Advertisement
Mayo Clinic Proceedings Home
MCP Digital Health Home

Pustular Psoriasis

      A woman in her 60s with no past dermatologic history presented with a 4-month history of a pustular eruption. Before onset she had received steroid injections for cervical radiculopathy, but there were no other new medications or illnesses. Dermatologic exam showed more than 90% body surface area involvement with erythematous patches studded with pustules and diffuse skin sloughing (Figure 1) (Supplemental Figure 1, available online at http://www.mayoclinicproceedings.org). Skin biopsy specimens showed subcorneal neutrophilic pustules with hypogranulosis (Figure 2). Direct immunofluorescence and organism stains were negative. White blood cell count was elevated to 28/μL with neutrophilic predominance. Creatinine was 2.15 mg/dL (baseline 0.76). She was diagnosed with generalized pustular psoriasis and admitted for intravenous fluids and intensive full-body wet wrap dressings, which led to improvement. Ixekizumab was initiated for long-term treatment after failure of acitretin to control symptoms.
      Figure thumbnail gr1
      Figure 1Erythematous patches studded with pustules covering extremities.
      Figure thumbnail gr2
      Figure 2Subcorneal neutrophilic pustule with hypogranulosis (original magnification ×40; hematoxylin and eosin stain).
      Generalized pustular psoriasis is a severe and uncommon psoriasis variant with onset in middle age. Patients may or may not have a prior history of psoriasis vulgaris. Medications, particularly systemic steroids, and infections are reported triggers. Cutaneous symptoms include erythematous, painful skin studded with small, sterile pustules accompanied by fever, malaise, and elevated white blood cell count with neutrophilic predominance. Extracutaneous manifestations include acute renal failure, cholestasis, cholangitis, and arthritis; these typically warrant inpatient admission and management.
      • Hoegler K.M.
      • John A.M.
      • Handler M.Z.
      • Schwartz R.A.
      Generalized pustular psoriasis: a review and update on treatment.
      Differential diagnosis includes acute generalized exanthematous pustulosis, which can have a similar clinical and histological presentation but is differentiated by history of recent antibiotic use and resolution within an average of 2 weeks.
      • Feldmeyer L.
      • Heidemeyer K.
      • Yawalkar N.
      Acute generalized exanthematous pustulosis: pathogenesis, genetic background, clinical variants and therapy.
      The traditional first-line treatment is acitretin.
      • Hoegler K.M.
      • John A.M.
      • Handler M.Z.
      • Schwartz R.A.
      Generalized pustular psoriasis: a review and update on treatment.
      ,
      • Kearns D.G.
      • Chat V.S.
      • Zang P.D.
      • Han G.
      • Wu J.J.
      Review of treatments for generalized pustular psoriasis.
      Biologics, particularly infliximab and interleukin 17 inhibitors (ixekizumab and secukinumab), have more recently shown efficacy in treatment of recalcitrant cases.
      • Hoegler K.M.
      • John A.M.
      • Handler M.Z.
      • Schwartz R.A.
      Generalized pustular psoriasis: a review and update on treatment.
      ,
      • Wang W.M.
      • Jin H.Z.
      Biologics in the treatment of pustular psoriasis.
      Interleukin 17 inhibitors have the added convenience of self-administered injections and a favorable side effect profile. Lastly, avoidance of systemic steroids is crucial to avoid disease flares.

      Potential Competing Interests

      The authors report no potential competing interests.

      Supplemental Online Material

      References

        • Hoegler K.M.
        • John A.M.
        • Handler M.Z.
        • Schwartz R.A.
        Generalized pustular psoriasis: a review and update on treatment.
        J Eur Acad Dermatol Venereol. 2018; 32: 1645-1651
        • Feldmeyer L.
        • Heidemeyer K.
        • Yawalkar N.
        Acute generalized exanthematous pustulosis: pathogenesis, genetic background, clinical variants and therapy.
        Int J Mol Sci. 2016; 17: 1214
        • Kearns D.G.
        • Chat V.S.
        • Zang P.D.
        • Han G.
        • Wu J.J.
        Review of treatments for generalized pustular psoriasis.
        J Dermatolog Treat. 2021; 32: 492-494
        • Wang W.M.
        • Jin H.Z.
        Biologics in the treatment of pustular psoriasis.
        Expert Opin Drug Saf. 2020; 19: 969-980