|Year : 1991 | Volume
| Issue : 5 | Page : 240-241
Pretibial epidermolysis bullosa with intraepidermal split
Sanjay Singh, AK Jha, Mohan Kumar, SS Pandey
Histology of a 26 year old woman with pretibial epidermolysis bullosa (PEB) revealed intraepidermal split. Her son had dystroplaic epidermolysis bullosa.
Keywords: Pretibial epidermolysis bullosa
|How to cite this article:|
Singh S, Jha A K, Kumar M, Pandey S S. Pretibial epidermolysis bullosa with intraepidermal split. Indian J Dermatol Venereol Leprol 1991;57:240-1
|How to cite this URL:|
Singh S, Jha A K, Kumar M, Pandey S S. Pretibial epidermolysis bullosa with intraepidermal split. Indian J Dermatol Venereol Leprol [serial online] 1991 [cited 2013 May 21];57:240-1. Available from: http://www.ijdvl.com/text.asp?1991/57/5/240/3693
Very few cases of PEB have been re ported,,., The clinical and pathological manifestations of this disorder remain unclear. The delayed age of onset (between 3 and 24 years) contrasts with other forms of hereditary EB,,, PEB has been described as an auto somal dominant, scarring type of EB in which light microscopy reveals subepidermal split.
| Case Report|| |
A 26-year-old Indian woman had a 20 year history of blistering eruption on pretibial regions and lower lateral part of thighs. The blisters appeared every time after minor trauma. Her nails, teeth and hair were normal. Bilateral erythematous plaques and erosions were seen [Figure - 1]. There were no milia_ Histology showed intraepidermal split [Figure - 2]. Rubbing the pretibial area with a cotton swab for about a minute induced a blister, the histology of which was the same.
Her 6-year-old son had blistering eruption since birth. The blisters were precipitated by minor trauma and were present over whole body mainly on the extensor surfaces except mucosae, face, palms and soles. Alongwith blisters, erythematous indurated plaques and milia were seen. All his nails were dystrophic. Teeth and hair were normal. Histology revealed subepidermal bulla. Except these two patients all other family members and both maternal and paternal relations were normal [Figure - 3].
Routine investigations like complete blood count, liver function tests, total serum proteins, albumin globulin ratio, serum electrolytes, urinalysis, serum creatinine, VDRL test, chest X-ray and electrocardiogram were normal in both patients.
| Comments|| |
All cases of PEB reported in the literature showed subepidermal blistering,,, thus putting them in dystrophic category. This case with features of PEB showed intraepidermal split. Ultrastructural studies are of no more help than in light microscopy in differentiating between simple (intraepidermal split) and dystrophic (subepidermal blister) forms of EB. The reasons, for reporting this case are, (1) this appears to be the first report of PEB with intraepidermal split, (2) scarring had developed at the site of some blisters, simple EB is said to be nonscarring and (3) patient's son had dystrophic EB.
| References|| |
|1.||Garcia-Perez A and Carapeto FJ : Pretibial epidermolysis bullosa : Report of two families and review of literature, Dermatologica, 1975; 150 122-128. |
|2.||Portugal H : Symmetrische Bullosis der unteren extremitaten, Haut, 1959; 10 : 170-173. |
|3.||Jones R R : Epidermolysis bullosa : Report of a family and discussion of the dominant dystrophic types, Clin Exp Dermatol, 1979; 4 : 303-308. |
|4.||Koike R, Mikoshiba H, Ikegawa S et al : Pure form of pretibial epidermolysis bullosa : Report of a fame ily, Rinsho Dermatol, 1984; 38 : 313-319. |
|5.||Furue M, Ando i, Inoue Y et al : Pretibial epidermolysis bullosa : Successful therapy with a skin graft, Arch Dermatol, 1986; 122 : 310-313. |
|6.||Pye R J : Bullous eruptions, in : Textbook of Dermatology, Fourth ed, Editors, Rook A, Wilkinson DS, Ebling FJG et al : Oxford University Press, Bombay, 1987; p 1619-1663. |
[Figure - 1], [Figure - 2], [Figure - 3]