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Proc. roy. Soc. Med. Volume 70 April 1977
Infantile Generalized Pustular Psoriasis Responding to Dapsone Richard Staughton MA MRCP (for Peter Copeman MD FRCP) (Westminster Hospital, London SWJ) D T, boy aged 14 months
History: Presented with a three-week history of a pustular rash around his neck, unresponsive to ampicillin and topical betamethasone valerate. On examination: His neck was surrounded by a complete collar of erythema 3 cm broad, studded with yellow pustules. Otherwise his skin, scalp and mucous membranes were clear. He was apyrexial and general examination was normal. There was a strong family history of atopy, but none of psor-
Fig 3 Section showing typical Kogoj abscess. H & E ( x 400)
iasis. A week later he became pyrexial, fractious and irritable, and his skin worsened dramatically. The collar of pustules began to migrate rapidly and presented the remarkable spectacle of a band of pustular psoriasis moving at 4-5 cm a day leaving behind an erythematous, scaly, wake (see Figs 1 & 2). The band spread upwards over his face and off the crown of his head, and down each limb in turn and off the tips of his digits. Moreover, rings surrounded the bony prominences of his elbows and knees, migrating off their summits.
Investigations: Abnormal: WBC 20 000/mm3, polymorphs showing toxic granulation; skin swabs, Staph. albus only; skin biopsy, classical pustular psoriasis (Fig 3); HLA typings - patient HLA 27, patient's father HLA 27 (no clinical
psoriasis). Normal: blood cultures, nose and throat swabs, all negative; full blood count (except for raised WBC); calcium; T cell function tests, response to phytohemagglutinin; polymorph function tests, candida killing and NBT; urea and electrolytes; liver function tests; plasma proteins and electrophoresis; complement levels; immunoglobulins. Fig 1 Generalized pustular psoriasis
Fig 2 Close-up of neck showing advancing pustular edge of rash and scaly wake
Progress and treatment: During the initial attack he remained toxic, ill and poikilothermic for the four weeks the pustules took to migrate off his skin. A second attack began two weeks later with fever and rigors; again a collar of pustular psoriasis appeared around his neck and migrated quickly over the whole skin surface. Cycles were repeated at two-weekly intervals and he derived little benefit from topical treatments. After three months there was no sign of remission and a trial of dapsone at 25 mg/day was started. Three beneficial effects were noted (see Fig 4): (1) Attacks became progressively less severe. (2) The frequency of attacks diminished from two-weekly, to four-weekly, to six-weekly. (3) The morphology of the lesions changed from pustules to small greasy plaques of psoriasis.
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Fig 4 Cyclical attacks ofpustular psoriasis. Effect of dapsone Side-effects were limited to initial central cyanosis with a methemoglobinemia of 23 % falling to less than 5 % after two weeks. There was evidence of mild hamolysis but the htmoglobin remained within normal limits. Dapsone was continued for nine months in all; for the last two months no attacks occurred and so the drug was stopped. Since then a few small plaques of psoriasis have appeared regularly at six-weekly intervals, chiefly on the lower legs. Discussion Pustular psoriasis in infancy is extremely rare. A review of the literature reveals a total of 18 cases beginning before the age of 2. Most cases appear to be of the annular scaling variety rather than the more dramatic von Zumbusch pustular type. Boys outnumber girls. Reports of treatment are uniformly depressing. Topical agents and antibiotics have little effect, systemic steroids are unhelpful and hazardous. Lyon (1975) reported benefit from intermittent courses of methotrexate in a 5-yearold child with cyclical attacks of pustular psoriasis reminiscent of our patient. We were prompted to try dapsone by MacMillan & Champion (1973), who reported dramatic improvement of pustular psoriasis in an adult treated with dapsone. The results in our patient were gratifying. Dapsone appears to offer an alternative to methotrexate, whose safety in children has yet to be established. REFERENCES Beylot C, Bioulac P, Julien B et Sourreil M P (1973) Annales de Dermatologie et de Syphiligraphie (Paris) 100, 121 Khanetal S A (1972) Archives of Dermatology 105, 67 Lyon J B (1975) British Journal of Dermatology 93, Suppl. No. 2, p 40 MacMillan A L & Champion R H (1973) British Journal of Dermatology 88, 183
Dr T J Ryan: It is very unusual for infantile, pustular psoriasis to endanger life. In most children it just grumbles on often having an annular pattern affecting chiefly the trunk. I wonder whether your patient i§ an example of local steroid withdrawal effects which would have settled within three or four weeks without any systemic treatment. Dr Harvey Baker: I would accept the diagnosis. The disease is rare in childhood and seen in infancy rather than later. In infants it is commoner in boys, whereas ordinary childhood psoriasis is commoner in girls. Annular forms are particularly seen in childhood. Occasionally methotrexate cannot be avoided.
Dr E J Moynahan: I showed a patient to this Section in 1961 whose pustular psoriasis began in infancy and in whom pustulosis was confined to the front of her lower legs for a number of years before it became generalized. The condition then became very difficult to control because of vertebral collapse during corticosteroid therapy and acute psychosis due to ACTH. Indeed she was moribund on more than one occasion, until rapid and complete relief was obtained with large doses of vitamin D. The regimen consisted of 600 000 units of vitamin D twice a week for one month and once a week thereafter until the lesions cleared. She has remained well, apart from a small area of pustulosis on each leg, for the past five years.
