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Rapid response of von Zumbusch psoriasis to cyclosporine
Brief communications Rapid response of von Zumbusch psoriasis to cyclosporine Mark S. Fradin, MD, Charles N. Ellis, MD, and John J. Voorhees, MD Ann Arbor, Michigan The efficacy of oral cyclosporine in the treatment of plaque-type psoriasis has been well documented in numerous trials,1-4 We describe a patient with generalized pustular (von Zumbusch) psoriasis who responded rapidly to cyclosporine. Case report. A 49-year-old man with a 3D-year history of intermittentgeneralized pustular psoriasis had an acute flare ofhis disease. He had recently discontinued oral methotrexate because of concerns about its long-term toxicity. A course of etretinate therapy 2 years before had led to an exacerbation of From the Department of Dermatology, University of Michigan Medical Center. Reprint requests: Mark S. Fradin. MD, Department of Dermatology, University of Michigan Medical Center, 1910 Taubman Center, 1500 E. Medical Center Dr., Ann Arbor, MY 48109-0314. *The PASI score (Psoriasis Area and Severity Index) is a numeric system that grades psoriasis according to the extent of erythema, induation. scaling, and percentage of body surface involved. The score ranges from 0 to 72; higher numbers indicate more severe disease. 5
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his disease. Examination revealed plaques of pustular psoriasis on 50% of the body (Fig. I, A); new psoriatic areas were developing daily. Involvement of the hands and feet, particularly the tips of the digits, was severe. In addition, the patient's psoriatic arthritis was flaring and was no longer responding to piroxicam. His baseline PASI* score was 33.6. After providing informed consent the patient began outpatient treatment with oral cyclosporine, 7.5 mg/kg per day. Within 24 hours he noted a subjective improvement in his skin lesions. By day 3 pustules were no longer present. After 1 month he reported marked improvement in the range of motion of the hands. His PASI score was 14.9 and the plaques were mild-moderate in severity. The patient's cyc1osporine dosage was decreased to 5.0 mg/kg per day. The patient continued to be examined biweekly, and, because of his excellent clinical response, dosage reductions continued. By week 16, he was receiving cyclosporine at a dosage of 3.5 mg/kg per day and his PASI score was 0; only posti:iJ.f1amrnatory hyperpigmentation remained (Fig. 1, B). The patient noticed that his nails had begun to grow normally. Continued dosage reductions of approximately 0.5 mg/kg every 2 weeks were made. At a dosage of 1.5 mg/kg per day, at week 20, the patient noted mild worsening ofhis psoriasis and increased joint pain. No pustular lesions were noted. A gradual increase in the cyclosporine dosage to 3.5 mg/kg per day resulted in remission, and his condition has been successfully maintained with that dosage for more than 8 months. Laboratory evaluations were performed at each visit and
Fig. 1. Patient at baseline (A) and after 16 weeks of cyclosporine treatment (B). 925
Journal of the American Academy of Dermatology
926 Brief communications showed no changes from baseline normal values. At week 4 the patient was noted to have hypertension. Verapamil, 240 mg/day orally, was given, with return of blood pressure to normotensive levels. Other side effects included perioral tingling, increased temperature sensitivity of the hands and feet, and hand tremor, all of which resolved spontaneously during continued treatment. Hypertrichosis was noted at week 12 and persisted throughout therapy. Discussion. Meinardi et a1. 6 used cyclosporine to treat a patient with a generalized pustular flare of psoriasis triggered by discontinuation ofmethotrexate therapy and noted dramatic improvement in the skin disease and in the psoriatic arthritis. Korstanje et a1. 7 found cyc1osporine in combination with oral prednisone to be effective in the treatment of a patient with pustular psoriasis and acrodermatitis continua of Hallopeau, but a 'high dosage of 8 mg/kg per day was needed to maintain remission. Zachariae and Thestrup-Pedersen 8 treated a woman with incapacitating pustular psoriasis of the palms and soles and was able to induce clearance for the first time in 10 years. Similar results were reported by Reitamo et a1. 9 Our findings support the dramatic and rapid efficacy of cyc1osporine in the treatment of generalized pustular psoriasis. In contrast to other authors, however, we found it possible to maintain remission in our patient with low mg/kg per 4ay). We suggest dosages of cyclosporine that fl slow decrease· in the cyclosporine dosage (by no more than 0.5 to 1.0 mg/kg per day ~onthly) may be more effective in maintaining remissions than a rapid decrease, particularly when the patient is already receiving low dosages of cyclosporine.
