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Pustular Necrotising Anghtis - A Personal Perspective
Journal of Tissue Viability Vol6 No 1
15
PUSTULAR NECROTISING ANGIITIS A PERSONAL PERSPECTIVE John Tanner General Practitioner, Pontypridd, Mid-Glamorgan
BACKGROUND Born: December 23rd 1946 Past medical history: Nil of note, except endoscopy in 1991 which showed gastritis but no evidence of peptic ulceration. It was treated with omeprazole.
Current medication: Mefloquine for protection against malaria on a holiday in India. I had, however, taken a course of antibiotics two weeks previously for a sore throat that was not responding to paracetamol after five days. The condition started in the third week in November with about a dozen or so eruptions on the forearm and on the dorsum of each foot. These were raised, red lesions with black/purple raised centres. The lesions on the feet were surrounded by multiple tiny petechiae. There was no itching and by the end of the week they settled down. Five days after the rash, there was an onset of pain in the right knee, which commenced very suddenly. I was quite comfortable at lunchtime but three hours later I was having difficulty walking with the knee being hot and swollen. The condition was affecting the right foot and beginning to affect the left foot. There was progressive synovial involvement over the next few days, now affecting both knees, feet and elbows. The achilles tendons became inflamed as did the dorsum of each hand with crepitus being palpable. Oedema developed in the feet and within 4-5 days was above the knees. Walking was now becoming progressively more difficult, because of the increasing pain. The redema was not helped by travelling back from India in a very cramped plane for ten hours. The next day, I was seen by the physicians and admitted to East Glamorgan General Hospital on the last Sunday in November. On Monday, lesions appeared on both lower limbs. Worse ones appeared on the buttocks, popliteal fossae and feet. The lesions were raised papules with raised purple/black centres. The borders were grey/blue and the centres had now started to become vesicular. The lesions on the lower limbs progressed in two ways. On the buttocks, popliteal fossae and feet - the little vesicles joined neighbouring lesions to form small bullae and the small bullae joined together to form very large bullae, especially on the feet, and smaller ones on the popliteal fossae and the buttocks. The lesions on the backs of the thighs and legs, apart from a few, tended to resolve.
At this point I was discharged home and I will discuss my medical management in hospital later on. By the end of week three, the skin on the feet, popliteal fossae and buttocks had completely broken down to reveal clearly defined ulcers with sharp edges and intensely red bases. The pain was now two-fold: that caused by the ulceration and also from the joints. Walking had now become very difficult and I spent most of the time in bed, although I was able to get to and from the bathroom without help. Thrombophlebitis was affecting both legs and thighs and this progressed to a situation where I needed help to move in bed, because of the pain. There was also marked muscular wasting owing to my immobility. I must say that apart from a time when the ulcers developed a Staphyllococcus aureus infection, I did not feel ill in myself. MANAGEMENT BY DOCTORS Whilst I was on holiday, I commenced taking piroxicam 20 mg, twice daily because of the inflammatory reaction. At the end of week two, after admission to hospital, I was prescribed diclofenac 150 mg daily and had bed rest. Because of the symmetry of the rash associated with the throat infection, a provisional diagnosis of Henoch-Schonlein purpura (HSP) was made.
