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Bullous pemphigoid associated with chronic renal allograft rejection

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Je-Ho Mun, MD, a Jeong-Min Kim, MD, b Hyun-Chang Ko, MD, a Byung-Soo Kim, MD, b and Moon-Bum Kim, MD b Department of Dermatology, Pusan National Uni- versity Yangsan Hospital, Yangsan, a and the Department of Dermatology, Pusan National University Hospital, Busan, b Republic of Korea Funding sources: None. Conflicts of interest: None declared. Correspondence to: Moon-Bum Kim, MD, Biomedical Research Institute, Pusan National University Hospital, 305 Guduk-ro, Seo-gu, Busan 602-739, Korea E-mail: [email protected] REFERENCES 1. Micali G, Lacarrubba F, Massimino D, Schwartz RA. Dermatos- copy: alternative uses in daily clinical practice. J Am Acad Dermatol 2011;64:1135-46. 2. Dubreuil A, Dompmartin A, Barjot P, Louvet S, Leroy D. Umbilical metastasis or Sister Mary Joseph’s nodule. Int J Dermatol 1998;37:7-13. 3. de Giorgi V, Alfaioli B, Massi D, Wnekowicz E, Sestini S, Papi F, et al. Solitary cutaneous metastasis as the first sign of relapse of thyroid carcinoma: a clinical, dermoscopic-pathologic case study. Dermatol Surg 2009;35:523-6. 4. Oiso N, Kimura M, Kawada A. A dermoscopic figure of polymorphous atypical vessels with color gradation: metastatic cutaneous carcinoma into the lymphatic vessels. Acta Derm Venereol 2011;91:737-8. http://dx.doi.org/10.1016/j.jaad.2012.11.020 Bullous pemphigoid associated with chronic renal allograft rejection To the Editor: We report a case of bullous pemphigoid (BP) in a young woman who presented with chronic renal allograft rejection, an unusual association. A 15-year-old girl developed nephrotic syndrome as a result of mesangial glomerulonephritis. She developed loss of renal function, requiring dialysis, and underwent kidney transplantation 3 years later. Ten years after the transplantation, she again pre- sented with renal function impairment. A kidney biopsy specimen demonstrated chronic rejection, and she was again submitted to dialysis. Four months later she presented for dermatologic consultation with blisters located on the trunk, thighs, arms, forearms, face, and oral mucosa (Fig 1). A skin biopsy specimen revealed subepidermal vesiculo- bullous dermatitis, with neutrophils and a mild superficial chronic infiltrate with eosinophils. Direct immunofluorescence revealed IgG and granular C3 at the dermoepidermal junction. Confirmation with enzyme-linked immunosorbent assay was not avail- able. The clinical and pathological findings were consistent with BP. The patient was treated with 100 mg of azathioprine and prednisone 1 mg/kg (60 mg) and complete remission of the lesions occurred within 1 month. She remained under main- tenance treatment for 1 year until she underwent a second kidney transplantation. BP is a chronic recurrent subepidermal vesiculo- bullous disease mediated by autoantibodies toward antigens located in the hemidesmosomes and lamina lucida of the dermoepidermal junction. 1 The usual clinical presentation consists of vesicles and tense blisters that heal without scarring. Oral mucosa involvement is uncommon. In most cases, BP is idiopathic. 1 The occurrence of BP in association with renal transplantation is rare. To our knowledge, 10 reports associating BP with renal transplant rejection have been published to date. In those cases, most were male (70%) and the age ranged between 6 and 52 years. Only 1 case followed acute rejection. The bullous eruption occurred after the onset of organ rejection and was usually preceded by a dose decrease or a withdrawal of immunosuppressive drugs. 2 Furthermore, clearance of the blisters was usually associated with the removal or spontaneous atrophy of the renal allograft. 3 This suggests immu- nologic activity within the renal graft may be in- volved in the pathogenesis of BP in these cases, 3 with cross-reactivity between the epidermal and glomer- ular basement membrane antigens. 1,4 The alpha-5 chain of type-IV collagen is present in the renal and epidermal basement membrane and has been sug- gested to be the target antigen for autoantibodies. 4 Fig 1. Bullous pemphigoid. Tense bullae with some crusts and erosions are seen over right thigh. JAM ACAD DERMATOL JUNE 2013 e192 Letters
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J AM ACAD DERMATOL

JUNE 2013e192 Letters

Je-HoMun, MD,a Jeong-Min Kim, MD,b Hyun-ChangKo, MD,a Byung-Soo Kim, MD,b and Moon-BumKim, MDb

Department of Dermatology, Pusan National Uni-versity Yangsan Hospital, Yangsan,a and theDepartment of Dermatology, Pusan NationalUniversity Hospital, Busan,b Republic of Korea

Funding sources: None.

