Von zumbusch disease is?
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Correct Answer:
Pustular psoriasis
Description:
ANSWER: (A) Pustular psoriasisREF: Bolognia 2nd ed Ch; 9Von zumbusc disease is a type of pustular psoriasisPUSTULAR VARIANTS OF PSORIASIS:Generalized pustular psoriasis: In generalized pustular psoriasis (GPP), the infiltration of neutrophils dominates the histologic picture, while erythemaand the appearance of sterile pustules dominate the clinical picture. GPP is an unusual manifestation of psoriasis, and triggering factors include pregnancy, rapid tapering of corticosteroids (or other systemic therapies), hypocalcemia, infections and, in the case of localized disease, topical irritants. GPP during pregnancy is also referred to as impetigo herpetiformis. There are four distinct patterns of GPP.von Zumbusch pattern: This is a generalized eruption starting abruptly with erythema and postulation. The skin is painful during this phase and the patient has a fever and feels ill. After several days, the pustules usually resolve and extensive scaling is observedAnnular pattern. The eruption is characterized by annular lesions, consisting of erythema and scaling with pustulation at the advancing edge. The lesions enlarge by centrifugal expansion over a period of hours to days, whilst healing occurs centrally. General malaise, localized tenderness and fever may occur.Exanthematic type. This is an acute eruption of small pustules, abruptly appearing and disappearing in a few days. It usually follows an infection or may occur as a result of administration of specific medications, e.g. lithium. Systemic symptoms usually do not occur. There is overlap between this form of pustular psoriasis and pustular drug eruptions, also referred to as acute generalized exanthematous pustulosis (AGEP).'Localized' pattern. Sometimes pustules appear within or at the edge of existing psoriatic plaques. This can be seen during the unstable phase of chronic plaque psoriasis and following the application of irritants, e.g. tars.Localized pustular psoriasis:Pustulosis of the palms and soles: It is characterized by 'sterile' pustules of the palmoplantar surfaces admixed with yellow-brown macules ,scaly erythematous plaques may also be seen. The minority of patients have chronic plaque psoriasis elsewhere. In contrast to the natural history of GPP, the pustules remain localized to the palmoplantar surfaces and the course of this disease is chronic. Focal infections and stress have been reported as triggering factors and smoking may aggravate the condition. Pustulosis of the palms and soles is one of the entities most commonly associated with sterile inflammatory bone lesions, for which there are several names: chronic recurrent multifocal osteomyelitis, pustulotic arthro-osteitis, and SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis). Several neutrophilic dermatoses are associated with SAPHO.Acrodermatitis continua of Hallopeau: This is a rare manifestation of psoriasis. Clinically, pustules are seen on the distal portions of the fingers and sometimes the toes. Pustulation is often followed by scaling and crust formation. Pustules may also form in the nail bed (beneath the nail plate) and there may be shedding of nail plates. Transition into other forms of psoriasis can occur and acrodermatitis continua may be accompanied by annulus migrans of the tongue.
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