O acometimento ungueal no pênfigo vulgar é um achado raro. Descrevemos cinco doentes com pênfigo vulgar e alterações ungueais. As alterações ungueais descritas no pênfigo vulgar são, em ordem decrescente de ocorrência: paroníquia crônica, onicomadese, onicólise, linhas de Beau e traqueoníquia. Paroníquia crônica foi a forma de acometimento ungueal encontrada em todos os doentes deste estudo e um deles apresentou, também, linhas de Beau. As manifestações cutâneo-mucosas e ungueais foram controladas, em todos os casos, com o uso de prednisona e/ou ciclofosfamida.Nail involvement in pemphigus vulgaris is rare. We describe 5 patients with pemphigus vulgaris presenting nail involvement. In this disease, nail manifestations present, by order of frequency, as chronic paronychia, onychomadesis, onycholysis, Beau's lines and trachyonychia. All our 5 cases presented with paronychia, and 1 of them also had Beau's lines. Treatment with prednisone and/or cyclophosphamide controlled mucocutaneous and nail manifestations in all cases

Cahali, Juliana Burihan

Kakuda, Everton Yuji Soyama

Maruta, Celina Wakisaka

Santi, Cláudia Giuli

English

Nail involvement in pemphigus vulgaris is rare. We describe 5 patients with pemphigus vulgaris presenting nail involvement. In this disease, nail manifestations present, by order of frequency, as chronic paronychia, onychomadesis, onycholysis, Beau's lines and trachyonychia. All our 5 cases presented with paronychia, and 1 of them also had Beau's lines. Treatment with prednisone and/or cyclophosphamide controlled mucocutaneous and nail manifestations in all cases.O acometimento ungueal no pênfigo vulgar é um achado raro. Descrevemos cinco doentes com pênfigo vulgar e alterações ungueais. As alterações ungueais descritas no pênfigo vulgar são, em ordem decrescente de ocorrência: paroníquia crônica, onicomadese, onicólise, linhas de Beau e traqueoníquia. Paroníquia crônica foi a forma de acometimento ungueal encontrada em todos os doentes deste estudo e um deles apresentou, também, linhas de Beau. As manifestações cutâneo-mucosas e ungueais foram controladas, em todos os casos, com o uso de prednisona e/ou ciclofosfamida

Cadernos Espinosanos (E-Journal)

