Studies in Abnormal Human Sensitivity to Light I

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STUDIES IN ABNORMAL HUMAN SENSITIVITY TO LIGHT I. PRTJRIGO AEST1VALIS, ECZEMA SOLARE AND URTICARIA PHOTOGENICA. REPORT OF 17CASESAND REVIEW OF THE L1TERATTJRE STEPHAN EPSTEIN, M.D.' Marshfield, Wisconsin (Received for publication May 6, 1942) Prurigo aestivalis, eczema solare and urticaria photogenica form a special group among those dermatoses which are considered as caused by light. Rela- tively little is known about them. They have received only short mention in most textbooks; few investigators have concerned themselves with experimental studies. The following is a report of clinical and experimental investigations of these conditions which will be presented in four parts. I. Clinical report. II. Experimental study of light sensitivity. III. Passive transfer in prurigo aestivalis. IV. Photoallergic concept of prurigo aestivalis. I The confusion which exists in regard to diagnosis and classification of these conditions warrants a short review of their clinical diagnosis.2 1. Prurigo aestivalis:Thiscondition, also known as chronic polymorphic light eruption, dermatopathia photogenica, erythema perstans solare, was first described by Hutchinson as summer prurigo in 1888. It is characterized by re- current eruptions of prurigo-like papules and nodules on those parts of the body which are exposed to the sunlight, face, neck, arms, hands, jugular triangle and to some extent, the lower legs (Figs. 1—3). The first symptom is burning and itching of the skin followed by erythema with more or less urticaria-like swelling. Combination with urticaria has been observed. The clinical picture is varied and may change in the same patient. The lesions of the face frequently present a papular dermatitis which may resemble lupus erythematosus; in other cases ve- sicular lesions may occur. Prurigo aestivalis usually begins in early spring with the first sunny days and lasts until fall. In some cases, eruptions occur in winter, following exposure to sun. Phases of sensitivity and apparently partial or temporary desensitization may alternate; not infrequently patients are able to tolerate the sun during the hot summer months. Prurigo aestivalis occurs pre- dominantly among the female sex. Usually only one member of a family is affected, but in some instances heredity has been reported. 2. Eczema solace: The term "eczema solare," introduced by Willan, was used by Kaposi generally for eczematoid dermatoses caused by sunlight. Veiel in 'From the Marshfield Clinic, Marshfield, Wis. 'The literature on this subject has been recently reviewed by Blum (a) and by Ep- stein (a). 187 ,1

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FIG. 1.PEUEIGOAESTIVALIS OF FACE AND NECK; CONFINED TO AREAS EKFOSED TO SUN (CASE 5)

FIG.2.PEUEIGOAESTIVALIS OF BACK OF HAND (CASE 5) 188 ABNORMAL SENSITIVITY TO LIGHT 189

1887described under this name a Special type of sunlight dermatitis. His patient presented a combination of urticaria photogenica with more or less acute and papular dermatitis. The cases, which since have been reported as eczema solare or photodermatitis or under similar names, do not constitute a definite group. The only point in common is a more or less acute inflammatory dermatitis, following exposure to the sun. With our present, very incomplete

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Fic. 3. PRUEIGO AESTIVALIS OF NECK; CONFINED TO AEEAS EXPOSED TO AND TANNED BT SUN (CASE 3) knowledge, I believe at least four types of what is called eczema solare may be differentiated theoretically: a) Veiel's eczema solare with its combination with or close relation to urticaria pbotogenica. b) Eczema-like light dermatoses which partially or temporarily present the picture of prurigo aestivalis. c) Solar dermatitis with vesiculation but without combination with urticaria or prurigo. d) Cases which morphologically and etiologically belong to contact dermatitis. 190 THEJOURNAL OF iNVESTiGATIVE DERMATOLOGY

3. Urticaria photogenica: The first detailed report about urticaria photogenica (urticaria solaris) is that of Ward (1905). The number of subsequent reports of this condition is small. The clinical picture in general is quite uniform. Wheals appear at the sites which have been exposed to the light. Short exposure produces erythema, prolonged irradiation urticaria and finally angio- neurotic edema. Most cases represent instances of plain urticaria. Combina- tion of the urticarial eruption with dermatitis has been reported by Duke.

