Urgenţe dermatologice în neonatologie şi practica pediatrică

Introduction

Several studies have focused on dermatological diseases in children, but too few on dermatological emergencies, these being a challenge for both the physician and the patient.

The dermatological problems in children represent 30% of the visits to the doctor⁽¹⁾.

In a Swiss pediatric emergency department a wide range of dermatological diseases was identified for patients, most of which being inflammatory/allergic (42.9%) or infectious (31.8%)⁽²⁾.

The study from New Delhi found the prevalence of neonatal dermatological emergencies in the studied group as follows: epidermolysis bullosa (50%), collodian baby (33.3%), dermatological reactions associated with the intensive neonatal unit (18.7%), harlequin’s fetus (16.7%), infantile seborrheic dermatitis (12.5%); while the prevalence of post-neonatal dermatological emergencies was: drug reactions (56.4%), infections (28.2%) and others (15.4%)⁽³⁾.

Although it is a long list of dermatological emergencies, we will treat some of these diseases separately.

Erythroderma

Erythroderma (exfoliative dermatitis) is a generalized inflammatory disease with metabolic, hemodynamic and thermal implications. Erythroderma should be mentioned because it puts lives at risk; however, it occurs in less than 1% of cases of pediatric dermatology⁽⁴⁾.

The etiology disease in a child may be hereditary or acquired. Hereditary: keratom malign, Sjöngren-Larsson syndrome, Rud syndrome, Netherthon syndrome, congenital bullous erythroderma, rubella pilaris congenital pitiriasis. Acquired: Ritter von Rittersheim erythrodermia, Leiner Moussous erythroderma, toxic erythroderma, erythroderma of the Letterer-Siwe disease, erythroderma due to preexisting dermatoses such as psoriasis, mastocytosis, atopic dermatitis, lichen^(5,6). However, there are situations where the etiology remains unknown.
 

Erythroderma covers over 90% of the skin surface and the patient may have pruritus, cold sensation, fever over 39⁰C, increased basal metabolism, red to violet skin, edema can lead to vesicles, scaling occurs in 4-5 days or at 10-15 days, the skin is fattened, the patient may have chelation, stomatitis or conjunctivitis, hyperkeratosis of palms and soles and cracks. Most often, lymphadenopathy or splenomegaly occur^(5,7).

Among complications we include: hydroelectrolytic disorders, respiratory failure, heart failure, cachexia, renal failure, predisposition to infection. The presence of complications can cause a poor prognosis for the patient⁽⁸⁾.

Treatment is done with corticotherapy, but not when erythroderma is caused by psoriasis. Thermal comfort, hydroelectrolytic rebalancing, antibiotherapy, antipruriasis must be ensured⁽⁹⁾.

Collodion baby

Collodion baby (congenital ichthyosis) is a condition that occurs at birth, especially preterm. It is inherited by autosomal recessive genes and affects both sexes. The baby is wrapped in a glowing membrane that begins to crack in 48 hours and within a few days there is a generalized squamous erythema.

Collodion babies require intensive care; they need to stay in an incubator to regulate water and heat loss. The treatment consists of frequent application of moisturizers and mouth care, because the lips can become painful, but also the eyes that can dry out^(10,11).

Most patients evolve into various types of congenital ichthyosis, such as transglutaminase-1 deficient lamellar ichthyosis, trichotiodistrophy, Sjögren-Larsson’s syndrome or Gaucher disease⁽¹²⁾.

Even though mortality occurs in 10% of cases⁽¹⁰⁾, most cases spontaneously heal in the first few weeks of life.

Acute erythrodermic psoriasis

Acute erythrodermic psoriasis is a serious form of psoriasis with high risk of mortality and difficult to treat. It may be a new psoriasis or a complication of a preexisting psoriasis^(13,14).

Skin manifestations can be: erythema, edema, generalized pruritus, intense desquamation, nail and hair damage⁽¹⁵⁾. Systemic manifestations: obviously altered general condition, fever, chills, heart failure⁽¹⁶⁾.

