A fifty years old caucasian female patient with positive family history for hypertension and negative family history for malignancy, having hypertension controlled by lisinopril, amlodipine and bisoprolol fumarate, Diabetes mellitus type II (DM II) controlled by short acting regular insulin, and end stage renal disease (ESRD) on regular hemodialysis. She was admitted to the intensive care unit (ICU) with fever of unknown origin (FUO) of fourteen days duration associated with agitation, irritability, tachycardia (120 beats/minute), generalized weakness, anorexia, nausea, vomiting, diarrhea, scratch marks and maculopapular rash ( and ). Sepsis workup was done followed by infusion of empirical intravenous broad spectrum antibiotics with the dose adjusted according to renal function and systemic steroids were started with methyl prednisolone 40 milligrams intravenous infusion once daily.
Illustration shows erythroderma and scaly skin of the upper extremity.
Illustration shows erythroderma and scaly skin of the trunk.
On Day two, the patient developed severe upper epigastric pain. Upper gastrointestinal endoscopic biopsy confirmed her diagnosis with severe CMV esophagitis and duodenitis. Treatment was started with intravenous Ganciclovir at a dose of 1.25 milligrams/kilogram administered three times/week following each hemodialysis session.
On day three the maculopapular rash progressed to erythroderma, followed by development of bullous lesions all over the body associated with skin peeling, bleeding, positive Nikolsky’s sign and mucous membrane involvement (). Skin biopsy was done and the pathology showed extensive epidermal necrosis, focal subepidermal necrotic blisters and extensive vacuolar degeneration of dermoepidermal junction with separation of the epidermis from the dermis. The dermis showed melanin incontinence and moderate perivascular lymphocytic infiltrate in the absence of eosinophils, neutrophils and viral inclusions (). TEN was confirmed. All the immunoflourescence markers that were done on the skin biopsy showed negative staining with nonspecific granular deposition in the necrotic epidermis. The immunoflourescence markers included Immunoglobulin G (IgG), Immunoglobulin A (IgA), Immunoglobulin M (IgM) and Complement factor 3. On day 15, she developed pneumonia which was complicated by respiratory failure. Intubation and mechanical ventilation were initiated.
Illustration shows bullous lesions of the lower extremities associated with skin peeling, bleeding and positive Nikolsky’s sign.
Histopathological examination of the skin biopsy. The black arrows illustrate the pan epidermal necrosis and the red arrows demonstrate the separation of the epidermis from the dermis
On day 48 the patient, whose SCORTEN (severity-of-illness score) was five and expected mortality rate was 90%, passed away due to overwhelming sepsis, shock and multiorgan failure.