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Minimal change nephropathy associated with pemphigus vulgaris: a new relationship?

2004; Elsevier BV; Volume: 50; Issue: 4 Linguagem: Inglês

10.1016/s0190-9622(02)61624-x

1097-6787

Mark D. Herron, Donald E. Kohan, C. David Hansen,

Eosinophilic Disorders and Syndromes

A 3 Accepted for publication , . 7-year-old man presented with painful oral, nasal, and scalp erosions of 6 months' duration. Within 2 months, he developed lower extremity edema and a mild cough. He then noticed facial fullness and weight gain. Physical examination revealed periorbital edema as well as edema of the lower extremities. Desquamative gingivitis was prominent. Histopathology and immunofluorescence of skin supported the diagnosis of pemphigus vulgaris. Histopathology, immunofluorescence, and electron microscopy from a kidney biopsy specimen confirmed minimal change nephropathy.There has been virtually no description of the relationship between pemphigus vulgaris and kidney disease, even though the kidney is a frequent site of immune-mediated injury. Only one report exists in the literature that describes an association of pemphigus vulgaris with renal disease, and, in this case, a clear offending agent (d-penicillamine) was identified.1Shapiro M. Jimenez S. Werth V.P. Pemphigus vulgaris induced by d-penicillamine therapy in a patient with systemic sclerosis.J Am Acad Dermatol. 2000; 42: 297-299Abstract Full Text Full Text PDF PubMed Scopus (29) Google Scholar, 2Savill J.S. Chia Y. Pusey C.D. Minimal change nephropathy and pemphigus vulgaris associated with penicillamine treatment of rheumatoid arthritis.Clin Nephrol. 1988; 29: 267-270PubMed Google Scholar Our observation of minimal change nephropathy and pemphigus vulgaris occurring simultaneously in a patient in the absence of offending agents or other clinically apparent disease processes represents a novel finding (see Table I). Table ICutaneous diseases described with minimal change nephropathyDermatitis herpetiformis3Gaboardi F. Perletti L. Cambie M. Mihatsch M.J. Dermatitis herpetiformis and nephrotic syndrome.Clin Nephrol. 1983; 20: 49-51PubMed Google ScholarBehçet's syndrome4Malik G.H. Sirwal I.A. Pandit K.A. Behçet's syndrome associated with minimal change glomerulonephritis and renal vein thrombosis.Nephron. 1989; 52: 87-89Crossref PubMed Scopus (27) Google ScholarPartial lipodystrophy5Jacob CK, Date A, Shastry JC. Minimal change disease with partial lipodystrophy. Child Nephrol Urol 1988-89;9:116-7Google ScholarKimura's disease (subcutaneous eosinophilic lymphoid granuloma)6Matsumoto K. Katayama H. Hatano M. Minimal-change nephrotic syndrome associated with subcutaneous eosinophilic lymphoid granuloma (Kimura's disease).Nephron. 1988; 49: 251-254Crossref PubMed Scopus (31) Google ScholarWaldenström macroglobulinemia7Hory B. Saunier F. Wolff R. Saint-Hillier Y. Coulon G. Perol G. Waldenstrom macroglobulinemia and nephrotic syndrome with minimal change lesion.Nephron. 1987; 45: 68-70Crossref PubMed Scopus (26) Google ScholarYellow nail syndrome8Yanez S. Val-Bernal J.F. Fernandez-Llaca H. Yellow nails and minimal change nephrotic syndrome.Nephron. 1999; 83: 180-182Crossref PubMed Scopus (16) Google ScholarSystemic lupus erythematosus9Guery B. Martinez F. Beaufils H. Deray G. Minimal-change nephropathy associated with systemic lupus erythematosus.Nephrol Dial Transplant. 1997; 12: 2030-2031Crossref PubMed Scopus (9) Google ScholarSarcoidosis10Parry R.G. Falk C. Minimal-change disease in association with sarcoidosis.Nephrol Dial Transplant. 1997; 12: 2159-2160Crossref PubMed Scopus (16) Google Scholar Open table in a new tab The initial appearance of pemphigus vulgaris, followed by clinically apparent nephrotic syndrome, suggests the possibility of a causative effect of pemphigus vulgaris in the pathogenesis of minimal change nephropathy. Alternatively, an as yet unidentified additional process may have caused the simultaneous occurrence of the two disorders, although the absence of any other apparent disease makes this possibility quite speculative. Regardless of the nature of the primary immune insult, an important question is raised as to whether patients with pemphigus vulgaris and autoimmune bullous diseases should be screened for proteinuria. Unfortunately, there are no data on which to address this issue: proteinuria has not been studied in this population. One might argue that urinalyses are inexpensive, have no risk to the patient, and may help groups that are at risk for renal disease. We suggest that easily performed prospective studies could help resolve this issue. A 3 Accepted for publication , . 7-year-old man presented with painful oral, nasal, and scalp erosions of 6 months' duration. Within 2 months, he developed lower extremity edema and a mild cough. He then noticed facial fullness and weight gain. Physical examination revealed periorbital edema as well as edema of the lower extremities. Desquamative gingivitis was prominent. Histopathology and immunofluorescence of skin supported the diagnosis of pemphigus vulgaris. Histopathology, immunofluorescence, and electron microscopy from a kidney biopsy specimen confirmed minimal change nephropathy. There has been virtually no description of the relationship between pemphigus vulgaris and kidney disease, even though the kidney is a frequent site of immune-mediated injury. Only one report exists in the literature that describes an association of pemphigus vulgaris with renal disease, and, in this case, a clear offending agent (d-penicillamine) was identified.1Shapiro M. Jimenez S. Werth V.P. Pemphigus vulgaris induced by d-penicillamine therapy in a patient with systemic sclerosis.J Am Acad Dermatol. 2000; 42: 297-299Abstract Full Text Full Text PDF PubMed Scopus (29) Google Scholar, 2Savill J.S. Chia Y. Pusey C.D. Minimal change nephropathy and pemphigus vulgaris associated with penicillamine treatment of rheumatoid arthritis.Clin Nephrol. 1988; 29: 267-270PubMed Google Scholar Our observation of minimal change nephropathy and pemphigus vulgaris occurring simultaneously in a patient in the absence of offending agents or other clinically apparent disease processes represents a novel finding (see Table I). The initial appearance of pemphigus vulgaris, followed by clinically apparent nephrotic syndrome, suggests the possibility of a causative effect of pemphigus vulgaris in the pathogenesis of minimal change nephropathy. Alternatively, an as yet unidentified additional process may have caused the simultaneous occurrence of the two disorders, although the absence of any other apparent disease makes this possibility quite speculative. Regardless of the nature of the primary immune insult, an important question is raised as to whether patients with pemphigus vulgaris and autoimmune bullous diseases should be screened for proteinuria. Unfortunately, there are no data on which to address this issue: proteinuria has not been studied in this population. One might argue that urinalyses are inexpensive, have no risk to the patient, and may help groups that are at risk for renal disease. We suggest that easily performed prospective studies could help resolve this issue.