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Year : 2003  |  Volume : 51  |  Issue : 3  |  Page : 260-263

Malignant Atrophic Papulosis. Report of a Case with Multiple Ophthalmic Findings.

Department of Ophthalmology, St. John's National Academy of Health Sciences, Johnnagar, Bangalore, India

Correspondence Address:
Reji K Thomas
Department of Ophthalmology, St. John's National Academy of Health Sciences, Johnnagar, Bangalore
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Source of Support: None, Conflict of Interest: None

PMID: 14601855

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Malignant atrophic papulosis is a rare and fatal condition with multiple organ involvement. We describe a patient with progressive ocular and dermatological findings.

Keywords: Malignant atrophic papulosis, ophthalmo-logical and dermatological manifestations

How to cite this article:
Thomas RK, Nithyanandam S, Rawoof B A, Rajendran S C. Malignant Atrophic Papulosis. Report of a Case with Multiple Ophthalmic Findings. Indian J Ophthalmol 2003;51:260-3

How to cite this URL:
Thomas RK, Nithyanandam S, Rawoof B A, Rajendran S C. Malignant Atrophic Papulosis. Report of a Case with Multiple Ophthalmic Findings. Indian J Ophthalmol [serial online] 2003 [cited 2021 Jan 16];51:260-3. Available from: https://www.ijo.in/text.asp?2003/51/3/260/14670

Malignant atrophic papulosis is a rarely seen and often fatal condition. In 1941, Kohlmeier reported a case of what he believed to be Buerger's disease with characteristic skin lesions. Some months later, Degos independently described a similar case. Both patients died within one year of diagnosis following sudden intestinal perforation. Degos recognised the serious nature of this syndrome and named it malignant atrophic papulosis. It is commonly known as Degos' syndrome. Since then various case reports have been published. The disease is characterised mainly by its porcelain-like gross skin lesions, and the ophthalmic, central nervous system and gastrointestinal lesions. Lesions involving the lungs, heart, bladder and kidneys have also been described. [1],[2] We present a patient of malignant atrophic papulosis who had progressive ocular and dermatological manifestations.

  Case report Top

A 19-year-old male sought treatment for papules and ulcers on the trunk and extremities associated with a painful burning sensation. The lesions started as papules 3 years earlier over his lower extremities and gradually progressed to other areas sparing the face. Over time, the lesions became whitish in the centre. The vision in both eyes was normal. No significant family history was available.

General physical examination revealed pallor, bilateral significant inguinal lymphadenopathy and multiple erythematous papules on the limbs and trunk. The lower limbs were predominantly involved [Figure - 1]. A majority of the papules were umbilicated with a central whitish porcelain-like zone of atrophy and a pink, erythematous, well-demarcated border [Figure - 1]a. Some of the lesions were ulcerated with mucopurulent discharge [Figure - 1]b. Systemic examination was normal. Skin biopsy showed features consistent with Degos' disease.

At the initial ophthalmic examination, the visual acuity was 6/6; N6 in both eyes. In the right eye, the colour vision was abnormal using Ishihara's pseudo-isochromatic charts. Anterior segment findings were unremarkable and intraocular pressure was normal. Fundus examination showed a mild temporal pallor of the optic disc and the peripheral retina showed small areas of doubtful retinal pallor. Visual fields showed multiple areas of decreased retinal sensitivity and scotomata [Figure - 2]. The left eye was normal.

The patient presented two weeks later with progressive, painless loss of vision in the right eye. The visual acuity in the right eye was 6/18 with a relative afferent pupillary defect and gross colour vision abnormality. The eyelids developed atrophic patches. The bulbar conjunctiva had atrophic plaques and telangiectatic vessels [Figure - 1]c. The fundus showed increasing pallor of the optic disc with peripheral pale, patchy, chorioretinal lesions with few areas of pigment clumping. The visual field worsened [Figure - 2]. The left eye remained normal. The vision in the right eye progressed to no light perception (NLP) over six weeks, with an amaurotic pupil and total optic atrophy [Figure - 1]d. The lesions on the eyelids and the conjunctiva persisted. The left eye remained normal.

The patient was treated with oral steroids, dapsone, rifampicin and cyclophosphamide. The patient also received oral dispirin, dipyridamole, pentoxyfilline, ranitidine and broad-spectrum antibiotics. There was no significant improvement in the ocular or dermatological findings. Six weeks later, he developed hoarseness of voice with haemorrhagic lesions over the vocal cords and genital skin lesions. Oral cyclosporine was then started. The patient showed signs of improvement within a week, with no new skin lesions, disappearance of hoarseness of voice and the penile lesions. The ophthalmic manifestations did not improve. The patient was discharged against medical advice on treatment with oral corticosteroids, antibiotics and anticoagulants only. However, he was re-admitted a week later with fever and difficulty in breathing. He developed septicemia, respiratory distress, acute renal failure and septicemic shock. He required ventilatory support. Three days after admission, the patient expired due to multiple organ failure.

