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Histopathology of Porphyria Cutanea Tarda 133 HISTOPATHOLOGY OF PORPHYRIA CTJTANEA TARDA*t MATJRIMAURI FELDAKER, FELDAKER, M.D., MD., HAMILTON MONTGOMERY, M.D. AND LOUIS A. BRUNSTING, M.D.MD. We wish to report the results of the histopathologic examination of the skin in 11 patients \vithwith porphyria cutanea tardatarda whowho werewere examinedexamined betweenbetween 19461946 and 1954. The microscopic findings in two patients (numbers I nndand 2) havehnve beenbeen reported previously by Brunsting and Mason (1). A preliminary report of the histopathologic findings in the first 10 patients has been given by Brunsting (2). MATERIAL Eleven patients, 10 men and one woman, with porphyria cutanea tarda were included in this study. One or more cutaneous biopsy specimens from each patient were fixed with 10 per cent formalin and stained with hematoxylin and eosin,eosiu, various elastic-tissue stains such as elastin H (Grubler), and an modified aldehyde-fuchsin stain (3)t whichwhich wewe preferprefer toto thethe elastinelastin HH oror WeigertWeigert stain, stain van Gieson stain forfor collagen,collagen, periodicperiodic acid acid Schiff Schiff stain stain (P.A.S., (PAS., that is, Hotch- kiss-McManus stain) for polysaccharides with and without pretreatment with diastase, mucin stains such as mucin D (4) counterstained with 2 per cent indigo carmine, Mallory's potassium ferrocyanide stain for the demonstration of hemo- siderin, and silver nitrate stain counterstained with\vith hematoxylin to demonstrate melanin. Stains for amyloid, Maresch-Bielschowsky stain for reticulum fibers (Gitterfasern) arid the Giemsa stain to demonstrate mast cells were done on all specimens. Biopsy was performed on the dorsum of the hand or finger iiiin about 50 per cent of the patients and, with one exception, all specimens were removed from thethe usuallyusually exposed areas of the body. There waswas aa totaltotal ofof iSiS biopsybiopsy specimens.specimens. Approximately one half of the patients were in the 41 to 50-year age group, thethe youngestyoungest patient being aged 20 years andand thethe oldestoldest 6767 years.years. TheThe durationduration *Fromthethe SectionSection ofof Dermatology and Syphilology, MayoMayo C]inieClinic andand Mayo Foundation, Rochester, Minn. t Fellow in Dermatology, Mayo Foundation. The Mayo Foundation is a part of the Graduate School of the University of Minnesota. t The modified aldehyde-fuchsin stain is prepared as follows: The reagents used are (1) aldehyde-fuchsinaldehyde-fuehsin solution andand (2)(2) toluidinetoluidine blueblue 00 solution.solution. TheThe aldehyde-fuchsin aldehyde-fuehsin solution solution is prepared by combining 1 gm. of basic fuchsin, 200 cc. of 70 per cent ethanol, 2 cc. par- aldehyde, 2 cc. concentrated hydroch]orichydrochloric acid, and letting the preparation stand for 3 days until it becomes blue. Toluidine blue 0 solution is prepared from a mixture of 0.1 gm. tolui- dine blue 0 and 100 cc. of 0.2 per cent glacial acetic acid. The section is stained with aldehyde-fuchsin solution for 15 minutes, is rinsed with 95 per cent ethanol and then remains in tap water for 5 minutes. Then the slide is rinsed in distilled water and stained with toluidine blue 0 for 2 minutes. Two changes each of acetone and xylene complete the procedure. Elastic-tissue fibers stain a deep purple, and the toluidine blue stains mast cells purple, melanin green and nuclei blue. Received for puh]icationpublication October 20, 1954. 131 132 THEJOIJEIIALJOUEAL OF OF INVESTIGATIVE INVESTIGATIVE DERMATOLOGYDERMATOLOGY TABLE 1*1 ,S'ummaryof of data data for for 11 11 patients patients with with porphyriaporphyria cutaneacatanea torda Locationof Biopsy Case Sex U Resuits of Microscopic Examination ge, Lesions LocaonpfBioow MaleMala 20 P2 yearsyears Nose (Aug.,(Aug.. Some senile skin changes 1946) Forefinger No senile skin changes (Nov., 1946) Dorsum hand No senile skin changes (Nov., 1946) 2 Male 41 4 years Neck (Mar., Senile skin changes. Increased 1947) staining of collacin with both P.A.S. and mucinmacin stains Shoulder (Mar.,(Mar., NormalNormal skin 1947)1947) Neck (Mar., Senile skin changes as in neck 1954)1954) biopsy of Mar., 1947 Dorsunt 4th Subepidermal bulla with festoon- finger (Mar., ing of base of bulla. Increased 1954) staining ofof vessels atat base of bulla with P.A.S. stain. No senile skin changes 3 Male 65 6 months Dorsum hand Subepidermal bulla with festoon- (Apr., 1948) ing of base. P.A.S. and mucin stains increased in area of collacin beneath bulla 4 Male 48 2 years DorsumDorsum hand hand Intra-epidermal bulla. No senile (Sept.,(Sept., 1948) skin changes 5 Male 34 1 year Cheek (Oct., Senile skin changes. P.A.S. and 1948) mucin stains increased intensity in area of collacin Dorsum hand Senile skin changes. P.A.S. and (Oct., 1948) mucin stains more intense in regionregion ofof collacin (fig. 1). 6 Male 42 1 year L. neck (Aug., Senile skin changes. Difference in 1949) staining of aldehyde-fuchsinaldehyde4uchsin stain (elastic fibers pink, blue or violet) in area of collacin as compared to uniformnniform staining of elastic fibers with elastin H (Grubler).