DIFFERENTIAL DIAGNOSIS I do not intend to enter deeply the discussion on the nomenclature of conditions, the etiology of which is so little understood. In these papers the terms prurigo aestivalis, eczema solare and urticaria photogenica will be used in their strict morphological sense to designate a prurigo-like, an eczematoid and an urticaria-like eruption respectively. The classification of typical cases of prurigo aestivalis, eczema solare, and urticaria photogenica is simple. Difficulties are encountered chiefly in differ- entiating prurigo aestivalis from eczema solare and from hydroa vacciniformis. PRURIGO AESTIVALIS AND ECZEMA SOLARE The combination or alternation of prurigo-like and eczema-like eruptions has been noticed by several authors (Barber, Howitt and Knott; Haxthausen; Urbach and Konrad). For this reason Hausman and Haxthausen proposed the name of chronic polymorphic light eruptions to cover all these cases. It does not seem desirable to me that two conditions, morphologically so different as prurigo aestivalis and eczema solare, should be called by one name, even if transitions or combinations of the two types are not infrequent. PRURIGO AESTIVALIS AND HYDROA VACCINIFORMIS This differentiation presents a difficult problem. Typical cases of prurigo aestivalis (Hutchinson's summer prurigo) can be easily differentiated from hydroa vacciniformis (Hutchinson's summer eruption); yet there exists certainly some relation between these two diseases, the nature of which is not understood at the present time. This explains why some authors separate these two conditions as different diseases, whereas others claim that they are identical or at least very closely related. Pick based the differential diagnosis on the following criteria:

HYDROA VACCINIFOSSS&AS (SUMMER ERUPTIUN) PRURIUU AESTIVALIS (SUMSrnE PRURIGO)

1.Begins in early youth Begins at later age 2.Predilection for the male sex Predilection for the female sex 3.Primary lesion: eolliquative vesicle Primary lesion: itchingurticaria-like papule 4.Leaves scars Skin returns to normalcy in winter 5.Frequent itching and burning Always intensive pruritus 6.No scratch effects Always profuse lichenification on account of scratching 7.Relatively frequent hematoporphyrin No hematoporphyrin demonstrated ABNORMAL SENSITIViTY TO LIGHT 191

These criteria can no longer be recognized as valid for the following reasons: 1. About one-third of the cases of hydroa vacciniformis begin before the third year of life and about four-fifths before the fourteenth year. Prurigo aestivalis certainly does not start so frequently in childhood; but all five original cases of Hutchinson's which Pick recognizes as prurigo aestivalis started at the age of from eight to fifteen years; in some cases an even earlier beginning has been reported. 2. Preponderance of the male sex in hydroa vacciniformis is generally believed. This holds true for the familial cases which, however, amount only to about 10% of all observations. In these instances the relation between the sexes appears one to ten in favor of the males (Siemens). Actually there is a preponderance of the female sex; according to Senear and Fink's review of more than eighty cases of hydroa vacciniformis, the female sex leads two to one. 3.Itis now recognized that the primary lesion of hydroa vacciniformis is not a vesicle but of an urtjcarial nature. It is furthermore known that blisters do not appear with all eruptions of hydroa vacciniformis. 4. Mild eruptions and lesions of hydroa vacciniformis frequently heal without scars. On the other hand, scars are by no means rare in prurigo aestivalis. 7. The porphyrin question will not be discussed in detail in this paper as there is no proof as yet that the porphyrins play a causative role in either prurigo aestivalis or hydroa vacciniformis. It may suffice to mention that porphyrins have been demonstrated in cases of both conditions. The relation between hydroa vacciniformis and prurigo aestivalis may he summarized as follows: Typical cases of either condition present a clinical entity, easily recognized; however, there are no single criteria which will allow a hard and fast differentiation.

CASE REPORTS Seventeen cases of prurigo aestivalis, eczema solare, urticaria photogenica and their combinations are presented in table 1.

