Cap 16 Schwatz .

Pyoderma gangrenosum (PG)

Summary :
Lesion borders are purplish in color
debridement
surgical insult may trigger further
PG
split-thickness skin grafting

It is a relatively uncommon non-infectious Neutrophilic dermatosis. This disease is commonly associated with
inflammatory bowel disease, rheumatoid arthritis, hematologic malignancies, and monoclonal gammopathies.
Clinically, the condition is characterized by the presence of sterile pustules, which progress and ulcerate to variable
depths and dimension . Lesion borders are purplish in color with erythematous edges. Surgical debridement should be
used concomitantly with systemic therapy, as the surgical insult may trigger further PG. Wound closure can usually be
achieved with split-thickness skin grafting and temporary coverage with allografts or bioengineered skin substitutes.

Toxic Epidermal Necrolysis and StevenJohnson Syndrome

These inflammatory diseases represent a spectrum of an autoimmune reaction to stimuli such as drugs that result in
structural defects in the epidermal-dermal junction. The cutaneous manifestations of toxic epidermal necrolysis
syndrome (TENS) follow a prodromal period reminiscent of an upper respiratory tract infection.

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CHAPTER

16 THE SKIN AND SUBCUTANEOUS TISSUE A symmetrical macular eruption follows starting from the face
and trunk and spreading to the extremities. Typically, a Nikolsky sign develops in which lateral pressure causes the
epidermis to detach from the basal layer. The macular eruption evolves into blisters, causing an extensive superficial
partial-thickness skin injury with exposed dermis (Fig. 16-2). The process progresses for 7 to 10 days; reepithelialization occurs over 1 to 3 weeks. Mucosal and ocular surfaces may be involved in a similar fashion.
Immunosuppressed patients are at higher risk. TENS historically was considered to be the extreme of a spectrum, with
erythema multiforme and Stevens-Johnson syndrome (SJS) being less extensive forms of disease. Currently, erythema

multiforme is thought to be a separate entity, related to herpetic and Mycoplasma pneumoniae infection. TENS
involves more than 30% total body surface area; between 10% and 30% is considered the SJS-TEN overlap syndrome.
Prognosis is related to the extent of disease and related primarily to secondary infection and other intensive care unit
(ICU)-associated morbidity. With modern-day burn and ICU care, the mortality has declined significantly. The mildest
form of the disease is SJS, which clinically presents as second-degree burns appearing as erythema and blisters/bullae
of the oropharynx, anoderm, and torso. Less than 10% of total body surface area is involved with this disease. TENS is
driven by the same dermo-epidermal structural defects but consists of greater than 30% total body surface area. In
addition to the aforementioned, it affects the mouth, esophagus, small bowel, and colon, resulting in sloughing of
mucosa that may present as gastrointestinal bleeding and intestinal malabsorption. It also affects the eyes, genitalia,
and other mucosal surfaces. 18 The drugs most commonly associated with TENS-SJS include aromatic anticonvulsants,
sulfonamides, allopurinol, oxicams (nonsteroidal anti-inflammatory drugs), and nevirapine. The pathophysiology of
TENS is not completely understood; current theories involve apoptosis due to Fas-mediated mechanisms (a soluble or a
membrane-bound protein that causes apoptosis upon activation), granulysin (a proapoptotic protein that permits cellmediated cytotoxicity), and reactive oxygen species. There appears to be a genetic component, and genetic testing
before carbamazepine treatment is recommended in people of Han Chinese ancestry to exclude carriers of HLAB1502.
19 The two principles of TENS management include early withdrawal of the offending drug and supportive care (i.e.,
pain control, intravenous fluid, electrolyte repletion, prevention of skin infections, enteral feeds, and possible
respiratory support) in a burn unit. Despite drug withdrawal, noxious metabolites may persist. Wound care differs
between centers and focuses on debridement of devitalized tissue and coverage with nonadherent dressings. Temporary
skin coverage is sometimes needed until re-epithelialization is allowed to progress, reducing the probability of skin
infections and dehydration. Coverage can be achieved with biologic dressing (allograft skin), biosynthetic dressings
(Biobrane), and antimicrobial dressings (antibiotic or silver-impregnated such as Acticoat). A Woods lamp examination
every 1 hour should be performed to look for corneal sloughing. It should be noted that these diseases should be
distinguished from staphylococcal scalded skin syndrome, which clinically appears similar but is a result of exotoxins
produced after staphylococcal infections of nasopharynx or otitis media. Systemic treatment with steroids has fallen out
of favor due to increased sepsis rates, prolonged admission, and potentially higher mortality rates. Intravenous
immunoglobulin (IVIG) is thought to be a treatment given the presence of antiFas antibodies within IVIG. The
antagonistic antibodies inhibit Fas-mediated cell apoptosis. However, a high variability exists between batches with
this regard. There are mixed reports of IVIG treatment efficacy. A 2007 meta-analysis of nine IVIG trials concluded that
high-dose IVIG does, in fact, improve survival. 21 20 Other treatment protocols include plasmapheresis aimed at
decreasing cytokine and drug load, cyclosporine, cyclophosphamide, and antiTNF- antibodies.