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Hairy cell leukemia and pyoderma gangrenosum
300
Journal of the American Academy of Dermatology
Correspondence
The portal of entry of Pseudomonas septicemia is through the skin due to loss of normal mechanical function (as seen in bums, surgical wounds, and decubitus ulcers), as well as via the gastrointestinal or urinary tracts with or without surgical manipulations. The anatomic distribution of our ecthyma gangrenosum patients (anogenital and extremities) does not correspond to the usual distribution of hot tub folliculitis, namely, trunk, hips, and buttocks, which is aggravated by bathing suit-induced occlusion and maceration that enhances colonization and penetration. In true ecthyma gangrenosum, topical antiseptics likely provide only adjunctive therapeutic support in these patients' management since systemic therapy probably makes the greatest difference in controlling local and systemic P. aeruginosa infections. In considering a therapeutic approach to hot tub folliculitis, there is uncertainty whether adequate penetration to both deep follicular and nonfollicular abscesses can occur with topical antiseptic agents. 4 It is interesting that the lesions noted in the patients of EI Baze et al were serotyped in seven of fourteen cases. Their serotypes were similar to patients reported with hot tub folliculitis. Seven of fourteen positive similarities is not statistically significant. However, I doubt if it is really known yet whether other P. aeruginosa infections of the skin have prevalent serotypes as does hot tub folliculitis. Obviously, this is an area of needed research. In conclusion, I would not refer to the lesion described by El Baze et al as a localized form of ecthyma gangrenosum. Since they emphasize the similarities to hot tub folliculitis, perhaps they should consider a designation such as "advanced hot tub folliculitis" or "ecthyma gangrenosum-like eruptions."
Steven L. Greene, M.D. Group Health Cooperative of Puget Sound Olive Way Medical Center 509 Olive Way, Ste 555, Seattle, Wa 98101
REFERENCES 1. Greene SL, Su WPD, Muller SA: Ecthyma gangrenosum:
Report of clinical, histopathologic, and bacteriologic aspects of eight cases. J AM ACAD DERMATOL 11:781-787, 1984.
2. Fitzpatrick TB, Eisen AZ, Wolff K, et al: Gram-negative coccal and bacillary infections, in Dermatology in general medicine, ed. 2. New York, 1979, McGraw-Hill Book Co., pp. 1445-1459. 3. Rook A, WilkinsonDS, EblingFJG: Cutaneous vasculitis, in Textbook of dermatology, ed. 2. Oxford, 1979, Blackwell Scientific Publications, p. 1019.
4. Greene SL, Su WPD, Muller SA: Pseudomonas aeruginosa infections of the skin. Am Fam Physician 29: 193200, 1984.
Hairy cell leukemia and pyoderma gangrenosum To the Editor: We read with interest the recent article by Finan and colleagues (J AM ACAD DERMATOL 11: 788-797, 1984) describing the cutaneous findings in hairy cell leukemia, but unfortunately no mention of pyoderma gangrenosum in association with hairy cell leukemia was made in the discussion. Crow and Bowers I reported a patient with hairy cell leukemia who developed bullous hemorrhagic ulceration, a variant of pyoderma gangrenosum, and we report a second patient with hairy cell leukemia who developed pyoderma gangrenosum at the site of a percutaneous liver biopsy. Case report. A 62-year-old housewife with a 30year history of seropositive rheumatoid arthritis developed symptoms of anemia when her disease was in remission and 2 years after stopping penicillamine. Examination revealed a nontender splenomegaly 8 cm below the costal margin. Investigations showed a hemoglobin of 7.4 gm/dl and a peripheral leukocyte count of 5.3 X 10911iter with 31 % atypical vacuolated lymphocytes. Felty'S syndrome was excluded by a normal red blood cell survival time, and red cell mass and bone marrow aspiration confirmed the diagnosis of hairy cell leukemia. Five years later, an uncomplicated liver biopsy was done because liver function tests showed a persistently abnormal obstructive pattern with a hepatic alkaline phosphatase level of 1,155 IUlliter (normal, less than 70 IV/liter) and normal ultrasound and isotope scans. Histologic examination showed a normal hepatic architecture with leukemic infiltration of the sinusoids. One week later the patient complained of severe pain at the biopsy site, and then a tender, ulcerating area with an advancing bluish necrotic margin developed (Fig. 1). The ulcer reached a maximum size of 7 .5 x 8.5 cm, and repeated aerobic and anaerobic cultures of swabs and biopsy material were sterile. A clinical diagnosis of pyoderma gangrenosum was made and biopsy showed a pyoderma-like process associated with many atypical mononuclear cells in and around the blood vessels. Prednisolone, 80 mg/day, produced pain relief within 48 hours, and the ulcer healed in 3 weeks. Discussion. Pyoderma gangrenosum has been described in association with many myeloproliferative
ume 13 nber 2, Part 1 ~ust, 1985
Correspondence
301
Reply
:. 1. Pyoderma gangrenosum over the lateral aspect the right chest wall.
