Elderly-Onset Generalized Pustular Psoriasis without a Previous History of Psoriasis Vulgaris

Abstract

Generalized pustular psoriasis (GPP) is characterized by sudden fever and extensive erythema with pustules and occurs in patients with or without preceding psoriasis vulgaris. We report an 83-year-old man showing irregularly shaped erythema with pustules on the trunk and extremities. He initially had no fever and came to our clinic a few days after the onset of the skin lesions because of high fever and general malaise. We found an extension and new development of erythema and pustules on the whole body. The patient also manifested night delirium. Histological examination revealed neutrophil infiltration into the upper epidermis, which formed a spongiform pustule of Kogoj. Pustular fluid cultures were negative for bacteria. We diagnosed GPP without preceding psoriasis vulgaris. Mutation analysis revealed no significant mutations in IL36RN and CARD14. Previous reports indicated that onset of GPP at the age of 83 years is definitely rare. In older individuals, general disease characteristics include an atypical clinical course, an especially slow appearance and cure, and mental disorder. Our case also revealed such characteristics. Thus, it is necessary to be aware of the clinical course and mental problems in elderly patients with GPP.

Generalized pustular psoriasis in a 92‐year‐old man with a homozygous nonsense mutation in IL36RN

“…The onset of GPP at the age of 92 years in this case is very late, according to previous reports. A case of elderly‐onset GPP was reported in a 51‐year‐old patient, with a mutation in IL36RN ; another case was reported with onset at age 83 years, with no mutation found in IL36RN or caspase recruitment domain family member 14 ( CARD14 ) . Our case did not develop GPP until the age of 92 years, even with a homozygous pathogenic mutation of IL36RN .…”

“…A case of elderlyonset GPP was reported in a 51-year-old patient, with a mutation in IL36RN; 3 another case was reported with onset at age 83 years, with no mutation found in IL36RN or caspase recruitment domain family member 14 (CARD14). 4 Our case did not develop GPP until the age of 92 years, even with a homozygous pathogenic mutation of IL36RN. Thus, even patients with a pathogenic mutation of IL36RN may not develop GPP until old age.…”

Lack of association between CARD10/CARMA3 tag SNPs and psoriasis vulgaris in the southern Chinese population

“…Abundant evidence shows that single nucleotide polymorphisms (SNPs) of the caspase recruitment domain (CARD) family member 14 (CARD14) gene are implicated in this condition (Jordan et al, 2012a,b;Tsoi et al, 2012;Qin et al, 2014;Aizu et al, 2015). In a previous study, we also determined that the rs4722404 SNP of CARD11 can increase risk of early-onset psoriasis vulgaris (PsV; Shi et al, 2016).…”

Generalized pustular psoriasis: current management status and unmet medical needs in Japan

“…While there are several biologic agents available for the treatment of GPP in Japan, more robust data is needed to optimize the use of these agents in all patient types. Table 4 summarizes available evidence for the use of systemic treatments for GPP in different patient groups [1,[66][67][68][69][70][71][72][73][74][75][76]. To date, the approval of antagonists of IL-17 and IL-23 has been based on clinical trials that included mostly cases of moderate disease, so there is not adequate evidence to fully understand how these agents perform against GPP flares.…”

“…GPP can develop late in life. In published reports, the age of disease onset in Japanese adults ranges up to 83 years [73], and in the Malaysian cohort study, one patient had an age of onset of 81 years [23]. Careful evaluation of the possible impact of comorbidities is important when treating older patients, and a possible increased risk of adverse reactions should be considered.…”