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● Acute generalized exanthematous pustulosis: Severe cases of acute generalized exanthematous pustulosis (AGEP) may be difficult to differentiate from SJS/TEN. AGEP typically develops within a few days of exposure to the offending drug, most often a beta-lactam antibiotic, and resolves without treatment in 1 to 2 weeks after drug discontinuation. The histologic hallmark of AGEP is a spongiform, subcorneal, and/or intraepidermal pustule. ● Generalized bullous fixed drug eruption: Generalized bullous fixed drug eruption is an extremely rare form of fixed drug eruption characterized by widespread red or brown macules or plaques with overlying, large, flaccid bullae. In contrast with SJS/TEN, mucosal involvement is usually absent. Resolution generally occurs in 1 to 2 weeks after drug discontinuation. ● Phototoxic eruptions: Severe phototoxic eruptions may be confused with SJS/TEN. Important clues to the correct diagnosis include recent sun exposure, known phototoxic properties of certain medications, and the location of the lesions on sun-exposed areas. ● Staphylococcal scalded skin syndrome: Staphylococcal scalded skin syndrome (SSSS) is caused by epidermolytic toxins produced by certain strains of staphylococci and is usually seen in neonates and young children. SSSS presents with generalized erythema rapidly followed by flaccid Case study #3 Jared, a 21-year-old college student, presented to the emergency department with a prodromal phase of painful and pruritic rash involving his face and trunk. The onset of the rash was noted a week prior as small erythematous papules on his chest, and subsequently spread to his arms, legs, and facial region. Additionally, he complained of systemic symptoms, including feverishness, myalgia, dysphagia, and dysphonia. Relevant medical history was negative for any allergies, recent infections, or medication changes. During the examination, Jared appeared distressed and uncomfortable, and was observed to have a temperature of 101°F (38.3°C). He displayed bilateral conjunctivitis and a multitude of erythematous macules and papules that were present on his face, chest, and extremities. Some of these papules exhibited central necrosis and hemorrhagic crusting. Additionally, there were areas of epidermal detachment noted on his oral and ocular mucosa. The rash was most prominent on his trunk, back, and buttocks, with some sporadic involvement on his limbs. Based Syphilis A thorough sexual history is essential when evaluating a patient with a rash of unknown etiology. Genital or rectal ulcerations may be caused by various infectious agents, including syphilis, herpes simplex, lymphogranuloma venereum, chancroid, and donovanosis. These infections may not be associated with fever. Syphilis is an infection caused by the bacterium Treponema pallidum . Most new cases of syphilis are sexually acquired. Syphilis can have a variety of cutaneous manifestations. The primary stage is characterized by the chancre, a painless, often solitary, indurated genital ulceration with elevated, well- defined borders. The skin lesions of secondary syphilis include a generalized papular or maculopapular rash (rarely pustular) that also affects the palms of the hands and soles of the feet. Other lesions seen during this stage include condylomata lata: Flat, moist condylomata-like lesions that are gray, infectious, and located around the genitals, mouth, anus, and other moist areas

blisters and desquamation development. The mucous membranes are not involved. Histology reveals sloughing of only the upper layers of the epidermis, in contrast with the subepidermal split with full-thickness epidermal necrosis observed in SJS/TEN. ● Paraneoplastic pemphigus: Paraneoplastic pemphigus is a rare disorder that can represent the initial presentation of a malignancy or occur in a patient with a known neoplastic process, such as non-Hodgkin lymphoma in adults or Castleman’s disease in children. Patients may develop severe mucocutaneous disease with ocular and oral blisters and skin lesions. ● Linear IgA bullous dermatosis: Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease that may mimic TEN. Histology reveals a subepidermal blister with an underlying, neutrophil-predominant, dermal infiltrate. Direct immunofluorescence shows linear deposits of IgA along the basement membrane. ● Chikungunya fever: An atypical, SJS/TEN-like form of chikungunya fever characterized by fever and generalized, vesiculobullous eruption and superficial erosions has been reported in infants and young children. However, in contrast with SJS/TEN, mucosal involvement is generally absent, and the resolution of the skin manifestations occurs in most cases in 4 to 10 days. on Jared’s clinical presentation and examination findings, the medical team made a diagnosis of Stevens–Johnson syndrome (SJS). The severe form of the disease was indicated by the presence of mucosal involvement and epidermal detachment. Further laboratory testing showed elevated liver enzymes and leukocytosis, which are common in patients with SJS. Question What would be appropriate management of this patient? Discussion This patient should receive close monitoring and supportive care. Intravenous fluids, broad-spectrum antibiotics, and intravenous high-dose corticosteroids should be administered to decrease inflammation and stop infection. Supportive care should be performed for the patient’s eyes, to include topical lubricants and antibiotics to prevent ocular complications. Pain management is also a priority for this patient’s care. Secondary Syphilis Within weeks to a few months after the chancre develops, approximately 25% of individuals with untreated infection develop a systemic illness that represents secondary syphilis (Hicks & Clement, 2022). Patients with secondary syphilis may not have a history of a preceding chancre, since the primary infection may have been asymptomatic and/or gone unnoticed. Secondary syphilis can produce a wide variety of signs and symptoms, including dermatological. The rash can take almost any form, although vesicular lesions are uncommon. The rash is classically a diffuse, symmetric macular or papular eruption involving the entire trunk and extremities, including the palms of the hands and soles of the feet. Although the involvement of the palms and soles is an important clue to diagnosing secondary syphilis, localized lesions can also occur. Individual lesions are discrete copper, red, or reddish-brown and measure 0.5 to 2 cm in diameter. Although lesions are often scaly, they may be smooth (Hicks & Clement, 2022). In addition, nodular lesions also may be seen. On occasion, the rash may be pruritic.

(Hicks & Clement, 2022). Primary Syphilis (Chancre)

Following the acquisition of T. pallidum , the initial clinical manifestation of infection is a localized skin lesion termed a chancre. The median incubation period before the chancre appears is 21 days (range 3 to 90 days).

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Book Code: AUS3024

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