Psoriasis of von Zumbusch

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Psoriasis of von Zumbusch

Thais Jerez Jaime^I; Beatriz Alessi Rodrigues^II; Tatiana Jerez Jaime^III; Maria Lourdes Peris Barbo^IV; Deborah Regina Cunha Simis^V

^ISixth Year Medical Student of Faculdade de Ciências Médicas de Sorocaba - Pontifícia Universidade Católica de São Paulo (FCMS – PUCSP) – Sorocaba (SP), Brazil

^IISixth Year Medical Student of Faculdade de Ciências Médicas de Sorocaba - Pontifícia Universidade Católica de São Paulo (FCMS – PUCSP) – Sorocaba (SP), Brasil

IIIGraduate student in Dermatology at Hospital Universitário Pedro Ernesto - Universidade Estadual do Rio de Janeiro (HUPE - UERJ) – Rio de Janeiro (RJ), Brazil

IV Associate Professor of the Department of Morphology and Pathology of Pontifícia Universidade Católica de São Paulo (PUCSP) – Sorocaba (SP), Brazil

^VAssistant Professor of the Department of Medicine of Faculdade de Ciências Médicas da Pontifícia Universidade Católica de São Paulo (FCMS – PUCSP) – Sorocaba (SP), Brazil

^{Mailing Address}

ABSTRACT

Abstract: A disorder of unknown origin, psoriasis is characterized by erythemato-squamous eruption. Amongst its clinical and morphologic presentations there is acute generalized pustular psoriasis, termed the von Zumbusch variant, accompanied by generalized toxicity. It is often triggered when stopping steroids, in unstable psoriasis patients. Case report of atypical development that began during prednisone use; complete clearing of lesions and symptoms was achieved with 35 days of acitretin.

Keywords: Acitretin; Leukocytosis; Prednisone; Psoriasis

Von Zumbusch PsoriasisK

Psoriasis is a chronic inflammatory condition present in up to 3% of the population.^1-3 There are genetic components and erythemato-squamous skin lesions with variable distribution, and that may evolve with alterations in nails and joints.^1-4 Of unknown etiology, the condition is related to emotional factors, external irritants and infections.^1-3 It has universal distribution, a uniform gender distribution, and is rare among Indian and Afro-American descendents.^1,2 Age of initiation has a bimodal peak, the first of which in the third decade of life and the second around the fifth decade.^1,2 The juvenile variant, before 15 years of age, correlates with a higher percentage of the body affected, higher frequency of family cases and greater severity.¹

The diagnosis of psoriasis is essentially clinical, due to the typical lesions; however, less classical pictures may demand biopsies. ^1-3 Histopathology shows earlier lesions on the papillary dermis, vasodilatation with perivascular infiltrate1-2, followed by changes in the epidermis such as acanthosis and paraceratosis.^1-4

Regarding presentation, the condition may be classified as: vulgaris or in plaques, guttate or in drops, erythrodermic, pustular, arthropathic. ^1,2 There are special and atypical variants among which is a rare variant, an acute and generalized, pustular psoriasis called von Zumbusch.^1-3,5,6 It occurs in patients with psoriasis vulgaris, when submitted to aggravating factors, such as sunburn, use of lithium, antralin, tar, chloroquine, beta-blockers, non-steroid anti-inflammatory drugs, and mainly to sudden interruption of systemic or topical corticoid treatment.^1,2,6

We report the case of a male, Caucasian, 29 year-old patient who presented at the Emergency Room of Conjunto Hospitalar de Sorocaba, with a previous history of psoriasis vulgaris since the age of 12. He complained of intense burning and itching on his entire body surface, associated with difficulty to close his eyes and walk. He had been on continuous use of 20mg/day prednisone for six months, prescribed by another service. He did not have any lesions until the sudden development, seven days before, of a generalized erythematous picture. Upon admission, he was awake, afebrile, acyanotic and slightly dehydrated. Dermatological examination revealed generalized erythematous-squamous lesions with delicate scales and intense red erythema, in addition to countless ruptured pustules. (Figure 1). Physical examination revealed intense conjunctival purulent secretion. Initial management consisted of electrolyte replacement and initial weaning of prednisone in ten days, simultaneous to oral ciprofloxacin for eight days and to intravenous clindamycin for eight days. After the first weak of hospitalization, oral 30mg/day acitretin was introduced and maintained for 35 days. After eight days of hospitalization, pustules began to present a fetid secretion associated with fever (38°C – 41°C), suggesting secondary infection, and intravenous ceftriaxone was introduced for ten days.

The patient developed superficial keratitis due to non occlusion of the eyelid, and pneumonia for which specific treatment was introduced.

Histopathology confirmed the diagnosis of pustular psoriasis. There was complete remission of lesions after one month of treatment (Figure 2), without recurrences to date.

Generalized von Zumbusch psoriasis is characterized by the presence of pustules that, in addition to numerous and sterile, may easily burst.^1-3,6,7 Eruption is sudden on an already erythematous surface, and accompanied by dissemination.^1,6 It appears initially in flexion regions, and may affect nail beds.^1,6 Patients are affected systemically, with clinical deterioration, and there may be fever, muscle weakness, leukocytosis, hypocalcaemia and increased blood sedimentation rate, and it may sometimes be lethal.^1-3,6,7

Systemic treatment is mandatory when skin commitment is extensive, and acitretin may be used, and is particularly effective in the pustular variants. Another option is methotrexate or cyclosporine for fast control.^1-4,6-10 Patients generally do not present sequelas1-2, but there is a chance of recurrence. ^1,6

The case presented is a rare form of von Zumbusch psoriasis that erupted during systemic corticoid use and not after interruption, as commonly observed.^1,2,6

REFERENCES

Conflict of interest: None

Financial funding: None

How to cite this article: Jaime TJ, Rodrigues BA, Jaime TJ, Barbo MLP, Simis DRC. Psoríase de von Zumbusch. An Bras Dermatol. 2009;84(3):299-301.

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• 2. Sampaio SAP, Rivitti EA. Dermatologia. 3 ed. São Paulo: Artes Médicas; 2007. p. 227-41
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• 5. Proença NG, Freitas TP, Guidotti A, Seguim MC. An Bras Dermatol.1997;72:575-8
• 6. Habif TP. Dermatologia Clínica. 4 ed. São Paulo: Artmed; 2005. p. 227
• 7. Gontijo LC, Miranda LDC, Tristão RJ, Pelegrinelli FFF, Naves MC. Psoríase pustulosa generalizada (doença de von Zumbusch): apresentação atípica Relato de caso. An Bras Dermatol. 2005;80(Supl 2):S223-4
• 8. Berbis P. Acitrétine. Ann Dermatol Venereol. 2001;128:737-45
• 9. Berth-Jones J. The use of ciclosporin in psoriasis. J Dermatol Treat. 2005;16:258-77
• 10. Griffths CE, Clark CM, Chalmers RJ, Li Wan Po A, Williams HC. A systematic review of treatments for severe psoriasis. Health Technol Assess. 2000;4:1-125

Publication Dates

• Publication in this collection
  04 Aug 2009
• Date of issue
  July 2009