  Discussion Top

Malignant atrophic papulosis is a rare condition. Reported ocular findings include atrophic lesions of eyelids and conjunctiva, telangiectasia of the conjunctival vessels, episcleral plaques, choroidal infarcts, pigmentary choroiditis, optic atrophy, oculomotor nerve palsy, intermittent diplopia and papilloedema. These ocular abnormalities result both from direct ocular involvement and progressive central nervous system involvement.[3],[4] Telangiectasia and microaneurysms of conjunctival vessels with or without atrophic plaques are the most commonly reported ocular findings. Neuro-ophthalmic findings are due to CNS involvement.[4] However, changes like altitudinal or sectoral field defects and optic atrophy can be due to vascular lesions of the optic nerve head.[3] Congenital cataracts,[5] congenital glaucoma due to angle dysgenesis,[3] and retinochoroidal colobomas[3] have also been reported in patients with malignant atrophic papulosis.

Henkind et al[3] reported the histopathological changes occurring in the ocular tissues. Small calibre vessels in the affected areas including episcleral, retinal and optic nerve head vessels showed sub-endothelial fibrosis, endothelial proliferation, narrowing of the lumen and thrombosis. The muscular and adventitial layers were relatively normal. Fibrinoid necrosis was limited to the intima only.

The characteristic cutaneous features include crops of elevated papules, which soon become umbilicated with the depressed center taking on a whitish porcelain tint, with a dry, detachable scale. These "porcelain drops" are the sine qua non of Degos' syndrome. Classically Degos' syndrome is fatal within 2-3 years of onset of systemic involvement. Intestinal perforation with peritonitis[1] is the commonest cause of mortality. The aetiology of Degos' syndrome remains unclear. Various authors have suggested different theories, which includes a viral theory, a genetic predisposition, a focal coagulopathy, a vasculitis or a primary mucinosis.[1],[6],[7] This syndrome has also been reported with AIDS.[8] A thrombotic vasculopathy appears to account for the various manifestations of Degos' syndrome. The diagnosis is based mainly on clinical findings and a skin biopsy.

Ocular findings, though previously reported, are rarely encountered by ophthalmologists. We feel that more documentation of this rare condition and further studies are required to explain the findings in the eye. Considering the encouraging response of the dermatological lesions to cyclosporine, we feel that early treatment with this drug may help mitigate the ocular and systemic disease.

  References Top

Magrinat G, Kerwin KS, Gabriel DA. The clinical manifestations of Degos' syndrome. Arch Pathol Lab Med 1989;13:354-62.  Back to cited text no. 1
Durie BGM, Stroud JD, Kahn JA. Progressive systemic sclerosis with malignant atrophic papulosis. Arch Dermatol 1969;100:575-81.  Back to cited text no. 2
Henkind P, Clark WE. Ocular pathology in malignant atrophic papulosis. Am J Ophthalmol 1968;65:164-69.  Back to cited text no. 3
Lee DA, Su WP, Liesegang TJ. Ophthalmic changes of Degos' disease (Malignant Atrophic Papulosis). Ophthalmology 1984;91:295-99.  Back to cited text no. 4
Howard RO, Klaus SN, Savin RC, Featon R. Malignant atrophic papulosis. Arch Ophthalmol 1968;79:262-71.  Back to cited text no. 5
Kisch LS, Bruynzeel DP. Six cases of malignant atrophic papulosis (Degos' disease) occuring in one family. Br J Dermatol 1984;111:469-71.  Back to cited text no. 6
Katz SK, Mudd LJ, Roenigk HH. Malignant atrophic papulosis (Degos'disease) involving three generations of a family. J Am Acad Dermatol 1997;37:480-84.  Back to cited text no. 7
Requena L, Farina MC, Barat A. Degos' disease in a patient with acquired immuno deficiency syndrome. J Am Acad Dermatol 1998;38:852-56.  Back to cited text no. 8


  [Figure - 1], [Figure - 2]

This article has been cited by
1 Malignant atrophic papulosis: A case report with severe visual and neurological impairment
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2 Degosæ Disease
Cebeci, Z., Tuncer, S., Tugal-Tutkun, I.
Ophthalmology. 2009; 116(7): 1415-1415,e2


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