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The effect of certain types of trauma in epidermolysis bullosa
THE
EFFECT
OF
CERTAIN
TYPES OF TRAUMA BULLOSA
CLAUDIA t~. SI3/[0N, CINCINNATI,
H E differential diagnosis of bullous infants and children is often difficult. Any diagnostic aids which may help should be of considerable interest. A study of the various types of trauma able to evoke a bullous reaction in epidermolysis bullosa has been made. Little is known about the mechanism of bul]ae formation in epidermolysis bullosa. Engman and Mook1 were the first to believe that a developmental
T disorders in
Fig. 1 . - - D y s t r o p h i c epidermolysis bullosa. E p i d e r m o l y s i s produced by r u b b i n g with tongue blade.
M.D.
0HIO CASE REPORT
The patient was K. W., a one-yearold white female, with typical lesions of the dystrophic type of epidermolysis bullosa, present since birth. The patient had generalized lesions involving skin and mucous membranes and presented serous and hemorrhagic bullae, denuded and cr~usted lesions, scarring, and nail involvement. She was treated with pregnenSlone 100 mg. every day, which resulted in some improvement.
Fig.
2.--Epidermolysis produced most stantly by adhesive application.
con-
The following types of trauma were applied to skin areas of the back, which at the time were free from visible lesions. The patient did not receive any therapy during the performance of this procedure. 1. Multiple light tapping with a percussion hammer produced immediate, severe, and prolonged erytheIna. If the tapping was continued, denudation of the skin occurred. 2. Scraping with a tongue blade also of D e r m a t o l o g y and produced denudation of the skin (Fig. Medicine, U n i v e r s i t y 1). 750
defect of elastic tissue is present. But this has not been confirmed by other investigators.2, 3 The following is a description of various types of trauma which will produce lesions in a patient with epidermolysis bullosa, and again demonstrates a lack of cohesive force of the skin in this disease. From the Department Syphilology, College of of Cincinnati.
IN EPIDERMOLYSIS
SIMON :
EPIDERMOLYSIS BULLOSA
3. Application of adhesive tape resulted in a stripping off of the top layers of the skin, so that they adhered to the tape when it was removed (Fig. 2). This procedure was the most constant, as it could be reproduced over nearly the entire skin surface. 4. Multiple pinpricks produced minimal erythema, but no other lesions. 5. Intraeutaneous injection of 0.02 c.e. of hyaluronidase produced no abnormal reaction in the skin. A control injection of 0.02 e.c. of saline also did not produce any lesions. LeonP has made a somewhat similar s t u d y in epidermolysis bullosa simplex and concluded that the p r i m a r y disturbance in this disease resulted from a defect in elastic tissue development and possibly a hypersensitivity of cutaneous capillaries. These techniques of a t r a u m a test were applied to two other cases of epidermolysis bullosa with similar results, to clear skin of an infant with R i t t e r ' s disease with negative results , to generalized impetigo in two y o u n g chil-
751
dren with negative results, and also to a case of severe poison ivy dermatitis with equally negative results. CONCLUSIONS
T r a u m a from the pulling u p of a strip of adhesive tape has been found to produce u n i f o r m l y epidermolysis in two cases of epidermolysis bu]losa. This simple trauma test may be of help in the differential diagnosis of bullous diseases of the skin in infants and children. REFEREN
CES
1. Engman, M. F., and lViook, W. I-I.: A Study of Some Cases of Epidermolysis Bullosa With Remarks Upon Congenital Absence of Elastic Tissue, J. Cutan. Dis. 24: 55, 1903. 2. Allen, A. C. : Survey of Pathologic Studies of Cutaneous Diseases During World War II, Arch. Dermat. & Syph. 57: 19, 1948. 3. Guy, W. ~I. : Epidermolysis Bullosa, Arch. Dermat. & Syph. 15: 30, 1927. 4. Leoni, A.: Investigations of 1V[echanism of Bleb F o r m a t i o n in Epidermolysls Bu]losa Simplex, Ann. dermat, et syph. 10: 501, 1950.
