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Severe erytrodermic psoriasis in child twins: from clinical-pathological diagnosis to treatment of choice through genetic analyses: two case reports.

Campione E, Diluvio L, Terrinoni A, Orlandi A, Latino MP, Torti C, Pietroleonardo L, Botti E, Chimenti S, Bianchi L - BMC Res Notes (2014)

Bottom Line: Major histocompatibility complex, class I, Cw*06 was detected in both twins; we found no transglutaminase-1, no corneodesmosin, nor any Interleukin-36 receptor antagonist gene mutations.After three months of treatment, consistent clearing and significant improvement of their social and psychological behaviour were achieved.After over one year of continuous therapy with cyclosporine, the twins have still maintained the result obtained.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Tor Vergata University of Rome, Rome, Italy. campioneelena@hotmail.com.

ABSTRACT

Background: Pediatric erythroderma is a severe cutaneous disorder, which may pose diagnostic and therapeutic challenges. Psoriasis, ichthyoses, atopy, seborrhoeic dermatitis, pityriasis rubra pilaris, infections, metabolic diseases, drugs reaction, may cause erythroderma. The therapy should be tailored on each aetiology, if possible. The biochemical and metabolic imbalance should be corrected, and particular attention should be paid to the psychosocial behavior often related to this disfiguring disease.

Case presentation: Two 3 year-old Caucasian twins have been suffering from an unmanageable erythroderma since the age of 8 months. The diagnosis of psoriasis, already remarkably expressed in the father's family in three cases of fraternal twins, could be enforced for several points. Major histocompatibility complex, class I, Cw*06 was detected in both twins; we found no transglutaminase-1, no corneodesmosin, nor any Interleukin-36 receptor antagonist gene mutations. We performed a cutaneous histology, positive immunostaining for Lympho-epithelial Kazal-type-related inhibitor, dermoscopy and reflectance confocal microscopy. The twins had previously received systemic steroids, short cycles of low-dosage ciclosporine, followed by etanercept at the dosage of 0,8 mg/kg, without reliable results. Cyclosporine was then reconsidered at a dosage of 5 mg/kg/day with close blood monitoring. After three months of treatment, consistent clearing and significant improvement of their social and psychological behaviour were achieved. After over one year of continuous therapy with cyclosporine, the twins have still maintained the result obtained.

Conclusion: Pediatric erythroderma may pose a great challenge as a potentially life-threatening condition causing extreme distress in children, parents and pediatricians. In young patients it is mandatory to establish correct clinical and instrumental procedures, possibly supplemented by genetic analyses such as those we required, in order to determine an effective and safe therapy in terms of cost-benefit and put patients and family in the best condition to perform common daily activities.

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Clinical images during therapy. Residual redness of the face and slightly scaling, not infiltrated mild erythematous areas, during treatment.
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Fig8: Clinical images during therapy. Residual redness of the face and slightly scaling, not infiltrated mild erythematous areas, during treatment.

Mentions: Taken together, the clinical-pathological features, immunostaining, dermoscopy and RCM findings, genetic HLA-Cw*06 testing, with the high prevalence of psoriasis in the family, were all consistent with the diagnosis of pediatric psoriatic erythroderma. The twins were previously treated with emollients, topical and systemic steroids and short cycles of low-dosage cyclosporine, without any consistent result. Afterwards, etanercept, a tumor necrosis factor-alpha receptor inhibitor approved for psoriatic patients aged 8 years and over, was proposed. After informed consent as off-label treatment because of their young age, etanercept was started at a dosage of 0.8 mg/kg subcutaneously, once a week. After a short intermission because of an upper mild respiratory tract viral infection in both children, etanercept gave ineffective results and was then stopped after a total of 10 injections. Cyclosporine was reconsidered but prescribed at a dosage of 5 mg/kg/day as continuous treatment with close blood monitoring as scheduled. Acitretin, at a dosage of 0.2 mg/kg per day, was combined for one month only, because of the risk of bone anomalies. After one year of continuous treatment, consistent cutaneous clearing and reduction of pruritus with significant improvement of their psychological behaviour were achieved. Weak redness of the face, more evident in the sister, and slightly scaling, not infiltrated mild erythematous areas, without plaque appearance, are still persistent (Figures 7, 8).Figure 7


Severe erytrodermic psoriasis in child twins: from clinical-pathological diagnosis to treatment of choice through genetic analyses: two case reports.