DISCUSSION AND COMMENTS In general our cases fit in well with those previously reported. A few points will be emphasized. Clinical picture: 11 out of my 17 cases presented symptoms of only one clinical entity, either prurigo aestivalis, eczema solare or urticaria photogenica; 7 had to be classified as combination or transition types; 2 of these showed at various times the characteristics of all three forms of light sensitivity. These combinations occurred too often to be considered a chance result. The frequent associa- tion of ordinary prurigo, eczema and urticaria, so well known, and stressed recently by Zurhelle, lends itself as an analogy and points to common factors. Heredity: In prurigo aestivalis usually only one member of the family is affected; cases of heredity have been reported by Glaubersohn and Goldenberg, HUbner, Hutchinson, and Pick. My series contains one interesting instance of heredity: Mr. L. R. (case 9) suffered from typical eczema solare, his daughter (case 12) from prurigo aestivalis, combined occasionally with dermatitis. Ct TABLE 1 tcl Review of 17 cases of prnrigo aestivalis, eczema solare, urticaria photogenica and their combinations

1 2 3 4 5 6 8 9 10 AGE AT FIRST RESULTSOF TESTS OF LIGHT NO. OF CASE AND APPEAR- DIAGNOSIS LOCATION SEASONAL INCIDENCE RESIARES INITIALS SEX or;1%. AGE SENSITIVITF ERUP- S TION El 15 1.LM.* M Truck 41 30 Frurigo aestivalis Cheeks, neck, jugular March till Fall, occa- Thrapeutic UV treatment Positive family history of C driver triangle, hack of sionallyin winter. to ear producederuption allergy. Passivetrans- El arms after 2 to 3 hours fer negative z 2.B.J.E.* M Garage 51 40 Prurigo aestivalis Left side of facs, back March until Fall Fatient sometimes has owner of hands & extensor "bowel trouble" vs surfaceof lower arms SC 3.G.J.t M Railroad 52 7 Prurigo aestivalis Face, neck and hands Spring and Fall Hypersensitivity to UV. man (fig. 3) Provocation of prurigot z F Teacher 23 22 Prurigo aestivalis Face March until Fall Positive reaction to feath- El 4FF.5 El ers, housedust, tobacco. CA Cleared up in 1941 after S taking torantil. In 1940 same medication without )s.S result El I. A. B.5t M Farmer 35 32 l'rucigoaestivalis Face and exposed parts Spring to Fall, occa- Provocation of prurigo- Delayed reaction to choc- 51 of hands and arms sionally in winter like eruption.f olate, wheat, coru, bar- (see figs. I and 2) ley. No improvement El following elimination El diet 6. E. F.t M Student 14 Prurigo aestivalis Cheeks, nose, lips, be- Early spring, Mar. 1) Immediate pigmenta- Usually less seositive dur- C hind ears; occasion- even Feb. to June. tion ing hot summer months vs ally lesions on arms Reoccurs around 2) Prurigo-like eruptiont C Nov. and heals be- a -1 fore Christmas 7. E. P. F House- 20 Prurigo aestivalis Face Spring and summer Immediate pigmentatiou Patient suffered also from wife followsd uviol filtered cold urticaria UV irradiation. Other- wise normal reaction to mercury and carbon arc lamps S. A. M. A,5 F House- 13 47 Prorigo aestivalis Cheeks and eyelids All year around. Poaitive allergic history wife Spends winter in (urticaria, contact der- Florida matitia). Hysterectomy at age 43. Patient re- covered following treatment with follicnlar hormone 9. L. R.5 M Farmer 50 10 Eczemasolare Face and neck. Other Spring and Fall. Worse Pathologically increased Somewhat less severe in parts also when ex- in Spring; may occur reaction (dermatitis and recent years. Patient posed. Dermatitis in Winter edema) to ultraviolet noticed red color of urine wouldappear on his (sunburn) irradiation attimes hack through hole in shirt 10. H. Sch.t F house- 33 7 Eczema solare Face and arms Previously only its increased sensitivity to Sensitiaation of affected wife Spring. Now occa- unfiltered mercury arc parts ceases allegedly o siunally in Fall light (sunhurn radia- at the cad of May. Ex- tion) t pusureof previouslynon- diseased parts producca an eruption even after that time SI 11. W. E.5 M Student 7 7 Urticaria photogenica) Face and neck Window glass filteredsun- Urticaria appeared three light produced eroption weeks after sulfanilamide medication. Positive personal and family history of allergy. Loss of s< sensitivity after about ten days C 12. E. R.5 F Hosise- 17 12 Prurigo aestivalis at Face and arms; ankles Usually worse in Fall Non affected skin less sen- Rash appeared more fre- work times combined with also when exposed sitive to UV (sunburn quently before men- eczema solare irradiation) than normal struation. Eruption controls disappeared nearly en- tirely after pregnancy. Severity of eruption de- creased with the years. Father (ease 9) suffered from eczema solare 13. Mrs. C. K.5 F House- 47 45 Prurigo aestivalis and Face, jugular triangle May and Fall Sensitivity disappeared wife ecaema solare spontaneously about one year later, Two years later operation on ac- cosint of tb? of ovaries. Passive transfer nega- tivet I-' Cc TABLE 1—Concluded

