Top dermatologist talks about a case of rare disorded of Degos’ disease. This case study appeared in the ‘Journal of Pakistan Association of Dermatologists 2006; 16: 52-55.’
Degos’ disease: a rare disorder
Ikram ullah-Khan, Saima Rafiq
Department of Dermatology, Pakistan Institute of Medical Sciences, Islamabad.
Abstract Malignant atrophic papulosis (Degosí disease) is a rare multisystem lymphocytic vasculitis characterized by widespread thrombosis of small vessels not only in the skin, but also in the gastrointestinal, ocular and central nervous systems. We report a middle aged lady who has Degos’ disease. We diagnosed her on clinical and histological ground.
Key words Degos’ disease, malignant atrophic papulosis.
Introduction Malignant atrophic papulosis is a progressive vasculopathy causing occlusion of small and medium sized arteries.1,2 It is characterized by skin and gastrointestinal lesions, but neurological features are also frequent. The skin lesions are usually the first feature, and may be the only manifestation over many years.3
The etiology of Degos’ disease is not clear. It is a vasculitis, and a thrombotic disorder. In some cases, antiphospholipid antibodies are identified. In histopathology the early lesions show a superficial and deep perivascular, perineural and peri- appendageal chronic inflammatory cell infiltrate.4 Later, lesions show a classical ‘wedge-shaped’ pattern of sclerotic change in dermis which is usually only sparsely cellular. In skin, Degosí disease manifests as erythematous, pink or red papules. These papules heal to leave central, porcelain white atrophic scars. These papules usually have a peripheral telangiectatic rim.5 In the systemic variant of Degos’ disease gastrointestinal tract with intestinal perforations is the most sever complication. Other systems e.g. CNS, ocular, cardiovascular and pulmonary can also be involved.1 Skin lesions can be confused with guttate lichen sclerosus but the histopathology is diagnostic. No successful medical therapy for Degosí disease is known. Antiplatelet drugs e.g. aspirin, dipyridamol may reduce the number of lesions in some patients with only skin involvement.6 Surgery may help in cases of intestinal perforation.
Case report A 50-year-old lady presented with history of papular lesions on her legs for the last 1 year. Initially these lesions started as small pink-coloured papules on thighs. Within a few days these lesion increased in number involving lower legs and abdomen. These lesions healed after some days and new lesions appeared. On examination small pink-coloured papular lesions were seen on legs and abdomen. Some of the lesions had central atrophy with white scars. No other systems were involved. Skin histopathology showed epidermal atrophy, a wedge shaped area of necrosis in dermis and vessels walls were thick. Other laboratory investigations like blood complete, ESR and coagulation profile were normal
Discussion Malignant atrophic papulosis (Degos’ disease) was first described by Kohlmeier in 1941 17 and recognized as a specific entity by Degos’ in 1942.18 It is a rare disease. About 150 cases have been reported in the world literature. Broadly speaking Degos’ disease is a vasculopathy or an endovasculitis.7 It is a progressive, small and medium-size arterial occluding disease, leading to tissue infarction.8 Degosí disease occurs both in a limited benign cutaneous forms and in a lethal multiorgan, systemic variant.9 In systemic variant of Degos’ disease the gastrointestinal tract is affected in 50% of cases.10 20% of cases of systemic Degos’ disease involve the CNS. Systemic manifestation usually develop from weeks to year after the onset of the skin lesion, or in rare instances, they may precede the skin lesions. Because of the broad overlap in clinical and histological findings, High et al.contended in 2004 that Degos’ disease may not be a specific entity but, rather may represent a common end point to a variety of vascular insults, many of which have not been fully elucidated.11 In 2003, Bull et al. proposed that Degos’ disease is just a variant of lupus.8 Some authorities suggest that Degos’ disease involves a primary endothelial cell defect with secondary thrombosis, leading to infarctive changes. No evidence exists for antibodies to the component of endothelial cells. Although, in some cases antiphospholipid antibodies are isolated.12 Three possible mechanisms have been suggested: disturbance in immunity, viral infection and abnormality in clotting system of blood. This disorder usually occurs in adults and male to female ratio is approximately 3:1. The cutaneous eruption is constant and pathognomonic. These lesions are pink or yellow papule and heal with typical central porcelain-white zone of atrophy. In our patient there were pink papular lesions as well as healed lesion with central porcelain-like zone of atrophy.
