Malignant atrophy papules disease and intestinal perforation, perimenitis 1 case.

Clinical data patient male, 54 years old, 3 years ago the double lower limbs appeared several red papules, no itching, pain, and then spread to the whole body, with the torso and limbs as the main, without treatment.
no obvious cause of bloating or abdominal pain 5 months ago.
had been in a local hospital, diagnosed with "perimenitis", to give treatment, abdominal pain alleviated discharge.
3 months ago, the patient again appeared bloating, abdominal pain, admitted to the hospital gastroenterology surgery treatment, diagnosed as: abdominal pain check-up.
discharged from the hospital after being given relief from the symptoms of treatment.
3 d before the patient again appeared abdominal pain accompanied by fever, in the local hospital according to "periperitis" to give treatment, poor effect, after emergency admission to the hospital, fever, T39.6 degrees C, emergency diagnosis to give treatment after the treatment into the digestive department, diagnosis: abdominal pain, digestive tract perforation? Give Reberalazole sodium intestinal capsules and digestive hemolytic drugs, nutrition drugs and other treatment.
patients deny the history of hepatitis, tuberculosis, heart disease and other chronic diseases, denying the family genetic history associated with the disease.
Physical examination: divine, spiritual, T 38 degrees C, P 95 times / min, R 19 times / min, BP 125/80 mmHg, painful face, mid-abdominal pressure, anti-jumping pain, abdominal muscle tension, abdominal wall varicose veins, no gastrointestinal type, no peristaltic waves, no touching of the envelope.
the mobile turbid tone is negative and the intestinal tone is weakened.
Dermatology situation: torso, limbs multiple diameter 3 to 13 mm round, round red papules, the center is porcelain white umbilical cord-like atrophy depression, edge slightly bulging, part of the papules covered with scales, the outer hair thin blood vessels dilated (Figure 1).
laboratory examination: blood routine, urine routine, liver and kidney electrolyte, blood sink, C reaction protein are normal.
fiber protein 4. 16 g/L (2 to 4 g/L), D-g/L, D-gluten 1.132 mg/L (0 to 0.3 mg/L).
the standing abdomen flat piece shows: intestinal gas, right under the shin gas, consider intestinal perforation.
CT shows: double pneumonia, double-sided pleural cavity fluid and double lung expansion incomplete, abdominal cavity, pelvic fluid, left kidney stones, left kidney cyst, liver small cyst, abdominal cavity part of the small intestine wall thickening, abdominal cavity intestinal membrane more hair slightly lymph nodes.
examination showed: rising colon ulcers, rectal mucosa many places congestion, decay.
of the colon, cross-colon tissue pathology: mucosal chronic inflammation.
patients in hospital because of torso, limb rash dermatology consultation, consider "malignant atrophy papules disease?", the establishment of the organization of pathology examination.
dermatography performance: skin damage peripheral main branch-like blood vessels, carcinogenic blood vessels and irregular blood vessels, the central shape of irregular white unstrucular area (Figure 2).
Fore chest loss pathological examination shows: epiderm diagonalization excessive coliformization incomplete, the lower echiculated layer thinning, substrate cell liquefaction denaturation, derma shallow part collagen wedge transparent denaturation, a small number of lymphatic cell immersion around blood vessels, collagen arrangement disorder (Figure 3), special staining: AB-PAS (-) (Figure 4), diagnosed as malignant atrophic papulopathy.
was given aspirin intestinal tablets (100 mg, 1 time/d), Pan Shengdin (50 mg, 3 times/d) treatment, discharged from the hospital for 1 month follow-up, stable condition, after the patient lost the visit.
2 Discussion of malignant atrophy papules is a rare vascular disease of unknown origin that often affects multi-system organs and causes in vivo thrombosis or ischemicemia, as first described by Degos in 1942, and is also known as Degos disease.
This disease clinical can be divided into skin-type malignant atrophy papules disease and system-type malignant atrophy papules disease two kinds, skin type about 1/3, only the performance of skin lesions and no systematic lesions, can survive for more than 14 years.
systemic malignant atrophy papules often affect multi-system organs within weeks to several years after the rash appears, with a high fatality rate.
the cause of the disease is currently believed to be related to viral infections, autoimmune abnormalities, blood clotting function abnormalities, etc. , skin loss is mostly distributed in the torso and limbs, early red spots or red papules, and then gradually developed into a central atrophy of the umbilical depression of porcelain white papules, the outer side of the red embankment and accompanied by the expansion of the capillary tube.
