64
CORRESPONDENCE
References 1 Rosenberg SA. l^tze MT. Muul LM et al. A progress report on the treatment of 1 57 patients with advanced cancer using lymphokineactivated killer cells and interleukin-2 or high-dose interIeukin-2 alone. New Encjl I Med 1987; 316: 889-97. 2 Nagatani T. Kin ST. Baba N et al. A case of cutaneous T cell lymphoma treated with recombinant interleukin 2 (rIL-2). Ada Derm Venereol (Stockh). 1988: 68: 504-8. 3 Agnarsson BA. Vonderheid EC. Kadin ME. Cutaneous T cell lymphoma with suppressor/cytotoxic (CD8) phenotype: identification of rapidly progressive and chronic subtypes. / Am Acad Dennatol 1990; 22: 569-77. 4 Allison MA. Jones SE. McGuffey P. Phase II trial of outpatient interleukin-2 in malignant lymphoma. chronic lymphocytic leukemia, and selected solid tumors. / Clin Oncol 1989; 7: 75-80.
Capillary leak syndrome in generalized pustular psoriasis SIR. We were interested to read Dr McGregor's report of two patients with capillary leak syndrome complicating pustular psoriasis.' We would like to report a further case with a more favourable outcome. A 33-year-old obese lady was admitted urgently suffering from widespread erythrodermic psoriasis. Within 12 h of her admission she had developed pustulation of such severity that her skin peeled off in sheets leaving glazed, red, moist areas covering 90% of her body. She had first developed psoriasis with arthropathy in 1981. The psoriasis rapidly became widespread and difficult to control. She had received treatment with PUVA. methotrexate, azathioprine and etretinate with either lack of response or unacceptable side-effects. Fn 1989 following an acute flare of her psoriasis she was started on cyclosporin A which had been continued at a dose of 2-4 mg/ kg/day. Blood pressure and renal function had shown no deterioration and her skin had responded well. Six weeks prior to her admission she had felt vaguely unwell and in the week before admission had felt nauseated, and omitted her cyclosporin for 1-2 days. On admission her investigations showed normal renal and liver function: her neutrophil count was raised at 22 3 x 10^1 and her chest X-ray was normal. Over the next 3 days her skin improved with continued cyclosporin A and emollients. However, her renal function deteriorated (creatinine 414 /(mol/l). her chest X-ray showed fluffy shadowing and her blood pressure fell. She was therefore transferred to the intensivecare unit where she received positive inotropic support and mannitol. and her renal function improved. Her principal problem then became her gas exchange, with progressive hypoxia which was not controlled with continuous positive airways pressure. A decision was made to provide ventilatory support. The fluffy shadowing on X-ray, and the clear frothy fluid from her lungs suggested the diagnosis of the capillary leak syndrome. At the onset of pulmonary problems blood cultures were negative, with no evidence of Gram-negative sepsis. Later in the course of her illness multiple organisms were isolated from her chest {Streptococcus pneumoniae. coliforms) and skin
{Pseiidowonas. Staphylococcus aureus). Antibiotic therapy with vancomycin. gentarnicin and erythromycin, was given intravenously in accordance with the sensitivities of the isolated organisms and in addition the skin was treated with topical silver sulphadiazine (Flamazine®). Although her urine output was satisfactory there was a progressive rise in her serum creatinine and urate: she was therefore treated with haemoHltration. Her neutrophil count had also risen progressively reaching 6 6 x 1 0 ^ 1 . The initiation of haemofiltration coincided with a marked improvement in her skin which had remained erythrodermic despite intravenous cyclosporin A at doses dictated by her blood levels. Her neutrophil count also fell and it was thought that the haemofiltration had probably removed inflammatory mediators such as IL-l and TNF. Over the next 3 weeks the patient had a stormy course. Problems included unstable blood pressure, episodes of asystole necessitating resuscitation, and sepsis of both lungs and skin, particularly around intravenous lines. However, 21 days after transfer to the intensive-care unit the patient was extubated. and 3 days later was transferred to the ward: 10 days later she was well enough to be discharged. Prior to discharge her renal function, full blood count and pulmonary function tests showed minor abnormalities only, and her chest X-ray was clear. She was discharged on cyclosporin A 4 mg/kg/day which was increased to 5 mg/kg/ day when her skin flared a week following discharge. Six months after this dramatic episode she remains well. St John's Derwatology Centre, St Thomas' Hospital. London SEl 7EH. U.K.
S.E.HANDFIELD-JONES M.GARVEY
D.H.McGiBBON M.M.BLACK
Reference 1 McGregor JM, Barker JNWN. MacDonald DM. Pulmonary capillary leak syndrome complicating generalized pustular psoriasis; possible role of cytokines. Br I Dennatol 1991; 125: 472-4,
Successftil treatment of ulcerating livedo reticularis with infusions of prostacyclin SIR, Livedo reticularis associated with atrophie blanche (livedo reticularis with ulceration: livedoid vasculitis) is an uncommon condition characterized by purplish discoloration of the skin in a reticular pattern on the legs, leading to painful ulceration, and leaving ivory-white scars after healing.' Although this condition has been reported in association with several systemic diseases^ most cases seem to be idiopathic. The pathogenesis is thought to be related to thrombotic occlusion of small and medium-sized vessels of the dermis. Microscopically deposition of immunoglobulins, complement and fibrin has been demonstrated' but inflammatory changes are usually absent.'"^ Beneficial therapeutic effects have been reported for fibrinolytic agents, heparin, pentoxifylline and antiplatelet drugs'*"" and are possibly related to the proposed imbalance between coagulation and fibrinolysis in this disorder. However,
CORRESPONDENCE
References 1 Rosenberg SA. l^tze MT. Muul LM et al. A progress report on the treatment of 1 57 patients with advanced cancer using lymphokineactivated killer cells and interleukin-2 or high-dose interIeukin-2 alone. New Encjl I Med 1987; 316: 889-97. 2 Nagatani T. Kin ST. Baba N et al. A case of cutaneous T cell lymphoma treated with recombinant interleukin 2 (rIL-2). Ada Derm Venereol (Stockh). 1988: 68: 504-8. 3 Agnarsson BA. Vonderheid EC. Kadin ME. Cutaneous T cell lymphoma with suppressor/cytotoxic (CD8) phenotype: identification of rapidly progressive and chronic subtypes. / Am Acad Dennatol 1990; 22: 569-77. 4 Allison MA. Jones SE. McGuffey P. Phase II trial of outpatient interleukin-2 in malignant lymphoma. chronic lymphocytic leukemia, and selected solid tumors. / Clin Oncol 1989; 7: 75-80.
