Acta Clinica Belgica International Journal of Clinical and Laboratory Medicine
ISSN: 1784-3286 (Print) 2295-3337 (Online) Journal homepage: https://www.tandfonline.com/loi/yacb20
Pyoderma Gangrenosum in a Patient with Chronic Myeloid Leukemia F. De Keyser, G.E.G. Verhoef, J. Dewyspelaere & M.A. Boogaerts To cite this article: F. De Keyser, G.E.G. Verhoef, J. Dewyspelaere & M.A. Boogaerts (1990) Pyoderma Gangrenosum in a Patient with Chronic Myeloid Leukemia, Acta Clinica Belgica, 45:5, 340-342, DOI: 10.1080/17843286.1990.11718108 To link to this article: https://doi.org/10.1080/17843286.1990.11718108
Published online: 16 May 2016.
Submit your article to this journal
Citing articles: 3 View citing articles
Full Terms & Conditions of access and use can be found at https://www.tandfonline.com/action/journalInformation?journalCode=yacb20
340
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PYODERMA GANGRENOSUM IN A PATIENT WITH CHRONIC MYELOID LEUKEMIA
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F. De Keyser, G.E.G. Verhoef, J. Dewyspelaere, M.A. Boogaerts*
SUMMARY We report a case of pyodenna gangrenosum in association with chronic myeloid leukemia. The clinical and pathological features are reviewed and the relationship with hematological disorders is discussed. Acta Clin Belg. 45, 5: 340-2
INTRODUCTION Pyoderma Gangrenosum is an uncommon ulcerative skin di sease with distinctive clinical characteristics and a frequent association with systemic diseases, e.g. gastro-intestinal diseases with autoimmune aspects. It has also been associated with haematological disorders such as monoclonal gammopathies, myeloproliferative disorders and myelodysplastic syndromes ( 1-5).
(CML) in 1984. She was initially tr~ated with busulphan and remained well for a number of years.By 1989 increasing resistance to busulphaJl indicated a more accelerated phase of the disease· Three weeks before admission, she developed fever simultaneously with painful erythematot!S plaques at the right comer of the mouth and the right shoulder. The fever spontaneouslY disappeared and the skin lesions resolved. one month later, the patient presented again with fever (39°C) and a painful indurated erythematolls plaque on the abdomen centrated round an old sqtr of appendectomy. Formation of bullae occurred centrally with eventually ulceration and raised erythematous and undermined bordefS· The ulcer reached a maximum size of 8x8 cfll· (Fig. I)
We describe the occurrence of pyoderma gangrenosum in a patient with chronic myeloid leukemia. The characteristic'clinical features and natural history of this skin disease are illustrated.
CASE REPORT A 65-year-old woman presented with classical Phil adelphia-positive chronic myeloid leukemia *Universitair Ziekenhuis Gasthuisberg, Afdeling Hematologie, Herestraat 49, 30Q0 Leuven. Reprints request : G.E.G. Verhoef. Acta Clinica Belgica 45.5 ( 1990)
.
.
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Fig . 1 : A large ulcer of pyoderma gangrenos . /I consisting of central necrosis and ulceration wit ragged undermining edges.
PYODERMA GANGRENOSUM JN A PATIENT WITH CHRONIC MYELOID LEUKEMIA
Laboratory investigations revealed: ESR 150 lllmfhr, haemoglobin 8.6 g/dl, white blood cell I count 9.9 x 109/l with a differential of 10% metatnyelocytes, 8% myelocytes, 20% stab forms, 373 neutrophils, 20% lymphocytes and 5% 1 lllonocytes, and a platelet count of 174 x 109/l. Results of assays for circulating immune comPlexes, antinuclear antibodies and cryoglobulins Were negative. Repeated cultures of blood and 1 swabs of the ulcer were sterile.
. Skin biopsy showed a dense dermal tnf1ammatory infiltration, almost exclusively tb build up by neutrophils spreading into the of epidermis with formation of sub- and intraepidermal bullae (Fig.2). The diagnosis of e. PYoderma gangrenosum was made.
:d
341
The patient was initially treated with broad spectrum antibiotics without improvement of fever and skin lesions. As the diagnosis of pyoderma gangrenosum became clear, antibiotics were stopped. Treatment with oral prednisone (40 mg/day) was followed by a characteristic rapid response, i.e. pain relief within 48 hours with rapid resolution of fever and ulcer healing within 3 weeks. There was no recurrence of the skin lesions and the dose of prednisone was progressively reduced. The patient remained reasonably well.
