Cameo

Concurrent Development of Pemphigus Foliaceusand Psoriasis

CHANG W O O LEt, M.D., YOUNG SUK RO, M.D., )AE HONG KIM, M.D., AND JOONG HWAN KIM, M.D.

Ironi thi' Dvp^ulment of Dermatology, Haryyartg Universily.College of Medicine, Seoul. Korea

A 34-year-old Korean woman presented with a thief((implriint of pruritic patches on the trunk. She first noticedIhe lesions on her flank 2 weeks hefore her visit. Newk'sit)ns hdd gradually (Jevel()ppd on her .ihdomen. Oneweek after the onset of initi.il prurHit lesions, she founci:,everal white st.ily papules on her .irms. She h.id no drughistory relevant to her skin lesit)ns, nor Hid she htive anysystemic disease or infection at the time of her first visit.

On physical examination, the only pertinent findingswere confined to the skin. Her flank (Fig. 1), abdomen,and extremities showed discrete, hean-sized or larger ery-thematc)us eczematoid patches, a few vesicles, and whitescaly papular lesions. The Nikolsky sign was [>resent on thevesicular lesions, and the Auspitz sign was note(] on thescaly papular lesions. She did not have any oral lesions.

Biopsy studies of the lesional skin revealed two differentpathologit changes. In one specimen, from the eczematoidpatt h on the flank, upper epidermal hullae containing someneutrophils and acantholytic cells were seen. The dermisshowed a mild perivascular infiltration of lymphoid cells.Anolher specimen from a scaly papular lesion and onefrom the lesion of Koebner response revealed [Jtiraker;itosis,absence of the granular layer, exocytosis of granulocytes,and elongation of rete ridges in the epidermis. Elongation,ind edema of the papillae, dilatation of blood vessels, andinfiltratioti of lymphoid cells in the dermis were also seen.I he diret t immurioflu(jrescenl studies were performed withtwo different S[)ecimens. The perilesional skin of the ecze-matoid patch showed intercellular deposits of IgC high inthe epidermis. The scaly papular lesion revealed stainingsof IgG, IgA, and C.3 at the parakeratotic horny layer. Thebasketweave pattern of staining was not seen, which seemslo be due to the loss of scales containing immune deposits,the result of lupus band test conducted on the normal-appearing skin from ihe extensor forearm was negative.The indirec t immunolluorescent studies of the serum, using(lorinal flank skin as substrates, demonstrated circulatingintercellular antibodies at a titer of 1:80.

Significant laboratory data are as follows. There was anelevation of ESR of 35 mm/hr and an increase in the serum

s for correspondence: Chang Woo Lee, M.D.. Depiirlmentol Dermatology, Hanyang University Hospital, Sungdong-ku, Seoul133, Korea.

concentration of IgG of 2,200 mg/dL. The early morningserum zinc concentration was measured twice, with i\ninterval of 3 days, by atomic absorption spec tronietry andwas 50.1 Mg/dL and 50.4 pig/dl, respectively {the normalrange being 120 ± 16 ̂ g/tlL in this siudy}. The proportionof T-cells in the peripheral blood measured by the E-rosette technique was 42"/(). Other serologic studies, in-cluding a routine blood examination, were all within normallimits or negative. Swabs taken from vesicobuilous lesionsfor bacteritil cultures were sterile. The skin tests using ret:allantigens demonstrated cutaneous anergy to SK/SD, candi-din, and Trichophyton.

After the diagnosis of pemphigus foliaceus and psoriasiswas confirmed, the patient was treated with a combinationof azathioprlne and prednisone as well as supportive topicalcares. Zinc sulfate was administered orally lor 2 monthsuntil the clinical remission was evident.

Five monlhs alter the initial visit, the titer of intercellularantibodies was 1:10, and the f>atient hac! t)niy a fewpsoriatic papules. In addition, the serum concentration ofzinc and the number of T cells in ihe peripheral bloorj,unlike the abnormally decreased values obtained at thetime of ihe early stages of the patient's disease, were upto normal levels (128.1 ^g/dL and 80%, respectively). Theskin test reactions to SK/SD and candidin were positive.

