Correspondence Pyoderma gangrenosum in infancy
To the Editor: In their brief communication entitled, "Pyoderma Gangrenosum in Infancy: The Youngest Reported Patient," (J AM ACAD DERMATOL 1991;25:10910), Drs. Glass, Bancila, and Milgraum present a patient who they believe to have pyoderma gangrenosum. I suggest that their patient has a variant of Sweet's syndrome occurring in children as published by Esterly et a1. 1 and Hazen et al. 2 Several features suggest that this is likely to be Sweet's syndrome rather than pyoderma gangrenosum. 1. Itoccurred after an infectious/immunologic event. Many patients develop Sweet's syndrome, including children, after the immunologic stimulation of infection. Therefore I suspect that the immunization probably acted as the inciting event. 2. Significant leukocytosis with white blood cell count of 21,900/mm3 is one ofthe key features of Sweet's syndrome. 3. Fever is common in Sweet's syndrome. 4. Multiplicity of lesions is more common in Sweet's syndrome, less common in pyoderma gangrenosum. 5. Ithad lesions clinically consistent with Sweet's syndrome. In addition, the histologic picture of the skin biopsy specimen, showing a diffuse infiltrate of primarily polymorphonuclear cells, is compatible with that of Sweet's syndrome. . 6. The rapid response to steroids and/or dapsone IS compatible with that which occurs in Sweet's syndrome. The response is more rapid and generally occurs at lower doses than would be expected with severe pyoderma gangrenosum. . . 7. Pyoderma gangrenosum is usually aSSOCIated WI:h more undermining of the margins than was observed In the patient they described. We congratulate Drs. Glass, Banci1a and Milgraum.on the successful management of their patient but questIon whether the lesions in their patient could be compatible with those described as "Sweet's syndrome in childhood." Paul G. Hazen, MD 14701 Detroit Ave. Lakewood, OH 44107 REFERENCES 1. Levin DL, Esterly NB, Herman JJ, et aL The Sweet syndrome in children. J Pediatr 1981;99:73-8. . 2. Hazen PG, Kar EC, David BR, et aL Acute febn1e neutrophilic dermatosis in children. Arch Dermatol1983;119:9981002.
Reply
To the Editor: We appreciate the interest in our article shown by Hazen et al., who suggested that our patient was
more likely to have Sweet's syndrome than pyoderma gangrenosum. Unfortunately, in the months after our report, the patient took a dramatic turn for the worse and died. He had been referred to a pediatric cardiologist for evaluation of a newly noted murmur. Physical findings were consistent with aortic stenosis and an echocardiogram showed significant narrowing throughout the aortic arch with severe left ventricular hypertrophy. His erythrocyte sedimentation rate was 93 mm/hr and he was treated with alternate-day prednisone therapy on the basis of the tentative diagnosis of Beh~et's disease. He remained asymptomatic for the next few weeks, but further studies showed an increasing severity of the obstructive process with increasing left ventricular hypertrophy and involvement of the coronary and head vessels. The patient underwent surgery for repair of the distal ascending aorta, the aortic arch, and the proximal ascending aorta. During surgery, numerous adhesions were noted and the involved vessels were extremely fibrotic and adherent to the surrounding structures. The patient died several hours after surgery, presumably because of bleeding at the anastomotic sites. A biopsy specimen of the innominate artery and aortic tissue revealed an active necrotizing arteritis and aortitis with granulomatous features, including giant cells. Although the caus~ could not be determined, the plasmacytic nature of the mflammatory infiltrate suggested an immune-mediated process. According to the pathologist, Takayasu's ("pulse1ess") disease and granulomatous (temporal) aortitis would have a similar distribution; the inflammation in these conditions is granulomatous (C. C. Marboe, personal communication, January 1992). With this new information we are certain that the patient did not have Sweet's syndrome, as suggested by Hazen et al. The patient's lesions demonstrated pathergy. Although this has been reported in Sweet's syndrome,l, 2 it is far more common in pyoderma gangrenosum, particularly in children. 3 In addition, the severe, fatal granulomatous arteritis and aortitis in our patient has not been reported with Sweet's syndrome. However, there are several case reports of pyoderma gangrenosum associated with Takayasu's arteritis. 4 Therefore, although some features of the initial presentation of our case may also have been consistent with Sweet's syndrome, the profound pathergy and severe, large-vessel granulomatous vasculitis make the diagnosis of pyoderma gangrenosum associated with Takayasu's arteritis more likely.