1 2 3 4 3 9 10 AOE AT FIRST AFFEAR- RESULTS OF TESTS OF LIOHT NO OF CASE AND OCCUFA- DIAONOSIS LOCATION SEASONAL INCIDENCE REMARKS INITIALS SEX nON AOE ANCE OF SFNSITIVSTY EISSJF- TION H Si until No immed. reaction to uls- one summer 14.Mrs. L. E. T.* F HouSe- 50 40 Frurigo aestivalis with Face, arms, legs May Sept. During (1936) Cs wife urticarial swelling filtered mercury arc free of eruptios. Had a lamp; normal delayed suffered from lympho- Si reaction (sunburn) pathia venerea; positive z Frel test. Passive transfer negative Arms and Since Not For 17 and a 15. G. K. F Farmer's 44 21 Prnrigo aestivalis with legs; Spring (March) performed yrs. only legs H wife wheal-like swellings 1935 also face arms affected; later on face also. Positive fam- z ily history of allergy. H Si Passive transfer posi- Cl) tivef H 16. H. 54 F House- 41 4 Prurigo aestivalis, cc- Face, neck and arms Especially spring. I. Immed. urticarial reac- Change in sensitivity in 51 wife zema solare, urticar- May occur any sea- tion following uviol different yrs. In 1926 H is photogeniea son, even winter filtered mercury arc not affected by sun. In irradiation. 1935 more sensitive than Si 2. Pathological delayed ever reaction with unfil- Si tered DV irradiation. Si 3. Developmentof prurigo lesions in sites tested H previouslywith strong a erythema. a 51 17. M. K.t F Stenog- 31 21 Urticaria solare, cc- Face and all not well Chiefly in spring. Oc- Immediateurticarial reac- Repeatedexposure leads to < Fapher zema solare, prurigo covered parts of curs at any season, tion from unfilteredand diminishedsensitivity in body even in winter uviol filtered mercury summer arc lightt

* These cases have besn observed at the Marshfield Clinic, Marshfield, Wis.; the rest at the University Skin Clinic, Breslau (Director: Prof. M. Jessner), The histories and the results of the experimentalstudy of the light sensitivity of these csses will be reported in parts II and III, 'I' his case has been reported previously (Epstein Ed]). ABNORMAL SENSITIVITY TO LIGHT 195

