Overview
Malignant atrophic papulosis, also known as Dego’s disease and Kermiel’s syndrome, is a disease in which the lining of tiny arteries in the skin-intestinal tract or other organs becomes thrombosed.
Etiology
The cause of this disease is unknown, and is thought to be related to autosomal dominant inheritance, autoimmune abnormalities and reduced fibrinolytic activity, and lentiviral infections.
Symptoms
The incidence of the disease is highest in young adults, mostly in the age of 20 to 40 years, and is more common in men. The skin and intestines are usually involved, and the skin often comes first, with only skin damage in about 1/3 of cases. The central nervous system is involved in about 20% of cases, and in rare cases, the eyes, heart, kidneys and bladder may also be involved. Skin lesions are mainly distributed on the trunk and limbs, especially on the back and proximal limbs, and less on the face, hands and feet.
The primary damage is a hemispherical edematous red papule 2 to 15 mm in diameter. During the course of the disease some of the damage persists or absorbs and disappears, leaving small white skin atrophies, while most of the center rapidly necrose and ulcers developed. The remaining porcelain-white patches of skin atrophy are accompanied by grayish-white scales with narrow raised margins, which may be covered with dilated capillaries. Damage occurs in batches, as few as a few and as many as a hundred or more, scattered and less frequently fused. There are usually no conscious symptoms. It may last for months or years.
Intestinal damage occurs between 3 weeks and 10 years after the appearance of skin damage, mostly in the small intestine, large intestine, mesentery, and the stomach is also involved. The disease is insidious, with dyspepsia, diarrhea, or constipation in the early stages, followed by abdominal colic and blood in the stools, and finally by multiple perforations and peritonitis, which have a poor prognosis. Ocular damage is found in the eyelids and conjunctiva, with white avascular spots, ischemic degeneration of the choroid and retina, diplopia, optic disc edema, and optic nerve atrophy.
Examination
1. Physical signs
(1) Skin lesions: light red round edematous papules, occurring on the back of the trunk, etc. The lesions gradually form a central umbilical concavity, atrophy, and a characteristic porcelain-white appearance, with peripheral vasodilatation. The lesions occur in batches and gradually increase. The rash has no obvious discomfort.
(2) Intestinal necrosis As ischemic necrosis occurs in the small intestine, it causes severe abdominal pain, vomiting, diarrhea, and diffuse peritonitis caused by multiple intestinal perforations.
(3) Mucosal invasion Damage to the bulbar conjunctiva of the eye and oral mucosa occurs similar to skin damage.
2. Histopathology
Microarteries and small arteries are characterized by necrosis and occlusion leading to wedge-like necrosis.
3. Other auxiliary examinations
Tissue biopsy, the main lesion is endarteritis of small arteries in the lower dermis and subcutaneous fat tissue.
Diagnosis
Diagnosis can be made according to the etiology, clinical manifestations and relevant examinations.
Differential diagnosis
It should be differentiated from lymphomatoid papulosis, acute acneiform mossy furuncle and allergic cutaneous vasculitis.
Treatment
The main treatment is symptomatic. Indomethacin, aspirin and dipyridamole are usually used. Corticosteroids may be used, but the possibility of intestinal perforation should be borne in mind in advanced stages. Heparin can also be used.
Prevention
Strengthen nursing care and nutrition to improve the patient’s resistance and immunity. In order to prevent infection, isolation should be paid attention to, and contact with pathogens should be minimized. Avoid consanguineous marriages, premarital examination and fertility counseling.