CED
Clinical dermatology • Concise report
Clinical and Experimental Dermatology
Pyoderma gangrenosum and Wegener granulomatosis-like syndrome induced by cocaine nez-Gallo,1 C. Albarra n-Planelles,1 M. Linares-Barrios,1 C. Rodrıguez-Herna ndez,2 D. Jime A. Martınez-Rodrıguez,1 E. Garcıa-Moreno2 and R. Bravo-Monge3 Departments of 1Dermatology and 2Immunology, and 3Emergency Department, Puerta del Mar University Hospital, Cadiz, Spain doi:10.1111/ced.12207
Summary
Cocaine abuse is associated with various skin and rheumatological diseases that mimic primary autoimmune diseases, including retiform purpura with involvement of the ears, cocaine-induced midline destructive lesions (CIMDL), and eruptive pyoderma gangrenosum (PG). Previous reports have suggested the use of perinuclear antineutrophil cytoplasmic antibodies (pANCA) with specificity against human neutrophil elastase (HNE) to differentiate these cocaine-induced diseases from primary autoimmune diseases. We describe a case of a 54-year-old woman with a history of cocaine abuse, who had PG lesions on her legs with accompanying CIMDL and lung lesions similar to those seen in Wegener granulomatosis. Detection of HNE-positive pANCA, and improvement or clinical recurrence after cessation or consumption of cocaine, respectively, were key to differentiating this presentation from primary autoimmune disease.
Cocaine consumption has been related to a wide variety of clinical conditions, including cocaine-induced midline destructive lesions (CIMDL),1 cutaneous vasculitis induced by cocaine contaminated with levamisol,2 and eruptive pyoderma gangrenosum (PG).3 Some of these pathologies have been related to the presence of peri-nuclear antineutrophil cytoplasmic antibodies (pANCA) with specificity against human neutrophil elastase (HNE).4–6 We describe a case of autoimmunity due to cocaine, with cutaneous, pulmonary, and ear, nose and throat involvement. The primary key for the aetiological diagnosis was the detection of HNE-positive pANCA.
Report A 54-year-old woman presented with a 2-month history of multiple painful ulcers on both legs. During nez-Gallo, Department of Dermatology, Correspondence: Dr David Jime Puerta del Mar University Hospital, 21 Ana de Viya Avenue, Cadiz, 11009, Spain E-mail: [email protected] Conflict of interest: none declared. Accepted for publication 27 March 2013
878
Clinical and Experimental Dermatology (2013) 38, pp878–882
this period, she had been treated with topical and oral antibiotics with no improvement. Her general health was otherwise good, but she reported occasional episodes of dry cough and bloody sputum. She had been a user of cocaine by inhalation (approximately 3.5 g/week) for 5 years. On physical examination, multiple painful ulcers were seen on the patient’s legs. These were purplish in colour, had a cribriform morphology with a slightly raised edge, and varied between 10 and 50 mm in size (Fig. 1a). Each ulcer first developed as a pustule, which then developed into an ulcer within about 3 days (Fig. 1b). The patient also had a saddlenose nasal deformity, associated with extensive oronasal fistula in the palate (Fig. 1c), due to the cocaine abuse. On histological examination of a biopsy taken from the edge of a skin ulcer, a dense inflammatory infiltrate was seen in the dermis, with neutrophils and fibrin deposits in the vessels (Fig. 2). No granulomas or vasculitis were present. Direct immunofluorescence gave negative results, as did stains and cultures for bacteria, fungi and alcohol-resistant acid bacilli. Based on the clinical and histological findings, the patient was diagnosed as having PG, and further
ª 2013 British Association of Dermatologists
PG and WG-like syndrome induced by cocaine D. Jimenez-Gallo et al.