orders 1 •3 and trauma is often a precipitating factor. 4 •5 ~ believe that the trauma to the skin produced by the er biopsy led to the pyoderma gangrenosum in a ;ceptible individual. Although this patient may have ~n predisposed to pyoderma gangrenosum by both r hematologic and joint disease, we consider that the mer was more likely since her Rose-Waaler test was sitive at a titer of only 1/32 and she had required no ~dication for her joint disease during the preceding 2 ars. We would be interested in knowing whether other rmatologists have experienced the association of iry cell leukemia with pyoderma gangrenosum and : recommend that invasive procedures in patients with iry cell leukemia be kept to a minimum. M. H. A. Rustin, M.R.C.P. Department of Dermatology University College Hospital Gower St., London WC 1 6AU R. C. D. Staughton, M.R.C.P. E. N. Coomes, MD., F.R.C.P. St. Stephen's Hospital Fulham Rd., Chelsea, London SWlO 9TH <:FERENCES Crow KK, Bowers RE: Bullous haemorrhagic ulceration, a variant of pyoderma gangrenosum. Trans St Johns Hasp Dermatol Soc 60:142-151, 1974. Newell LM, Malkinson FD: Commentary: Pyoderma gangrenosum. Arch DermatoI1l8:769-773, 1982. Perry RO, Winkelmann RK: Bullous pyoderma gangrenosum and leukaemia. Arch Dermato1106:901-905, 1972. Mahood JM, Sneddon 18: Pyoderma gangrenosum complicating non-Hodgkin's lymphoma. Bf J Derrnatol 102: 223-225, 1980. Finkel sr, Janowitz HD: Trauma and the pyoderma gangrenosum of inflammatory bowel disease. Gut 22:410412, 1981.
To the Editor: We appreciate the letter by Drs. Rustin, Staughton, and Coomes and are glad that they add pyoderma gangrenosum to the list of cutaneous associations with hairy cell leukemia. It has been well recognized that pyoderma gangrenosum may be associated with leukemia or lymphoma. However, in our 113 cases of hairy cell leukemia, pyoderma gangrenosum was not found and, thus, not included in our table. We would like to comment on two of their statements in the "Discussion" section of the letter. First, they suggest that because the rheumatoid arthritis in their patient was inactive at the time of onset of pyoderma gangrenosum, the hairy cell leukemia was "more likel y" to have predisposed the patient to the pyoderma gangrenosum. This may be true-but one should realize that several authors have emphasized that the underlying disease found in association with pyoderma gangrenosum, be it rheumatoid arthritis, inflammatory bowel disease/· 3 or others, need not, and often is not, active coincident with flares in the skin disease. Two patients described by Holt et aP with classic seropositive rheumatoid arthritis and pyoderma gangrenosum had intermittent exacerbations of skin disease, unrelated to the activity or exacerbations of the joint disease. Edwards and Truelove 2 reported cases of pyoderma gangrenosum that persisted for years, with absent or minimal symptoms of colitis. Similarly, Johnson and Wilson 3 stressed this point in their series of seven patients with colitis and pyoderma gangrenosum. Thus, while the coincidence of rheumatoid arthritis, hairy cell leukemia, and pyoderma gangrenosum is quite interesting, and an immunologic link would appe<\r likely, it is better not to assume that the activities of the diseases need to paralIel each other to be of pathogenetic significance. Second, they conclude by recommending that "invasive procedures in patients with hairy cell leukemia be kept to a minimum." It is true that pyoderma gangrenosum patients should avoid invasive procedures, but this may not be true for hairy cell leukemia patients. For a large number of patients with hairy cell leukemia, splenectomy, while an invasive procedure, is the only major therapeutic intervention and has led to prolonged survival. In addition, invasive diagnostic procedures to establish the specific etiology of opportunistic infections to which these patients are particularly susceptible may be lifesaving and have been highly recommended. 4 Pyoderma gangrenosum in patients with hairy cell leukemia appears to be quite rare, as evidenced by the fact that it was absent in all 113 patients in our review, 5
Journal of the American Academy of Dermatology
Correspondence
