- Email: [email protected]
Malignant atrophic papulosis presenting as gastroparesis
3462
Letters to the Editor
Reprint requests and correspondence: M. C. Lasso de la Vega, Ph.D., Unidad de Farmacologı´a Clı´nica, Hospital General Universitario de Alicante, C/ Maestro Alonso, 109, 03010 Alicante, Spain. Received July 2, 2001; accepted July 6, 2001.
Malignant Atrophic Papulosis Presenting as Gastroparesis TO THE EDITOR: Malignant atrophic papulosis (Degos’ disease, Kohlmeier-Degos’ syndrome, progressive arterial mesenterial vascular occlusive disease) is a rare multisystem disorder characterized by occlusive vasculopathy (1). Involvement of the GI tract usually takes the form of vascular and mucosal disease (1–3); severe gastroparesis without mucosal inflammation has not been reported previously. A 48-yr-old man initially presented with a 3-month history of dyspepsia, early satiety, and occasional heartburn. An initial esophagogastroduodenoscopy was normal. Over the next 3 months his symptoms progressed, with the development of postprandial nausea, epigastric pain, regurgitation, and vomiting. Over this period he lost 12 kg in weight. Gastroscopy at this stage revealed significant food residue in the stomach, despite a 12-h fast, but no mucosal disease or obstruction as far as the third part of the duodenum. A solid-phase radioisotope gastric emptying study confirmed severe gastroparesis: after 150 min, 95% of the meal remained in the stomach (normal ⬍ 20%). A barium followthrough showed only delayed transit from the stomach with no obstructive lesions. Autonomic function testing demonstrated impaired sympathetic and parasympathetic cardiovascular responses, and peripheral nerve conduction studies were consistent with mononeuritis multiplex. Over the next 4 wk he developed multiple erythematous, cutaneous papules, with the pathognomonic central porcelain-white area typical of Degos’ disease. Skin biopsy was confirmatory (4). Examination of the cerebrospinal fluid, magnetic resonance imaging of the brain and spinal cord, and mesenteric angiography were all normal. There was initially some improvement in the patient’s vomiting with a combination of i.v. erythromycin, rectal domperidone, and subcutaneous octreotide, but after 4 wk there were worsening symptoms and weight loss; endoscopy was repeated. On this occasion the anterior wall of the stomach was abnormal, with areas of dusky discoloration as well as small erythematous papules similar to the cutaneous features extending throughout all of the duodenum (biopsy was not performed because of the risk of perforation) (1). In view of the possibility of further traumatizing the inflamed mucosa, total parenteral nutrition was established rather than enteral tube feeding. Treatment with i.v. corticosteroids, heparin, cyclosporin, tacrolimus, aspirin, and clopidrogel had no impact on his skin lesions or abdominal symptoms. Four weeks later there was a sudden deterioration in his condition. At laparotomy a perforated
AJG – Vol. 96, No. 12, 2001
segment of jejunum was excised. A further laparotomy was required 3 days later, to repair a perforation in the anterior wall of the stomach. The patient died of multiorgan failure. Malignant atrophic papulosis is an uncommon multisystem disease of unknown etiology (5). Although the cutaneous lesions are regarded as characteristic, the vasculopathy can affect any organ system. This patient had dyspeptic symptoms for 6 months before the appearance of the rash. Severe gastroparesis was confirmed, and there was no evidence of other conditions known to cause autonomic neuropathy. GI involvement, usually manifest by mucosal lesions that can bleed or perforate, and neurological involvement are both common in malignant atrophic papulosis (5, 6). Usually cutaneous manifestations precede GI or neurological (2, 4). No effective treatment has been described, although a variety of immunosuppressive and antithrombotic therapies have been tried (7). Death often arises via GI complications. The sequence of events in this case strongly suggests that malignant atrophic papulosis initially presented as gastroparesis, possibly due to localized neuropathy of the autonomic system or enteric neurons. In the future gastroparesis should be considered in the differential diagnosis of abdominal pains in patients with malignant atrophic papulosis. I. L. P. Beales, M.D. Medical School University of East Anglia Norwich, United Kingdom
REFERENCES 1. Casparie MK, Meyer JW, van Huystee BE, et al. Endoscopic and histopathologic features of Degos’ disease. Endoscopy 1991;23:231–3. 2. Fruhwirth J, Mischinger HJ, Werkgartner G, et al. KohlmeierDegos’s disease with primary intestinal manifestation. Scand J Gastroenterol 1997;32:1066 –70. 3. Lankisch MR, Johst P, Scolapio JS, Fleming CR. Acute abdominal pain as a leading symptom for Degos’ disease (malignant atrophic papulosis). Am J Gastroenterol 1999;94:1098 –9. 4. Snow JL, Muller SA. Degos syndrome: Malignant atrophic papulosis. Semin Dermatol 1995;14:99 –105. 5. McFarland HR, Wood WG, Drowns BV, Meneses AC. Papulosis atrophicans maligna (Kohlmeier-Degos disease): A disseminated occlusive vasculopathy. Ann Neurol 1978;3:388 –92. 6. Burrow JN, Blumbergs PC, Iyer PV, Hallpike JF. KohlmeierDegos disease: A multisystem vasculopathy with progressive cerebral infarction. Aust N Z J Med 1991;21:49 –51. 7. Leslie TA, Goldsmith PC, Thompson AJ, Dowd PM. Degos disease and spastic paraplegia. Clin Exp Dermatol 1993;18: 344 – 6.
Reprint requests and correspondence: I. L. P. Beales, M.D., Medical School, University of East Anglia, Norwich NR4 7TJ, United Kingdom. Received July 2, 2001; accepted July 6, 2001.
