Stevens Syndrome – Johnson, drug rash with eosinophilia and systemic manifestations, fulminant purpura, acute generalized exanthematous pustulosis, pyoderma gangrenosum: what do all these pathologies have in common?
In the article estet-portal.com you can get acquainted in detail with drug-inducedsystemic activation of immunological reactions, the primary manifestation of which is skin manifestations. Early diagnosis and timely administration of immunosuppressive therapy can save the patient from death.
- Skin manifestation #1 — Stevens–Johnson Syndrome
- Skin manifestation #2 — Drug eruption with eosinophilia and systemic involvement
- Skin manifestation #3 — Fulminant purpura
- Skin manifestation #4 — Acute generalized exanthematous pustulosis
- Skin manifestation #5 — Pyoderma gangrenosum
Skin manifestation #1 — Stevens–Johnson Syndrome
In adults, Stevens-Johnson syndrome usually develops in response to drug administration in the previous 3 weeks.
The combination of trimethoprim and sulfamethoxazole has been identified as the most common trigger in the United States, but other drugs have also been associated with this disorder. The incidence of the syndrome cited in the literature is one in 1 million, but anecdotal evidence suggests that Stevens-Johnson syndrome occurs more frequently.
Morbidity and mortality can be quite high. Hospitalization is mandatory and, depending on the severity, admission to an intensive care unit or a burn unit may be required.
Wound care is critical.
Patients with Stevens-Johnson syndrome usually die from sepsis, which develops due to the destruction of the skin barrier and exposure of the dermis. The syndrome is associated with long-term rehabilitation due to mucosal lesions.
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The syndrome is complicated by the formation of scars on the cornea, esophagus, vaginal mucosa and urethra, so the most aggressive and effective therapy should be selected for the treatment of these patients.
Multidisciplinary management includes ophthalmic, gynecological and urological care.
Skin manifestation #2 — Drug eruption with eosinophilia and systemic involvement
The mortality rate for DRESS is about 10%.
Drug rush with eosinophilia and systemic symptoms (drug rush with eosinophilia and systemic symptoms — DRESS) is characterized by the presence of general malaise, rash, eosinophilia, damage to the liver, kidneys and heart, which is accompanied by an increase in intracellular enzymes from the group of aminotransferases: alanine mitrosferase (ALT) and aspartate aminotransferase (AST). The reaction usually occurs 3-6 weeks after the trigger drug is taken.
Sometimes the general condition of the patient does not correlate with the severity of the lesion, which can mislead the clinician.
Patients require long-term immunosuppression at relatively high doses. Some complications, such as certain forms of autoimmune diseases that are associated with the thyroid gland, require continued monitoring after the end of the acute episode.
Skin manifestation #3 — Fulminant purpura
One of the features of purpura fulminans is the presence of areas of dark purple skin that do not fade when pressed.
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Patients with this disorder are at high risk of developing disseminated intravascular coagulation and deep necrosis of the affected skin.
An interdisciplinary approach with a team of dermatologists and infectious disease specialists allows for a quick and accurate diagnosis.
Early antibiotics is essential because death is usually not due to fulminant purpura but due to the addition of infectious agents. Skin manifestation #4 — Acute generalized exanthematous pustulosis
Acute generalized exanthematous pustulosis — AGEP is associated with taking
narcotic drugs
. Usually within a day or two after taking the trigger drug, patients will pustule against the background of hyperemic skin. The pathology can look very similar to pustular psoriasis, so timely diagnosis is essential.
How allergic dermatosis manifests itself: features of the clinical picture
Systemic steroids or systemic immunosuppressants
are drugs of choice. Skin manifestation #5 — Pyoderma gangrenosum
Pyoderma gangrenosum — it is a
neutrophilic dermatosis
in which white blood cells infiltrate the skin and cause ulcers. Pyoderma gangrenosum is often confused with infection, even after a biopsy, but this mistake can be devastating.
aggravation of the course of pyoderma gangrenosum
. Pyoderma gangrenosum is actually an inflammatory process that can only be stopped by
immunosuppression
. Timely recognition of the signs of fatal skin pathologies can save lives.
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