Pustular Psoriasis: Lakes of Sterile Pustules…

Generalized Pustular Psoriasis is also referred to as von Zumbusch psoriasis.  Traditionally the onset is sudden with formation of “lakes of pus” along the nails, palms and at the edges of psoriatic plaques.  Erythema (redness) occurs in the flexures before the generalized eruption appears, followed by generalized erythema and more pustules.  The pustules are sterile–no bacterial infection is associated with Pustular Psoriasis within the pustules.  Pediatric cases have been reported in infants and children such as the case shown here.  Recent research suggests that Pustular Psoriasis is distinct from psoriasis with a different pattern of immune dysfuntion.   Predisposing factors include infection, withdrawal from systemic steroids and certain medications such as lithium, aspirin, indomethacin, iodides, coal tar, terbinafine, minocycline, hydroxychloroquine, acetazolamide and some beta blockers.

Pustular Psoriasis has been associated with abnormalities in the cytokine system (messenger proteins), specifically interleukin-36-receptor-antagonist signaling, due to gene mutations in IL36RN.  Other skin conditions have been associated with IL36RN mutations, such as AGEP (acute generalized erythematous pustulosis), palmoplantar psoriasis and acrodermatitis continua of Hallopea.  About 10% of patients have a prior history of psoriasis.  In these patients CARD14 gene gain-of-function defects have been reported.

Pruritus (itching) and intense burning are common symptoms.  The lips may be red and scaly and superficial ulcerations of the tongue and mouth may occur.  Geographic or fissured tongue, as shown above, often occurs.  These patients are frequently very ill with fever, erythroderma, hypocalsemia, and chachexia (wasting and malnutrition).  Some develop acute respiratory distress syndrome (ARDS) which requires intensive care management.  Other complications include pneumonia, congestive heart failure, and hepatitis.

TREATMENT:  The treatment of choice is acitretin and isotretinoin (accutane).  Cyclosporine, methotrexate and a variety of biologic agents (infliximab, etanercept, ustekinumab, adalimumab and anakinra) have been shown to be effective.  To read more about Pustular Psoriasis click HERE and HERE.  To read more about Psoriasis in general click HERE.