Case report of generalized pustular psoriasis of von Zumbusch associated with hypothyroidism (2014)

Type of publication:
Conference abstract

*Mirhadi S., Moazenzade M.

British Journal of Dermatology, December 2014, vol./is. 171/6(e147-e148), 0007-0963 (December 2014)

Generalized pustular psoriasis (GPP), a rare subtype of psoriasis, is characterized by abrupt extensive pustular eruptions with potentially fatal outcome. This report describes a case of GPP associated with hypothyroidism. A 28-year-old Iranian woman presented 5 years ago with pustular lesions with underlying erythematous background, which spread over > 50% of her body and was particularly severe in her calf and lower abdomen. The size of lesions was 4-20 cm. She also had systemic symptoms including fever, rigors, arthralgia, loss of appetite and severe depression. In addition, she had peripheral oedema and lymphadenopathy in the submental and anterior cervical chain. She had no significant past medical history. Her family history included mild localized psoriasis in her mother. She was admitted on five occasions, for 2 weeks, at intervals of 6-12 months with acute relapses. The attacks occurred mainly during the winter and summer. No drugs have been implicated. Skin biopsy confirmed pustular psoriasis. Unbroken pustules were sterile. The main biochemical abnormalities were elevated erythrocyte sedimentation rate, leucocytosis, iron-deficiency anaemia, hyperlipidaemia and hypoproteinaemia in the acute phase. During her third attack, it was noticed that she had hypothyroidism. She was treated with low-dose systemic corticosteroid for her acute attacks. Both the skin lesions and peripheral oedema started to respond on day 5 of steroids. Weaning of steroids was commenced in week 3. Among systemic drugs, she tolerated only a retinoid (Neotigason) and she had longer remissions with retinoids. Following the correction of her anaemia, hypothyroidism and peripheral circulation using tensile bandage, and additional family support, her condition is now improving. She occasionally gets small localized lesions that respond well to combined topical steroid with mupirocin. Following resolution of her pustules, topical Daivonex (calcipotriol) and steroid are used. The plan is to reduce the dose of Neotigason gradually. Multiple trigger factors for GPP have been described, including low thyroid activity. GPP is associated with autoimmune conditions such as hypothyroidism. Its prognosis is better in younger age and when the pustular psoriasis is preceded by ordinary psoriasis. In conclusion, we report a typical case of GPP with systemic symptoms, raised inflammatory markers, leucocytosis, hypoalbuminaemia, anaemia, hyperlipidaemia and hypothyroidism. Pharyngitis and emotional stress were identified as triggers. Her management was quite complicated and the disease finally responded to steroids, retinoid and calcipotriol. This patient did not develop any life-threatening complication.

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