Sex incidence: Most statistics about prurigo aestivalis reveal an outspoken preponderance of the female sex; according to Sarateanu 58 out of 64 cases of the literature since 1920 were female. In my material the ratio of males to females was 5 to 9. Etioiogj: There is no doubt that sensitization to sunlight plays a decisive role in these cases; this aspect will be discussed in part II. Allergic phenomena are also observed; positive, personal or family, histories of allergy have been reported by Beinhauer, Frei, Jausion, Schaumann and Lindholm; Touraine; Tjrbach. Three of my cases (no. 7, 8 and 11) gave personal histories of allergy. Several authors emphasized a negative allergic history in their cases. The results of passive transfer tests, and the discussion of prurigo aestivalis from a photoallergic angle will be given in part III and IV of these papers. I shall mention here only other causal and contributory factors: Several authors consider the causal significance of pathological changes of internal organs or general disturbances. Sellei noticed increased sedimentation rates in three of four cases of prurigo aestivalis and in two cases of eczema solare. Barber, Howitt and Knott found dysbacteria of the intestines; cultures yielded among others enterococci, staphylococcus aureus, streptococcus faecalis, and anaerobe bacteria. Disturbances of hormonal functions were detected by Selisky and Riccioni. Lancaster believes that women with dermatoses due to light lack follicular hormone. One of my patients (case 13) suffered from tuber- culosis of the ovaries and tubes. D'Amato's observation is of interest in this respect: Hypersensitivity to light appeared in a woman at the time of her menarche at age 16; this hypersensitivity disappeared after x-ray castration; 2 years later the menstruation returned and the sun-eruption recurred. Sonek reported quite a series of cases of prurigo aestivalis in patients who suffered from lymphogranuloma venereum. He noticed an increased light reaction at the site of a Frei test. One of my patients (case 14) also presented the history and evidence of an old infection with lymphogranuloma venereum; the Frei test was positive. THERAPY As a whole, the treatment of prurigo aestivalis, eczema solare and urticaria photogenica is not satisfactory as yet; the former apparently more amenable to therapy than the latter two. Numerous procedures have been tried with various results. Desensitization: Increasing doses of ultraviolet rays have been given successfully even in cases due to hypersensitivity to different wave lengths. Murayama treated his patients with good results with their own serum which had been irradiated with ultraviolet rays. Protection against sunlight: Veiel recommended red veils. Ointments and lotions containing substances which absorb ultraviolet, like tannic acid, resorcin, have been used; they have helped considerably in some of my cases. Resorcin internally, 0.15 to 0.25 grams three times a day has been recommended by several authors. I have not been impressed with the results of this treatment in prurigo aestivalis. 196 THE JOURNAL OF INVESTIGATLVE DERMATOLOGY

Hormonal treatment has been used extensively by Selisky with excellent results. Lancaster was satisfied with the results of the treatment of prurigo with fol— licular hormone. One of my patients (case 8) seemed benefited by this therapy as she did not have further attacks in the following years. Similar therapy failed in another case (case 14). Injection of 2000 units of estrogenic substance twice a week gave almost entire relief to Hadley's patient who suffered from urticaria photogenica. A nti-infectious treatment: The elimination of infectious foci is favored by several authors. Barber and collaborators recommend sanitation of pathological intestinal conditions. They prescribe hydrochloric acid in case of hypo- acidity, enemas, laxatives, and especially treatment with autogenous vaccines. Urbach suggests a preparation derived from bacterium coli "Mutafior" of Nissle, furthermore elimination of meat and animal proteins combined with administration of sugar and liver extract as suggested by Schreus and Carrie. Treatment with sulfaguanidin might be indicated where dysbacteria is present as this drug acts chiefly on the intestinal tract; apparently it has not been tried so far. Other procedures: Treatment with histamine and histaminase seemed promis- ing to O'Leary in a few cases of prurigo aestivalis. One of my patients (case 4) cleared up completely a few weeks after she took histaminase (torantil) in 1941. Whether this is just a coincidence or due to the therapy I cannot say; in the previous year the same treatment was not successful. The oral administration of histaminase failed to alter the sensitiveness to light in Arnold's case of urticaria photogenica. It should be remembered that the potency of torantil apparently is not uniform and this might explain the results to some extent. Treatment with gold chloride is recommended by Lomholt and Haxthausen, autohemotherapy and autourotherapy by Jausion, sodium thiosulf ate by others. Ohmori reports a case successfully treated with this drug in combination with external use of resorcin. Very good results with nicotinic acid amide (niacin amide) have been reported recent]y by Civatte,Tzank and Sidi, Keining and Ritter.

SUMMARY 1. 17 cases of prurigo aestivalis, eczema solare and urticaria photogenica are reported. 2. 11 observations deal with simple cases of these conditions, the rest represent transitions and combinations of these forms of light sensitivity. 3. The observations are discussed and compared with the literature in regard to heredity, sex incidence, etiology and therapy. 4. The problem of light sensitivity and of allergic phenomena in these conditions will be discussed in the following papers. References will be found after part IV.