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erythropoietic protoporphyrin Hepatobiliary disease appears damage to one case  2017-6-19 19:30


erythropoietic porphyria appear original one case

hepatobiliary damage

journal of dermatology 1998, volume 31 issue 4 case reports

author: li shide zhang rongliang zhuai xia guo peng

units: 277500 zaozhuang city, north tan skin treatment centers (li shide zhang rongliang cubbon); tengzhou city people's hospital of pediatrics (zhu aixia)

patients female, 7 years old. due to the face, hand, foot and back erythema, blisters repeated attacks four years, increasing with fever, abdominal pain, anorexia 15 days, on september 20, 1996 to our hospital. since the spring of 1992 followed by the ministry of the sun, hand, foot and her own erythema, blisters associated with burning, itching, dermatitis according to a local hospital with chlorpheniramine, astemizole, vitamin b6 and a new hydrogen-pine ointment treatment results are poor, the disease when light weight. usually very little outdoor activity and mild, when the sun again after the skin lesions increased again, exposed parts of edema, erythema, blisters and bullae, then erosion, dry, scab, leaving pigmentation and superficial scarring. 15 days the day before yesterday after-sun face, limbs exposed parts appear swollen, purple-red spots, blisters, blood blisters and bullae, with chills, fever, abdominal pain, bloating, and anorexia. no previous history of chronic diarrhea, mental disorders and urine abnormalities, no history of acute and chronic infectious hepatitis, no history of photosensitive drugs and food history, no history of blood transfusion and blood products. parents of non-consanguineous marriage, family, no similar patients.

examination: body temperature 38.6 ℃, conscious, suffering face. axillary, inguinal lymph nodes with mild swelling, tenderness. sclera stained teeth to normal. no abnormal heart and lung, abdominal bulge, liver youlei 8cm, xiphoid 10cm, quality and tough, smooth surface, tenderness, percussion pain liver, spleen under the left rib cage 4cm, no shifting dullness. dermatology: the puffy face, eye, cheek, upper lip purple-red spots, blisters, little erosion and crusting, diffuse pigmentation, and pitting scars, like worm-eaten; swollen limbs, hands that showed a sausage-like , hand, foot and back, forearm, leg diffuse erythema, erosion, bullae, blood blisters, and superficial ulcers of varying sizes, the surface with purulent discharge, ulcers without tenderness to the left leg as the weight (figure 1).

laboratory tests: wbc 6.5 × 109 / l, neutrophils 0.72, lymphocytes 0.28, esr 15mm/1h, hbsag-negative, alt 60u, total protein 48g / l, albumin 26g / l, globulin 22g / l; anti-hav igm, anti-hepatitis c virus, hepatitis c virus rna, anti-hepatitis e virus igm, igg, anti-cytomegalovirus and eb virus antibody were negative. serum total bilirubin 120μmol / l, serum bilirubin qualitative test was positive biphasic response, fetoprotein negative, serum igg, igm, igd, iga and c3, c4 and ch50 were normal. hematoporphyrin qualitative test was positive, qualitative fluorescent erythropoietic porphyria positive, negative characterization of urinary porphyrins. cornea without k-f rings. b-: left hepatic lobe thickness 8.5cm, right lobe thickness 13.5cm, a clear outline of the liver, liver spot density, blood vessels is unclear, the portal vein showed unclear; gallbladder wall thickness, rough double-impact sound through the poor ; spleen thickness of 4.8cm, substantial internal echoes, homogeneous. skin histopathology: subepidermal blister, dermal, papillary layer of the vessel wall and surrounded by a large deposition of amorphous material transparent proteins, pas staining strongly positive.

diagnosis: erythropoietic porphyria primary, secondary jaundice, cirrhosis, chronic cholecystitis.

figure 1 erythropoietic porphyria original clinical

(received :1997 -10-08 revised :1998-01-08)