Juvenile generalized circinate pustular psoriasis treated with oral cyclosporin A

ARTICLE

Reports from Scandinavia suggest the onset of psoriasis before the age of 16 years in about 45% of patients [1]. Plaque and guttate psoriasis are the most frequently observed variants to psoriasis in childhood [2]. Pustular psoriasis is a rare disease. Among 104 cases with generalized pustular psoriasis from the UK there were only four children under the age of 11 years [3]. Another large study from the United States reviewing 63 patients with generalized pustular psoriasis included 13 patients with an onset earlier than 18 years of age [4]. The most common form is generalized pustular psoriasis of the circinate or annular type.

There is no standardized treatment for juvenile pustular psoriasis supported by evidence-based medicine, but in general there is little serious morbidity, in contrast to adults. The condition in most patients is well controlled with topical therapy. In some patients, however, the disease takes a more serious course and systemic therapy is mandatory. Cyclosporin A (CsA) is effective in adults with severe psoriasis but has previously not been systematically investigated in children. In childhood psoriasis only one case report has been published as far as we know [5]. We report on a 17-year-old boy suffering from generalized pustular psoriasis since birth, who was treated with CsA with good success.

Case report

A 17-year old boy was admitted to our hospital with a generalized pustular eruption on an erythematous base of two weeks duration, in combination with an streptococcal angina tonsillaris.

History: There was a positive family history of pustular (sister) and non-pustular (father) psoriasis. He suffered from neonatal pustular psoriasis. Because of severe febrile and toxic pustular psoriasis the boy was treated with etretinate from 1987 to 1992. He was in partial remission with minimal to moderate residual disease until spring 1999.

Clinical examination: A severely ill boy was admitted with fever but normal physical and mental development. His body weight was 63 kg. On examination widespread pustular eruptions on an erythematous base were observed on his trunk (Fig. 1). On the head a heavy scaling with marked thickening of the skin was observed. On the limbs numerous erythemato-squamous plaques, in part with annular pustulosis, were found. The genitals were also involved. The initial PASI scored for 40.7 points.

Laboratory investigations: C-reactive protein 30.4 mg/l, leukocytosis of 16.3 Gpt/l. Normal results were obtained for creatinin, creatinin clearance, urea, transaminases.

Treatment and course: The angina was treated with oral penicillin 0.8 mio U 3 x daily for ten days. Psoriasis could not be controlled by topical combination therapy with amcinomide lotio, calcipotriol lotio, and urea ointments. Therefore systemic prednisolone therapy with 60 mg/d and CsA 200 mg/d in a microemulsion formulation (Sandimmun Optoral^®; Novartis Germany) was initiated. Because of temporary blood pressure increase (160/ 100 mmHg), the CsA dosage was reduced (2 x 50 mg/d). During the following 12 weeks prednisolone was tapered down to 5 mg/day. Topical corticosteroids were no longer necessary, but calcipotriol and urea ointments were continued on an outpatient basis. The pustular lesions completely disappeared and PASI dropped down to 4.8 score points (Fig. 2).

During a follow-up of 15 months, no relapse was observed and only a limited skin involvement was noted. Monitoring was performed regularly as recommended by the manufacturer. Blood pressure and serum creatinine remained within the normal range. No clinical signs of immunosuppression were observed. The residual psoriatic lesions were of the erythemato-squamous type. No pustules were observed during follow-up.

Discussion

Generalized pustular psoriasis before the age of 18 years is a rare disease. Less than 100 cases have been reported in the literature. Despite the fact that in most cases the disease is well-controlled by topical corticosteroids and/or calcipotriol, some patients need systemic therapy. In case reports and uncontrolled retrospective studies, low-dose methotrexate [6], etretinate [5, 7], PUVA therapy [2, 5] and sulphones [5, 8] have been used with success in several patients. Recently, the Chinese herb Lei-Gong-teng was employed in 26 patients with childhood generalized pustular psoriasis in a dosage range between 70 to 210 mug tripterlide [9]. We have no experience with this drug. But we feel that available data are not sufficient for a recommendation.

In the past the patient presented herein received a partial remission with etretinate. However, the side-effects such as dryness and increased sensitivity of skin and mucous membranes were no longer tolerable for him. Because of a severe relapse of his generalized pustular psoriasis we used a combination of systemic corticosteroids with CsA to interrupt the pustular eruption. The initial dosage of 3.2 mg CsA per kg body weight and day had to be decreased because of rising blood pressure. After dosage reduction to 1.6 mg per kg and day the treatment was well tolerated in a long-term run. Corticosteroids, both systemic and topical, could be tapered down. The psoriasis type was changed from the severe generalized pustular one to nummular psoriasis vulgaris. Our experience is in accordance with another case report where a 9-year-old boy with generalized pustular psoriasis was treated with 3 mg CsA for 11 months. A complete remission was induced [5].

CsA is widely used in pediatric patients as a primary immunosuppressant in renal and liver transplantation [10, 11]. There is also evidence for its efficacy and tolerability in severe childhood atopic dermatitis [12, 13].

Maintenance CsA therapy is useful in patients with severe psoriasis [14]. Continuous CsA therapy is more efficient than intermittent long-term therapy [15].

We observed a good clinical response with good tolerability in our patient. The effect seems to be more delayed than in atopic dermatitis and maintenance therapy was preferred, not a short-time course.

CsA seems to be a treatment option in patients before the age of 18 years after informed consent, when they suffer from a severe type of psoriasis and other therapies are not sufficient or applicable, when they have no contraindications, when they and their family are compliant, and the monitoring is performed on a regular base.

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