Dermatological Emergencies: General Considerationschapter 1

The explosion of new medical knowledge and resultant technologies for patient care has changed the scope of dermatology. Dermatologists play a key role in diagnosis and management of skin disorders in intensive care units and emergency departments as well as in the more traditional outpatient arena. All practicing dermatologists must now not only be familiar with the classic dermatological disorders but also be able to recognize, evaluate, and treat emergencies in dermatology.

We must salute the forethought of Shuster, the great dermatologist, who dealt with ‘systemic manifestations of cutaneous disorders’ way back in 1967. His treatise on this subject is the basis of coinage of the term ‘acute skin failure’ and rationale for intensive care in dermatology.

The concept of dermatologic intensive care unit (DICU) is also gaining wider acceptance, as it has become mandatory to manage patients with acute skin failure, e.g. toxic epidermal necrolysis (TEN), etc., in an aseptic 2environment with multispecialty consultation and all the necessary gadgets for maintaining vitals, fluid and electrolyte balance. It is almost akin to ‘burn ward’. In fact dermatological emergencies are still managed in burn ward in many parts of the world.

Burn injury affects skin integrity and partial thickness burns usually blister. In patients with burn, the barrier function against fluid loss is totally damaged. Burn injury is a dynamic process that peaks at about 3 days after the thermal trauma. TEN is a widespread immunological injury leading to epidermal loss in sheets leaving a dark red oozing dermis as compared to the burn injury which involves the deep dermis too. TEN carries worse prognosis than burns of the same extent because of systemic involvement like hepatitis, hematological abnormalities, and subclinical interstitial edema culminating into frank pulmonary edema. Required quantity of fluid replacement also differs in both the conditions. Hence, it was right by Professor Rene Touraine to shove a unit devoted to the intensive care of dermatological diseases way back in 1976 amidst the criticism he faced from his colleagues.

In the medical field ‘emergency’ stands for ‘immediate action’ to tackle the unforeseen (expected or unexpected) complication of the disease process or those arising from use of the therapeutic agents.

In a secondary analysis of the Intensive Care National Audit & Research Centre (ICNARC) Case Mix Programme Database (Critical care 2008), data was extracted for 476,224 admissions to 178 intensive care units (ICU) in England, Wales and Northern Ireland participating in the programme over the time period from December 1995 to September 2006. They identified admissions with dermatological conditions from the primary and secondary reasons for admission to ICU, and total 2,245 dermatological admissions were identified. Conditions included infections (e.g. cellulitis, necrotizing fasciitis), dermatological malignancies, and acute skin failure (e.g. Stevens–Johnson 3syndrome [SJS], TEN, and autoimmune blistering diseases).

The incidence of SJS and TEN are better characterized, and have been estimated at 0.4 to 1.2 and 1.2 to 6 million person years, respectively. There is a slightly increased risk in females. Mortality from erythema 4multiforme (EM) does not exist but may occur in 10% for patients with SJS, approximately 30% for patients with SJS/TEN-overlap and almost 50% for patients with TEN. For SJS, SJS/TEN-overlap and TEN together the mortality rate is almost 25%. In order to evaluate the mortality due to SJS/TEN, time of death in relation to the onset of the reaction, age of the patient, underlying diseases and the amount of skin detachment have to be considered. Medications most commonly associated with these conditions are sulfonamide and non-sulfonamide antibiotics, anticonvulsants and nonsteroidal anti-inflammatory drugs. Of these medications, sulfonamides have the highest risk. In the era of HIV/AIDS and increased incidence of associated oppurtunistic infections, the list of drugs causing severe cutaneous drug reaction is expanding. Abacavir hypersensitivity is a well-known entity. Nevirapine (antiretroviral) and lamotrigine (antiepileptic) are the newer drugs added to the list of drugs causing SJS/TEN.

In an overview of the major causes responsible for erythroderma from 13 reviews quoted in Mark Lebwohl's ‘Difficult diagnoses in dermatology’, approximately half of all the cases were secondary to primary dermatological condition (eczema, psoriasis, pemphigus foliaceus, lichen planus and pityriasis rubra pilaris), with remaining cases occurring due to systemic drug reactions, malignancy and of undetermined etiology. In a study involving 91 patients with erythroderma, the disease specific mortality was 18%.

Ten to 25% of angioedema patients are due to angiotensin-converting-enzyme inhibitor (ACEI) therapy, occurring in 1–2/1000 new users.

Prompt recognition and diagnosis with subsequent appropriate treatment might improve the prognosis in both the categories. Table 1.2 presents the list of conditions which may result in dermatological emergency.

Irvine (1991) was the first person to define the skin failure as ‘loss of normal temperature control with inability to maintain the core body temperature and failure to prevent percutaneous loss of fluid, electrolytes and protein with resulting imbalance, and failure of mechanical barrier to prevent penetration of foreign materials’ and proposed that it was a real entity comparable to any other major organ system dysfunction.

The principal pathomechanism of ASF has been summarized in Figure 1.1.

Regular skin swab from different body sites should be taken to know the organism, which may be the cause of infection and to decide upon appropriate antibiotic. Antibiotics are indicated if there is sign of sepsis. Some authors administer broad spectrum antibiotics empirically, if there is leukopenia associated with TEN.

In the event of inadequate oral intake, nasogastric feeding is advised (in patients without any evidence of malabsorption). If intravenous fluid has to be administered, peripheral line should be through the uninvolved skin. Intravenous fluids administered at similar rate like patients with burn in a patient with ASF may end up with pulmonary edema. Hence, it is important to estimate fluid loss and requirement in each individual patient with ASF by repeated clinical assessment. In the first 24 hours, the fluid administration may be estimated according to the extent of skin loss.

Temperature control is an important issue in ASF. The room temperature should be increased to prevent shivering and calorie loss. This may be achieved by air conditioners or infrared light in resource poor setup. Optimum moisture can be maintained by boiling a large kettle in the corner of room.

Noninvasive hemodynamic monitoring by pulse oximeter is preferred especially when the skin on the arms is involved (difficult to record blood pressure with sphygmomanometer). Close monitoring of urine output and vital parameters is essential.11

Multidisciplinary approach is called for to address all the known causes of mortality like secondary infection and septicemia, fluid and electrolyte imbalance, hypovolemic shock, cardiovascular failure, pulmonary edema and disseminated intravascular coagulation. Physicians, surgeons, pediatricians and anesthetists play important role in the management of patients with ASF. These goals can be achieved in a setup of DICU. Dermatologists need to know when and where to utilize the cross-consultation of fellow medical colleagues. It is also mandatory to know the basic minimum about the procedures necessary for revival of the patient in case need arises.

In conclusion, patients with dermatological emergencies deserve special attention and management protocol. Establishment of DICU is a positive step towards achieving this goal. Organization of a dedicated and coordinated multi-specialty member team is the essential initial step for establishment of DICU. Various stages of establishment of DICU has been discussed in chapter 7.