A case of ? epidermolysis bullosa
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Clinical and Experimental Dermatology (1977) 2, 409. Clinical meeting ofthe St John's Hospital Dermatologieal Society: 4 November 1976 A case of ? epidermolysis bullosa PETER GOODWIN* AND ROBIN EADYt * Department of Dermatology, Middlesex Hospital, Mortimer Street^ London wi, and t Institute of Dermatology, Homerton Grove, London E9 Accepted for publication 6 July History Mr M.K. a 46-year-old Cypriot has suffered from a blistering tendency since childhood. Blisters, often provoked by trauma, appeared anywhere on the trunk and limbs and healed without scarring. Since puberty this problem has usually been confined to the legs where some blisters have initiated ulcers that have healed with scarring and pigmentary changes. During a recent hospital admission, for excision and grafting of a well differentiated squamous carci- noma from non-scarred skin on the dorsum of the hand, blistering was provoked by the application and removal of sticking plaster and by chaffing between a bandage and the skin. At the same time multiple large blisters reappeared on both legs. In 1969 Prednisone, 60 mg daily appeared to suppress new blister formation but a relapse occurred when the dose was reduced to 40 mg. There is no known family history of blistering and mucosal surfaces have not been involved. Examination Revealed a healthy male with skin changes confined to the legs which were scaly with scarring and pigmentary changes. There were blisters filled with straw-coloured fluid varying in size from I to 10 cm diameter and several ulcers of varying size (Fig. i). Mucosal surfaces were unaffected. Fingernails were normal, but a dermatophyte was isolated from dystrophic toenails. Investigations (i) Histological examination of a fresh lesion showed a subepidermal blister with numerous 409
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Transcript of A case of ? epidermolysis bullosa
Clinical and Experimental Dermatology (1977) 2, 409.
Clinical meeting ofthe St John's Hospital Dermatologieal Society: 4 November 1976
A case of ? epidermolysis bullosa
PETER GOODWIN* AND ROBIN EADYt
* Department of Dermatology, Middlesex Hospital, Mortimer Street^ London w i , andt Institute of Dermatology, Homerton Grove, London E9
Accepted for publication 6 July
History
Mr M.K. a 46-year-old Cypriot has suffered from a blistering tendency since childhood.Blisters, often provoked by trauma, appeared anywhere on the trunk and limbs and healedwithout scarring. Since puberty this problem has usually been confined to the legs where someblisters have initiated ulcers that have healed with scarring and pigmentary changes. Duringa recent hospital admission, for excision and grafting of a well differentiated squamous carci-noma from non-scarred skin on the dorsum of the hand, blistering was provoked by theapplication and removal of sticking plaster and by chaffing between a bandage and the skin.At the same time multiple large blisters reappeared on both legs.
In 1969 Prednisone, 60 mg daily appeared to suppress new blister formation but a relapseoccurred when the dose was reduced to 40 mg. There is no known family history of blisteringand mucosal surfaces have not been involved.
Examination
Revealed a healthy male with skin changes confined to the legs which were scaly with scarringand pigmentary changes. There were blisters filled with straw-coloured fluid varying in sizefrom I to 10 cm diameter and several ulcers of varying size (Fig. i). Mucosal surfaces wereunaffected. Fingernails were normal, but a dermatophyte was isolated from dystrophictoenails.
Investigations
(i) Histological examination of a fresh lesion showed a subepidermal blister with numerous
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Figure i. Tense blisters arc seen adjacent to an ulcer on the leg. Scarring is also present.
Figure 2. Histology of a fresh lesion showing a sub-epidermal blister with numerous inflammatory cells (neutrophi'Is and eosinophils) present in the upper dermis and lower epidermij
Epidermolysis buUosa 411
Figure 3. Transmission E M showing sub-epidermal blister formation. The roof of the blistercavity (C) is formed by the basal surface of the epidermis (E). The basal lamina (arrows)remains at the floor of the cavity, overlying the dermal connective tissue (D). N, neutrophilC X 4900).
Figure 4. Direct immunofluorescence showing positive IgG basement membrane zoneimmunofluorescence.
412 P.Goodwin and R.Eady
inflammatory cells (neutrophils and eosinophils) present in the upper dertnis and lowerepidermis (Fig. 2).
(2} Transmission electron microscopy (Fig. 3) showed that the roof of the blister cavitywas formed by the basal surface of the epidermis. The basal lamina remained at the floor ofthe cavity overlying dermal connective tissue.
(3) Direct immunofluorescence (Fig. 4) showed positive IgG and fibrin basement mem-brane zone fluorescence. Circulating antibodies were not detected.
Treatment
A trial of azathioprine failed to prevent new blister formation. The dose was increased to200 mg daily and continued for 3 months. Local treatment consisted of drainage of blisterfluid with firm compression bandaging of the legs.
Discussion
Basement membrane immunofluorescence in this patient's skin has to be explained. The longhistory of blistering often provoked by trauma and an unconvincing response to immuno-suppressants favour a diagnosis of epidermolysis bullosa (EB). This histological and electron-microscopical findings would support a diagnosis of EB simplex, but might also suggestpemphigoid. We are unaware of other reports of positive basement membrane immunofluores-cence in EB but feel that it could be secondary to an infiammatory process. On clinical groundswe favour a diagnosis of EB simplex. The usual absence of blisters from sites other than thelegs suggest that this is an unusual localized form of EB.
Acknowledgments
We are grateful to Dr R.H.Meara for allowing us to publish this case and to Dr Martin Blackfor help with immunofluorescence investigations.