(a)
(b)
(c)
Figure 1 Clinical images. (a) Painful violet-edged ulcers on the
legs; (b) pustule with violet-coloured halo on the leg; (c) saddlenose deformity with palatal oronasal fistula.
investigations were performed. Chest radiography showed pulmonary infiltrates in the middle and lower lobes of the right lung, accompanied by an interstitial lung pattern (Fig. 3a). Computed tomography (CT) of
(a)
the chest identified bilateral pulmonary nodules and cavitation (Fig. 3b), and a facial CT scan showed extensive midline destruction associated with the oronasal fistula (Fig. 3c). Full blood count, coagulation and biochemistry tests were normal. Results for the following were negative or within normal limits: anti-Sm, anti-RNP, anti-Ro, anti-La, anti-Scl70, anticentromere, anti-b2-glycoprotein, anticardiolipin and antihistone antibodies, antidouble-stranded DNA, cryoglobulins, complement levels, rheumatoid factor, and urine sedimentation tests. Investigations for syphilis, hepatitis B and C, human immunodeficiency virus and tuberculosis (TB) were also negative. Circulating pANCA without specificity for myeloperoxidase (MPO) or proteinase (PR)3 was found, at a titre of 1 : 80 (Fig. 3d). Because of the patient’s history of cocaine abuse, a specific search was performed for atypical ANCAs that were positive for elastase. The patient was also positive for antinuclear antibodies, at a titre of 1 : 40. Given the pulmonary findings, we conducted fibrobronchoscopy, which did not indicate malignant disease, and tested for bacteria, fungi and acid-alcohol resistant bacilli. Histology of a lung biopsy showed nonspecific interstitial pulmonary disease. The final diagnosis was PG and Wegener granulomatosis (WG)-like syndrome induced by cocaine, based on destruction of the midline of the nose and palate,
(b)
Figure 2 (a) Ulceration and deep and dense inflammatory infiltrate; (b) neutrophils in the inflammatory infiltrate associated with fibrin
deposits in the vessels. Haematoxylin and eosin, original magnification (a) 940 (b) 9200.
ª 2013 British Association of Dermatologists
Clinical and Experimental Dermatology (2013) 38, pp878–882
879
PG and WG-like syndrome induced by cocaine D. Jimenez-Gallo et al.
(a)
(b)
(c)
(d) Figure 3 (a) Radiograph showing the
presence of pulmonary infiltrates in the middle and lower lobes of the right lung. (b,c) Computed tomograph scan scan showing (b) pulmonary infiltrates in the right lung accompanied by a cavitated nodule at the lingula of the left lung, and (c) extensive destruction of the midline of the upper respiratory tract associated with an oronasal fistula. (d) Granulocyte immunofluorescence fixed in ethanol showing a perinuclear pattern of antinuclear cytoplasmic antibodies to elastase (original magnification 9600).
accompanied by the fixed pulmonary infiltrates and bilateral cavitary pulmonary nodules. Cocaine was further implicated as the cause of the illness because of the patient’s clinical improvement or relapse after cessation or consumption, respectively, along with an increase in pANCA titres to 1 : 160 after cocaine abuse and the detection of HNE-specific ANCAs. Since the initial presentation, our patient has had further and increasingly severe episodes of PG, associated with resumption of cocaine abuse. The most recent flare was accompanied by significant retiform purpura and involvement of the legs and ears. Currently the disease is stable with bolus administration of cyclophosphamide, despite continued cocaine consumption, verified by drug-testing of the patient’s urine. An increase in cocaine abuse worldwide is leading to a rise in associated pathology.7 Inhalation of cocaine can result in nasal inflammation and necrosis because of its vasoconstrictor effect, microtraumas caused by the cocaine itself and accompanying substances, and an increased incidence of infections caused by Staphylococcus aureus.1,4 These factors are known to cause CIMDL. The main problem arises in differentiating CIMDL from primary WG.
880
Clinical and Experimental Dermatology (2013) 38, pp878–882
For this purpose, Trimarchi et al.5 evaluated clinical, radiological, histopathological and serological findings in patients with a diagnosis of WG, and compared them with cases of CIMDL. The degree of nasal and paranasal destruction was more severe in patients with CIMDL; however, lung and kidney involvement and destruction of the ears and orbit were more common in WG. Compared with CIMDL, WG was associated with a higher frequency of fever, malaise, arthralgias and myalgias, and also of laboratory abnormalities such as increased incidence of acutephase reactants, microhaematuria, proteinuria and renal damage. Radiologically, nasal-septum perforation and involvement of other nasal structures was more frequent in patients with CIMDL. Histologically, the presence of nonspecific changes in the biopsies was also greater in CIMDL. Biopsies from patients with WG showed findings such as microabscesses, granulomas or multinucleated giant cells at the extravascular level, with deep and localized necrosis. An important finding to aid in the differential diagnosis of WG and CIMDL is the presence of ANCAs. The antigens of classic ANCA-associated vasculitis are PR3 and MPO. The presence of PR3 supports the diagnosis of
ª 2013 British Association of Dermatologists
PG and WG-like syndrome induced by cocaine D. Jimenez-Gallo et al.