DISCUSSION Pyoderma gangrenosum is associated with various diseases including solid tumors, autoimmune diseases and bowel disorders. It may also precede, concur, or follow detection of haematological diseases such as idiopathic myelofibrosis, hairy cell leukemia, myelodysplastic syndromes and myeloproliferative diseases. Pyodermagangrenosum is essentially a clinical diagnosis. It is often not recognized and several diagnostic difficulties cause the diagnosis to be delayed. The problems arise from the clinical context in which the lesions develop. Most skin lesions in leukemic patients are either haemorrhagic or infectious, and our case was initially treated as such. Pyoderma gangrenosum usually starts as a papule or vesicle which, during a period of several days becomes pustular and breaks down, leaving a necrotic ulcer with a spreading erythematous border. The edge of the lesion is ragged and undermined, producing an overhanging ledge of tissue with a blue-red color (6). Although it can occur at any site, there is a clear predilection for the lower limbs or, as in our patient, around an old scar. The histopathology of pyoderma gangrenosum is not specific, but may help the physician to rule out other di sorders that may clinically appear Acta Clinica Belgica 45.5 ( 1990)
PYODERMA GANGRENOSUM JN A PATJEN1 WITH CHRONIC MYELOID LEUKEMIA
342
as a cutaneous ulceration. The site from which the biopsy is taken is critical to the histopathologic findings that may be observed (I). In the peripheral zone of erythema the predominant pattern is .that of a lymphocytic infiltrate, endothelial swelling, and fibrinoid necrosis of the vessel walls. Centrally; the findings are those of polymorphonuclear leucocytes and necrosis of the epidermis and papillary dermis. The presence or absence of vasculitis is a point of considerable confusion (7).
SAMENV ATIING Wij beschrijven een patiente met pyoderrl1 3 gangrenosum waarbij de aandacht wordt gevestigd op de associatie met hematologische aandoeningen· Tevens beschrijven wij de klinische kenmerken en pathologische bevindingen.
RESUME Nous rapportons le cas d'une patiente presentant un pyoderma gangrenosum dans le cadre d'un syndrorlle myeloproliferatif, en detaillant l'histoire naturelle et les aspects cliniques propres.
In the differential diagnosis of pyoderma gangrenosum, one should consider disseminated infections and entities such as blastomycosis, streptococcal gangrene, necrotizing vasculitis, and drug related ulcers (eg. bromoderma, warfarin necrosi s). Sweet's syndrome (acute febrile neutrophilic dermato s is) and pyoderma gangrenosum might represent, apparently, the ultimate ends of a common basic pathological reactivity of the skin and, as a matter of fact, have been observed simultaneously in some patients
We wish to thank ProfessorC. De Wolf-Peeters for her helpful comments on the histological studies.
(8).
1. Powell FC, Schroeter AL, Su, WPD, Perry
The pathogenesis of pyoderma gangrenosum is unknown . However, an underlying aberration in the immune system has been suggested by the assessment of a deficient granulocytic function in many patients with this sk in disease (7). Corticosteroids remain the cornerstone of the treatment of pyoderma gangrenosum, although alternative agents have been proposed. The beneficial response with corticosteroids is even considered as an essential diagnostic criterium by many clinicians. Topical treatments are usually ineffective. Recognition of the association of pyoderma gangrenosum and hematological diseases is important to ensure early diagnosis and appropriate th era py . Furthermore, the development of pyoderma gangrenosum in patients without a recognized di sease indicates the need for careful follow-up to excl~de an under) ying hematological di sease. Acta Clinica Belgica 45.5 ( 1990)
Si
ACKNOWLEDGEMENTS
REFER ENCES
2.
3.
4.
5.
6.
J-10·
Pyoderma gangrenosum: A review of 86 patients· Quart J Med. 1985, 55: 173-86. Hay CRM, Messenger AG, Cotton DWK. et n1· Atypical bullou s pyoderma gangrenosu~ associated with myeloid malignancies. J C! 111 Pathol. 1987; 40: 387-92. Romano J, Safai B. Pyoderma gangrenosurn an~ myeloproliferative disorders: Report of a case an . review of the literature. Arch Intern Med.1979; 139· 932-4. ·1e Cooper PH, Innes DJ, Greer KE. Acute febfl d neutrophilic dermatosis (Sweet's syndrome) an . myeloproliferative disorders. Cancer. 1983; 5 1· 1518- 26. Cohen PR, Talpaz M, Kurz'rock R. Malignanc)'d associated Sweet's syndrome: review of the wort literature. J Clin Onco/. 1988; 6: 1887-97. 1 Hickman JG . Pyode rma gangrenosurn. Cli '
Derma to/; 1983; I: 102-13. . ·c~ 7. SchwaegerleSM,Bergfeld WF,SenitzerD,T1d!1 d RT. Pyodermagangrenosum: A review.I AmAC11 Dermatol. 1988; 18: 559-68. ·iic
8. Caughman W, Stem R, Haynes H. Neutroph 1 1 dermatosis of myeloproliferative disorders. 1 }.II Acad Dermatol. 1983; 9:751-8.
