Generalized Pustular Psoriasis.

Abstract

SUMMARY.— A series of 104 cases of generalized pustular psoriasis has been studied. The syndrome occurs predominantly in the second half of life, affecting both sexes. Two quite distinct sub‐groups were discernible. In the first the pre‐pustular phase of psoriasis began early in life, was typical through‐out and was prolonged. At least one third of these cases were apparently precipitated by the withdrawal of systemic corticosteroid therapy. Others were provoked by pregnancy or infection. It is likely that this type is usually extraneously provoked. In the second, the psoriasis was of late onset and atypical, acral or flexural patterns predominating in the pre‐pustular phase. In these progress to generalized pustular disease was rapid and apparently spontaneous. Four clinical patterns of generalized pustular phase were apparent and have been named the Zumbusch, annular, localized and exanthematic types. The Zumbusch type was characterized by widespread fiery erythema, sheeted pustulation and scarlatiniform peeling, accompanied by malaise, fever and often leucocytosis. The annular type was a more low‐grade sub‐acute affection characterized by gyrate and annular pustular lesions and little systemic disturbance. The exanthematic type arose de novo, usually as a single short‐lived episode following infection or drug exposure. In the localized type restricted areas of pustular psoriasis developed in and around ordinary psoriatic plaques. Two thirds were erythrodermic at some stage and one third had polyarthritis. Oral lesions occurred in 5 and hypocalcaemia in 5 during pustular phases. The different clinical patterns of pustular psoriasis have been interpreted in terms of the balance between vascular hypertrophy, epidermopoiesis and leucocytic immigration into the epidermis.

Acrodermatitis Continua of Hallopeau Is a Clinical Phenotype of DITRA: Evidence that It Is a Variant of Pustular Psoriasis

“…Many have long considered ACH to be a localized variant of pustular psoriasis based on several reports of ACH progressing to GPP [4,5,6]. Our findings of homozygous c.338C>T (p.Ser113Leu) mutation in both siblings provide more evidence that ACH and GPP lie on a spectrum of a solitary condition.…”

“…It has long been debated whether ACH is a distinct entity or a variant of generalized pustular psoriasis (GPP). It was shown that some cases of ACH progressed later on to GPP [4,5,6]. Recently, mutations in the IL36RN gene, which encodes interleukin (IL)-36 receptor antagonist (IL-36Ra), were shown to be associated with familial GPP, a condition termed DITRA (deficiency of IL-36Ra).…”

Generalized Pustular Psoriasis of Pregnancy (Impetigo herpetiformis)

“…Baker and Ryan [1], found only 15 patients without any previous manifestation of psoriasis in their large study on 104 patients with generalized pustular psoriasis. Other accompanying symptoms described in impetigo herpetiformis are hormonal dysregulation with possible placental insufficiency and hypercalcemia as described in hyperparathyroidism (mostly after injury of the parathyroid glands during strumectomy) [5, 6, 7].…”

“…Besides other causes of a disseminated spread of pustules and blisters, differential diagnosis includes also impetigo herpetiformis, of which nearly 100 cases have been reported until now. This disease has been observed in pregnancy with and without hormonal dysregulations [1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14]. In the more recent literature, this disease is considered to be a generalized manifestation of pustular psoriasis [5, 6, 14].…”

Ciprofloxacin-Induced Acute Generalized Exanthematous Pustulosis Mimicking Bullous Drug Eruption Confirmed by a Positive Patch Test

“…In 1968, Baker and Ryan [8 ]assumed for the first time that this disease represents a distinct entity. Further characterization has been outlined by Beylot et al [9] and Roujeau et al [10] who additionally separated AGEP from pustular psoriasis and underlined drugs as its main etiology.…”