Table 1 Features of documented cases of pyoderma gangrenosum (PG) associated with cocaine use.
Source Roche et al., 20083
Present case
Gender/age, years
Cocaine consumption, years
Predominant location of PG
ANCA
Effective treatment Infliximab 5 mg/kg at weeks 0, 2, and 6 followed by infusion every 8 weeks, accompanied by methotrexate 15 mg/week Infliximab 5 mg/kg at weeks 0, 2, and 6 followed by infusion every 8 weeks, accompanied by topical tacrolimus. Cyclophosphamide bolus of 15 mg/kg every 2 weeks (3 pulses) followed by 15 mg/kg every 3 weeks (6 pulses)
M/30
2
Back
Negative
M/38
10
Back
Negative
F/54
5
Legs
HNE ANCA positive
Accompanying symptoms None
None
Destruction of midline, presence of retiform purpura and lung involvement
ANCA, antineutrophil cytoplasmic antibodies; HNE, human neutrophil elastase.
WG, while MPO is associated with Churg–Strauss granulomatosis and microscopic polyangiitis. pANCA is almost always associated with MPO, and cytoplasmic ANCA is typically associated with PR3.8 However, there are other atypical ANCAs that are also normally associated with a perinuclear pattern (i.e. pANCA), for which are not performed in routine laboratory immunology. Their investigation requires specific suspicion by the clinician. These atypical ANCAs include HNE, lactoferrin, azuricidin, catalase K, bactericidal/permeability-increasing (BPI) protein, cathepsin G, defensin and lysozyme.1,5,6 HNE is an atypical ANCA that has been structurally and functionally related with PR3 ANCA, and for this reason could favour the simulation of WG in our patient. HNE ANCA was recently described as a marker of CIMDL; however, it is rarely detected in patients with WG.4–6 The differential diagnosis for midline destructive lesions includes, among others, CIMDL, WG, extranodal lymphoma, squamous cell carcinoma, midline malignant reticulosis, TB, syphilis, leishmaniasis, blastomycosis and actinomycosis.1,9 Another interesting detail of this case is the relationship between cocaine abuse and development of PG. The two cases described by Roche et al.3 in 2008 are the only similar cases published to date (Table 1). In conclusion, we report a case in which the clinical features were triggered by cocaine abuse accompanied by a raised titre of HNE ANCA. The similarity of these autoantibodies to PR3 ANCA, together with the toxic effects of cocaine, could explain the destruction of the midline and lung lesions simulating WG. The lesions of PG, a neutrophilic dermatosis, could also be related to the presence of these antibodies. To our knowledge, this
ª 2013 British Association of Dermatologists
is only the third case of PG reported, and the first to include pulmonary involvement mimicking cocaineinduced WG, illustrating the various clinical features that produce autoimmunity related to this drug. The raised titre of elastase ANCAs seemed to be related to the cocaine abuse and worsening of the PG in this case. This case highlights the importance of HNE ANCA for diagnosis, and the incorporation of cocaine abuse in the aetiology of PG.
Learning points ● Retiform purpura, destruction of the midline,
and PG are syndromes associated with cocaine consumption. ● pANCA with specificity against HNE is a marker of dermatological and rheumatic diseases induced by cocaine. ● Clinicoimmunological correlation allows differentiation of WG from the clinical syndrome induced by cocaine. ● Cocaine should be considered in the list of causes of PG. ● Cessation of cocaine abuse is the most effective treatment, but cyclophosphamide may be useful if the patient continues to use the drug.
References 1 Stahelin L, Fialho SC, Neves FS et al. Cocaine-induced midline destruction lesions with positive ANCA test mimicking Wegener’s granulomatosis. Rev Bras Reumatol 2012; 52: 434–7.