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ISSN: 1784-3286 (Print) 2295-3337 (Online) Journal homepage: https://www.tandfonline.com/loi/yacb20
Pyoderma Gangrenosum in a Patient with Chronic Myeloid Leukemia F. De Keyser, G.E.G. Verhoef, J. Dewyspelaere & M.A. Boogaerts To cite this article: F. De Keyser, G.E.G. Verhoef, J. Dewyspelaere & M.A. Boogaerts (1990) Pyoderma Gangrenosum in a Patient with Chronic Myeloid Leukemia, Acta Clinica Belgica, 45:5, 340-342, DOI: 10.1080/17843286.1990.11718108 To link to this article: https://doi.org/10.1080/17843286.1990.11718108
Published online: 16 May 2016.
Submit your article to this journal
Citing articles: 3 View citing articles
Full Terms & Conditions of access and use can be found at https://www.tandfonline.com/action/journalInformation?journalCode=yacb20
340
1~
n
I~
PYODERMA GANGRENOSUM IN A PATIENT WITH CHRONIC MYELOID LEUKEMIA
~
I:
F. De Keyser, G.E.G. Verhoef, J. Dewyspelaere, M.A. Boogaerts*
SUMMARY We report a case of pyodenna gangrenosum in association with chronic myeloid leukemia. The clinical and pathological features are reviewed and the relationship with hematological disorders is discussed. Acta Clin Belg. 45, 5: 340-2
INTRODUCTION Pyoderma Gangrenosum is an uncommon ulcerative skin di sease with distinctive clinical characteristics and a frequent association with systemic diseases, e.g. gastro-intestinal diseases with autoimmune aspects. It has also been associated with haematological disorders such as monoclonal gammopathies, myeloproliferative disorders and myelodysplastic syndromes ( 1-5).
(CML) in 1984. She was initially tr~ated with busulphan and remained well for a number of years.By 1989 increasing resistance to busulphaJl indicated a more accelerated phase of the disease· Three weeks before admission, she developed fever simultaneously with painful erythematot!S plaques at the right comer of the mouth and the right shoulder. The fever spontaneouslY disappeared and the skin lesions resolved. one month later, the patient presented again with fever (39°C) and a painful indurated erythematolls plaque on the abdomen centrated round an old sqtr of appendectomy. Formation of bullae occurred centrally with eventually ulceration and raised erythematous and undermined bordefS· The ulcer reached a maximum size of 8x8 cfll· (Fig. I)
We describe the occurrence of pyoderma gangrenosum in a patient with chronic myeloid leukemia. The characteristic'clinical features and natural history of this skin disease are illustrated.
CASE REPORT A 65-year-old woman presented with classical Phil adelphia-positive chronic myeloid leukemia *Universitair Ziekenhuis Gasthuisberg, Afdeling Hematologie, Herestraat 49, 30Q0 Leuven. Reprints request : G.E.G. Verhoef. Acta Clinica Belgica 45.5 ( 1990)
.
.
µf11
Fig . 1 : A large ulcer of pyoderma gangrenos . /I consisting of central necrosis and ulceration wit ragged undermining edges.
PYODERMA GANGRENOSUM JN A PATIENT WITH CHRONIC MYELOID LEUKEMIA
Laboratory investigations revealed: ESR 150 lllmfhr, haemoglobin 8.6 g/dl, white blood cell I count 9.9 x 109/l with a differential of 10% metatnyelocytes, 8% myelocytes, 20% stab forms, 373 neutrophils, 20% lymphocytes and 5% 1 lllonocytes, and a platelet count of 174 x 109/l. Results of assays for circulating immune comPlexes, antinuclear antibodies and cryoglobulins Were negative. Repeated cultures of blood and 1 swabs of the ulcer were sterile.
. Skin biopsy showed a dense dermal tnf1ammatory infiltration, almost exclusively tb build up by neutrophils spreading into the of epidermis with formation of sub- and intraepidermal bullae (Fig.2). The diagnosis of e. PYoderma gangrenosum was made.
:d
341
The patient was initially treated with broad spectrum antibiotics without improvement of fever and skin lesions. As the diagnosis of pyoderma gangrenosum became clear, antibiotics were stopped. Treatment with oral prednisone (40 mg/day) was followed by a characteristic rapid response, i.e. pain relief within 48 hours with rapid resolution of fever and ulcer healing within 3 weeks. There was no recurrence of the skin lesions and the dose of prednisone was progressively reduced. The patient remained reasonably well.