Although more than 25% of cases are due to psoriasis, it is important to make a differential diagnosis with other causes of erythroderma⁽¹⁷⁾; if necessary, skin biopsy can be performed.

Regarding the treatment of erythrodermic psoriasis, the most commonly used methods are systemic retinoid, methotrexate, and cyclosporine⁽¹⁸⁾. The decision to implement the treatment and the monitoring should be done in a multidisciplinary team from which the dermatologist should not miss.

Toxic shock syndrome (TSS)

The toxic shock syndrome is determined by an immune reaction caused by the deficiency of antibodies against the streptococcal or staphylococcal toxin.

At birth, S. aureus can be transmitted from an intrauterine or acquired infection during delivery. The most common risk factor of toxic shock syndrome in pediatrics is varicella, that can lead to group A streptococcal skin infection, favoring the onset of toxic shock syndrome⁽⁴⁾.

Symptoms of toxic shock may be: faster influenza flu symptoms, skin reaction mimicking sunburn, erythroderma, skin necrosis, desquamation (during healing, days 7-14), fever, vomiting, diarrhea, syncope, hypotension, sepsis. It can cause complications of the lungs, liver and kidneys. Possible major complications: acute respiratory distress syndrome, disseminated intravascular coagulation, renal failure⁽¹⁹⁾.

The diagnosis is clinical, usually the serum levels of transaminases, bilirubin and creatinine being increased. Differential diagnosis is done with Gram-negative septic shock, staphylococcal toxic shock syndrome, severe dengue.

Because toxic shock syndrome has a rapid evolution, therapy should be initiated as soon as possible. Intravenous fluids, oxygen therapy and intravenous antibiotics are given.

Mortality occurs in 30-70% of cases, sometimes even in cases where treatment has been established correctly⁽²⁰⁾.

DRESS syndrome

DRESS syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) is also known as hypersensitivity reaction. It is a condition endangering the patient’s life, with organ dysfunctions.

Dress syndrome is due to drug reactions, most often found as a delayed reaction to antiepileptic drugs⁽²¹⁾.

The patient has a diffuse cutaneous eruption that can progress to erythroderma, lymphadenopathy, fever, visceral involvement, eosinophilia, and possibly atypical lymphocytes^(21,22).

Systemic damage may include liver, kidneys, lungs and gastrointestinal disorders; later, patients may develop insipid diabetes, thyroiditis and adrenal insufficiency⁽²³⁾.

The differential diagnosis in children is made with infectious diseases, immunological diseases and neoplastic diseases.

Immediate patient hospitalization and stopping the­rapy that triggered the syndrome are required. It is usually necessary to administer systemic corticoste­roids for a short period of time; antipyretics and topical corticosteroids may also be used.

The administration of intravenous immunoglobulins has been shown to be ineffective⁽²⁴⁾.

Purpura fulminans

Purpura fulminans is a life-threatening condition, being a hematological emergency. It is a very severe infection with a high mortality rate, the most severe and most common form being caused by meningococcus.

The patient presents the destruction of soft tissues and necrotic lesions that may even include the bones. Due to  the alteration of endothelial vascular function, thrombotic and hemorrhagic lesions increase⁽²⁵⁾.

For treatment use: antibiotics, corticoids, vascular filling and catecholamines; depending on the evolution, between 10 days and 3 weeks, the necrotic lesions will be debrided and if an amputation is necessary, the psychological counseling of the child will be mandatory⁽²⁶⁾.

From the point of view of complications, there is a great possibility of organ failure and the skin will change very rapidly from scattered petechiae to gangrene⁽²⁷⁾.

Conclusions

Although intensive management and care are the responsibility of the pediatrician, it is necessary to collaborate with the dermatologist for diagnosis confirmation, disease prevention and early treatment of life-threatening conditions.  

Conflict of interests: The authors declare no conflict of interests.