(QrUbler). P.A.S.P.A.S. andand mucin stained more intensely in area of collacin L. neck (Feb.,(Feb., Senile skin changes 1954)1954) 7 Male 46 41 years NeckNeck (May,(May, Senile skin changes 1952) HISTOPATHOLOGY OF PORPHYRIA CUTANEA TARDA 133 TABLE 1—Cont. Duration of Case Sex Age, Cutaneousneous Location off BiopsyBiopsy Results of Microscopic Examination Years Lesions Specimen 8 Male 67 9 years Index finger Subepidermal buflabulla with festoon- (Aug., 1952) ing of base. No senile skin changes (fig. 2a and b) 9 Male 48 1 yearsDorsum hand Senile skin changes. P.A.S. and (Jan., 1953) mucin stains increased in area of collacin (fig. 3a, b, c and d) 10 Male 5757 1 year Dorsum hand SubepidermalSuhepidermal bulla with festoon- (July, 1953) ing of base. No senile skin changes. P.A.S. stains thickened vessel walls underneath the bulla more initenselyintensely 11 Female 5858 19 years Neck (Apr., Senile skin changes. MucinI\iucin stains 1954) collacincollacin moremore intensely.intensely. P.A.S.P.A.S. revea]s increased stain of vessel walls * Ilistopathologicfindings in patients 11 andand 22 (Mar.,(Mar., 1947)1947) werewere reportedreported iiiin referencereference 1.1. Photomicrographs of biopsies inin patientspatients 11 (Mar.,(Mar., 1945)1945) andand 6 6 reported reported in in reference 2. FIG. 1. Dorsum of hand. Positive increased staining with P.A.S. of prickle-cell layer, "basement membrane" between epidermis and dermis, collacin, sweat duct and vessel walls and of connective tissue about sebaceous glands. With pretreatment by diastase there was disappearance of the staining of the rete malphigii, but the positive staining of the other structures8t ructures waswas unalteredunaltered (P(P.A.S. .A.S. [Hotchkiss-McManusl; X X145). 145). 134 TilEJOURNAL OF INVESTIGATIVE DERMATOLOGY :4,,-. r : r :—- -$$- ,c 1• " 41• ;tC Fia.Fm. 2. Dorsum of index finger. a. Subepidermal tension bulla. The projection of the papillary bodies into the bullahulla in the manner of villi has been called "festooning.''"festooning." No senile skin changes were demonstrated (hematoxylin and eosin; X75). b. Edge of bulla. Note early detachment of the papillary bodies from the epidermis to form subepidermal tension bulla with festooning. Dilatation, tortuositytortoosity and increased staining of the thick- ened capillaries underneathnnderneath thethe bullabnlla (P.A.S.(P.A.S. Hotchkiss-McMarius];Hotchkiss-McManus]; X75).X75). of cutaneous lesions varied from a few months to 19 years. The bullae were present for a varying length of time before biopsy, from several hours' to days' duration. A summary of the data for these patients is giver'given in table 1. COMMENT In the histopathologic examination of the skin from 11 patients with porphyria cutaneaeutanea tarda we frequently noted in nonbullous lesions, and in several instances underneath the bullae, changes in the connective and elastic tissue to form homogeneous masses, namely, an apparent merging of connective and elastic tissue to form collacin (TJnna).(Tlnna). The periodic acid SchiffSchiff P.A.S.P.A.S. (Hotchkiss-(Hotchkiss- McManus) stain in sections not pretreatedprctreated with diastase usually was positive in these areas of homogeneoushomogeneous tissuetissue andand alsoalso inin adjacentadjacent swollenswollen andand ffrag- rag- mented elastic fibers. In a few instances there was positive staining of the prickle- cell layer of the epidermis. Stains for mucin may also be positive in the homog- enized zones. These staining reactions were not constantly positive and in some instances were negative. A newer elastic-tissue stain (aldehyde-fuchsin stain), in which toluidine blue is employed as a counterstain, reveals the collacin HISTOPATHOLOGY OF PORPHYRIA CIJTANEA TARDA 135 S $1, ! 11 rGwaTbc.w1 FIG.3. Dorsum of of hand. hand. LI. a. Keratotic pluggingplugging of sweat duct, acanthosis, pale-staining homogeneous area of connective tissue at junction of middle and upper thirds of cutis. Mild cellular infiltrate. Dilatation and tortuosity of capillaries and lymphatics in the upper cutis (hematoxylin and eosin; X75). b. Zone of merging of collagen and elastic fibers stain- ing darkly (collacin,(eollaein, Unna)Uana) andand normalnormal elasticelastic fibersfibers deeperdeeper inin thethe dutis.cutis. The changes are comparable to those seen in senile skin (elastin H; X75). c. Increased staining of the area of collacin but not of walls of capillaries which are dilated (P.A.S. [Hotchkiss-McManus];[Hotchkiss-MeManus]; X75). d. Increased staining ofof areaarea ofof eollaeincollacin (mucin(mucin D;D; X75).x75). 136 THE JOURNAL JOURNAL OF OF INVESTIGATIVE INVESTIGATIVE DERMATOLOGY DERMATOLOGY to vary from pink to purple to blue and to stain lightly or intensely. The sig- nificance of this is not known andand isis subjectsubject toto furtherfurther study.study. ThisThis isis inin contrast contrast to old elastic-tissue stains in which homogeneous masses of merged fibers of con- nective and elastic tissue stainedstained aa deepdeep purplepurple oror blackblack dependingdepending onon whetherwhether elastin H (Grubler) or Weigert's stain was employed.
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