DISCUSSION Pyoderma gangrenosum is associated with various diseases including solid tumors, autoimmune diseases and bowel disorders. It may also precede, concur, or follow detection of haematological diseases such as idiopathic myelofibrosis, hairy cell leukemia, myelodysplastic syndromes and myeloproliferative diseases. Pyodermagangrenosum is essentially a clinical diagnosis. It is often not recognized and several diagnostic difficulties cause the diagnosis to be delayed. The problems arise from the clinical context in which the lesions develop. Most skin lesions in leukemic patients are either haemorrhagic or infectious, and our case was initially treated as such. Pyoderma gangrenosum usually starts as a papule or vesicle which, during a period of several days becomes pustular and breaks down, leaving a necrotic ulcer with a spreading erythematous border. The edge of the lesion is ragged and undermined, producing an overhanging ledge of tissue with a blue-red color (6). Although it can occur at any site, there is a clear predilection for the lower limbs or, as in our patient, around an old scar. The histopathology of pyoderma gangrenosum is not specific, but may help the physician to rule out other di sorders that may clinically appear Acta Clinica Belgica 45.5 ( 1990)
PYODERMA GANGRENOSUM JN A PATJEN1 WITH CHRONIC MYELOID LEUKEMIA
342
as a cutaneous ulceration. The site from which the biopsy is taken is critical to the histopathologic findings that may be observed (I). In the peripheral zone of erythema the predominant pattern is .that of a lymphocytic infiltrate, endothelial swelling, and fibrinoid necrosis of the vessel walls. Centrally; the findings are those of polymorphonuclear leucocytes and necrosis of the epidermis and papillary dermis. The presence or absence of vasculitis is a point of considerable confusion (7).
SAMENV ATIING Wij beschrijven een patiente met pyoderrl1 3 gangrenosum waarbij de aandacht wordt gevestigd op de associatie met hematologische aandoeningen· Tevens beschrijven wij de klinische kenmerken en pathologische bevindingen.
RESUME Nous rapportons le cas d'une patiente presentant un pyoderma gangrenosum dans le cadre d'un syndrorlle myeloproliferatif, en detaillant l'histoire naturelle et les aspects cliniques propres.
In the differential diagnosis of pyoderma gangrenosum, one should consider disseminated infections and entities such as blastomycosis, streptococcal gangrene, necrotizing vasculitis, and drug related ulcers (eg. bromoderma, warfarin necrosi s). Sweet's syndrome (acute febrile neutrophilic dermato s is) and pyoderma gangrenosum might represent, apparently, the ultimate ends of a common basic pathological reactivity of the skin and, as a matter of fact, have been observed simultaneously in some patients
We wish to thank ProfessorC. De Wolf-Peeters for her helpful comments on the histological studies.
(8).
1. Powell FC, Schroeter AL, Su, WPD, Perry
The pathogenesis of pyoderma gangrenosum is unknown . However, an underlying aberration in the immune system has been suggested by the assessment of a deficient granulocytic function in many patients with this sk in disease (7). Corticosteroids remain the cornerstone of the treatment of pyoderma gangrenosum, although alternative agents have been proposed. The beneficial response with corticosteroids is even considered as an essential diagnostic criterium by many clinicians. Topical treatments are usually ineffective. Recognition of the association of pyoderma gangrenosum and hematological diseases is important to ensure early diagnosis and appropriate th era py . Furthermore, the development of pyoderma gangrenosum in patients without a recognized di sease indicates the need for careful follow-up to excl~de an under) ying hematological di sease. Acta Clinica Belgica 45.5 ( 1990)
Si
ACKNOWLEDGEMENTS
REFER ENCES
2.
3.
4.
5.
6.
J-10·
Pyoderma gangrenosum: A review of 86 patients· Quart J Med. 1985, 55: 173-86. Hay CRM, Messenger AG, Cotton DWK. et n1· Atypical bullou s pyoderma gangrenosu~ associated with myeloid malignancies. J C! 111 Pathol. 1987; 40: 387-92. Romano J, Safai B. Pyoderma gangrenosurn an~ myeloproliferative disorders: Report of a case an . review of the literature. Arch Intern Med.1979; 139· 932-4. ·1e Cooper PH, Innes DJ, Greer KE. Acute febfl d neutrophilic dermatosis (Sweet's syndrome) an . myeloproliferative disorders. Cancer. 1983; 5 1· 1518- 26. Cohen PR, Talpaz M, Kurz'rock R. Malignanc)'d associated Sweet's syndrome: review of the wort literature. J Clin Onco/. 1988; 6: 1887-97. 1 Hickman JG . Pyode rma gangrenosurn. Cli '
Derma to/; 1983; I: 102-13. . ·c~ 7. SchwaegerleSM,Bergfeld WF,SenitzerD,T1d!1 d RT. Pyodermagangrenosum: A review.I AmAC11 Dermatol. 1988; 18: 559-68. ·iic
8. Caughman W, Stem R, Haynes H. Neutroph 1 1 dermatosis of myeloproliferative disorders. 1 }.II Acad Dermatol. 1983; 9:751-8.
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