A severe case of erythrodermic psoriasis associated with advanced nail and joint manifestations: a case report
© Teran et al; licensee BioMed Central Ltd. 2010
Received: 21 October 2009
Accepted: 15 June 2010
Published: 15 June 2010
Erythrodermic psoriasis is a rare generalized clinical presentation of psoriasis in children and adults. Its systemic involvement and a diverse range of clinical findings in the joint and nails are commonly described. A high index of suspicion and an exhaustive differential diagnosis involving other causes of erythroderma should be initially considered.
We present the case of a 9-year-old native Hispanic girl with severe erythrodermic psoriasis associated with uncommon advanced nail and joint manifestations. Our patient showed an excellent response to methotrexate medication.
This case shows clinical features not commonly described or reported in severe cases of erythrodermic psoriasis, including severe and rare nail and arthritic findings in a pediatric scenario.
Erythrodermic psoriasis is considered a rare clinical presentation. It may arise from any type of psoriasis and occurs in adults, children and babies . It is well known that trauma, infections and drugs, such as lithium, antimalarials, trimethoprim and sulfamethoxazole, as well as environmental, psychological and metabolic factors can trigger psoriasis and the erythrodermic form of the disease [2, 3].
The generalized manifestations of the disease are erythema, edema, desquamation and systemic compromise (fever, dehydration, malaise and malnutrition) . This disease ranges from mild to severe, as in the case of our patient
Nail malformations, ranging from mild pitting with yellowish discoloration to severe onychodystrophy, are seen in all types of the disease. These symptoms are more pronounced in the fingernails than in the toenails. In addition, several well-designed studies have demonstrated an association between nail lesions and psoriatic arthritis .
The treatment of erythrodermic psoriasis should include rigorous control of a patient's hydration and nutrition. Clinical trials have proven the efficacy of systemic agents such as cyclosporin A or methotrexate, which should be started as soon as a diagnosis is made [6, 7]. Although less used in severe cases, new immunomodulators such as alefacept, efalizumab, etanercept and infliximab, are recommended in mild cases. Their use in severe erythrodermic cases has only been reported incidentally .
A 9-year-old native Hispanic girl with a history of plaque psoriasis was admitted to the emergency department of the Hospital Abina Patiño with a 4-week history of progressive skin desquamation. The condition started in her extremities and face, and then rapidly spread over her whole body.
Three months before she presented to our hospital, our patient was clinically diagnosed with plaque psoriasis and had been treated with topical corticosteroids and moisturizing creams. She had no pathological birth or related family history. Her parents also denied that she had experienced trauma, drugs, infections or any other similar triggers for her condition.
The initial management of our patient's condition included systemic treatment with methotrexate, fluid resuscitation, and well-controlled food intake. At the same time, her conjunctivitis and blepharitis were treated with artificial tears and antibiotics, respectively. Her skin was completely desquamated in the following two weeks and she achieved full skin recovery one month after she commenced treatment. Her arthritis also recovered in the first month of treatment and her malnutrition was progressively addressed with nutritional support. Her nail recovery, however, took a longer time and treatment to achieve any notable improvement.
Our case report illustrates clinical features that are not commonly described and reported in severe cases of erythrodermic psoriasis, including severe and rare nail malformations, and arthritic findings in a pediatric scenario.
The time it took for our patient's condition to evolve from one type to another was short, and without any history of the triggering factors commonly described in the literature. The diagnosis of erythrodermic psoriasis is based on the clinical features and history of her psoriasis, as well as on histopathological examinations of our patient's tissue specimens. Any final diagnosis should include a differential diagnosis which includes severe skin reaction secondary to drugs, atopic reaction, infections, and malignancies such as lymphoma and mycosis fungoides which can be clinically indistinguishable from a severe form of psoriasis. Histopathological studies are also generally needed to achieve a definitive diagnosis.
Written informed consent was obtained from the patient's next-of-kin for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Farber EM, Nall L: Childhood psoriasis. Cutis. 1999, 64 (5): 309-314.PubMedGoogle Scholar
- Dika E, Bardazzi F, Balestri R, Maibach HI: Environmental factors and psoriasis. Curr Probl Dermatol. 2007, 35: 118-135. full_text.View ArticlePubMedGoogle Scholar
- Fry L, Baker BS: Triggering psoriasis: the role of infections and medications. Clin Dermatol. 2007, 25 (6): 606-615. 10.1016/j.clindermatol.2007.08.015.View ArticlePubMedGoogle Scholar
- Naldi L, Gambini D: The clinical spectrum of psoriasis. Clin Dermatol. 2007, 25 (6): 510-518. 10.1016/j.clindermatol.2007.08.003.View ArticlePubMedGoogle Scholar
- Lavaroni G, Kokelj F, Pauluzzi P, Trevisan G: The nails in psoriatic arthritis. Acta Derm Venereol Suppl (Stockh). 1994, 186: 113-Google Scholar
- Krueger G, Ellis CN: Psoriasis: recent advances in understanding its pathogenesis and treatment. J Am Acad Dermatol. 2005, 53 (1 Suppl 1): S94-S100. 10.1016/j.jaad.2005.04.035.View ArticlePubMedGoogle Scholar
- Leman J, Burden D: Psoriasis in children: a guide to its diagnosis and management. Paediatr Drugs. 2001, 3 (9): 673-680. 10.2165/00128072-200103090-00005.View ArticlePubMedGoogle Scholar
- Ceović R, Pasić A, Lipozencić J, Murat-Susić S, Skerlev M, Husar K, Kostović K: Treatment of childhood psoriasis. Acta Dermatovenerol Croat. 2006, 14 (4): 261-264.PubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.