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Benign Chronic Bullous Dermatosis of Childhood." Are These Immunologic Diseases?

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Benign Chronic Bullous Dermatosis of Childhood. THE J OUItNAL OF INVEST IGATIVE DERMATOLOGY. 65:447-450, 1975 Vol. 65, No.5 Copyrig ht © 1975 by The Willia ms & Wilkins Co. Printed in U.S.A. JUVENILE DERMATITIS HERPETIFORMIS VERSUS "BENIGN CHRONIC BULLOUS DERMATOSIS OF CHILDHOOD." ARE THESE IMMUNOLOGIC DISEASES? TADEUSZ P. CHqRZELSK I, M.D., STAFANIA JABLONSKA, M .D ., ElmsT E. BEUTNER, PHD., EWA MACIEJOII'SI( A, M.D., AND MAlliA JAIlZAI3EI\ - CI-IOIlZELSKA , PHD. Department of Dermatology, Warsaw School of M edicine. Warsaw, Poland, and Department of Microbiology, State University of N ew York at Buffalo, Buffalo, N ew York , U. S. A. Seven cases of juvenile dermatitis herpetiformis have been investigated. Immunofluores­ cence a nd histologi c studies were made in all and jej unal biopsies in three. Immunopathologic results were positive in all cases including one that had previously been reported to be negative. Two groups could be distinguished according to clinical a nd histologic criteria, response to sulfapyridine, and character of the immunoglobulin depOSits. The first corresponded to dermatitis herpeti['ormis (DH) of adults, with characteristic lesions of the jejunal mucosa; the second corresponded either to bullous pemphigoid (BP), although in the majority of the cases without circulating anti basement-membrane antibodies, or to a mixed type with the combined features 0[' DH and BP. Repeated biopsies with seri al sections are essential for demonstrating immune depo its. The question arises whether any immunologically negative cases of " benign chronic bullous dermatosis of childhood" actuall y exist. In a previous paper (1] we have noted that informa tion was contributed by repeated immunologic . venile dermatitis herpetiformis (JDH) is a het­ investigations a nd inte tinal biopsy. For exa mple, in one ~~ogeneous synd~ome. On t~e st ~en~th of clinical, case (B.K.) with chronic vesicul ar eruptions that had )1. 'stologic, and Immunologic cntena as well as been studied previously 11 j, 30 consecutive sections of to sulfapyridine and sulfones, JDH may one s kin biopsy speci men were negative for [g Ao While ~sponse the same was true of the first 8 sections of anot her ~e classified either as dermatitis herpetiformis s pecimen of this patient, the next 5 sections had gra nula r (DB) or bullous pemphigoid (BP). In 3 of 8 cases, deposits of IgA characteristic of DH. Thus, in other cases ' mmunofluorescence (IF) studies gave negative additional IF examinations were made if initial s tudies of ~esults , so these cases could be considered beni gn skin biopsies yielded negative findings. hronic bullous dermatosis of childhood [2- 4]. The The cases were divided into those corresponding to DH ~xistenCe of clinical, histologic, and immunologic or BP according to clinical criteria (in DH, characteristic ~ ap of childhood has been confirmed (4- 6 ]. Cases small vesicle and papules in typical sites), hi tologic corresponding to DH of adults have also been features (in DH, microabscesses in derma l papillae in the neighborhood of lesions), a nd response to Sulfapyridine described [7- 9], but without imm .un~patholo g ic and/or sulfones (dramatic in DH). Intestinal biopsies confirmation of granular IgA depOS its III the der­ were obtained in 4 cases. mal papill ae. Serum and tissue [F studies were made in each case. In the present paper we report further observa- The methods were those we have previously described tions of cases that correspond to typical DH or BP [10 ,11 j. The cha racteristics of the conjugates used are f adults or combine the clinical, histologic , and desG,ribed in Figures 1 a nd 6. ~mm unologic features of both. We have found that I case may be negative in a single IF study but may RESULTS ~e positive. when the I~ t.est is rep~ated ; t.he The relation of IF studies to clinical and histo­ difficulties III demonstratmg lInmunolog lc depOSits logic findings as well as sulfone responses are tnay be a .source of the in~onsiste.ncy in classi.fica­ recorded in Tables I and II. Table I detail 2 cases, tion withIll the group of chrol11C nonhereditary which meet all the criteria for DH. IF studies I:mUous diseases of childhood [4] . demonstrated exclusively IgA deposits in the unin­ volved skin and none in the lesions (Fig. 1) . Jejunal MATERIALS AND METHODS biopsies were typical of DH, showing flattened Seven cases, including one that was covered in the mucosal villi. previoUs paper [1], were studied. Significant diagnostic In Table II are summarized the findings in 5 cases which corresponded more to BP a lthough This research was s upported by a grant from the Polish they also showed some clinical features of DH Academ:y of Sciences, 09.32.10, and the Summer Hill (Figs. 2-4). Hi tology was of the BP type: the foundatiOn. blisters were subepidermal (Fig. 5) and revealed no Reprint requests to: Dr. T. P. Chorzelskl , Department of Dermatology, Warsaw School of Medicine, 02-008 microabscesses. Response to sulfapyrid ine was not Warsaw, Poland. apparent. 