229REV. HOSP. CLÍN. FAC. MED. S. PAULO 57(5):229-234, 2002SEPTEMBER-OCTOBERFrom the Department of Dermatology,Hospital das Clínicas, Faculty of Medicine,University of Sao Paulo.NAIL MANIFESTATIONS IN PEMPHIGUS VULGARISJuliana Burihan Cahali, Everton Yuji Soyama Kakuda, Cláudia Giuli Santi andCelina Wakisaka MarutaRHCFAP/3098CAHALI JB et al. - Nail manifestations in pemphigus vulgaris. Rev. Hosp. Clín. Fac. Med. S. Paulo 57(5):229-234, 2002.Nail involvement in pemphigus vulgaris is rare. We describe 5 patients with pemphigus vulgaris presenting nailinvolvement. In this disease, nail manifestations present, by order of frequency, as chronic paronychia, onychomadesis,onycholysis, Beau’s lines and trachyonychia. All our 5 cases presented with paronychia, and 1 of them also had Beau’s lines.Treatment with prednisone and/or cyclophosphamide controlled mucocutaneous and nail manifestations in all cases.DESCRIPTORS: Pemphigus vulgaris. Paronychia. Beau’s lines. Prednisone. Cyclophosphamide.INTRODUCTIONPemphigus vulgaris is anautoimmune, mucocutaneous vesicu-lobullous disease1. It is characterizedby intraepidermal bullae withsuprabasal acantholysis and IgGautoantibody against a desmosomal-associated glycoprotein, desmoglein12,3. Although pemphigus vulgaris mayoccur at any age, its most common on-set is in the fourth, fifth, and sixth dec-ades4,5. Involvement of the nail inpemphigus vulgaris is rarely de-scribed. We describe 5 patients withchronic paronychia as a manifestationof pemphigus vulgaris.CASE REPORTSCase 1A 65-year-old white woman pre-sented in 1991 with oral erosions andcutaneous lesions on the whole body.Histological examination of the af-fected skin revealed suprabasalclefting containing acantholytic cells.Epidermal intercellular deposition ofIgG was noted on direct immunofluo-rescence. After the diagnosis of pem-phigus vulgaris, she was treated with1 mg/kg daily of oral prednisone,which resulted in clinical resolution ofboth skin and oral mucosa lesions. In1996, free of mucocutaneous lesions,on a daily maintenance dose of 10 mg/day of prednisone, she developed ery-thema, edema, pustules, inflammation,and suppuration of the proximal andlateral nail folds of several fingernailsand toenails associated with Beau’slines of the 2nd, 3rd, and 4th digits onthe left hand (Fig. 1). Gram stain andpotassium hydroxide preparations ofnail drainage were negative. Bacterialand fungal cultures disclosed Staphy-lococcus aureus, but no fungi. Topi-cal and oral antibiotic therapy pro-duced no beneficial effect. A biopsyspecimen of the nail fold of the rightfirst toe revealed suprabasal cleftingand acantholysis. Direct immunofluo-rescence showed positive intercellulardeposition of IgG and C3. Indirect im-munofluorescence was positive at adilution of 1/10240. Thus, we consid-ered the paronychia to be a clinicalmanifestation of pemphigus vulgaris.Mucocutaneous lesions of pemphigusvulgaris occurred 2 weeks after the nailinvolvement. She was treated with anew cycle of prednisone 1 mg/kg/day,resulting in clinical resolution of mu-cocutaneous and nail lesions with noresidual damage on the nails.Case 2A 15-year-old white man withpemphigus vulgaris diagnosed in1993 presented initially with ulcera-tions in the oral cavity and simultane-ous involvement of the nails. The lat-ter was characterized by erythema,edema, inflammation, and bullae, withpurulent drainage on 1st and 4th rightfingers and 1st and 2nd left fingers (Fig.230REV. HOSP. CLÍN. FAC. MED. S. PAULO 57(5):229-234, 2002 SEPTEMBER-OCTOBER2). Skin lesions of pemphigus vulgarisappeared 1 month later. Histologicalexamination of the affected skin re-vealed suprabasal clefting and acan-tholysis. Direct and indirect immun-ofluorescence of perilesional skinshowed epidermal intercellular depo-sition of IgG and C3. Gram stain andpotassium hydroxide preparations ofthe drainage were negative, as werebacterial and fungal cultures. Nail bi-opsy was not performed. He was treatedwith a 1mg/kg daily dose of oral pred-nisone. Mucocutaneous and nail le-sions improved simultaneously withthis therapy, with no permanent dam-age on the nail.Case 3A 27-year-old white woman withpemphigus vulgaris diagnosed in Au-gust 2000 presented initially with skinlesions, oral erosions, and nail in-volvement characterized by erythema,edema, and purulent drainage from theproximal nail fold of the right first fin-ger (Fig. 3). Gram stain and potassiumhydroxide preparations of the drain-age were negative as were bacterialand fungal cultures. She was diag-nosed as having pemphigus vulgarison the basis of a skin biopsy and di-rect immunofluorescence. Indirect im-munofluorescence was positive at adilution of 1/1280. Nail biopsy wasnot performed. There was a rapid re-sponse of