EFFECT
OF
CERTAIN
TYPES OF TRAUMA BULLOSA
CLAUDIA t~. SI3/[0N, CINCINNATI,
H E differential diagnosis of bullous infants and children is often difficult. Any diagnostic aids which may help should be of considerable interest. A study of the various types of trauma able to evoke a bullous reaction in epidermolysis bullosa has been made. Little is known about the mechanism of bul]ae formation in epidermolysis bullosa. Engman and Mook1 were the first to believe that a developmental
T disorders in
Fig. 1 . - - D y s t r o p h i c epidermolysis bullosa. E p i d e r m o l y s i s produced by r u b b i n g with tongue blade.
M.D.
0HIO CASE REPORT
The patient was K. W., a one-yearold white female, with typical lesions of the dystrophic type of epidermolysis bullosa, present since birth. The patient had generalized lesions involving skin and mucous membranes and presented serous and hemorrhagic bullae, denuded and cr~usted lesions, scarring, and nail involvement. She was treated with pregnenSlone 100 mg. every day, which resulted in some improvement.
Fig.
2.--Epidermolysis produced most stantly by adhesive application.
con-
The following types of trauma were applied to skin areas of the back, which at the time were free from visible lesions. The patient did not receive any therapy during the performance of this procedure. 1. Multiple light tapping with a percussion hammer produced immediate, severe, and prolonged erytheIna. If the tapping was continued, denudation of the skin occurred. 2. Scraping with a tongue blade also of D e r m a t o l o g y and produced denudation of the skin (Fig. Medicine, U n i v e r s i t y 1). 750
defect of elastic tissue is present. But this has not been confirmed by other investigators.2, 3 The following is a description of various types of trauma which will produce lesions in a patient with epidermolysis bullosa, and again demonstrates a lack of cohesive force of the skin in this disease. From the Department Syphilology, College of of Cincinnati.
IN EPIDERMOLYSIS
SIMON :
EPIDERMOLYSIS BULLOSA
3. Application of adhesive tape resulted in a stripping off of the top layers of the skin, so that they adhered to the tape when it was removed (Fig. 2). This procedure was the most constant, as it could be reproduced over nearly the entire skin surface. 4. Multiple pinpricks produced minimal erythema, but no other lesions. 5. Intraeutaneous injection of 0.02 c.e. of hyaluronidase produced no abnormal reaction in the skin. A control injection of 0.02 e.c. of saline also did not produce any lesions. LeonP has made a somewhat similar s t u d y in epidermolysis bullosa simplex and concluded that the p r i m a r y disturbance in this disease resulted from a defect in elastic tissue development and possibly a hypersensitivity of cutaneous capillaries. These techniques of a t r a u m a test were applied to two other cases of epidermolysis bullosa with similar results, to clear skin of an infant with R i t t e r ' s disease with negative results , to generalized impetigo in two y o u n g chil-
751
dren with negative results, and also to a case of severe poison ivy dermatitis with equally negative results. CONCLUSIONS
T r a u m a from the pulling u p of a strip of adhesive tape has been found to produce u n i f o r m l y epidermolysis in two cases of epidermolysis bu]losa. This simple trauma test may be of help in the differential diagnosis of bullous diseases of the skin in infants and children. REFEREN
CES
1. Engman, M. F., and lViook, W. I-I.: A Study of Some Cases of Epidermolysis Bullosa With Remarks Upon Congenital Absence of Elastic Tissue, J. Cutan. Dis. 24: 55, 1903. 2. Allen, A. C. : Survey of Pathologic Studies of Cutaneous Diseases During World War II, Arch. Dermat. & Syph. 57: 19, 1948. 3. Guy, W. ~I. : Epidermolysis Bullosa, Arch. Dermat. & Syph. 15: 30, 1927. 4. Leoni, A.: Investigations of 1V[echanism of Bleb F o r m a t i o n in Epidermolysls Bu]losa Simplex, Ann. dermat, et syph. 10: 501, 1950.