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REFERENCES I. Ellis CN, Gorsulowsky DC, Hamilton TA, et al. Cyclosporine improves psoriasis in a double-blind study. JAMA 1986;256:3110-6. 2. Fry L, Griffiths CEM, Powles AV, et al. Long-term cyclosporine in the management of psoriasis. Transplant Proc 1988;20(suppI4):23-5. 3. Van Joost Th, Bos JD, Heule F, et al. Low-dose cyclosporine in severe psoriasis: a double-blind study. Br J Dermatol 1988;118:183-90. 4. Meinardi MM, Bos JD. Cyclosporine maintenance therapy in psoriasis. Transplant Proc 1988;20(suppl 4):42-8. 5. Fredriksson T, Patterson V. Severe psoriasis: oral therapy with a new retinoid. Dermato1ogica 1978;157:238-44. 6. Meinardi MM, Westerhof W, Bos JD. Generalized pustular psoriasis (von Zumbusch) responding to cyclosporine. Br J Dermatol 1987;116:269-70. 7. Korstanje MJ, Bessems PJMJ, Hulsmans RFHJ, et al. Pustular psoriasis and acrodermatitis continua (Hallopeau) need high doses of systemic ciclosporin A. Dermatologica 1989;179:90-1. 8. Zachariae H, Thestrup-Pedersen K. Ciclosporin A in acrodermatitis continua. Dermatologica 1987;175:29-32. 9. Reitamo S, Puska P, Lassus A. Cyclosporin in the treatment of palma-plantar pustulosis. Br J Dermatoll989;120:857.
Exacerbation of Darier's disease by lithium carbonate Gayle P. Milton, MD,a Gary L. Peck, MD,a luian-luian L. Fu, MD, PhD,b John J. DiGiovanna, MD,a James J. Nordlund, MD,b lames H. Thomas, MD,c and Susan F. Sanders, MD* Bethesda, Maryland, and Cincinnati, Ohio Lithium exacerbates preexisting psoriasis and acne and produces a variety of cutaneous eruptions. 1 In 1986 Clark et al. 2 described a patient with Darier's disease that flared during lithium therapy and exacerbated on rechallenge. In this report we present another case of Darier's disease, well controlled with etretinate, that markedly worsened on two separate occasions after lithium ingestion and improved after its discontinuation. From the Dermatology Branch, National Cancer Institute, Bethesda,' and the Departments ofDermato!ogY' and Psychiatry,C University of Cincinnati College of Medicine. Reprint requests: Gary L. Peck, MD, Dermatology Braneh, National Cancer Institute, Bldg. 10, Room 12N238, National Institutes of Health, Bethesda, MD 20892. *Dr. Sanders is in private practice, Cincinnati, Ohio.
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Fig. 1. Within 1 month oflithium carbonate treatment, severe flare of Darier's disease is present with marked hyperkeratosis and infection with exudation, despite etretinate therapy (1 mg/kg/day). Skin of back had been free of Darier's disease before initiation of lithium therapy.
16/4/22994
his disease. Examination revealed plaques of pustular psoriasis on 50% of the body (Fig. I, A); new psoriatic areas were developing daily. Involvement of the hands and feet, particularly the tips of the digits, was severe. In addition, the patient's psoriatic arthritis was flaring and was no longer responding to piroxicam. His baseline PASI* score was 33.6. After providing informed consent the patient began outpatient treatment with oral cyclosporine, 7.5 mg/kg per day. Within 24 hours he noted a subjective improvement in his skin lesions. By day 3 pustules were no longer present. After 1 month he reported marked improvement in the range of motion of the hands. His PASI score was 14.9 and the plaques were mild-moderate in severity. The patient's cyc1osporine dosage was decreased to 5.0 mg/kg per day. The patient continued to be examined biweekly, and, because of his excellent clinical response, dosage reductions continued. By week 16, he was receiving cyclosporine at a dosage of 3.5 mg/kg per day and his PASI score was 0; only posti:iJ.f1amrnatory hyperpigmentation remained (Fig. 1, B). The patient noticed that his nails had begun to grow normally. Continued dosage reductions of approximately 0.5 mg/kg every 2 weeks were made. At a dosage of 1.5 mg/kg per day, at week 20, the patient noted mild worsening ofhis psoriasis and increased joint pain. No pustular lesions were noted. A gradual increase in the cyclosporine dosage to 3.5 mg/kg per day resulted in remission, and his condition has been successfully maintained with that dosage for more than 8 months. Laboratory evaluations were performed at each visit and
Fig. 1. Patient at baseline (A) and after 16 weeks of cyclosporine treatment (B). 925