the mucocutaneous and naillesions to prednisone 1 mg/kg daily,with resolution of the nail lesions.Case 4A 44-year-old white woman withpemphigus vulgaris diagnosed in Au-gust 2000 presented initially with skinand oral lesions. Nail lesions occurred3 months later, with erythema andedema on the lateral and posterior nailfolds of her 1st, 2nd, and 3rd left andright fingers (Fig. 4). She noticed ex-acerbation of the mucocutaneous le-sions 2 weeks after the development ofthe paronychia. She was diagnosed ashaving pemphigus vulgaris on the ba-sis of a skin biopsy and direct immun-ofluorescence. Indirect immunofluo-rescence was positive at a dilution of1/2560. Nail biopsy was not per-formed. Gram stain and potassium hy-droxide preparations of the nail werenegative, as were bacterial and fungalcultures. Mucocutaneous and nail le-sions improved with oral prednisone 1mg/kg daily associated with endov-enous cyclophosphamide (900 mg/dose, monthly) and methylprednisone(300 mg/day during 3 consecutivedays in the 2nd month of the treatment).Case 5A 39-year-old white woman withpemphigus vulgaris diagnosed in1990 presented initially with oral ero-sive lesions. Skin lesions appeared 2months later. One month after that, theFigure 2 - CASE 2: Erythema, edema, and bullous lesions, with purulent drainage on 1st and 4thright fingernails and 1st and 2nd left fingernails.Figure 1 - CASE 1: Erythema, edema, pustules, and hemorrhagic crusts on the nail folds of herfingers. Beau’s lines on 3rd and 4th left fingernails.231REV. HOSP. CLÍN. FAC. MED. S. PAULO 57(5):229-234, 2002SEPTEMBER-OCTOBERnail involvement began. It was char-acterized by erythema and edema onall 20 nails, with purulent drainage ofher left and right first fingers. Nail in-volvement preceded an exacerbationof mucocutaneous disease. She was di-agnosed as having pemphigus vulgarison the basis of a skin biopsy and di-rect immunofluorescence. Nail biopsywas not performed. Gram stain and po-tassium hydroxide preparations werenegative. Bacterial and fungal culturesdisclosed Staphylococcus aureus, butno fungi. Oral antibiotic therapy pro-duced no beneficial effect on the paro-nychia. Mucocutaneous lesions andparonychia improved markedly withoral prednisone (1 mg/kg daily) andendovenous cyclophosphamide (0.5 gto 1 g/dose, monthly).DISCUSSIONPemphigus vulgaris is anautoimmune, mucocutaneous vesicu-lobullous disease1,5. Nail disease hasbeen reported in a variety of manifes-tations: chronic paronychia, Beau’slines, trachyonychia, onychomadesis,onycholysis, subungueal hemorrhage,nail plate discoloration, pitting, ony-choschizia, and nail dystrophy1,6-15.Engineer et al.1 summarized all reportsin the English literature on nail in-volvement in patients with pemphigusvulgaris (15 patients). The most fre-quent alterations were paronychia(60%) and onychomadesis (33%). Our5 patients had paronychia, and 1 ofthem had Beau’s lines. According tothe literature, fingernails were more of-ten involved than toenails (9 had fin-gernails involved, 5 had finger andtoenails involved, and 1 had only toe-nails involved). In our series, no onehad toenail involvement alone (Table1). In the previously reported cases inFigure 3 - CASE 3: Erythema, edema, andpurulent drainage on the first right finger.Figure 4 - CASE 4: Erythema and edema on the lateral and posterior nail folds of her 1st, 2nd,and 3rd left and right fingers, with purulent drainage.Table 1 - Clinical and laboratory features of the patients.PATIENT 1 PATIENT 2 PATIENT 3 PATIENT 4 PATIENT 5sex/age F/65 M/15 F/27 F/44 F/39nail lesion paronychia and paronychia paronychia paronychia paronychiaBeau’s lines fingernails fingernails fingernails finger and toenailsfinger and toenailsGram stain negative negative negative negative negativeCulture S. aureus no growth no growth no growth S. aureusonset of paronychia before generalized part of initial part of initial before generalized before generalizedexacerbation presentation presentation exacerbation exacerbationhistology of suprabasal not done not done not done not doneperiungueal clefting withdisease acantholysisDirect epidermal not done not done not done not doneImmunofluorescence intercellularof periungueal disease deposition of IgG232REV. HOSP. CLÍN. FAC. MED. S. PAULO 57(5):229-234, 2002 SEPTEMBER-OCTOBERthe literature, the nail changes oc-curred independently of the extent ofdisease (67% had mucocutaneous le-sions, 27% had cutaneous lesions, andjust 6% had only mucosal disease). Allof our patients had mucocutaneousdisease. Nail involvement is due tobullous lesions in the nail bed, nailmatrix, or nailfold1 as part of the dis-ease process8. Nail disease can be partof the initial presentation along withmucosal and cutaneous lesions (47%of all cases reported), can