The portal of entry of Pseudomonas septicemia is through the skin due to loss of normal mechanical function (as seen in bums, surgical wounds, and decubitus ulcers), as well as via the gastrointestinal or urinary tracts with or without surgical manipulations. The anatomic distribution of our ecthyma gangrenosum patients (anogenital and extremities) does not correspond to the usual distribution of hot tub folliculitis, namely, trunk, hips, and buttocks, which is aggravated by bathing suit-induced occlusion and maceration that enhances colonization and penetration. In true ecthyma gangrenosum, topical antiseptics likely provide only adjunctive therapeutic support in these patients' management since systemic therapy probably makes the greatest difference in controlling local and systemic P. aeruginosa infections. In considering a therapeutic approach to hot tub folliculitis, there is uncertainty whether adequate penetration to both deep follicular and nonfollicular abscesses can occur with topical antiseptic agents. 4 It is interesting that the lesions noted in the patients of EI Baze et al were serotyped in seven of fourteen cases. Their serotypes were similar to patients reported with hot tub folliculitis. Seven of fourteen positive similarities is not statistically significant. However, I doubt if it is really known yet whether other P. aeruginosa infections of the skin have prevalent serotypes as does hot tub folliculitis. Obviously, this is an area of needed research. In conclusion, I would not refer to the lesion described by El Baze et al as a localized form of ecthyma gangrenosum. Since they emphasize the similarities to hot tub folliculitis, perhaps they should consider a designation such as "advanced hot tub folliculitis" or "ecthyma gangrenosum-like eruptions."
Steven L. Greene, M.D. Group Health Cooperative of Puget Sound Olive Way Medical Center 509 Olive Way, Ste 555, Seattle, Wa 98101
REFERENCES 1. Greene SL, Su WPD, Muller SA: Ecthyma gangrenosum:
Report of clinical, histopathologic, and bacteriologic aspects of eight cases. J AM ACAD DERMATOL 11:781-787, 1984.
2. Fitzpatrick TB, Eisen AZ, Wolff K, et al: Gram-negative coccal and bacillary infections, in Dermatology in general medicine, ed. 2. New York, 1979, McGraw-Hill Book Co., pp. 1445-1459. 3. Rook A, WilkinsonDS, EblingFJG: Cutaneous vasculitis, in Textbook of dermatology, ed. 2. Oxford, 1979, Blackwell Scientific Publications, p. 1019.
4. Greene SL, Su WPD, Muller SA: Pseudomonas aeruginosa infections of the skin. Am Fam Physician 29: 193200, 1984.
Hairy cell leukemia and pyoderma gangrenosum To the Editor: We read with interest the recent article by Finan and colleagues (J AM ACAD DERMATOL 11: 788-797, 1984) describing the cutaneous findings in hairy cell leukemia, but unfortunately no mention of pyoderma gangrenosum in association with hairy cell leukemia was made in the discussion. Crow and Bowers I reported a patient with hairy cell leukemia who developed bullous hemorrhagic ulceration, a variant of pyoderma gangrenosum, and we report a second patient with hairy cell leukemia who developed pyoderma gangrenosum at the site of a percutaneous liver biopsy. Case report. A 62-year-old housewife with a 30year history of seropositive rheumatoid arthritis developed symptoms of anemia when her disease was in remission and 2 years after stopping penicillamine. Examination revealed a nontender splenomegaly 8 cm below the costal margin. Investigations showed a hemoglobin of 7.4 gm/dl and a peripheral leukocyte count of 5.3 X 10911iter with 31 % atypical vacuolated lymphocytes. Felty'S syndrome was excluded by a normal red blood cell survival time, and red cell mass and bone marrow aspiration confirmed the diagnosis of hairy cell leukemia. Five years later, an uncomplicated liver biopsy was done because liver function tests showed a persistently abnormal obstructive pattern with a hepatic alkaline phosphatase level of 1,155 IUlliter (normal, less than 70 IV/liter) and normal ultrasound and isotope scans. Histologic examination showed a normal hepatic architecture with leukemic infiltration of the sinusoids. One week later the patient complained of severe pain at the biopsy site, and then a tender, ulcerating area with an advancing bluish necrotic margin developed (Fig. 1). The ulcer reached a maximum size of 7 .5 x 8.5 cm, and repeated aerobic and anaerobic cultures of swabs and biopsy material were sterile. A clinical diagnosis of pyoderma gangrenosum was made and biopsy showed a pyoderma-like process associated with many atypical mononuclear cells in and around the blood vessels. Prednisolone, 80 mg/day, produced pain relief within 48 hours, and the ulcer healed in 3 weeks. Discussion. Pyoderma gangrenosum has been described in association with many myeloproliferative
ume 13 nber 2, Part 1 ~ust, 1985
Correspondence
301
Reply
:. 1. Pyoderma gangrenosum over the lateral aspect the right chest wall.