Letters to the Editor
Reprint requests and correspondence: M. C. Lasso de la Vega, Ph.D., Unidad de Farmacologı´a Clı´nica, Hospital General Universitario de Alicante, C/ Maestro Alonso, 109, 03010 Alicante, Spain. Received July 2, 2001; accepted July 6, 2001.
Malignant Atrophic Papulosis Presenting as Gastroparesis TO THE EDITOR: Malignant atrophic papulosis (Degos’ disease, Kohlmeier-Degos’ syndrome, progressive arterial mesenterial vascular occlusive disease) is a rare multisystem disorder characterized by occlusive vasculopathy (1). Involvement of the GI tract usually takes the form of vascular and mucosal disease (1–3); severe gastroparesis without mucosal inflammation has not been reported previously. A 48-yr-old man initially presented with a 3-month history of dyspepsia, early satiety, and occasional heartburn. An initial esophagogastroduodenoscopy was normal. Over the next 3 months his symptoms progressed, with the development of postprandial nausea, epigastric pain, regurgitation, and vomiting. Over this period he lost 12 kg in weight. Gastroscopy at this stage revealed significant food residue in the stomach, despite a 12-h fast, but no mucosal disease or obstruction as far as the third part of the duodenum. A solid-phase radioisotope gastric emptying study confirmed severe gastroparesis: after 150 min, 95% of the meal remained in the stomach (normal ⬍ 20%). A barium followthrough showed only delayed transit from the stomach with no obstructive lesions. Autonomic function testing demonstrated impaired sympathetic and parasympathetic cardiovascular responses, and peripheral nerve conduction studies were consistent with mononeuritis multiplex. Over the next 4 wk he developed multiple erythematous, cutaneous papules, with the pathognomonic central porcelain-white area typical of Degos’ disease. Skin biopsy was confirmatory (4). Examination of the cerebrospinal fluid, magnetic resonance imaging of the brain and spinal cord, and mesenteric angiography were all normal. There was initially some improvement in the patient’s vomiting with a combination of i.v. erythromycin, rectal domperidone, and subcutaneous octreotide, but after 4 wk there were worsening symptoms and weight loss; endoscopy was repeated. On this occasion the anterior wall of the stomach was abnormal, with areas of dusky discoloration as well as small erythematous papules similar to the cutaneous features extending throughout all of the duodenum (biopsy was not performed because of the risk of perforation) (1). In view of the possibility of further traumatizing the inflamed mucosa, total parenteral nutrition was established rather than enteral tube feeding. Treatment with i.v. corticosteroids, heparin, cyclosporin, tacrolimus, aspirin, and clopidrogel had no impact on his skin lesions or abdominal symptoms. Four weeks later there was a sudden deterioration in his condition. At laparotomy a perforated
AJG – Vol. 96, No. 12, 2001
segment of jejunum was excised. A further laparotomy was required 3 days later, to repair a perforation in the anterior wall of the stomach. The patient died of multiorgan failure. Malignant atrophic papulosis is an uncommon multisystem disease of unknown etiology (5). Although the cutaneous lesions are regarded as characteristic, the vasculopathy can affect any organ system. This patient had dyspeptic symptoms for 6 months before the appearance of the rash. Severe gastroparesis was confirmed, and there was no evidence of other conditions known to cause autonomic neuropathy. GI involvement, usually manifest by mucosal lesions that can bleed or perforate, and neurological involvement are both common in malignant atrophic papulosis (5, 6). Usually cutaneous manifestations precede GI or neurological (2, 4). No effective treatment has been described, although a variety of immunosuppressive and antithrombotic therapies have been tried (7). Death often arises via GI complications. The sequence of events in this case strongly suggests that malignant atrophic papulosis initially presented as gastroparesis, possibly due to localized neuropathy of the autonomic system or enteric neurons. In the future gastroparesis should be considered in the differential diagnosis of abdominal pains in patients with malignant atrophic papulosis. I. L. P. Beales, M.D. Medical School University of East Anglia Norwich, United Kingdom
REFERENCES 1. Casparie MK, Meyer JW, van Huystee BE, et al. Endoscopic and histopathologic features of Degos’ disease. Endoscopy 1991;23:231–3. 2. Fruhwirth J, Mischinger HJ, Werkgartner G, et al. KohlmeierDegos’s disease with primary intestinal manifestation. Scand J Gastroenterol 1997;32:1066 –70. 3. Lankisch MR, Johst P, Scolapio JS, Fleming CR. Acute abdominal pain as a leading symptom for Degos’ disease (malignant atrophic papulosis). Am J Gastroenterol 1999;94:1098 –9. 4. Snow JL, Muller SA. Degos syndrome: Malignant atrophic papulosis. Semin Dermatol 1995;14:99 –105. 5. McFarland HR, Wood WG, Drowns BV, Meneses AC. Papulosis atrophicans maligna (Kohlmeier-Degos disease): A disseminated occlusive vasculopathy. Ann Neurol 1978;3:388 –92. 6. Burrow JN, Blumbergs PC, Iyer PV, Hallpike JF. KohlmeierDegos disease: A multisystem vasculopathy with progressive cerebral infarction. Aust N Z J Med 1991;21:49 –51. 7. Leslie TA, Goldsmith PC, Thompson AJ, Dowd PM. Degos disease and spastic paraplegia. Clin Exp Dermatol 1993;18: 344 – 6.
Reprint requests and correspondence: I. L. P. Beales, M.D., Medical School, University of East Anglia, Norwich NR4 7TJ, United Kingdom. Received July 2, 2001; accepted July 6, 2001.