The following cases were also presented:
(1) Systemic Sclerosis with Peripheral Neuropathy (2) Cicatricial Pemphigoid Dr C Kennedy (for Dr S C Gold) (St George's Hospital, London SWI) Sezary Syndrome Dr R Staughton (for Dr H T Calvert and Dr P Samman) (Westminster Hospital, London SWI)
Proc. roy. Soc. Med. Volume 70 April 1977
Infantile Generalized Pustular Psoriasis Responding to Dapsone Richard Staughton MA MRCP (for Peter Copeman MD FRCP) (Westminster Hospital, London SWJ) D T, boy aged 14 months
History: Presented with a three-week history of a pustular rash around his neck, unresponsive to ampicillin and topical betamethasone valerate. On examination: His neck was surrounded by a complete collar of erythema 3 cm broad, studded with yellow pustules. Otherwise his skin, scalp and mucous membranes were clear. He was apyrexial and general examination was normal. There was a strong family history of atopy, but none of psor-
Fig 3 Section showing typical Kogoj abscess. H & E ( x 400)
iasis. A week later he became pyrexial, fractious and irritable, and his skin worsened dramatically. The collar of pustules began to migrate rapidly and presented the remarkable spectacle of a band of pustular psoriasis moving at 4-5 cm a day leaving behind an erythematous, scaly, wake (see Figs 1 & 2). The band spread upwards over his face and off the crown of his head, and down each limb in turn and off the tips of his digits. Moreover, rings surrounded the bony prominences of his elbows and knees, migrating off their summits.
Investigations: Abnormal: WBC 20 000/mm3, polymorphs showing toxic granulation; skin swabs, Staph. albus only; skin biopsy, classical pustular psoriasis (Fig 3); HLA typings - patient HLA 27, patient's father HLA 27 (no clinical
psoriasis). Normal: blood cultures, nose and throat swabs, all negative; full blood count (except for raised WBC); calcium; T cell function tests, response to phytohemagglutinin; polymorph function tests, candida killing and NBT; urea and electrolytes; liver function tests; plasma proteins and electrophoresis; complement levels; immunoglobulins. Fig 1 Generalized pustular psoriasis
Fig 2 Close-up of neck showing advancing pustular edge of rash and scaly wake
Progress and treatment: During the initial attack he remained toxic, ill and poikilothermic for the four weeks the pustules took to migrate off his skin. A second attack began two weeks later with fever and rigors; again a collar of pustular psoriasis appeared around his neck and migrated quickly over the whole skin surface. Cycles were repeated at two-weekly intervals and he derived little benefit from topical treatments. After three months there was no sign of remission and a trial of dapsone at 25 mg/day was started. Three beneficial effects were noted (see Fig 4): (1) Attacks became progressively less severe. (2) The frequency of attacks diminished from two-weekly, to four-weekly, to six-weekly. (3) The morphology of the lesions changed from pustules to small greasy plaques of psoriasis.
Section of Dermatology
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Fig 4 Cyclical attacks ofpustular psoriasis. Effect of dapsone Side-effects were limited to initial central cyanosis with a methemoglobinemia of 23 % falling to less than 5 % after two weeks. There was evidence of mild hamolysis but the htmoglobin remained within normal limits. Dapsone was continued for nine months in all; for the last two months no attacks occurred and so the drug was stopped. Since then a few small plaques of psoriasis have appeared regularly at six-weekly intervals, chiefly on the lower legs. Discussion Pustular psoriasis in infancy is extremely rare. A review of the literature reveals a total of 18 cases beginning before the age of 2. Most cases appear to be of the annular scaling variety rather than the more dramatic von Zumbusch pustular type. Boys outnumber girls. Reports of treatment are uniformly depressing. Topical agents and antibiotics have little effect, systemic steroids are unhelpful and hazardous. Lyon (1975) reported benefit from intermittent courses of methotrexate in a 5-yearold child with cyclical attacks of pustular psoriasis reminiscent of our patient. We were prompted to try dapsone by MacMillan & Champion (1973), who reported dramatic improvement of pustular psoriasis in an adult treated with dapsone. The results in our patient were gratifying. Dapsone appears to offer an alternative to methotrexate, whose safety in children has yet to be established. REFERENCES Beylot C, Bioulac P, Julien B et Sourreil M P (1973) Annales de Dermatologie et de Syphiligraphie (Paris) 100, 121 Khanetal S A (1972) Archives of Dermatology 105, 67 Lyon J B (1975) British Journal of Dermatology 93, Suppl. No. 2, p 40 MacMillan A L & Champion R H (1973) British Journal of Dermatology 88, 183
Dr T J Ryan: It is very unusual for infantile, pustular psoriasis to endanger life. In most children it just grumbles on often having an annular pattern affecting chiefly the trunk. I wonder whether your patient i§ an example of local steroid withdrawal effects which would have settled within three or four weeks without any systemic treatment. Dr Harvey Baker: I would accept the diagnosis. The disease is rare in childhood and seen in infancy rather than later. In infants it is commoner in boys, whereas ordinary childhood psoriasis is commoner in girls. Annular forms are particularly seen in childhood. Occasionally methotrexate cannot be avoided.
Dr E J Moynahan: I showed a patient to this Section in 1961 whose pustular psoriasis began in infancy and in whom pustulosis was confined to the front of her lower legs for a number of years before it became generalized. The condition then became very difficult to control because of vertebral collapse during corticosteroid therapy and acute psychosis due to ACTH. Indeed she was moribund on more than one occasion, until rapid and complete relief was obtained with large doses of vitamin D. The regimen consisted of 600 000 units of vitamin D twice a week for one month and once a week thereafter until the lesions cleared. She has remained well, apart from a small area of pustulosis on each leg, for the past five years.
The following cases were also presented:
(1) Systemic Sclerosis with Peripheral Neuropathy (2) Cicatricial Pemphigoid Dr C Kennedy (for Dr S C Gold) (St George's Hospital, London SWI) Sezary Syndrome Dr R Staughton (for Dr H T Calvert and Dr P Samman) (Westminster Hospital, London SWI)
