Synonym: lues maligna, syphilis maligna praecox
Clinical features:
(1) The patient develops generalized and pleomorphic skin lesions that may occur in successive waves, with papules, pustules, ulcers, plaques, nodules and crusted lesions. The lesions may be particularly heavy over the face and scalp. Skin lesions may be pruritic.
(2) The patient develops a severe systemic reaction that may include fever, hepatitis, hepatosplenomegaly and/or lymphadenopathy.
(3) The patient may have a history of a primary chancre or phagedenic ulcer.
(4) The patient often responds rather quickly to penicillin therapy. A significant percent of patients develop a Jarisch-Herxheimer reaction.
Laboratory features:
(1) Serologic tests for syphilis may be negative or positive, sometimes with extremely high titers (raising the possibility of prozone effect).
(2) Spirochetes may be absent or hard to demonstrate.
(3) A skin biopsy may show:
(3a) a perivasculitis which may be rich in lymphocytes and/or plasma cells
(3b) thrombosis in small arteries
(3c) pseudolymphoma
(3d) a granulomatous inflammatory response