MAP's tissue pathology is manifested as early derepkin shallow layer and deep blood vessels around the inflammatory cell immersion, typical damage is the derosaur shallow layer to deep wedge necrosis, infarction site substrate of the small arterial wall inflammatory cell immersion, can have thrombosis, late epiderm shrinkage thinning, derial collagen hardening, derapiosis area mucosal protein deposition.
MAP skin mirror performance is rarely reported in literature, skin mirror performance is related to pathological changes, leather collagen degeneration and necrosis corresponding to white unstructured areas, peripheral capillary tube dilation corresponding to vascular structure.
dermatological performance of patients in this case: skin damage peripheral main branch-like blood vessels, carcinogenic blood vessels and irregular blood vessels, the shape of the central irregular white unstrucular area.
When the gastrointestinal tract is affected by severe abdominal pain, bloating, vomiting, periperitis, intestinal perforation, intestinal obstruction, etc. , and when the central nervous system is affected, it can be shown as meningitis, cerebral thrombosis, spinal corditis, limb and facial abnormalities; Pleuritis can be seen when the lungs are affected, and upper lid sagging, optic nerve inflammation, double vision, squinting, paralysis of the eye muscles, etc. can be seen when the outer nerves are affected.
common causes of death are intestinal perforation, perimenitis, cerebral hemorrhage.
This case of patients rash 3 years, since the disease many visits, did not pay attention to skin performance, only to give treatment for the disease, this visit to the rash as a diagnostic clue, combined with skin damage tissue pathology examination diagnosis as a systematic MAP, suggesting that repeated abdominal pain, peripiditis patients should pay attention to the accompanying skin damage, as early as possible clear diagnosis, timely treatment.
MAP can be based on typical skin loss and dermatological tissue pathology results to make a diagnosis, skin loss should be noted with allergenic vasculitis, acute acne-like moss-like rash and lymphoma-like papules disease identification.
The main manifestations of allergenic hemorrhage tubeitis are the lower limb-based tentacles, blisters, necrosis and ulcers and other polyformed rashes, can appear digestive tract symptoms, pathologists should be shown as vascular endocutral cell swelling, vascular wall fibroprotein seepage, degeneration and necrosis, red blood cell spillage, vascular wall and surrounding white blood cells soaked and accompanied by nuclear fragmentation, combined with clinical characteristics and pathological examination, this disease is easier to identify.
Acute acne-like moss-like rash to the whole body repeated erythema, papules, blisters, necrosis and knots as clinical manifestations, after the remaining acne-like scarring, pathological signs of keratin neutral granulocyte abscesses, epiderma endometriosis cell necrosis, substrate cell liquefament and dermatitis around the change.
Lymphoma-like papules are manifested in recurrent torso, limb-based papules, noumps, ulcers, necrosis and colons, pathological manifestations of a large number of lymphocytes soaked in the dermis, visible changes in lymphocytes, can have pro-epidermis phenomenon.
acute acne-like moss-like rash and lymphoma-like papules do not combine digestive tract symptoms, and skin damage and pathology are different from MAP, and MAP is easier to identify.
There is currently no effective treatment plan for malignant atrophic papules disease, mainly for the treatment of anticoagulant and anti-platelet therapy: such as aspirin, methyn, bithane damo, hexone cocoa base, chlorpyridine, huafarin and heparin, immunosuppressant treatment: thiopental, methotrexate, cyclosporine A; Guo and others have reported that 1 case of children in the routine application of aspirin on the basis of the application of glucostrogen, while intravenous injection of immunoglobulin, after the condition is stable.
Eeulizumab is a drug used to treat thrombosis of microvascular disease, and Eeulizumab has been documented to be effective in relieving symptoms when treating MAP.
This patient was treated with aspirin intestinal tablets (100 mg, 1 time/d), Pan Shengding (50 mg, 3 times/d), followed up 1 month after discharge, the patient's condition was stable, and the patient was visited.
In summary, according to the torso and limbs near the end of the main, multiple, central depression, surrounding bulging with red-hot porcelain white papules, combined with tissue pathology examination can diagnose MAP, such as the emergence of perimenitis, intestinal perforation and other systemic symptoms, can diagnose system-type MAP, at this time the prognosis difference, high mortality rate.
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