precede aflare of the pre-existing disease (33%),or can be the only sign of the disease(20%). In our study, nail involvementpreceded a flare of the disease in 3cases and was part of the initial pres-entation in 2 cases (Figs. 5 and 6).We thought bacteria and fungiwere secondary invaders6 and thechronic paronychia would not be re-solved with antibiotic or antifungaltherapy. Another possibility that couldbe included among the differential di-agnosis is herpetic whitlow, since thesepatients are chronically under the useof immunosuppressive drugs. This di-agnosis could be ruled out once theparonychia was resolved with theproper pemphigus control. In ourcases, nail biopsy was performed onlyin case 1. In the others, we did not takebiopsy specimens because the diagno-sis of pemphigus vulgaris had alreadybeen made histologically and by directand indirect immunofluorescence ofthe affected skin, and there is a knownassociation of pemphigus vulgaris withnail disease.CONCLUSIONWe present 5 patients with pemphi-gus vulgaris and nail involvement.This involvement can be part of theinitial presentation, precede a flare ofthe pre-existing disease, or can be theonly sign of the disease.It is important in patients withpemphigus vulgaris and paronychia tobe aware that the nail changes can bea manifestation of the autoimmunedisease, and in these cases, treatmentdirected against fungi, bacteria, andviruses may not be effective.Figure 6 - Onset of nail disease in this series.Figure 5 - Onset of nail disease, according to the literature.233REV. HOSP. CLÍN. FAC. MED. S. PAULO 57(5):229-234, 2002SEPTEMBER-OCTOBERREFERENCES1. ENGINEER L, NORTON LA & AHMED R - Nail involvement inpemphigus vulgaris. J Am Acad Dermatol 2000; 43: 529-35.2. CHAMPION RH, BURTON JL, BURNS DA et al. - Textbook ofDermatology. 6th ed. Oxford, Blackwell Science, 1998. p.1849-1855.3. EYRE RW & STANLEY JR - Identification of pemphigus vulgarisantigen extracted from normal human epidermis andcomparison with pemphigus foliaceus antigen. J Clin Invest1988; 81: 807-12.4. SAMPAIO SAP & RIVITTI EA - Dermatologia. Brasil. 2th ed.São Paulo, Artes Médicas, 2000. p. 229-248.RESUMO RHCFAP/3098CAHALI JB e col. - Manifestaçõesungueais no pênfigo vulgar. Rev.Hosp. Clín. Fac. Med. S. Paulo57(5):229-234, 2002.O acometimento ungueal nopênfigo vulgar é um achado raro. Des-crevemos cinco doentes com pênfigovulgar e alterações ungueais. As alte-rações ungueais descritas no pênfigovulgar são, em ordem decrescente deocorrência: paroníquia crônica,onicomadese, onicólise, linhas deBeau e traqueoníquia. Paroníquia crô-nica foi a forma de acometimentoungueal encontrada em todos os doen-tes deste estudo e um deles apresen-tou, também, linhas de Beau. As ma-nifestações cutâneo-mucosas eungueais foram controladas, em todosos casos, com o uso de prednisona e/ou ciclofosfamida.DESCRITORES: Pênfigo vulgar.Paroníquia. Linhas de Beau.Prednisona. Ciclofosfamida.5. MARTINS CR, SQUIQUERA HL & DIAZ LA - Pemphigus vulgarisand pemphigus foliaceus. Curr Probl Dermatol 1989; 1: 33-61.6. KIM BS, SONG KY, YOUN JI et al. - Paronychia – A manifestationof pemphigus vulgaris. Clin Exp Dermatol 1996; 21: 315-7.7. De BERKER D, DALZIEL K, DAWBER RPR et al. - Pemphigusassociated with nail dystrophy. Br J Dermatol 1993; 129:461-4.8. PARAMESWARA YR & NAIK RPC - Onychomadesis associatedwith pemphigus vulgaris. Arch Dermatol 1981; 117: 759-60.234REV. HOSP. CLÍN. FAC. MED. S. PAULO 57(5):229-234, 2002 SEPTEMBER-OCTOBER9. BAUMAL A & ROBINSON M - Nail bed involvement in pemphigusvulgaris. Arch Dermatol 1973; 107 :751.10.DHAWAN SS, ZAIAS N & PENA J - The nail fold in pemphigusvulgaris. Arch Dermatol 1990; 126: 1374-5.11.AKIYAMA C, SOU K, FURUYA T, SAITOH A el al. - Paronychia:a sign heralding an exacerbation of pemphigus vulgaris. J AmAcad Dermatol 1993; 29: 494-6.12.RIVERA DIAZ R, LLAMAZARES JA, PERALTO JLR et al. - Nailinvolvement in pemphigus vulgaris. Int J Dermatol 1996; 35:581-2.13.FULTON RA, CAMPBELL I, CARLYLE D et al. - Nail bedimmunofluorescence in pemphigus vulgaris. Acta DermVenereol (Stockh) 1983; 63: 170-2.14.LEROY D, LEBRUN J, MAILLARD V et al. - Pemphigus végétanta type clinique de dermatite pustuleuse chronique de hallopeau.Ann Dermatol Venereol 1982; 109: 549-55.15.STONE OJ & MULLINS JF - Vegetative lesions in pemphigus.Dermatol Int 1966; 5: 137-40.Received for publication on February 25, 2002.

Nail manifestations in pemphigus vulgaris

Scientific Electronic Library Online - Brazil

Manifestações ungueais no pênfigo vulgar

https://www.revistas.usp.br/rhc/article/download/30273/32157

Manifestações ungueais no pênfigo vulgar

Abstract

Similar works

Full text

Available Versions

Cadernos Espinosanos (E-Journal)

Scientific Electronic Library Online - Brazil