Clinical and Experimental Dermatology (2013) 38, pp878–882
881
PG and WG-like syndrome induced by cocaine D. Jimenez-Gallo et al.
2 Ullrich K, Koval R, Koval E et al. Five consecutive cases of a cutaneous vasculopathy in users of levamisole-adulterated cocaine. J Clin Rheumatol 2011; 17: 193–6. 3 Roche E, Martınez-Mench on T, S anchez-Carazo JL et al. Two cases of eruptive pyoderma gangrenosum associated with cocaine use. Actas Dermosifiliogr 2008; 99: 727– 30. 4 Rachapalli SM, Kiely PD. Cocaine-induced midline destructive lesions mimicking ENT-limited Wegener’s granulomatosis. Scand J Rheumatol 2008; 37: 477– 80. 5 Trimarchi M, Gregorini G, Facchetti F et al. Cocaine-induced midline destructive lesions: clinical, radiographic, histopathologic, and serologic features and their differentiation from Wegener granulomatosis. Medicine (Baltimore) 2001; 80: 391–404.
882
Clinical and Experimental Dermatology (2013) 38, pp878–882
6 Wiesner O, Russell KA, Lee AS et al. Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaine-induced midline destructive lesions but not autoimmune vasculitis. Arthritis Rheum 2004; 50: 2954–65. 7 Salas-Espındola Y, Peniche-Castellanos A, L opez-Gehrke I, Mercadillo-Perez P. Leukocytoclastic vasculitis related to cocaine use. Actas Dermosifiliogr 2011; 102: 825–7. 8 Walsh NM, Green PJ, Burlingame RW et al. Cocaine-related retiform purpura: evidence to incriminate the adulterant, levamisole. J Cutan Pathol 2010; 37: 1212–19. 9 Daggett RB, Haghighi P, Terkeltaub RA. Nasal cocaine abuse causing an aggressive midline intranasal and pharyngeal destructive process mimicking midline reticulosis and limited Wegener’s granulomatosis. J Rheumatol 1990; 17: 838–40.
ª 2013 British Association of Dermatologists
Clinical dermatology • Concise report
Clinical and Experimental Dermatology
Pyoderma gangrenosum and Wegener granulomatosis-like syndrome induced by cocaine nez-Gallo,1 C. Albarra n-Planelles,1 M. Linares-Barrios,1 C. Rodrıguez-Herna ndez,2 D. Jime A. Martınez-Rodrıguez,1 E. Garcıa-Moreno2 and R. Bravo-Monge3 Departments of 1Dermatology and 2Immunology, and 3Emergency Department, Puerta del Mar University Hospital, Cadiz, Spain doi:10.1111/ced.12207
Summary
Cocaine abuse is associated with various skin and rheumatological diseases that mimic primary autoimmune diseases, including retiform purpura with involvement of the ears, cocaine-induced midline destructive lesions (CIMDL), and eruptive pyoderma gangrenosum (PG). Previous reports have suggested the use of perinuclear antineutrophil cytoplasmic antibodies (pANCA) with specificity against human neutrophil elastase (HNE) to differentiate these cocaine-induced diseases from primary autoimmune diseases. We describe a case of a 54-year-old woman with a history of cocaine abuse, who had PG lesions on her legs with accompanying CIMDL and lung lesions similar to those seen in Wegener granulomatosis. Detection of HNE-positive pANCA, and improvement or clinical recurrence after cessation or consumption of cocaine, respectively, were key to differentiating this presentation from primary autoimmune disease.
Cocaine consumption has been related to a wide variety of clinical conditions, including cocaine-induced midline destructive lesions (CIMDL),1 cutaneous vasculitis induced by cocaine contaminated with levamisol,2 and eruptive pyoderma gangrenosum (PG).3 Some of these pathologies have been related to the presence of peri-nuclear antineutrophil cytoplasmic antibodies (pANCA) with specificity against human neutrophil elastase (HNE).4–6 We describe a case of autoimmunity due to cocaine, with cutaneous, pulmonary, and ear, nose and throat involvement. The primary key for the aetiological diagnosis was the detection of HNE-positive pANCA.