I asked ifl could have steroid therapy as the non-steroidal antiinflammatory drugs were having no effect on the condition, which was, in my opinion, getting worse day by day. It was thought that if the diagnosis was of HSP, steroid therapy could prolong the condition. A dermatological opinion was sought. I was advised to have potassium permanganate baths. The lesions were dressed with a non-adherent dressing and I was discharged home on this regime. Because of the increasing pain, I was given co-codamol together with a non-steroidal anti-inflammatory drug and an opiate for analgesia, before having the dressings changed. Three weeks into the illness, now leading to the second week in December, and with the inflammation worsening, my GP and myself again asked the hospital if I could have steroid therapy. I had read Diaz et al's 1973 paper on pustular necrotising angiitis 1 which suggested this course of action. Although my condition seemed to be a mirror image of the cases described I was once again advised against steroid therapy. After six weeks (we were now in the first week in January), with no healing taking place and the inflammatory response
16 Journal of Tissue Viability Vol6 No l getting worse, I took matters into my own hands with the support of my GP. Yet again against hospital advice, I placed myself on a regime of 80 mg of prednisolone daily. After ten days, mobility had returned so that I could walk and what really pleased me was that the ulcers had changed in appearance from deep red to pink and were now showing signs of healing. At this point I encountered a little 'hiccough' .... Whilst the district nurse was dressing the wounds in the popliteal fossae, I developed severe right lower chest pain which, after a short while, moved into the epigastrium and was associated with board rigidity of the abdomen. I thought to myself.... 'I have developed a pulmonary embolism and I have perforated owing to the high prednisolone intake'. Admission to East Glamorgan Hospital resulted in a laparotomy which showed no abnormality and three days post-operatively ventilation/perfusion scanning confirmed a right sided embolus. I was heparinised and started on a course of warfarin, 40 mg prednisolone, ranitidine 150 mg twice daily and analgesia. Over the next three weeks the ulceration had virtually fully healed and the dose of prednisolone was reduced to 20 mg daily, dropping by 5 mg per fortnight over the next month. By the beginning of April, all my medication was stopped. About that time, the skin around the medial malleolus of the left ankle and the lateral malleolus of the right began to break down, pain started to reappear on walking, and within a week or two, two ulcers about the size of ten pence pieces had formed. I commenced taking 40 mg prednisolone daily and after some weeks, on signs of healing, reduced this to 20 mg, then 15 mg and I am now on a maintenance dose of 10 mg daily, as the ulcers have now nearly healed. This is my current situation.
NURSING CARE The lesions were initially treated with potassium permanganate baths and covered with non-adherent dry dressings. My practice nurses were concerned about the ulcers and asked a dermatologist to call. We were advised that the ulcers should be dressed with a hydrogel and paste bandage. The district nurses then took over my care at home and the service I received was excellent. Two nurses spent approximately 2 hours daily dressing and cleaning the ulcers. They were, however concerned as to the best management due to the extent of the ulcers. They therefore asked Mrs Mair Fear, Tissue Viability Nurse, for her expert opinion on how this situation should be handled. The treatment was then changed to hydrocolloid paste in the cavities and hydrocolloid wafer used on appropriate sites. This dressing regime was continued until the ulcers had healed which was towards the end of February. When the ulceration reformed, towards the end of March/beginning of April on reducing and stopping steroid therapy, I was able to manage myself, dressing the wounds with hydrogel applied to the affected areas. By mid August, I was only covering the sites with a non adherent dry dressing, purely as a
protective agent. By mid September there was no need for anything to be applied.
RESULTS OF INVESTIGATIONS The only abnormal findings were a raised ESR of 78, CPR 28, and an ASO titre of over 800. These were the only results I had at the time of my initial discharge and I was happy to take the advice of the consultants who advised against steroid therapy. However, a histology report was received after my discharge which reads:
Sections of skin show an essentially normal epidermis covering dermis in which there is a marked perivascular inflammatory infiltrate consisting ofneutrophils, prominent eosinophils and some mononuclear cells. A small amount of nuclear dust is noted. The appearances are in keeping with those of a leukocytoclastic vasculitis'. The dermatologist also comments in the notes that:
'The histology confirms the diagnosis of leukocytoclastic vasculitis, the immunofluorescent was essentially negative making THE DIAGNOSIS OF HENOCH-SCHONLEIN PURPURA VERY UNLIKELY'. Had I known this at the time, I would have started taking steroids even against hospital advice.
CONCLUSIONS I feel that in cases such as this that biopsy is of vital importance as is rapid commencement of steroid therapy. For me, the effect of the illness were three months off work, a laparotomy, a pulmonary embolus and locum fees of £6,000. I still have a nagging thought in my head that the outcome might have been better had I initiated steroid therapy after my first two days in hospital. It is now the end of September and I am looking forward to the day I can come off the steroid therapy.
Address for Correspondence Dr JTanner Pare Canol Surgery, Central Park, Church Village, Nr Powtypridd Mid Glamorgan CF38 1RJ References · 1 Diaz L, Thomas T et al. Pustular necrotizing angiitis, Archives of Dermatology, 1973; 108: 104-118.