Gastrointestinal lesions usually occur a few months after the onset of skin lesions. GI tract involvement may remain asymptomatic. Sometimes, patient suffer only from dyspepsia, but usually it is an abdominal emergency that reveals the intestinal involvement and leads to perforation.1 The gastrointestinal tract is involved in about 50% of patients. In our patient the skin lesions were present for the last one year but there was no evidence of gastrointestinal complaint on history and examination. Neurologic manifestations of Degos’ disease are observed in 20% of patients. They include hemiparesis, aphasia, multiple cranial nerve involvement, monoplegia and seizures.13 In our patient there were no neurologic abnormalities. A variety of ocular findings occur in Degos’ disease. In 1986, Sibillat et al.14 reported that ophthalmologic symptoms were present in 35 of 105 observations published. They are posterior subcapsular cataracts, visual field defects, third cranial nerve palsies, papilledema and scleral plaques.15 In our patient there was no eye involvement on ophthalmoscopic examination. In 1997, Katz et al.16 described a familial variant of Degos’ disease. In our patient there was no family history of such disease. No successful medical therapy is known. Antiplatelet drugs may reduce the number of new lesions in some patients with only skin involvement. Other treatments include: topical corticosteroids pheniormin, iodohydroxyquinoline, sulphonamides, heparin, azathioprine, methotrexate, cyclosporine, tacrolimus and pentoxifylline.
References 1. Snow JL, Muller SA. Degos syndrome: malignant atrophic papulosis. Semin Dermatol 1995; 14: 99-105. 2. Chatham WW. Miscellaneous forms of vasculitis. In: Ball GV, Bridges SL Jr, eds. Vasculitis. Oxford: Oxford University Press; 2002. 513-32. 3. Su WPD, Schroeter AL, Lee DA et al. Clinical and histologic findings in Degos syndrome. Cutis 1985; 35: 131- 8. 4. Harvell JD, Williford PL, White WL. Benign cutaneous Degos’ disease: a case report with emphasis on histopathology as papules chronologically evolve. Am J Dermatopathol 2001; 23: 116-23. 5. Barriere H. Papulose atrophiante maligne (Maladie de Degos). Ann Dermatol Venereol 1978; 105: 733. 6. Drucker CR. Malignant atrophic papulosis: response to antiplatelet therapy. Dermatologica 1990; 180: 90- 2. 7. Farrell AM, Moss J, Costello C et al. Benign cutaneous Degos’ disease. Br J Dermatol 1998; 139: 708-12. 8. Ball E, Newburger A, Ackerman AB. Degos’ disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se. Am J Dermatopathol 2003; 25: 308-20. 9. Coskun B, Saral Y, Cicek D, Ozercan R. Benign cutaneous Degos’ disease: a case report and review of the literature. J Dermatol 2004; 31: 666-70. 10. 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. 11. High WA, Aranda J, Patel SB et al. Is Degos’ disease a clinical and histological end point rather than a specific disease? J Am Acad Dermatol 2004; 50: 895-9. 12. Asherson RA, Cervera R: Antiphospholipid syndrome. J Invest Dermatol 1993; 100: 21S-27S. 13. Subbiah P, Wijdicks E, Muenter M et al. Skin lesion with a fatal neurologic outcome (Degos’ disease). Neurology 1996; 46: 636-40. 14. Sibillat M, Avril MF, Charpentier P et al. [Malignant atrophic papulosis (Degos’ disease): clinical review. Apropos of a case]. J Fr Ophtalmol 1986; 9: 299-304. 15. Egan R, Lessell S. Posterior subcapsular cataract in Degos disease. Am J Ophthalmol 2000; 129: 806-7. 16. Katz SK, Mudd LJ, Roenigk HH Jr. Malignant atrophic papulosis (Degos’ disease) involving three generations of a Journal of Pakistan Association of Dermatologists 2006; 16: 52-55. 55 family. J Am Acad Dermatol 1997; 37: 480-4. 17. Kohlmeier W. Multiple Hautnekrosen bei thrombangiitis obliterans. Arch Dermatol et Syphilol 1941; 181: 783-4. 18. Degos R. Malignant atrophic papulosis. Br J Dermatol 1979; 100: 21-36.
Address for correspondence
Dr. Ikran Ullah Khan, Department of Dermatology, Pakistan Institute of Medical Sciences, Islamabad.