Report A 54-year-old woman presented with a 2-month history of multiple painful ulcers on both legs. During nez-Gallo, Department of Dermatology, Correspondence: Dr David Jime Puerta del Mar University Hospital, 21 Ana de Viya Avenue, Cadiz, 11009, Spain E-mail: [email protected] Conflict of interest: none declared. Accepted for publication 27 March 2013
878
Clinical and Experimental Dermatology (2013) 38, pp878–882
this period, she had been treated with topical and oral antibiotics with no improvement. Her general health was otherwise good, but she reported occasional episodes of dry cough and bloody sputum. She had been a user of cocaine by inhalation (approximately 3.5 g/week) for 5 years. On physical examination, multiple painful ulcers were seen on the patient’s legs. These were purplish in colour, had a cribriform morphology with a slightly raised edge, and varied between 10 and 50 mm in size (Fig. 1a). Each ulcer first developed as a pustule, which then developed into an ulcer within about 3 days (Fig. 1b). The patient also had a saddlenose nasal deformity, associated with extensive oronasal fistula in the palate (Fig. 1c), due to the cocaine abuse. On histological examination of a biopsy taken from the edge of a skin ulcer, a dense inflammatory infiltrate was seen in the dermis, with neutrophils and fibrin deposits in the vessels (Fig. 2). No granulomas or vasculitis were present. Direct immunofluorescence gave negative results, as did stains and cultures for bacteria, fungi and alcohol-resistant acid bacilli. Based on the clinical and histological findings, the patient was diagnosed as having PG, and further
ª 2013 British Association of Dermatologists
PG and WG-like syndrome induced by cocaine D. Jimenez-Gallo et al.
(a)
(b)
(c)
Figure 1 Clinical images. (a) Painful violet-edged ulcers on the
legs; (b) pustule with violet-coloured halo on the leg; (c) saddlenose deformity with palatal oronasal fistula.
investigations were performed. Chest radiography showed pulmonary infiltrates in the middle and lower lobes of the right lung, accompanied by an interstitial lung pattern (Fig. 3a). Computed tomography (CT) of
(a)
the chest identified bilateral pulmonary nodules and cavitation (Fig. 3b), and a facial CT scan showed extensive midline destruction associated with the oronasal fistula (Fig. 3c). Full blood count, coagulation and biochemistry tests were normal. Results for the following were negative or within normal limits: anti-Sm, anti-RNP, anti-Ro, anti-La, anti-Scl70, anticentromere, anti-b2-glycoprotein, anticardiolipin and antihistone antibodies, antidouble-stranded DNA, cryoglobulins, complement levels, rheumatoid factor, and urine sedimentation tests. Investigations for syphilis, hepatitis B and C, human immunodeficiency virus and tuberculosis (TB) were also negative. Circulating pANCA without specificity for myeloperoxidase (MPO) or proteinase (PR)3 was found, at a titre of 1 : 80 (Fig. 3d). Because of the patient’s history of cocaine abuse, a specific search was performed for atypical ANCAs that were positive for elastase. The patient was also positive for antinuclear antibodies, at a titre of 1 : 40. Given the pulmonary findings, we conducted fibrobronchoscopy, which did not indicate malignant disease, and tested for bacteria, fungi and acid-alcohol resistant bacilli. Histology of a lung biopsy showed nonspecific interstitial pulmonary disease. The final diagnosis was PG and Wegener granulomatosis (WG)-like syndrome induced by cocaine, based on destruction of the midline of the nose and palate,
(b)
Figure 2 (a) Ulceration and deep and dense inflammatory infiltrate; (b) neutrophils in the inflammatory infiltrate associated with fibrin
deposits in the vessels. Haematoxylin and eosin, original magnification (a) 940 (b) 9200.
ª 2013 British Association of Dermatologists
Clinical and Experimental Dermatology (2013) 38, pp878–882
879
PG and WG-like syndrome induced by cocaine D. Jimenez-Gallo et al.