15
PUSTULAR NECROTISING ANGIITIS A PERSONAL PERSPECTIVE John Tanner General Practitioner, Pontypridd, Mid-Glamorgan
BACKGROUND Born: December 23rd 1946 Past medical history: Nil of note, except endoscopy in 1991 which showed gastritis but no evidence of peptic ulceration. It was treated with omeprazole.
Current medication: Mefloquine for protection against malaria on a holiday in India. I had, however, taken a course of antibiotics two weeks previously for a sore throat that was not responding to paracetamol after five days. The condition started in the third week in November with about a dozen or so eruptions on the forearm and on the dorsum of each foot. These were raised, red lesions with black/purple raised centres. The lesions on the feet were surrounded by multiple tiny petechiae. There was no itching and by the end of the week they settled down. Five days after the rash, there was an onset of pain in the right knee, which commenced very suddenly. I was quite comfortable at lunchtime but three hours later I was having difficulty walking with the knee being hot and swollen. The condition was affecting the right foot and beginning to affect the left foot. There was progressive synovial involvement over the next few days, now affecting both knees, feet and elbows. The achilles tendons became inflamed as did the dorsum of each hand with crepitus being palpable. Oedema developed in the feet and within 4-5 days was above the knees. Walking was now becoming progressively more difficult, because of the increasing pain. The redema was not helped by travelling back from India in a very cramped plane for ten hours. The next day, I was seen by the physicians and admitted to East Glamorgan General Hospital on the last Sunday in November. On Monday, lesions appeared on both lower limbs. Worse ones appeared on the buttocks, popliteal fossae and feet. The lesions were raised papules with raised purple/black centres. The borders were grey/blue and the centres had now started to become vesicular. The lesions on the lower limbs progressed in two ways. On the buttocks, popliteal fossae and feet - the little vesicles joined neighbouring lesions to form small bullae and the small bullae joined together to form very large bullae, especially on the feet, and smaller ones on the popliteal fossae and the buttocks. The lesions on the backs of the thighs and legs, apart from a few, tended to resolve.
At this point I was discharged home and I will discuss my medical management in hospital later on. By the end of week three, the skin on the feet, popliteal fossae and buttocks had completely broken down to reveal clearly defined ulcers with sharp edges and intensely red bases. The pain was now two-fold: that caused by the ulceration and also from the joints. Walking had now become very difficult and I spent most of the time in bed, although I was able to get to and from the bathroom without help. Thrombophlebitis was affecting both legs and thighs and this progressed to a situation where I needed help to move in bed, because of the pain. There was also marked muscular wasting owing to my immobility. I must say that apart from a time when the ulcers developed a Staphyllococcus aureus infection, I did not feel ill in myself. MANAGEMENT BY DOCTORS Whilst I was on holiday, I commenced taking piroxicam 20 mg, twice daily because of the inflammatory reaction. At the end of week two, after admission to hospital, I was prescribed diclofenac 150 mg daily and had bed rest. Because of the symmetry of the rash associated with the throat infection, a provisional diagnosis of Henoch-Schonlein purpura (HSP) was made.