orders 1 •3 and trauma is often a precipitating factor. 4 •5 ~ believe that the trauma to the skin produced by the er biopsy led to the pyoderma gangrenosum in a ;ceptible individual. Although this patient may have ~n predisposed to pyoderma gangrenosum by both r hematologic and joint disease, we consider that the mer was more likely since her Rose-Waaler test was sitive at a titer of only 1/32 and she had required no ~dication for her joint disease during the preceding 2 ars. We would be interested in knowing whether other rmatologists have experienced the association of iry cell leukemia with pyoderma gangrenosum and : recommend that invasive procedures in patients with iry cell leukemia be kept to a minimum. M. H. A. Rustin, M.R.C.P. Department of Dermatology University College Hospital Gower St., London WC 1 6AU R. C. D. Staughton, M.R.C.P. E. N. Coomes, MD., F.R.C.P. St. Stephen's Hospital Fulham Rd., Chelsea, London SWlO 9TH <:FERENCES Crow KK, Bowers RE: Bullous haemorrhagic ulceration, a variant of pyoderma gangrenosum. Trans St Johns Hasp Dermatol Soc 60:142-151, 1974. Newell LM, Malkinson FD: Commentary: Pyoderma gangrenosum. Arch DermatoI1l8:769-773, 1982. Perry RO, Winkelmann RK: Bullous pyoderma gangrenosum and leukaemia. Arch Dermato1106:901-905, 1972. Mahood JM, Sneddon 18: Pyoderma gangrenosum complicating non-Hodgkin's lymphoma. Bf J Derrnatol 102: 223-225, 1980. Finkel sr, Janowitz HD: Trauma and the pyoderma gangrenosum of inflammatory bowel disease. Gut 22:410412, 1981.
To the Editor: We appreciate the letter by Drs. Rustin, Staughton, and Coomes and are glad that they add pyoderma gangrenosum to the list of cutaneous associations with hairy cell leukemia. It has been well recognized that pyoderma gangrenosum may be associated with leukemia or lymphoma. However, in our 113 cases of hairy cell leukemia, pyoderma gangrenosum was not found and, thus, not included in our table. We would like to comment on two of their statements in the "Discussion" section of the letter. First, they suggest that because the rheumatoid arthritis in their patient was inactive at the time of onset of pyoderma gangrenosum, the hairy cell leukemia was "more likel y" to have predisposed the patient to the pyoderma gangrenosum. This may be true-but one should realize that several authors have emphasized that the underlying disease found in association with pyoderma gangrenosum, be it rheumatoid arthritis, inflammatory bowel disease/· 3 or others, need not, and often is not, active coincident with flares in the skin disease. Two patients described by Holt et aP with classic seropositive rheumatoid arthritis and pyoderma gangrenosum had intermittent exacerbations of skin disease, unrelated to the activity or exacerbations of the joint disease. Edwards and Truelove 2 reported cases of pyoderma gangrenosum that persisted for years, with absent or minimal symptoms of colitis. Similarly, Johnson and Wilson 3 stressed this point in their series of seven patients with colitis and pyoderma gangrenosum. Thus, while the coincidence of rheumatoid arthritis, hairy cell leukemia, and pyoderma gangrenosum is quite interesting, and an immunologic link would appe<\r likely, it is better not to assume that the activities of the diseases need to paralIel each other to be of pathogenetic significance. Second, they conclude by recommending that "invasive procedures in patients with hairy cell leukemia be kept to a minimum." It is true that pyoderma gangrenosum patients should avoid invasive procedures, but this may not be true for hairy cell leukemia patients. For a large number of patients with hairy cell leukemia, splenectomy, while an invasive procedure, is the only major therapeutic intervention and has led to prolonged survival. In addition, invasive diagnostic procedures to establish the specific etiology of opportunistic infections to which these patients are particularly susceptible may be lifesaving and have been highly recommended. 4 Pyoderma gangrenosum in patients with hairy cell leukemia appears to be quite rare, as evidenced by the fact that it was absent in all 113 patients in our review, 5