Stevens-Johnson Syndrome (SJS) / Toxic Epidermal Necrolysis (TEN) |
- Prodrome of fever, malaise, and sore throat followed by painful, rapidly spreading red or purpuric macules.
- Blistering and extensive skin detachment, often starting on the face and trunk.
- Mucous membrane involvement with painful erosions in the mouth, eyes, and genitals.
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- Clinical diagnosis based on skin findings and patient history (often related to recent drug exposure).
- Skin biopsy shows full-thickness epidermal necrosis.
- Laboratory tests to monitor organ function and electrolyte levels.
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- Immediate discontinuation of the offending drug.
- Supportive care in a burn unit or ICU, including fluid and electrolyte management.
- Systemic corticosteroids or IV immunoglobulin (IVIG) may be considered in severe cases.
- Ophthalmologic consultation for eye involvement and long-term monitoring for sequelae.
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Necrotizing Fasciitis |
- Rapidly spreading erythema, severe pain, and swelling often out of proportion to physical findings.
- Fever, crepitus, and skin necrosis with bullae formation.
- Progresses to systemic toxicity, including hypotension and multiorgan failure.
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- Clinical diagnosis, often confirmed with surgical exploration showing necrotic fascia.
- CT or MRI may show gas in the soft tissues.
- Blood cultures, wound cultures, and laboratory tests including CBC, CRP, and creatine kinase (CK).
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- Immediate broad-spectrum IV antibiotics (e.g., piperacillin-tazobactam, clindamycin, vancomycin).
- Urgent surgical debridement of necrotic tissue.
- Intensive care support for septic shock and multiorgan failure.
- Hyperbaric oxygen therapy may be considered as an adjunctive treatment.
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Staphylococcal Scalded Skin Syndrome (SSSS) |
- Fever, irritability, and generalized erythema with tenderness.
- Large, flaccid bullae that easily rupture, leaving behind denuded skin.
- Commonly affects infants and young children.
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- Clinical diagnosis based on characteristic skin findings.
- Positive Nikolsky sign (skin sloughing with gentle pressure).
- Blood cultures may identify Staphylococcus aureus; skin biopsy shows a split in the epidermis at the granular layer.
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- Immediate IV antibiotics effective against Staphylococcus aureus (e.g., oxacillin, nafcillin, or vancomycin).
- Supportive care with fluid and electrolyte management, similar to burn care.
- Wound care to prevent secondary infections.
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Angioedema |
- Sudden onset of deep swelling in the skin, mucous membranes, and subcutaneous tissues, often affecting the face, lips, and airway.
- May be associated with urticaria and can lead to airway obstruction.
- Common triggers include medications (e.g., ACE inhibitors), foods, insect stings, and hereditary angioedema.
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- Clinical diagnosis based on history and physical examination.
- Measurement of C1 esterase inhibitor levels if hereditary angioedema is suspected.
- Monitoring of airway status and oxygen saturation.
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- Airway management is the first priority; intubation may be necessary.
- Administration of epinephrine, antihistamines, and corticosteroids for allergic angioedema.
- For hereditary angioedema, C1 esterase inhibitor concentrate or fresh frozen plasma may be administered.
- Observation and supportive care in a monitored setting.
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Disseminated Intravascular Coagulation (DIC) with Purpura Fulminans |
- Sudden onset of widespread purpura, petechiae, and ecchymoses, often with necrosis and gangrene.
- Associated with severe sepsis, trauma, malignancy, or obstetric complications.
- Signs of systemic bleeding, thrombosis, and organ failure.
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- Clinical diagnosis supported by laboratory findings of DIC (prolonged PT, aPTT, low platelets, elevated D-dimer).
- Blood cultures and other investigations to identify the underlying cause.
- Skin biopsy may show thrombotic occlusion of small vessels.
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- Treat the underlying cause (e.g., antibiotics for sepsis, delivery for obstetric causes).
- Supportive care with IV fluids, blood products, and management of organ dysfunction.
- Heparin or antithrombin III may be considered in some cases of DIC.
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Erythroderma |
- Widespread erythema and scaling affecting more than 90% of the body surface area.
- May be associated with pruritus, fever, and malaise.
- Common causes include psoriasis, atopic dermatitis, drug reactions, and cutaneous T-cell lymphoma.
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- Clinical diagnosis based on extensive skin involvement.
- Skin biopsy may help determine the underlying cause.
- Blood tests to assess for infection, organ dysfunction, and electrolyte imbalances.
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- Identify and treat the underlying cause (e.g., discontinue offending drug, treat infection).
- Supportive care with fluid and electrolyte management, temperature regulation, and nutritional support.
- Topical corticosteroids and emollients for skin symptoms.
- Hospitalization is often required for severe cases.
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Acute Generalized Exanthematous Pustulosis (AGEP) |
- Sudden eruption of numerous small, non-follicular pustules on a background of erythema.
- Often associated with fever, leukocytosis, and malaise.
- Commonly triggered by drugs (e.g., beta-lactam antibiotics, calcium channel blockers).
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- Clinical diagnosis based on characteristic skin findings and recent drug exposure.
- Skin biopsy shows subcorneal pustules and spongiform pustules in the epidermis.
- Blood tests may show leukocytosis and elevated inflammatory markers.
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- Immediate discontinuation of the offending drug.
- Supportive care, including antipyretics and fluid management.
- Topical or systemic corticosteroids may be used in severe cases.
- Most cases resolve within 1-2 weeks after stopping the causative drug.
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Herpes Simplex Virus (HSV) Encephalitis |
- Fever, headache, confusion, seizures, and focal neurological deficits.
- May be associated with vesicular lesions on the skin or mucous membranes.
- Rapid progression to coma and death if untreated.
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- CSF analysis showing elevated protein, lymphocytic pleocytosis, and normal glucose levels.
- PCR testing of CSF for HSV DNA is the gold standard for diagnosis.
- MRI of the brain may show temporal lobe involvement.
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- Immediate initiation of IV acyclovir.
- Supportive care, including seizure management and monitoring for increased intracranial pressure.
- Close monitoring in an ICU setting due to the potential for rapid deterioration.
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