447 448 CI'IORZELSKI ET AI. Vol. 65, No.5 TABLE 1. Cases of adu.lt dermatitis herpetiformis type Character of IF Studies skin les ions Histopathol- Response Duration Biopsy ogy (micro- to sulfa- Jejunal Patient Age of the Large py ridin e (years) disease Adu lt abscesses biopsy (years) bull ae in dermal or Serum Ski n Unin vo lved DH of BP papillae) sulfones lesions skin type type IgG IgA IgG IgA B.K. 9 4 + - + + + - - - - ++ '< grade gra nula r III M.P. 5 1 + + + + + - N .D ." N.D . - ++ '< occasion - gra nula r a ll y " N.D. Not d one TABLE II . Cases with features of buliolL S pemphigoid or m ixed forms (BP- DH type) Character of IF studies skin lesions Dura - Histo path- Response tion ology (m ic ro- to sulfa- Biopsy Patient. Age J ejunal (years ) of the Adu lt Large abscesses pyridine biopsy dis- bull ae in dermal or Serum Skin Uninvolved ease DH ofBP papillae) sulfones lesions skin type type IgG IgA [gG IgA W .J. '< 6 severa l + - - or " N.D ! 640,320,80 +++ - +++ - mont h; S . K. 4 1.5 y r - + - - N.D . - ++ N .D . ++ - Z. W. d 7 several - + - - sli ght - + +/++ ++ ++ days fl a t tening of villi in som e places R. W. 2 ca I y r - + - - or normal - ++ +++ +++ ++/+++ W.S. d to severa l + + + + N.D . - - + - + days U Sulfa pyrid ine added to prednisone (15 m g da il y) cont roll ed the disease; no effect with s ulfa pyridine a nd/or d iaminodiphenyl s ulfone (DDS) a lone_ • N.D. = Not done . Immunopathologic studies demonstrated con­ tinuous deposits chiefl y of IgG (Fig. 6) in 2 cases with accompanying IgA. In 1 case circulating a ntibasement-membra ne antibodies were present in a titer up to 640, whereas immunoglobulins at the dermal- epidermal junction were only of the IgG class. Interestingly, this case showed clinical features co rresponding both to BP and DH (see Fig. 2). J ejunal bi opsy was made in 2 cases and was normal in 1; in the other it showed slightly f1 attened villi in some sites (g rade 1) . ase W. S., which was of mi xed type (see Fig. 4), had cutane­ F IG. l. Direct immunofluorescence of the uninvolved ous lesions of DH type with large bull ae, mi croab­ ski n in t he vicini ty of t he lesions . G ra nula r IgA d eposits in t he dermal pa pillae. Conjugate: Goat ga mma globulin scesses, and a favorable response to sulfapyridine. a nt ihuman 19A, 1 unil/ml ; d ilut ion 1/4; mola r F/P 3.4. IF studies demonstrated deposits of IgA in linear Final magnification X 1000. arrange ment . NoV . 1975 BULLOUS DERMATOSES OF CHILDHOOD 449 F IG. 3. Case Z. W. (Table II). Lesions resembling both bull ous pemphigo id and dermatitis herpetif'ormis with occasional large bull ae. FIG. 2. Case W. J. (Table II). Clinical features consist· ent with dermatitis herpetiformis and bullou pem· phigOld . DISCUSSION Our present investigations confirm the existence f cases of JDH which fu ll y co rrespond to DH of ~dults, clinically and histologically, in response to ulfapyridine and/or sulfones, and with demon­ ~trate d intestinal lesions. Immunopathologic investigation demonstrated granular IgA deposits 'n dermal papillae in t he neighborhood of lesions, ~hich is of diagnostic significance [1 2- 16). A F"IG. 4. Case W. S. (Table II). Cutaneous lesio,>s of particularly noteworthy case was that of patient B. bu ll ous pemphigo id and partially of dermatitis herlJeti­ K. reported in a previous paper [1 ) as correspond­ formis type. 'ng to benign bullous dermatosis of childhood [2,4 ] ~ecause of negative immunopathologic find ings. Microabscesses in dermal. papillae abo~t lesi?ns case, irc ul ating antibodies against. the basen)'ent were absent. However, t his also occurs III tYP ical membrane similar to those reported by others DB cases. T here was, however, a favora ble re­ [1 ,5, 6) have been detected. In the majority of our sponse to s ulfap~r idin e. When reexamin ~ d, t he cases corre ponding to BP, circulating antibodies patient had tYP ical granular IgA depOS its and have not been seen, as has been reported by Faber microabscesses in dermal papillae. Jejunal biopsy and van Joost [17). The in testin al biopsies are corroborated the diagnosis of DH. Much as in DH usually normal in thi group. of adults, a single biopsy may supply no evidence The mixed-type cases with the features of DH of immune deposits.
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