Increased skin fragility

Introduction Increased skin fragility often occurs in skin porphyria. If it can be protected from light after the onset, the swelling can be eliminated within 2 to 5 days, and the skin fragility is increased. After the stimulation, the line-like epidermis peeling and crusting and the needle-to-sesame-sized worm-like depression scar appear.

Cause

An intermediate product in the synthesis of porphyrin heme. The synthesis of heme begins in the mitochondria of bone marrow and hepatocytes, and is synthesized from the mitochondria rich in glycine and succinate by -aminolevulinic acid (ala) synthetase, and then from bilirubin (pbg). The synthetase is dehydrated to form pbg, a monopyrrole precursor of porphyrin. Pbg is deaminated to form hydroxymethyl bile nucleus, a small part of which is converted into uroporphyrinogen i, and most of them are produced by uroporphyrinogen iii synthase to form urinary porphyrinogen iii, further decarboxylation to produce fecal sputum The porphyrin iii is further oxidized to the protoporphyrinogen ix and the protoporphyrin ix, and then complexed with iron to form heme under the action of ferrous chelatase.

an examination

Related inspection

chromosome

Skin porphyria is reported in China less. According to our statistics, there were only 145 cases from 1953 to 1996, of which epp accounted for 75.2% (109 cases), pct had 28 cases, cep had 3 cases, and pv had 1 case. 1 case. No hc reports.

1. Erythropoietic proto-porphyria (epp) is the most common skin porphyria disease, often with a family history, and is autosomal dominant. The incidence is mostly in childhood, mostly 4 to 10 years old, and adult cases are rare. It is characterized by an acute photosensitivity reaction. After 5 to 30 minutes of sun exposure, the exposed parts of the face and back of the hand have a tingling or burning sensation, followed by patchy swelling and erythema, depending on the intensity and time of the sun, and the upper part of the cheeks and the back of the fingers. The ventral side of the week and fingertips may have ecchymoses or even blistering and nail peeling. If it can be protected from light after the onset, the swelling can be eliminated within 2 to 5 days, and the skin fragility is increased. After the stimulation, the line-like epidermis peeling and crusting and the needle-to-sesame-sized worm-like depression scar appear.

Generally shallower. After repeated episodes over several years, the skin gradually thickens, the face looks like waxy, orange-like nose, pale and thick lips, and the reddish mucous membrane is thick and thick, and is connected with the perioral skin. Radial cracks and scars (Figure 2). Skin thickening of the back of the hand often begins at the metacarpophalangeal joint and the proximal interphalangeal joint, and is knuckle-like. In addition, the back of the hand is even more so on the side of the tiger's side, gradually thickening the moss, and the groove pattern is obvious, showing a pebble paving appearance. The neck is mostly rhomboid skin. Occasionally, skin pigmentation and thickening of the ankle hair. The entire face is weather-beaten old and old.

Differential diagnosis

What should be distinguished from CEP is dystrophic bullous epidermolysis, which is often destroyed, but its occurrence and lesions are related to trauma and collision without photosensitivity, and no red teeth and red urine. . EPF must be differentiated from acne-like vesicular disease, the age of onset, light-sensitive history and family history are similar, but the onset and performance of skin lesions are significantly different from the phototoxic damage of EPP, the main itching and no burning pain, skin The lesions are mainly papules and vesicles without obvious swelling and ecchymosis. The formed acne-like scars and EPP are different in superficial and concave, and the incidence is mostly relieved after puberty without repeated episodes of EPP. Sexual skin thickening performance. Early diagnosis of PCT is often difficult because there is often no clear history of sun allergy. The epidermal erosion of the face needs to be differentiated from artificial dermatitis. The blister and blister lesions should be differentiated from niacin deficiency. The scar and the malignant rash should be differentiated from acquired bullous epidermolysis. When hairy or pigmentation damage is dominant, it needs to be differentiated from related endocrine diseases. The main point of diagnosing PCT is that these above performances should not be isolated and should be considered together.

For patients with suspected skin porphyria disease, porphyrin examination in urine and red blood cells must be performed. The positive detection of porphyrin analysis is decisive for the diagnosis and differential diagnosis of this disease.

Skin porphyria is reported in China less. According to our statistics, there were only 145 cases from 1953 to 1996, of which epp accounted for 75.2% (109 cases), pct had 28 cases, cep had 3 cases, and pv had 1 case. 1 case. No hc reports.

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