I asked ifl could have steroid therapy as the non-steroidal antiinflammatory drugs were having no effect on the condition, which was, in my opinion, getting worse day by day. It was thought that if the diagnosis was of HSP, steroid therapy could prolong the condition. A dermatological opinion was sought. I was advised to have potassium permanganate baths. The lesions were dressed with a non-adherent dressing and I was discharged home on this regime. Because of the increasing pain, I was given co-codamol together with a non-steroidal anti-inflammatory drug and an opiate for analgesia, before having the dressings changed. Three weeks into the illness, now leading to the second week in December, and with the inflammation worsening, my GP and myself again asked the hospital if I could have steroid therapy. I had read Diaz et al's 1973 paper on pustular necrotising angiitis 1 which suggested this course of action. Although my condition seemed to be a mirror image of the cases described I was once again advised against steroid therapy. After six weeks (we were now in the first week in January), with no healing taking place and the inflammatory response
16 Journal of Tissue Viability Vol6 No l getting worse, I took matters into my own hands with the support of my GP. Yet again against hospital advice, I placed myself on a regime of 80 mg of prednisolone daily. After ten days, mobility had returned so that I could walk and what really pleased me was that the ulcers had changed in appearance from deep red to pink and were now showing signs of healing. At this point I encountered a little 'hiccough' .... Whilst the district nurse was dressing the wounds in the popliteal fossae, I developed severe right lower chest pain which, after a short while, moved into the epigastrium and was associated with board rigidity of the abdomen. I thought to myself.... 'I have developed a pulmonary embolism and I have perforated owing to the high prednisolone intake'. Admission to East Glamorgan Hospital resulted in a laparotomy which showed no abnormality and three days post-operatively ventilation/perfusion scanning confirmed a right sided embolus. I was heparinised and started on a course of warfarin, 40 mg prednisolone, ranitidine 150 mg twice daily and analgesia. Over the next three weeks the ulceration had virtually fully healed and the dose of prednisolone was reduced to 20 mg daily, dropping by 5 mg per fortnight over the next month. By the beginning of April, all my medication was stopped. About that time, the skin around the medial malleolus of the left ankle and the lateral malleolus of the right began to break down, pain started to reappear on walking, and within a week or two, two ulcers about the size of ten pence pieces had formed. I commenced taking 40 mg prednisolone daily and after some weeks, on signs of healing, reduced this to 20 mg, then 15 mg and I am now on a maintenance dose of 10 mg daily, as the ulcers have now nearly healed. This is my current situation.
NURSING CARE The lesions were initially treated with potassium permanganate baths and covered with non-adherent dry dressings. My practice nurses were concerned about the ulcers and asked a dermatologist to call. We were advised that the ulcers should be dressed with a hydrogel and paste bandage. The district nurses then took over my care at home and the service I received was excellent. Two nurses spent approximately 2 hours daily dressing and cleaning the ulcers. They were, however concerned as to the best management due to the extent of the ulcers. They therefore asked Mrs Mair Fear, Tissue Viability Nurse, for her expert opinion on how this situation should be handled. The treatment was then changed to hydrocolloid paste in the cavities and hydrocolloid wafer used on appropriate sites. This dressing regime was continued until the ulcers had healed which was towards the end of February. When the ulceration reformed, towards the end of March/beginning of April on reducing and stopping steroid therapy, I was able to manage myself, dressing the wounds with hydrogel applied to the affected areas. By mid August, I was only covering the sites with a non adherent dry dressing, purely as a
protective agent. By mid September there was no need for anything to be applied.
RESULTS OF INVESTIGATIONS The only abnormal findings were a raised ESR of 78, CPR 28, and an ASO titre of over 800. These were the only results I had at the time of my initial discharge and I was happy to take the advice of the consultants who advised against steroid therapy. However, a histology report was received after my discharge which reads:
Sections of skin show an essentially normal epidermis covering dermis in which there is a marked perivascular inflammatory infiltrate consisting ofneutrophils, prominent eosinophils and some mononuclear cells. A small amount of nuclear dust is noted. The appearances are in keeping with those of a leukocytoclastic vasculitis'. The dermatologist also comments in the notes that:
'The histology confirms the diagnosis of leukocytoclastic vasculitis, the immunofluorescent was essentially negative making THE DIAGNOSIS OF HENOCH-SCHONLEIN PURPURA VERY UNLIKELY'. Had I known this at the time, I would have started taking steroids even against hospital advice.
CONCLUSIONS I feel that in cases such as this that biopsy is of vital importance as is rapid commencement of steroid therapy. For me, the effect of the illness were three months off work, a laparotomy, a pulmonary embolus and locum fees of £6,000. I still have a nagging thought in my head that the outcome might have been better had I initiated steroid therapy after my first two days in hospital. It is now the end of September and I am looking forward to the day I can come off the steroid therapy.
Address for Correspondence Dr JTanner Pare Canol Surgery, Central Park, Church Village, Nr Powtypridd Mid Glamorgan CF38 1RJ References · 1 Diaz L, Thomas T et al. Pustular necrotizing angiitis, Archives of Dermatology, 1973; 108: 104-118.