Campione E, Diluvio L, Terrinoni A, Orlandi A, Latino MP, Torti C, Pietroleonardo L, Botti E, Chimenti S, Bianchi L - BMC Res Notes (2014)

Clinical images during therapy. Residual redness of the face and slightly scaling, not infiltrated mild erythematous areas, during treatment.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4300562&req=5

Fig8: Clinical images during therapy. Residual redness of the face and slightly scaling, not infiltrated mild erythematous areas, during treatment.
Mentions: Taken together, the clinical-pathological features, immunostaining, dermoscopy and RCM findings, genetic HLA-Cw*06 testing, with the high prevalence of psoriasis in the family, were all consistent with the diagnosis of pediatric psoriatic erythroderma. The twins were previously treated with emollients, topical and systemic steroids and short cycles of low-dosage cyclosporine, without any consistent result. Afterwards, etanercept, a tumor necrosis factor-alpha receptor inhibitor approved for psoriatic patients aged 8 years and over, was proposed. After informed consent as off-label treatment because of their young age, etanercept was started at a dosage of 0.8 mg/kg subcutaneously, once a week. After a short intermission because of an upper mild respiratory tract viral infection in both children, etanercept gave ineffective results and was then stopped after a total of 10 injections. Cyclosporine was reconsidered but prescribed at a dosage of 5 mg/kg/day as continuous treatment with close blood monitoring as scheduled. Acitretin, at a dosage of 0.2 mg/kg per day, was combined for one month only, because of the risk of bone anomalies. After one year of continuous treatment, consistent cutaneous clearing and reduction of pruritus with significant improvement of their psychological behaviour were achieved. Weak redness of the face, more evident in the sister, and slightly scaling, not infiltrated mild erythematous areas, without plaque appearance, are still persistent (Figures 7, 8).Figure 7

Bottom Line: Major histocompatibility complex, class I, Cw*06 was detected in both twins; we found no transglutaminase-1, no corneodesmosin, nor any Interleukin-36 receptor antagonist gene mutations.After three months of treatment, consistent clearing and significant improvement of their social and psychological behaviour were achieved.After over one year of continuous therapy with cyclosporine, the twins have still maintained the result obtained.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Tor Vergata University of Rome, Rome, Italy. campioneelena@hotmail.com.

ABSTRACT

Background: Pediatric erythroderma is a severe cutaneous disorder, which may pose diagnostic and therapeutic challenges. Psoriasis, ichthyoses, atopy, seborrhoeic dermatitis, pityriasis rubra pilaris, infections, metabolic diseases, drugs reaction, may cause erythroderma. The therapy should be tailored on each aetiology, if possible. The biochemical and metabolic imbalance should be corrected, and particular attention should be paid to the psychosocial behavior often related to this disfiguring disease.

Case presentation: Two 3 year-old Caucasian twins have been suffering from an unmanageable erythroderma since the age of 8 months. The diagnosis of psoriasis, already remarkably expressed in the father's family in three cases of fraternal twins, could be enforced for several points. Major histocompatibility complex, class I, Cw*06 was detected in both twins; we found no transglutaminase-1, no corneodesmosin, nor any Interleukin-36 receptor antagonist gene mutations. We performed a cutaneous histology, positive immunostaining for Lympho-epithelial Kazal-type-related inhibitor, dermoscopy and reflectance confocal microscopy. The twins had previously received systemic steroids, short cycles of low-dosage ciclosporine, followed by etanercept at the dosage of 0,8 mg/kg, without reliable results. Cyclosporine was then reconsidered at a dosage of 5 mg/kg/day with close blood monitoring. After three months of treatment, consistent clearing and significant improvement of their social and psychological behaviour were achieved. After over one year of continuous therapy with cyclosporine, the twins have still maintained the result obtained.

Conclusion: Pediatric erythroderma may pose a great challenge as a potentially life-threatening condition causing extreme distress in children, parents and pediatricians. In young patients it is mandatory to establish correct clinical and instrumental procedures, possibly supplemented by genetic analyses such as those we required, in order to determine an effective and safe therapy in terms of cost-benefit and put patients and family in the best condition to perform common daily activities.

Show MeSH
Related in: MedlinePlus