(a)
(b)
(c)
(d) Figure 3 (a) Radiograph showing the
presence of pulmonary infiltrates in the middle and lower lobes of the right lung. (b,c) Computed tomograph scan scan showing (b) pulmonary infiltrates in the right lung accompanied by a cavitated nodule at the lingula of the left lung, and (c) extensive destruction of the midline of the upper respiratory tract associated with an oronasal fistula. (d) Granulocyte immunofluorescence fixed in ethanol showing a perinuclear pattern of antinuclear cytoplasmic antibodies to elastase (original magnification 9600).
accompanied by the fixed pulmonary infiltrates and bilateral cavitary pulmonary nodules. Cocaine was further implicated as the cause of the illness because of the patient’s clinical improvement or relapse after cessation or consumption, respectively, along with an increase in pANCA titres to 1 : 160 after cocaine abuse and the detection of HNE-specific ANCAs. Since the initial presentation, our patient has had further and increasingly severe episodes of PG, associated with resumption of cocaine abuse. The most recent flare was accompanied by significant retiform purpura and involvement of the legs and ears. Currently the disease is stable with bolus administration of cyclophosphamide, despite continued cocaine consumption, verified by drug-testing of the patient’s urine. An increase in cocaine abuse worldwide is leading to a rise in associated pathology.7 Inhalation of cocaine can result in nasal inflammation and necrosis because of its vasoconstrictor effect, microtraumas caused by the cocaine itself and accompanying substances, and an increased incidence of infections caused by Staphylococcus aureus.1,4 These factors are known to cause CIMDL. The main problem arises in differentiating CIMDL from primary WG.
880
Clinical and Experimental Dermatology (2013) 38, pp878–882
For this purpose, Trimarchi et al.5 evaluated clinical, radiological, histopathological and serological findings in patients with a diagnosis of WG, and compared them with cases of CIMDL. The degree of nasal and paranasal destruction was more severe in patients with CIMDL; however, lung and kidney involvement and destruction of the ears and orbit were more common in WG. Compared with CIMDL, WG was associated with a higher frequency of fever, malaise, arthralgias and myalgias, and also of laboratory abnormalities such as increased incidence of acutephase reactants, microhaematuria, proteinuria and renal damage. Radiologically, nasal-septum perforation and involvement of other nasal structures was more frequent in patients with CIMDL. Histologically, the presence of nonspecific changes in the biopsies was also greater in CIMDL. Biopsies from patients with WG showed findings such as microabscesses, granulomas or multinucleated giant cells at the extravascular level, with deep and localized necrosis. An important finding to aid in the differential diagnosis of WG and CIMDL is the presence of ANCAs. The antigens of classic ANCA-associated vasculitis are PR3 and MPO. The presence of PR3 supports the diagnosis of
ª 2013 British Association of Dermatologists
PG and WG-like syndrome induced by cocaine D. Jimenez-Gallo et al.
Table 1 Features of documented cases of pyoderma gangrenosum (PG) associated with cocaine use.
Source Roche et al., 20083
Present case
Gender/age, years
Cocaine consumption, years
Predominant location of PG
ANCA
Effective treatment Infliximab 5 mg/kg at weeks 0, 2, and 6 followed by infusion every 8 weeks, accompanied by methotrexate 15 mg/week Infliximab 5 mg/kg at weeks 0, 2, and 6 followed by infusion every 8 weeks, accompanied by topical tacrolimus. Cyclophosphamide bolus of 15 mg/kg every 2 weeks (3 pulses) followed by 15 mg/kg every 3 weeks (6 pulses)
M/30
2
Back
Negative
M/38
10
Back
Negative
F/54
5
Legs
HNE ANCA positive
Accompanying symptoms None
None
Destruction of midline, presence of retiform purpura and lung involvement
ANCA, antineutrophil cytoplasmic antibodies; HNE, human neutrophil elastase.