Alan T. Glass, BA Joseph W. Gaffney, MD Sandy S. Milgraum, MD University ofMedicine and Dentistry of New Jersey New Brunswick, New Jersey
275
To the Editor: In their brief communication entitled, "Pyoderma Gangrenosum in Infancy: The Youngest Reported Patient," (J AM ACAD DERMATOL 1991;25:10910), Drs. Glass, Bancila, and Milgraum present a patient who they believe to have pyoderma gangrenosum. I suggest that their patient has a variant of Sweet's syndrome occurring in children as published by Esterly et a1. 1 and Hazen et al. 2 Several features suggest that this is likely to be Sweet's syndrome rather than pyoderma gangrenosum. 1. Itoccurred after an infectious/immunologic event. Many patients develop Sweet's syndrome, including children, after the immunologic stimulation of infection. Therefore I suspect that the immunization probably acted as the inciting event. 2. Significant leukocytosis with white blood cell count of 21,900/mm3 is one ofthe key features of Sweet's syndrome. 3. Fever is common in Sweet's syndrome. 4. Multiplicity of lesions is more common in Sweet's syndrome, less common in pyoderma gangrenosum. 5. Ithad lesions clinically consistent with Sweet's syndrome. In addition, the histologic picture of the skin biopsy specimen, showing a diffuse infiltrate of primarily polymorphonuclear cells, is compatible with that of Sweet's syndrome. . 6. The rapid response to steroids and/or dapsone IS compatible with that which occurs in Sweet's syndrome. The response is more rapid and generally occurs at lower doses than would be expected with severe pyoderma gangrenosum. . . 7. Pyoderma gangrenosum is usually aSSOCIated WI:h more undermining of the margins than was observed In the patient they described. We congratulate Drs. Glass, Banci1a and Milgraum.on the successful management of their patient but questIon whether the lesions in their patient could be compatible with those described as "Sweet's syndrome in childhood." Paul G. Hazen, MD 14701 Detroit Ave. Lakewood, OH 44107 REFERENCES 1. Levin DL, Esterly NB, Herman JJ, et aL The Sweet syndrome in children. J Pediatr 1981;99:73-8. . 2. Hazen PG, Kar EC, David BR, et aL Acute febn1e neutrophilic dermatosis in children. Arch Dermatol1983;119:9981002.
Reply
To the Editor: We appreciate the interest in our article shown by Hazen et al., who suggested that our patient was
more likely to have Sweet's syndrome than pyoderma gangrenosum. Unfortunately, in the months after our report, the patient took a dramatic turn for the worse and died. He had been referred to a pediatric cardiologist for evaluation of a newly noted murmur. Physical findings were consistent with aortic stenosis and an echocardiogram showed significant narrowing throughout the aortic arch with severe left ventricular hypertrophy. His erythrocyte sedimentation rate was 93 mm/hr and he was treated with alternate-day prednisone therapy on the basis of the tentative diagnosis of Beh~et's disease. He remained asymptomatic for the next few weeks, but further studies showed an increasing severity of the obstructive process with increasing left ventricular hypertrophy and involvement of the coronary and head vessels. The patient underwent surgery for repair of the distal ascending aorta, the aortic arch, and the proximal ascending aorta. During surgery, numerous adhesions were noted and the involved vessels were extremely fibrotic and adherent to the surrounding structures. The patient died several hours after surgery, presumably because of bleeding at the anastomotic sites. A biopsy specimen of the innominate artery and aortic tissue revealed an active necrotizing arteritis and aortitis with granulomatous features, including giant cells. Although the caus~ could not be determined, the plasmacytic nature of the mflammatory infiltrate suggested an immune-mediated process. According to the pathologist, Takayasu's ("pulse1ess") disease and granulomatous (temporal) aortitis would have a similar distribution; the inflammation in these conditions is granulomatous (C. C. Marboe, personal communication, January 1992). With this new information we are certain that the patient did not have Sweet's syndrome, as suggested by Hazen et al. The patient's lesions demonstrated pathergy. Although this has been reported in Sweet's syndrome,l, 2 it is far more common in pyoderma gangrenosum, particularly in children. 3 In addition, the severe, fatal granulomatous arteritis and aortitis in our patient has not been reported with Sweet's syndrome. However, there are several case reports of pyoderma gangrenosum associated with Takayasu's arteritis. 4 Therefore, although some features of the initial presentation of our case may also have been consistent with Sweet's syndrome, the profound pathergy and severe, large-vessel granulomatous vasculitis make the diagnosis of pyoderma gangrenosum associated with Takayasu's arteritis more likely.
Alan T. Glass, BA Joseph W. Gaffney, MD Sandy S. Milgraum, MD University ofMedicine and Dentistry of New Jersey New Brunswick, New Jersey
275