Journal of the American Academy of Dermatology
926 Brief communications showed no changes from baseline normal values. At week 4 the patient was noted to have hypertension. Verapamil, 240 mg/day orally, was given, with return of blood pressure to normotensive levels. Other side effects included perioral tingling, increased temperature sensitivity of the hands and feet, and hand tremor, all of which resolved spontaneously during continued treatment. Hypertrichosis was noted at week 12 and persisted throughout therapy. Discussion. Meinardi et a1. 6 used cyclosporine to treat a patient with a generalized pustular flare of psoriasis triggered by discontinuation ofmethotrexate therapy and noted dramatic improvement in the skin disease and in the psoriatic arthritis. Korstanje et a1. 7 found cyc1osporine in combination with oral prednisone to be effective in the treatment of a patient with pustular psoriasis and acrodermatitis continua of Hallopeau, but a 'high dosage of 8 mg/kg per day was needed to maintain remission. Zachariae and Thestrup-Pedersen 8 treated a woman with incapacitating pustular psoriasis of the palms and soles and was able to induce clearance for the first time in 10 years. Similar results were reported by Reitamo et a1. 9 Our findings support the dramatic and rapid efficacy of cyc1osporine in the treatment of generalized pustular psoriasis. In contrast to other authors, however, we found it possible to maintain remission in our patient with low mg/kg per 4ay). We suggest dosages of cyclosporine that fl slow decrease· in the cyclosporine dosage (by no more than 0.5 to 1.0 mg/kg per day ~onthly) may be more effective in maintaining remissions than a rapid decrease, particularly when the patient is already receiving low dosages of cyclosporine.
«4
REFERENCES I. Ellis CN, Gorsulowsky DC, Hamilton TA, et al. Cyclosporine improves psoriasis in a double-blind study. JAMA 1986;256:3110-6. 2. Fry L, Griffiths CEM, Powles AV, et al. Long-term cyclosporine in the management of psoriasis. Transplant Proc 1988;20(suppI4):23-5. 3. Van Joost Th, Bos JD, Heule F, et al. Low-dose cyclosporine in severe psoriasis: a double-blind study. Br J Dermatol 1988;118:183-90. 4. Meinardi MM, Bos JD. Cyclosporine maintenance therapy in psoriasis. Transplant Proc 1988;20(suppl 4):42-8. 5. Fredriksson T, Patterson V. Severe psoriasis: oral therapy with a new retinoid. Dermato1ogica 1978;157:238-44. 6. Meinardi MM, Westerhof W, Bos JD. Generalized pustular psoriasis (von Zumbusch) responding to cyclosporine. Br J Dermatol 1987;116:269-70. 7. Korstanje MJ, Bessems PJMJ, Hulsmans RFHJ, et al. Pustular psoriasis and acrodermatitis continua (Hallopeau) need high doses of systemic ciclosporin A. Dermatologica 1989;179:90-1. 8. Zachariae H, Thestrup-Pedersen K. Ciclosporin A in acrodermatitis continua. Dermatologica 1987;175:29-32. 9. Reitamo S, Puska P, Lassus A. Cyclosporin in the treatment of palma-plantar pustulosis. Br J Dermatoll989;120:857.
Exacerbation of Darier's disease by lithium carbonate Gayle P. Milton, MD,a Gary L. Peck, MD,a luian-luian L. Fu, MD, PhD,b John J. DiGiovanna, MD,a James J. Nordlund, MD,b lames H. Thomas, MD,c and Susan F. Sanders, MD* Bethesda, Maryland, and Cincinnati, Ohio Lithium exacerbates preexisting psoriasis and acne and produces a variety of cutaneous eruptions. 1 In 1986 Clark et al. 2 described a patient with Darier's disease that flared during lithium therapy and exacerbated on rechallenge. In this report we present another case of Darier's disease, well controlled with etretinate, that markedly worsened on two separate occasions after lithium ingestion and improved after its discontinuation. From the Dermatology Branch, National Cancer Institute, Bethesda,' and the Departments ofDermato!ogY' and Psychiatry,C University of Cincinnati College of Medicine. Reprint requests: Gary L. Peck, MD, Dermatology Braneh, National Cancer Institute, Bldg. 10, Room 12N238, National Institutes of Health, Bethesda, MD 20892. *Dr. Sanders is in private practice, Cincinnati, Ohio.
16/4/21327
Fig. 1. Within 1 month oflithium carbonate treatment, severe flare of Darier's disease is present with marked hyperkeratosis and infection with exudation, despite etretinate therapy (1 mg/kg/day). Skin of back had been free of Darier's disease before initiation of lithium therapy.