Capillary leak syndrome in generalized pustular psoriasis SIR. We were interested to read Dr McGregor's report of two patients with capillary leak syndrome complicating pustular psoriasis.' We would like to report a further case with a more favourable outcome. A 33-year-old obese lady was admitted urgently suffering from widespread erythrodermic psoriasis. Within 12 h of her admission she had developed pustulation of such severity that her skin peeled off in sheets leaving glazed, red, moist areas covering 90% of her body. She had first developed psoriasis with arthropathy in 1981. The psoriasis rapidly became widespread and difficult to control. She had received treatment with PUVA. methotrexate, azathioprine and etretinate with either lack of response or unacceptable side-effects. Fn 1989 following an acute flare of her psoriasis she was started on cyclosporin A which had been continued at a dose of 2-4 mg/ kg/day. Blood pressure and renal function had shown no deterioration and her skin had responded well. Six weeks prior to her admission she had felt vaguely unwell and in the week before admission had felt nauseated, and omitted her cyclosporin for 1-2 days. On admission her investigations showed normal renal and liver function: her neutrophil count was raised at 22 3 x 10^1 and her chest X-ray was normal. Over the next 3 days her skin improved with continued cyclosporin A and emollients. However, her renal function deteriorated (creatinine 414 /(mol/l). her chest X-ray showed fluffy shadowing and her blood pressure fell. She was therefore transferred to the intensivecare unit where she received positive inotropic support and mannitol. and her renal function improved. Her principal problem then became her gas exchange, with progressive hypoxia which was not controlled with continuous positive airways pressure. A decision was made to provide ventilatory support. The fluffy shadowing on X-ray, and the clear frothy fluid from her lungs suggested the diagnosis of the capillary leak syndrome. At the onset of pulmonary problems blood cultures were negative, with no evidence of Gram-negative sepsis. Later in the course of her illness multiple organisms were isolated from her chest {Streptococcus pneumoniae. coliforms) and skin
{Pseiidowonas. Staphylococcus aureus). Antibiotic therapy with vancomycin. gentarnicin and erythromycin, was given intravenously in accordance with the sensitivities of the isolated organisms and in addition the skin was treated with topical silver sulphadiazine (Flamazine®). Although her urine output was satisfactory there was a progressive rise in her serum creatinine and urate: she was therefore treated with haemoHltration. Her neutrophil count had also risen progressively reaching 6 6 x 1 0 ^ 1 . The initiation of haemofiltration coincided with a marked improvement in her skin which had remained erythrodermic despite intravenous cyclosporin A at doses dictated by her blood levels. Her neutrophil count also fell and it was thought that the haemofiltration had probably removed inflammatory mediators such as IL-l and TNF. Over the next 3 weeks the patient had a stormy course. Problems included unstable blood pressure, episodes of asystole necessitating resuscitation, and sepsis of both lungs and skin, particularly around intravenous lines. However, 21 days after transfer to the intensive-care unit the patient was extubated. and 3 days later was transferred to the ward: 10 days later she was well enough to be discharged. Prior to discharge her renal function, full blood count and pulmonary function tests showed minor abnormalities only, and her chest X-ray was clear. She was discharged on cyclosporin A 4 mg/kg/day which was increased to 5 mg/kg/ day when her skin flared a week following discharge. Six months after this dramatic episode she remains well. St John's Derwatology Centre, St Thomas' Hospital. London SEl 7EH. U.K.
S.E.HANDFIELD-JONES M.GARVEY
D.H.McGiBBON M.M.BLACK
Reference 1 McGregor JM, Barker JNWN. MacDonald DM. Pulmonary capillary leak syndrome complicating generalized pustular psoriasis; possible role of cytokines. Br I Dennatol 1991; 125: 472-4,
Successftil treatment of ulcerating livedo reticularis with infusions of prostacyclin SIR, Livedo reticularis associated with atrophie blanche (livedo reticularis with ulceration: livedoid vasculitis) is an uncommon condition characterized by purplish discoloration of the skin in a reticular pattern on the legs, leading to painful ulceration, and leaving ivory-white scars after healing.' Although this condition has been reported in association with several systemic diseases^ most cases seem to be idiopathic. The pathogenesis is thought to be related to thrombotic occlusion of small and medium-sized vessels of the dermis. Microscopically deposition of immunoglobulins, complement and fibrin has been demonstrated' but inflammatory changes are usually absent.'"^ Beneficial therapeutic effects have been reported for fibrinolytic agents, heparin, pentoxifylline and antiplatelet drugs'*"" and are possibly related to the proposed imbalance between coagulation and fibrinolysis in this disorder. However,
