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2003, Number 1

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Rev Mex Pediatr 2003; 70 (1)

Epidermolysis bullosa. Clinical review

Miranda-Gómez A, Frías-Ancona G, Hierro-Orozco S
Full text How to cite this article

Language: Spanish
References: 11
Page: 32-36
PDF size: 95.14 Kb.


Key words:

Epidermolysis bullosa, blisters and vesicles in early childhood.

ABSTRACT

Epidermolysis bullosa is a group of inherited disorders of the epithelial basement membrane zone, mainly presenting during early childhood. The most important clinical manifestation, is the presence of blisters and vesicles over the skin and the mucous membranes, specially after direct minor trauma and friction. Is very difficult to make a punctual diagnosis between epidermolysis subtypes; however, in this review we present the three main types epidermolysis bullosa. We explain for each one topography, morphology, histologic affection level, mode of inheritance, existing mutations, and differential diagnosis. Finally we described the best therapeutic options for these kind of diseases.


REFERENCES

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  2. Hovnanian A, Christiano AM, Vitto J. The molecular genetics of dystrophic epidermolysis bullosa. Arch Dermatol 1993; 129: 1566-9.

  3. Bergman R. Immunohistopathologic diagnosis of epidermolysis bullosa. Am J of Dermatopathol 1999; 21: 185-92.

  4. Epstein EH Jr. Molecular genetics of epidermolysis bullosa. Science 1992; 256: 799-804.

  5. Smith LT. Ultrastructural findings in epidermolysis bullosa. Arch Dermatol 1993; 129: 1578-84.

  6. Leigh IM, Lane EB. Mutations on the genes for epidermal keratins in epidermolysis bullosa and epidermolytic hyperkeratosis. Arch Dermatol 1993; 129: 1571-7.

  7. Lim KK, Su WPD, McEvoy MT, Pittelkow MR. Generalized gravis junctional epidermolysis bullosa: case report, laboratory evaluation and review of recent advances. Mayo Clinic Proc 1996; 71: 863-8.

  8. Marinkovich MP. The molecular genetics of basement membrane diseases. Arch Dermatol 1993; 129: 1557-65.

  9. Horn HM, Tidman MJ. The clinical spectrum of epidermolysis bullosa simplex. Br J Dermatol 2000; 142: 468-72.

  10. Luke MC, Darling TN, Hsu R, Summers RM, Smith JA, Solomon BI et al. Mucosal morbidity in patients with epidermolysis bullosa acquistica. Arch Dermatol 1999; 135: 954-9.

  11. Fine JD. Management of acquired bullous skin diseases. N Engl J Med 1999; 333: 1475-84.




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Rev Mex Pediatr. 2003;70