WG, while MPO is associated with Churg–Strauss granulomatosis and microscopic polyangiitis. pANCA is almost always associated with MPO, and cytoplasmic ANCA is typically associated with PR3.8 However, there are other atypical ANCAs that are also normally associated with a perinuclear pattern (i.e. pANCA), for which are not performed in routine laboratory immunology. Their investigation requires specific suspicion by the clinician. These atypical ANCAs include HNE, lactoferrin, azuricidin, catalase K, bactericidal/permeability-increasing (BPI) protein, cathepsin G, defensin and lysozyme.1,5,6 HNE is an atypical ANCA that has been structurally and functionally related with PR3 ANCA, and for this reason could favour the simulation of WG in our patient. HNE ANCA was recently described as a marker of CIMDL; however, it is rarely detected in patients with WG.4–6 The differential diagnosis for midline destructive lesions includes, among others, CIMDL, WG, extranodal lymphoma, squamous cell carcinoma, midline malignant reticulosis, TB, syphilis, leishmaniasis, blastomycosis and actinomycosis.1,9 Another interesting detail of this case is the relationship between cocaine abuse and development of PG. The two cases described by Roche et al.3 in 2008 are the only similar cases published to date (Table 1). In conclusion, we report a case in which the clinical features were triggered by cocaine abuse accompanied by a raised titre of HNE ANCA. The similarity of these autoantibodies to PR3 ANCA, together with the toxic effects of cocaine, could explain the destruction of the midline and lung lesions simulating WG. The lesions of PG, a neutrophilic dermatosis, could also be related to the presence of these antibodies. To our knowledge, this
ª 2013 British Association of Dermatologists
is only the third case of PG reported, and the first to include pulmonary involvement mimicking cocaineinduced WG, illustrating the various clinical features that produce autoimmunity related to this drug. The raised titre of elastase ANCAs seemed to be related to the cocaine abuse and worsening of the PG in this case. This case highlights the importance of HNE ANCA for diagnosis, and the incorporation of cocaine abuse in the aetiology of PG.
Learning points ● Retiform purpura, destruction of the midline,
and PG are syndromes associated with cocaine consumption. ● pANCA with specificity against HNE is a marker of dermatological and rheumatic diseases induced by cocaine. ● Clinicoimmunological correlation allows differentiation of WG from the clinical syndrome induced by cocaine. ● Cocaine should be considered in the list of causes of PG. ● Cessation of cocaine abuse is the most effective treatment, but cyclophosphamide may be useful if the patient continues to use the drug.
References 1 Stahelin L, Fialho SC, Neves FS et al. Cocaine-induced midline destruction lesions with positive ANCA test mimicking Wegener’s granulomatosis. Rev Bras Reumatol 2012; 52: 434–7.
Clinical and Experimental Dermatology (2013) 38, pp878–882
881
PG and WG-like syndrome induced by cocaine D. Jimenez-Gallo et al.
2 Ullrich K, Koval R, Koval E et al. Five consecutive cases of a cutaneous vasculopathy in users of levamisole-adulterated cocaine. J Clin Rheumatol 2011; 17: 193–6. 3 Roche E, Martınez-Mench on T, S anchez-Carazo JL et al. Two cases of eruptive pyoderma gangrenosum associated with cocaine use. Actas Dermosifiliogr 2008; 99: 727– 30. 4 Rachapalli SM, Kiely PD. Cocaine-induced midline destructive lesions mimicking ENT-limited Wegener’s granulomatosis. Scand J Rheumatol 2008; 37: 477– 80. 5 Trimarchi M, Gregorini G, Facchetti F et al. Cocaine-induced midline destructive lesions: clinical, radiographic, histopathologic, and serologic features and their differentiation from Wegener granulomatosis. Medicine (Baltimore) 2001; 80: 391–404.
882
Clinical and Experimental Dermatology (2013) 38, pp878–882
6 Wiesner O, Russell KA, Lee AS et al. Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaine-induced midline destructive lesions but not autoimmune vasculitis. Arthritis Rheum 2004; 50: 2954–65. 7 Salas-Espındola Y, Peniche-Castellanos A, L opez-Gehrke I, Mercadillo-Perez P. Leukocytoclastic vasculitis related to cocaine use. Actas Dermosifiliogr 2011; 102: 825–7. 8 Walsh NM, Green PJ, Burlingame RW et al. Cocaine-related retiform purpura: evidence to incriminate the adulterant, levamisole. J Cutan Pathol 2010; 37: 1212–19. 9 Daggett RB, Haghighi P, Terkeltaub RA. Nasal cocaine abuse causing an aggressive midline intranasal and pharyngeal destructive process mimicking midline reticulosis and limited Wegener’s granulomatosis. J Rheumatol 1990; 17: 838–40.
ª 2013 British Association of Dermatologists
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