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Generalized Pustular Psoriasis (generalized + pustular_psoriasis)

Distribution by Scientific Domains

Medical Sciences	100%

Selected Abstracts

A dramatic response to a single dose of infliximab as rescue therapy in acute generalized pustular psoriasis of von Zumbusch associated with a neutrophilic cholangitis

AUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 1 2010
Nisha Suyien Chandran
ABSTRACT Generalized pustular psoriasis of von Zumbusch is an unstable, inflammatory form of psoriasis, with the hallmark of neutrophil infiltration in cutaneous as well as extracutaneous lesions. It is often recalcitrant, making treatment difficult. Tumour necrosis factor-, antagonists including infliximab have been used with success in treating recalcitrant cases. We report a case of a 48-year-old Chinese female patient with a long-standing history of poorly controlled generalized pustular psoriasis which was resistant to multiple therapies. During a severe flare, a single dose of infliximab resulted in rapid clearing of cutaneous lesions, together with resolution of liver function abnormalities that are likely secondary to neutrophilic cholangitis. Subsequent maintenance therapy with acitretin allowed remission of pustular disease for 7 months. This demonstrates the efficacy of single-dose infliximab for both cutaneous lesions and systemic hepatic involvement in generalized pustular psoriasis. [source]

Generalized pustular psoriasis complicated by acute respiratory distress syndrome

BRITISH JOURNAL OF DERMATOLOGY, Issue 2 2006
M.R. Griffiths
No abstract is available for this article. [source]

Bullous and non-bullous ichthyosiform erythroderma associated with generalized pustular psoriasis of von Zumbusch type

BRITISH JOURNAL OF DERMATOLOGY, Issue 5 2001
S. Ingen-Housz-Oro
Bullous ichthyosiform erythroderma (BIE) and non-bullous ichthyosiform erythroderma (NBIE) are rare congenital ichthyoses. Generalized pustular psoriasis (GPP) of von Zumbusch type is a rare and severe form of psoriasis marked by desquamative and pustular erythroderma associated with fever and altered general conditions. We report two adults with an ichthyosis typical of BIE in the first case and NBIE in the second, without any previous history of psoriasis, who presented with a severe and relapsing GPP of von Zumbusch type. Using current knowledge of the genetic relationship between psoriasis and congenital ichthyoses, we discuss the possibility of a common physiopathological link between congenital ichthyoses and GPP, and examine the possible therapeutic problems resulting from this pathological association, especially in BIE. [source]

Psoriasis in childhood and adolescence: evaluation of demographic and clinical features

PEDIATRICS INTERNATIONAL, Issue 6 2006
MUAMMER SEYHAN
Abstract Background: The present study was aimed to define the gender ratio, familial occurrence, age of onset, precipitating factors, clinical types, nail and joint involvement of psoriasis in childhood and adolescence in Turkey. Methods: A total of 61 children with psoriasis under 18 years old were evaluated retrospectively, for age, gender, age of disease onset, family history, concomitant disease, the clinical type of psoriasis, clinical localization, nail and joint involvement and treatment modalities. Results: Of the patients, 23 (37.70%) were boys and 38 (62.30%) were girls. Mean age was 9.28 ± 4.02 years in girls and 11.18 ± 3.85 years in boys (9.96 ± 4.03 years in all children). Mean age at the onset of the disease was 6.81 ± 4.11 years in girls and 7.03 ± 4.28 years in boys (6.89 ± 4.14 years in all patients). In 14 (23%) cases, a positive family history was detected. The most frequent probable triggering factors were upper respiratory tract infections (14.8%) and positive throat culture for A group ß-hemolytic streptococcus (21.3%). Frequency of emotional stress and psychiatric morbidity were 54% and 9.8%, respectively. The most frequent localizations at onset were trunk (44.3%), extremities (54.0%), and scalp (36.0%). Three children (4.9%) had a history of dissemination from psoriatic diaper rash. In total, 51 (83.6%) patients presented with psoriasis vulgaris, eight (13.1%) with generalized pustular psoriasis, and the remaining two (3.3%) with erythrodermic psoriasis. Conclusion: The incidence of psoriasis among dermatological patients in childhood and adolescence was 3.8%. The disease tends to appear earlier in girls than boys. The authors suggested that stress and upper respiratory infections are the most important triggering factors in childhood and adolescence psoriasis. [source]

A dramatic response to a single dose of infliximab as rescue therapy in acute generalized pustular psoriasis of von Zumbusch associated with a neutrophilic cholangitis

Bullous and non-bullous ichthyosiform erythroderma associated with generalized pustular psoriasis of von Zumbusch type

Systemic immunosuppressant therapy in childhood

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 4 2002
David Atherton
The indications for systemic immunosuppressant therapy are much the same in children as in adults. Perhaps the most important difference is the need in a child to consider more carefully the patient's likely future therapy requirements. This need reflects a justifiable anxiety concerning the longer-term toxicity associated with some of these drugs. It is obvious that special attention should be paid to the dosage regimens that are appropriate in children. However, otherwise the principles of treatment are essentially the same as in adults. This talk will focus on the use of azathioprine in atopic eczema, methotrexate in psoriasis and linear morphoea, and intravenous methylprednisolone in severe muco-cutancous erythema multiforme and toxic epidermal necrolysis. The value of azathioprine as a treatment for severe childhood eczema was greatly increased by the elucidation of the metabolic pathways for this drug, and by the development of an assay for thiopurine methyl transferase to allow detection of those at greatest risk of myelosuppression. We now treat children with normal TPMT levels with 3 mg/kg per day with gratifying therapeutic response and limited requirement for monitoring of blood counts and liver function. More recently we have successfully treated TPMTHL heterozygotes with doses of around 1.5 mg/kg per day. We now consider azathioprine as superior to cyclosporin as a systemic therapy for atopic eczema. The value of methotrexate in adults with plaque psoriasis and generalized pustular psoriasis is well established. It is equally useful in children with these disorders, and the most appropriate dosage appears to be in the region of 0.3,0.4 mg/kg as a single weekly dose. Children generally tolerate oral therapy well. Methotrexate also appears helpful in arresting the progression of linear morphoea, both in the case of coup de sabre lesions and progressive hemi-facial atrophy, and in limb lesions that are interfering with joint mobility or are causing profound lipoatrophy. Intravenous methylprednisolone appears to be of value in several acute dermatoses in childhood, but is most commonly used at Great Ormond Street Hospital in the hope of arresting progression of severe muco-cutaneous erythema multiforme and toxic epidermal necrolysis. Various dose regimens are used in children, but in our unit we use a dose of 20,30 mg/kg per day, up to a maximum of 500 mg, for 3 successive days. Each dose is given over period of 2 h with frequent monitoring of vital signs, particularly blood pressure. [source]