks after initiation of an offending drug (Table 3).

The rash is typically diffuse morbilliform and may progress to blisters and erythroderma. Nikolsky’s sign is absent. Visceral involvement may include hepatitis, carditis, pneumonitis, interstitial nephritis, and thyroiditis. This syndrome has a 10% mortality rate in the literature, most commonly due to fulminant hepatitis with liver necrosis.

Common causative drugs include anticonvulsants such as carbamazepine, phenobarbital, phenytoin, and lamotrigine, as well as sulfonamides.

The offending medication should be withdrawn immediately. Supportive treatment includes antipyretics for fever and aggressive wound care for exfoliated areas of skin. When possible, admission to an intensive care or burn unit is advised.

Topical steroids can be used for symptomatic relief, but systemic corticosteroids are generally needed gradually over 6 to 8 weeks. Other immunosuppressants have been used successfully, including IVIg and plasmapheresis, but there is little evidence to justify their routine use.

Purpura fulminans is a hematological emergency that occurs in children when hemorrhagic infarction of the skin and disseminated intravascular coagulation (DIC) occur.

The most severe form is known as acute infectious purpura fulminans and occurs in severe acute sepsis. It is a cardinal feature of meningococcal septicemia. Less commonly, it is seen in sepsis due to Streptococcus, Haemophilus and Staphylococcus, especially in asplenic patients.

It can rapidly progress to multi-organ failure. Skin manifestations progress rapidly from scattered petechiae to purpura and finally necrosis and gangrene.

The distal extremities are the most affected, generally symmetrical. Meningitis, DIC, and acute respiratory distress syndrome (ARDS) are common complications.

Most infantile hemangiomas will not need treatment, and many more will not constitute emergencies. Those that begin to affect vital structures, e.g. eye or airway, diaper area that is easily damaged or those that will cause a poor cosmetic result should be urgently referred to pediatric dermatology.

Topical timolol can be used for small flat lesions and oral propranolol is the treatment of choice for the rest. A cardiac and general examination is required before starting propranolol and it is useful to consider whether the child has an associated syndrome (Table 4).

Babies with multiple small hemangiomas require a thorough examination to rule out heart or liver failure and a liver ultrasound to look for hemangiomas in the liver. If present, these lead to high mortality and are an indication for urgent treatment with propranolol.

Epidermolysis bullosa (EB) is a group of genetic disorders that result in fragility of the skin and mucosa leading to blistering. There are four main types of EB: simple EB, junctional EB, dystrophic EB and Kindler syndrome depending on the level of cleavage within the skin. It is very difficult to differentiate between types of BE and detailed prognostic information should not be given until biopsy results are available.

Blisters may present at birth or soon after or large unroofed areas may be present especially on the lower extremities. It is important to consider EB in the differential diagnosis of neonatal blisters while excluding infectious causes.

In England, there are two regional centers for EB based at Birmingham Children’s Hospital and Great Ormond Street. An appropriate center should be contacted without delay for advice. They operate an outreach service by sending packs of dressings by express courier, arriving as soon as possible to the neonatal unit to demonstrate skin care and perform the skin biopsy.

Table 1. Treatment of baby collodion and harlequin ichthyosis.

Table 2.SCORTEN criteria for SSJ/NET

Table 3. RegiSCAR inclusion criteria for DRESS syndrome. Three of the four highlighted criteria are required for diagnosis

Table 4. Syndromes associated with hemangiomas.