Conflicts of interest: None declared.

Correspondence to: Moon-Bum Kim, MD, BiomedicalResearch Institute, Pusan National UniversityHospital, 305 Guduk-ro, Seo-gu, Busan 602-739,Korea

E-mail: [email protected]

Fig 1. Bullous pemphigoid. Tense bullae with some crustsand erosions are seen over right thigh.

REFERENCES

1. Micali G, Lacarrubba F, Massimino D, Schwartz RA. Dermatos-

copy: alternative uses in daily clinical practice. J Am Acad

Dermatol 2011;64:1135-46.

2. Dubreuil A, Dompmartin A, Barjot P, Louvet S, Leroy D.

Umbilical metastasis or Sister Mary Joseph’s nodule. Int J

Dermatol 1998;37:7-13.

3. de Giorgi V, Alfaioli B, Massi D, Wnekowicz E, Sestini S, Papi F,

et al. Solitary cutaneous metastasis as the first sign of relapse of

thyroid carcinoma: a clinical, dermoscopic-pathologic case

study. Dermatol Surg 2009;35:523-6.

4. Oiso N, Kimura M, Kawada A. A dermoscopic figure of

polymorphous atypical vessels with color gradation: metastatic

cutaneous carcinoma into the lymphatic vessels. Acta Derm

Venereol 2011;91:737-8.

http://dx.doi.org/10.1016/j.jaad.2012.11.020

Bullous pemphigoid associated with chronicrenal allograft rejection

To the Editor:We report a case of bullous pemphigoid(BP) in a young woman who presented with chronicrenal allograft rejection, an unusual association.

A 15-year-old girl developed nephrotic syndromeas a result of mesangial glomerulonephritis. Shedeveloped loss of renal function, requiring dialysis,and underwent kidney transplantation 3 years later.Ten years after the transplantation, she again pre-sented with renal function impairment. A kidneybiopsy specimen demonstrated chronic rejection,and she was again submitted to dialysis.

Four months later she presented for dermatologicconsultationwith blisters locatedon the trunk, thighs,arms, forearms, face, and oral mucosa (Fig 1). A skinbiopsy specimen revealed subepidermal vesiculo-bullous dermatitis, with neutrophils and a mildsuperficial chronic infiltrate with eosinophils. Directimmunofluorescence revealed IgG and granular C3at the dermoepidermal junction. Confirmation with

enzyme-linked immunosorbent assay was not avail-able. The clinical and pathological findings wereconsistent with BP. The patient was treated with100 mg of azathioprine and prednisone 1 mg/kg(60 mg) and complete remission of the lesionsoccurred within 1 month. She remained under main-tenance treatment for 1 year until she underwent asecond kidney transplantation.

BP is a chronic recurrent subepidermal vesiculo-bullous disease mediated by autoantibodies towardantigens located in the hemidesmosomes and laminalucida of the dermoepidermal junction.1 The usualclinical presentation consists of vesicles and tenseblisters that heal without scarring. Oral mucosainvolvement is uncommon. In most cases, BP isidiopathic.1

The occurrence of BP in association with renaltransplantation is rare. To our knowledge, 10 reportsassociating BP with renal transplant rejection havebeen published to date. In those cases, most weremale (70%) and the age ranged between 6 and 52years. Only 1 case followed acute rejection. Thebullous eruption occurred after the onset of organrejection and was usually preceded by a dosedecrease or a withdrawal of immunosuppressivedrugs.2 Furthermore, clearance of the blisters wasusually associated with the removal or spontaneousatrophy of the renal allograft.3 This suggests immu-nologic activity within the renal graft may be in-volved in the pathogenesis of BP in these cases,3 withcross-reactivity between the epidermal and glomer-ular basement membrane antigens.1,4 The alpha-5chain of type-IV collagen is present in the renal andepidermal basement membrane and has been sug-gested to be the target antigen for autoantibodies.4

Fig 1. Red fingers syndrome of the left hand. A, Chronic,diffuse distal interphalangeal erythema and onycholysis.B, Persistent hypothenar eminence erythema.