Comment

Many combination cases of pemphigus with thy-moma or other autoimmune disorders have beenreported.'^ Psoriasis is one of the common skindiseases not universally believed to be an autoimmunedisorder, but an immunologic phenomenon, such asdysfunctional or decreased number ol' blood T cells,has been reported in patients suffering from pso-riasis.* *'

It is interesting that this patient showed hypozinc-emia, a decreased number of T-cells in the peripheralblood, and cutaneous anergy before treatment, all ofwhich returned to normal after remission. Our patientdid not belong to any of the clinical situations resultingin hypozincemia,''" and it is unusual to see hypozinc-emia in a patient with pemphigus foliaceus or psoriasisof the discoid type. The mechanism of the transientabnormalities of T-cells in this patient is hard to

316

No. 5 PEMPHIGUS FOLtACEUS AND PSORIASIS tee e( al. 317

FIC. I. Erythematous eczematoid patches and white scalypapular lesions on the flank.

explain, but the psorialic process could be consideredan implicating factor affecting the T-celL''

The toncurrenco of pemphigus foliaceus and pso-riasis might suggest that the underlying cause of thesetwo diseases is an immunologic disturbance, perhapsinduced by transient hypozincemia. Alternatively,

hypozincemia and suspected T-cell immunodepres-sion could be associated conditions or secondaryphenomena, since there is no evidence to suggestthat hypozincemia or defective T-cells predisposethe patient to the development of pemphigus orpsoriasis. The concurrent development oi those twodiseases might be merely coincidental.

ReferencesKH. Barnes WR. Thymoma associated with erythroplasia,

bullous skin eruption, and LE cell preparation. Ann InternMed. 1964;61:H)8 315.

2. Diaz t A, GKimb RW, Silva 1 |r. A syndrome o( multiple immuneautore.it tivily. Arch Dermatol. 1980;l 16:77-79,

3. Guilhou )|, MeynadifT ), Clol ), et al. Immunological aspec t ofpsoriasis. II. Dissociated impairment of thymus-depcndentIvmphotytes. Br | t)frm.itol. I976;9f;:295-3O1,

4. Clinski W, Obaiek S, langer A, et al. Defective function of Tlymphocytes in psoriasis. ) Invest Dermatol. 1978,70:105-110.

5. Sauder DN, Bailin PL, Sundeen |, et al. Suppressor cell (unctionm psoriasis. Arch Qermatol. 1980;! 16:51-55.

(>. Baker BS. Swam AF. Valdimarsson 11, «M a\. Tccll subpnpu lat ionsin the hlood ,v\d skin ot pdlirnls wjlh psoriasis. Br IDermatol. 1984:1 H):}7-44.

7. Cagfiiano V, S{ hnit/ler R, Strauss W, et al. Zinc deficiency ina patient with retarded growth, hypogon.idism. hypogam-maglohulint-mi.i, .intl t hronic inleciion. Am | Med Sci.1969,257:305 319.

8. Halsied |A, Smith |C Ir, Plasma zinc in health and disease.Lancet 1970;1:322 324.

ANA Tests

Antinuclear antibodies (ANA) were first detected by the LE cell phenomenon, a test thatrequires bare nuclei, an IgG anti-DNA-nucleoprotein antibody, and nrutrophil polyniorphs.Although not often done nowadays the LE cell tesi is reasonably specific for systemic lupuserythematosus (SLE). The immunofliiorescence test for ANA, introduced by Holborow in 1957,with rat liver as substrate, is more sensitive but less specific, encompassing a wide range ofantibodies to various nuclear components. Various staining patterns may be observed andthese correlate to some degree with different parts of the spectrum of connective tissuedisorders. Antibody to histone, present in virtually all cases of drug induced SLE, produces adiffuse staining ol the nuclei; double-stranded DNA antibodies, a p.irtit ul.ir feature of SLE, tendto be associated with perinuclear staining; nucleolar staining, which detects 4S-6S nucleotus-specific RNA, is usually associated with systemic sclerosis; while a speckled staining patterntends to be a feature of mixed connective tissue disease, Sjogren's syndrome, or sclerodermaand may be a manifestation of antibody to ribonudeoprotein. When a rapidly dividing cell linesuch as HEp-2 or WiU is used as substrate further antibodies may be revealed by immunoflu-orescence, often in patients previously regarded .is having seronegative SLE. For example, thesmall nucleoprotein .intigen Ko is cytoplasmic in interstitial cells but intranuclear in HEp-2; andantibodies to the PCNA antigen present in 5'M) of SLE sera, and to the centromeric antigenpresent in the sera of 90% of patients with the CRST (calcinosis, Raynaud's, scterodactyly,telangectasia) syndrome, are detectable only in rapidly proiitVrating cells. Recent work hasunravelled the antinuclear puzzle at a molecular level and has provided insights into theassociated diseases.—Antinuclear antibodies. Lancet. 1983;2:6}l.