TWO CASES OF EPIDERMOLYSIS BULLOSA

AgnesF. Savill
1906 The Lancet  
86 extremely doubtful if the plague bacillus can be held responsible for the full development of the bubo. In fact, my own experience leads me to believe that buboes are the result of the local growth of the bacillus pestisplus pyogenic bacteria, and the latter by their more active growth cause a full development of the bubo with all its typical pathological appearances and eventually crowd out the actual bacillus pestis itself. Hong-Kong. EPIDERMOLYSIS bullosa is still so rarely seen .that the
more » ... rely seen .that the record of the two following cases occurring in the same family is of interest, especially from a histological and therapeutical point of view. CASE 1.-The patient, a single woman, aged 26 years, was sent to me for opinion on August 2nd, 1905. She had suffered all her life from the formation of bullae, occurring chiefly on those parts of the body exposed to friction or traumatism. She was the youngest member of a family of eight children. Both her parents were healthy and only in the case of a brother (the subject of Case 2) was there a history of any similar skin disease. There was no history of syphilis either as regarded the patient or her family but "consumption" had been present in the family of the father. The condition of the skin was as follows. On the anterior fold of the right axilla was a bulla as large as a shilling ; it was tense and slightly cloudy. Over an irregular area of almost three inches long the anterior axillary fold was reddened and in parts was covered by the drying skin of former bulla3. A similar condition, but less marked, existed on the left anterior axillary fold. Two bullse. each of the size of a sixpenny-piece, were seen on the right arm near the extensor surface of the elbow and one on the inner aspect of the calf. All were over a day old and two had a slight halo of surrounding redness. Fine white scars, remnants of frequently recurring or long standing bullas in former years, existed on various parts of the body either alone, as on the face, or accompanied by brownish pigmentation or redness, as on the legs and axillary folds. The most serious changes existed on the shins. The lower third of both legs was practically covered with brown pigmentation and fine scarring. No varicose veins were present. Over the right shin the pigmented and cicatricial tissue extended over an area five and three-quarter inches long and four inches broad. Small pigment patches were present on the back and at the waist level where bullse had previously occurred Previo1is history of the b2clle.-The skin at birth was healthy. When the patient was three days old the mother noticed a " blister " as large as half-a-crown on one leg, and two days later smaller ones on both eyelids. Then the face and head were constantly affected until the child was six years old. The mother states that as soon as one was healing the child would fall down and knock her face or head and then they started afresh. At one time the mother kept the child's head covered constantly with a bonnet and the hands in gloves, as she observed that blisters always followed knocks and scratching. The face and head had been rarely affected since childhood. At seven years of age the legs began to be constantly affected by bulla3, and ever since that time the bulla; which occurred on the legs lasted the longest and recurred more frequently than on any other situation. The waist was, after the legs, the most often affected until four years ago, when the patient adopted a looser style of clothing. The armpits were affected when the sleeves were tight or the lining thick. The arms were rarely attacked and the hands never. The soles of the feet were not so often involved as the legs. Btil)a3 had occurred a few times in the mouth. Bullae on the face or upper part of the trunk lasted a short time, about a week ; bullse on the legs might last for weeks or months. Walking or standing about caused bulla3 to arise on the legs or soles. Bullse might appear even on parts with cicatricial tissue. The bullae had always been larger and more frequent in summer ; in winter they had sometimes been entirely absent for a week or two. Stout and all forms of alcohol aggravated the condition; the patient did not think that indigestion affected their occurrence in any way, As regarded subjective sensations there was no itching until the bul!a3 were drying up. The patient might be unconscious of the presence of bu1Jae on the upper part of the body, but she might have much pain when they occurred on the foot or on the leg when she had to walk or to stand. She bad, indeed, been obliged to lie in bed for several weeks at a time to hasten the healing of bullea on the legs. Slight pressure did not cause the formation of bul]se, but friction applied for a minute in warm or mild weather would cause a bulla to appear several hours later. No milium-like cysts were present. The nails showed the dystrophy noted in all recorded cases of this disease. All the nails of the fingers and toes were defective and had been so since the patient was three years old. They were raised, ribbed, and discoloured, thickened at the base, pale, brittle, and irregular at the free edge. General health.-The patient had had good general health, though she complained of being readily tired. She perspired freely and readily and suffered greatly from the cold but had never had chilblains. When excited she flushed over a sharply defined patch at the angle of the jaw and side of the neck. The heart and lungs were normal. Menstruation was regular but scanty. The patient had never had scarlet fever or measles. Treatment and subsequent clinical history.-It appeared to me that treatment must be directed to the improvement of the cutaneous circulation and toning up of the small bloodvessels. With that aim the patient was ordered to take four grains of extract of ergot thrice daily. She adhered to this treatment during the whole of the hot month of August and she very definitely stated that she felt stronger and that the bullaa were fewer in number and less in size during that month. She omitted the ergot from Sept. 5th to 12th and during that week she developed more and larger bullæ than she had experienced during all August. From Sept. 12th to 20th she resumed the ergot and reported that she had fewer bull se and " took much longer walks than usual without any bad effect." From Sept. 20th to 27th she had no ergot and when I saw her on the latter date several bullæ had formed. A specimen from a bulla smaller than a threepenny-piece, three days old, with a slight red halo, situated high up on the right calf, was taken for microscopical examination. A photograph of the legs was taken and this showed a tense hæmorrbagic bulla near the left internal malleolus and a flabby cloudy bulla on the sole of the left foot. On Oct. 25th the patient was seen again. She had taken ergot from Sept. 27th to Oct. 15th and had therefore taken no ergot for ten days before Oct. 25th. Only one bulla, however, had arisen since ; this freedom she attributed to the fact that the weather had been dry and cold. On the 28th she developed a feverish cold and cough and was confined to bed for several days. When convalescing during the first days of November she found a bulla starting on the extensor aspect of the right elbow and though she at once took double doses of ergot another bulla developed on the opposite elbow. The weather at the same time, it should be noticed, became warm and damp. When the patient was seen on Nov. 71h she still felt very weakly. The roof of the bulla on the right elbow was taken for microscopical examination. Despite this disappointing recurrerce the patient very decidedly said that since she had been under the present line of treatment she had been better in every way than she had been for years. She had previously given up all hope of improvement of the bullous eruption, having been under the care of many medical men and having attended many hospitals without beneficial result When seen on Nov. 22nd she said she had been free from bullæ until the 20th, when two small ones developed on the toes while she was out walking. From November, 1905, to March, 1906, she continued the use of vascular tonics ; as prolonged exhibition of ergot produced headaches she was only able to take ergot for a week or a fortnight at a time and during the intervals (of similar length) she took nux vomica. Durlng the warmer weeks of May and June 1906, she bad several bullm, some being of considerable size. The nails continued to make progress in appearance. She reported herself as being improved in general health and freer from bulla3 than she had been for years. CASE 2 -The patient (a brother to the patient in Case 1), a married man, aged 44 years, had suffered from the formation of bullea all his life. He was first seen on Oct. 25th,
doi:10.1016/s0140-6736(01)32585-0 fatcat:7y72j7oy4zeixnzjrvglafsdhi