J AM ACAD DERMATOL

VOLUME 68, NUMBER 6Letters e193

To fit the clinical events, it might be hypothesizedthat these antibodies remain harmless as long as theimmunosuppressive treatment is operative.5

Despite oral cavity involvement, which is unusualin BP, the lack of scarring with resolution suggests BPrather than mucous membrane pemphigoid. In ourpatient, complete remission of the mucocutaneouslesions was achieved with the use of immunosup-pressive drugs. Spontaneous resolution has alsobeen reported in the literature. However, lesionswere treated only temporarily in most of the casesand nephrectomy or atrophy of the graft was neededto clear the bullous lesions.2 It is important for thedermatologist to be aware of the association be-tween BP and chronic renal allograft rejection andknow that in most cases the lesions were onlycleared after nephrectomy.

Juliano Peruzzo, MD, Lia Dias Pinheiro Dantas,MD, and Marcia Zampese, MD

Department of Dermatology, Hospital de Cl�ınicasde Porto Alegre, Brazil

Funding sources: None.

Conflicts of interest: None declared.

Correspondence to: Juliano Peruzzo, MD, Depart-ment of Dermatology, Hospital de Cl�ınicas dePorto Alegre, Rua Ramiro Barcelos, 2350eSantaCec�ılia, Porto Alegre, RS, Brazile CEP: 90035-000

E-mail: [email protected]

REFERENCES

1. Tessari G, Barba A, Chieregato C, Tonini M, Boschiero L. Bullous

eruption during chronic renal allograft rejection. Dermatology

2002;204:307-8.

2. Rodr�ıguez-Caruncho C, Bielsa I, Bay�es B, Guinovart R, Fern�an-

dez-Figueiras T, Ferr�andiz C. Bullous pemphigoid associated

with chronic renal allograft rejection: resolution after trans-

plantectomy. J Am Acad Dermatol 2011;65:e89-90.

3. Yamazaki S, Yokozeki H, Katayama I, Komai R, Hashimoto T,

Nishioka K. Childhood bullous pemphigoid associated with

chronic renal allograft rejection. Br J Dermatol 1998;138:547-8.

4. Sofi AA, Gottwald L, Bohman K. Bullous pemphigoid associated

with acute renal allograft rejection. Transplantation

2010;89:368.

5. Devaux S, Michot C, Mourad G, Guillot B, Dereure O. Chronic

renal graft rejection-associated bullous pemphigoid: a cross-

reactive immune response? Acta Derm Venereol 2011;91:82-3.

http://dx.doi.org/10.1016/j.jaad.2012.11.023

Red fingers syndrome in a patient withpseudolymphoma

To the Editor: A 31-year-old woman presented with a10-year history of erythematous plaques on the faceconsistent with cutaneous pseudolymphoma. She

incidentally was noted to also have dusky discolor-ation of her distal fingers and hypothenar eminencesbilaterally (Fig 1), but not toes, and without associ-ated pain, numbness, or accentuation in the cold.Onycholysis was noted on several fingernails, butclubbing or periungual telangiectasias were notpresent. The patient related that the redness wasnot present at birth and appeared during her adultyears before her pseudolymphoma lesions. She alsonoted that the redness appeared on the fingers andpalms simultaneously and did not wax and wane.She was not using any prescribed or over-the-counter medications at the time of onset or atpresentation. She further denied use of alcohol orillicit drugs and was otherwise without medicalproblems. Screening serologies for HIV, hepatitis Bvirus (HBV), hepatitis C virus (HCV), Lyme diseaseand mononucleosis produced unremarkable find-ings. A biopsy was offered, but the patient declinedas the redness was asymptomatic. She had completeclearance of pseudolymphoma lesions with inter-feron alfa-2b therapy,1 but no change in the distalfinger discoloration was observed.

Red fingers syndrome (RFS) is a rare vasculardisorder first described in 1996 by Pech�ere et al2 in9 patients with HIV and coinfection with HBVand/or HCV. RFS is characterized by chronic,asymptomatic, well-delineated erythema of the dis-tal pulp areas of the fingers and toes, often


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