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|Year : 2000 | Volume : 66 | Issue : 5 | Page : 249-250 |
Epidermolysis bullosa pruriginosa
Devakar Yesudia, SGS Krishnan, M Jayaraman, Janaki
Epidermolysis bullosa pruriginosa is a recently described variant of epidermolysis bullosa dystrophica. It is characterised by pruritic nodular prurigo like lesions, milia and with a histopathology of a subepidermal blister. We report 3 cases of this new variant.
Keywords: Epidermolysis bullosa pruriginosa
|How to cite this article: |
Yesudia D, Krishnan S, Jayaraman M, Janaki. Epidermolysis bullosa pruriginosa. Indian J Dermatol Venereol Leprol 2000;66:249-50
|How to cite this URL: |
Yesudia D, Krishnan S, Jayaraman M, Janaki. Epidermolysis bullosa pruriginosa. Indian J Dermatol Venereol Leprol [serial online] 2000 [cited 2014 Mar 6];66:249-50. Available from: http://www.ijdvl.com/text.asp?2000/66/5/249/4936
| Introduction || || |
Epidermolysis bullosa is the term applied to a group of disorders in which the primary feature is the formation of blisters following trivial trauma. Recently, a new variant termed epidermolysis bullosa pruriginosa has been reported. We present three cases belonging to this subtype.
| Case Reports || || |
Case no 1. A 26 -year- old woman presented with pruritic skin lesions on the extensor aspects of forearms and legs since the age of 14 years. Some lesions started as reddish papules while on other sites she developed blisters. Both lesions resolved to give similar pruriginous lesions. On examination, many prurigo like lesions were seen with some showing a white spot at the summit suggestive of milium [Figure - 1] Differential diagnosis of prurigo nodularis and hypertrophic LP was considered. Biopsy showed hyperkeratosis, irregular acanthosis and a subepidermai bulla. Millia were seen in some sections. No therapy was effective.
Case no 2. A 36-year- old man presented with an itchy pigmented rash over all extremities since .I the age of 12. On examination multiple pigmented papules and plaques were seen varying in size from 0.5-1.5 cms. They were discrete and closely set on the shins. All limbs and the scalp were involved.
He also had white papules in the oral mucosa. Some nails on the hands showed dystrophy and discolouration. Histology revealed hyperkeratosis, acanthosis, subepidermal edema with dilated blood vessels and an inflammatory infiltrate in the dermis. Treatment with topical steroids under occlusion showed no improvement.
Case no 3. A 24-year-old woman, first cousin of case no. 2 presented with multiple pigmented pruritic skin lesions over her shins, extensor aspect of arms and dorsa of hands. Some of the lesions started as small vesicles which broke down to heal with these lesions. Examination revealed hyperkeratotic papules and plaques in the distribution mentioned. Histopathology showed subepidermal bullae with milia in several sections.
| Discussion || || |
Epidermolysis bullosa pruriginosa is a newly characterised variant of dystrophic epidermolysis in which patients present with pruritic, lichenified or nodular prurigo-like lesions, occasional trauma induced blistering, excoriations, milia and nail dystrophy. Lesions are most evident on the limbs, particularly the shins. Intact blisters are rarely seen.
Pruritus is the most dominant symptom in these cases. All 3 of our patients had severe itching. Similar cases of E. bullosa with pruritus and lichenified plaques have been reported before. Positive family history was noted in three of the eight cases first described under the title of epidermolysis bullosa pruriginosa. Two of our cases were related giving evidence of a possible genetic aetiology.
Histopathology of the lesion reported from the original article showed hyperkeratosis, mild acanthosis, disruption of the dermoepidermal junction and frank subepidermal blister formation in some areas. Moderate perivascular lympho histiocytic inflammatory infiltrate was seen. Milia were observed in several sections. Our cases showed all these features. Ultrastructurally, there were alterations in the number and structure of anchoring fibrils in the lesional and perilesional skin consistent with a diagnosis of dystrophic epidermolysis bullosa. We did not perform EM studies in our cases.
The unique combination of lichenified plaques and papules preceded by blisters and histopathology of subepidermal bulla with milia is very suggestive of EB pruriginosa. However, electron microscopy is needed for a definitive diagnosis. We recommend that this be added to the differential diagnosis of lichenified plaque on the shin, even though it is at present a rare condition.
Figures [Figure - 1]