Introduction:
Malignant atrophic papulosis is also known as Kohlmeier-Degos disease or just Degos disease. It is rare in which thrombo-obliterative vasculopathy can affect the skin, gastrointestinal tract, and central nervous system.
There are two forms of the disease stated in the literature. One is the life-threatening systemic form, and the other is the benign monosymptomatic cutaneous form. Thus atrophic papulosis is a better name for the disease than malignant atrophic papulosis.
Atrophic papulosis is further classified as malignant atrophic papulosis and benign cutaneous disease. The characteristic of the disease is papular skin lesions with central porcelain-white atrophy and a surrounding telangiectatic rim which can be considered pathognomonic.
What Causes Malignant Atrophic Papulosis?
The causes of malignant atrophic papulosis are:
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Kohlmeier first described the etiology of the disease in 1941, and it remains elusive.
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Several theories suggest that family members are genetically predisposed to the disease. The inheritance was meant to be autosomal.
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Apart from genetic manifestations, three hypotheses are suggested as the cause of malignant atrophic papulosis coagulopathy, vasculitis, and a primary defect of the endothelial cells. These causes of atrophic papulosis are not mutually exclusive and occur depending on the environment, creating an appropriate condition.
Which Population Is Most Affected by Malignant Atrophic Papulosis?
Atrophic papulosis affects mainly in the second and fifth decades of an individual's life. The occurrence of atrophic papulosis in newborns is found to be very rare. Although there is a slight female preponderance of the disease, it is not explicitly gendered.
What Does Atrophic Papulosis Look Like?
Atrophic papulosis look like the following:
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The lesion shows a wedge-shaped area of the ischemic dermis (restricted blood flow) with a perivascular lymphohistiocytic infiltrate at the edge of the ischemic area.
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There is also a slightly hyperkeratotic and atrophic overlying epidermis.
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Edema and mucin are deposited in the earlier lesion of atrophic papulosis, whereas sclerosis is often present in the later lesions.
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Early lesions of atrophic papulosis resemble lupus erythematosus, and the later lesions resemble lichen simplex chronicus atrophicus.
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Thrombosis may also lead to vascular damage in the base of the lesion.
What Are the Symptoms Seen in Malignant Atrophic Papulosis?
The symptoms of malignant atrophic papulosis are:
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The cutaneous lesions at first appear as small erythematous papules of size 2.0 mm to 5.0 mm on the trunk or the extremities.
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Over 2 to 4 weeks, the lesion gets depressed in the center and gradually evolves into pathognomonic large papules between 0.5 to 1.0 cm. The lesion has a porcelain white center and an erythematous telangiectatic rim around it.
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The scalp, face, soles, and palm rarely involve atrophic papulosis.
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In cases where there is extracutaneous involvement, systemic diseases can develop either suddenly or after many years of cutaneous involvement.
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Cases may report abdominal pain, gastrointestinal bleeding, numbness, tingling sensations, respiratory or cardiac symptoms, and vision changes depending on the organ affected.
How to Diagnose Malignant Atrophic Papulosis?
The diagnosis of malignant atrophic papulosis includes the following:
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The diagnosis of atrophic papulosis depends on finding the pathognomonic skin lesion on the physical examination of the patient and its histopathological evaluation. There are no serum markers or lab abnormalities for atrophic papulosis, so no lab tests are required.
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An organ evaluation is required if the symptoms indicate specific organ involvement. For example, if the gastrointestinal tract is involved, it is essential to have a fecal occult blood test, colonoscopy, laparoscopy, or esophagogastroduodenoscopy.
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In cases of central nervous system involvement, magnetic resonance imaging (MRI) of the brain should be performed.
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In cases affecting the respiratory organs, thorax computed tomography (CT) can be performed.
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In cases of ocular involvement, an ocular fundus examination is suggested.
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In cases of kidney involvement, kidney function tests should be performed.
What Is the Treatment for Malignant Atrophic Papulosis?
None of the treatment procedures are proven effective for treating malignant atrophic papulosis. However, some medications and anticoagulants help to facilitate blood perfusion. Dipyridamole, Pentoxifylline, acetylsalicylic acid, Ticlopidine, and heparin are some drugs that achieved partial regression in cases of skin lesions. Thus these drugs can be considered the first line of treatment in newly diagnosed cases of malignant atrophic papulosis. Eculizumab helps to decrease the C5b-9 membrane attack complex deposition and improves the intestinal lesion but does not affect the systemic progression of the disease. Treprostinil can be given subcutaneously in cases of Eculizumab-resistant malignant atrophic papulosis, which has intestinal and cerebral manifestations. Immunosuppressive therapy and fibrinolytic therapy with cyclosporin A, Azathioprine, or corticosteroids are unsuccessful in treating malignant atrophic papulosis.
As the disease is potentially life-threatening and severe, annual follow-up is mandatory. However, some studies and authors also recommend having a biannual follow-up for the first seven years of malignant atrophic papulosis and then reducing it annually. The follow-up monitoring should include magnetic brain examination, skin examination, gastroscopy, colonoscopy, chest X-ray, and abdominal ultrasound.
Which Diseases Are Similar to Malignant Atrophic Papulosis?
The disease similar to malignant atrophic papulosis are:
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Lupus Erythematosus: It is an autoimmune disorder in which the body's immune system attacks healthy cells and tissues. As a result, it can affect organs like the skin, joints, brain, kidneys, and many more. The symptoms of the disease include muscle and joint pain, fever, rashes, stomach pain, chest pain, fatigue, and headaches.
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Dermatomyositis: It a rare inflammatory disease marked by muscle weakness and a distinctive rash on the skin. The symptoms include a violet-colored or dusky red-colored rash on the eyelids, face, elbows, knuckles, knees, and chest.
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Atrophic Blanche: A pattern of scarring occurs secondary to the clinicopathologic entity known as livedoid vasculopathy. It is a chronic condition that can be recurrent, painful, and develop into ulcers on the lower leg, ankle, and foot dorsal. Atrophic blanche can be present as a white atrophic stellate scar with peripheral telangiectasias.
Conclusion:
Malignant atrophic papulosis is a rare condition of vascular tissues that affects the skin, gastrointestinal tract, central nervous system, and other body organs. The malignant type of atrophic papulosis is life-threatening and needs regular follow-up. The disease's treatment is not yet proven, but the symptoms should be controlled, and follow-ups should be done strictly.
