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Background

Polyvinylpyrrolidone (PVP) was once used as a plasma expander and has been used as a component of hair sprays, skin care products, and even as a food additive. The problem is this compound is water soluble but collects in tissue and cannot be excreted from the body. Over time, the material may collect in a variety of organs, causing storage disease like signs and symptoms and organ dysfunction.

OUTLINE

Epidemiology  
Disease Associations  
Pathogenesis  
Laboratory/Radiologic/
Other Diagnostic Testing
 
Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
Special Stains/
Immunohistochemistry/
Electron Microscopy
 
Differential Diagnosis  
Prognosis  
Treatment  
Commonly Used Terms  
Internet Links  

GROSS APPEARANCE/
CLINICAL VARIANTS
CHARACTERIZATION
GENERAL  
VARIANTS  
BONE MARROW  
Bone marrow failure and myelofibrosis in a case of PVP storage disease.

Dunn P, Kuo T, Shih LY, Wang PN, Sun CF, Chang MJ.

Department of Internal Medicine, Chang Gung Memorial Hospital, Taipei, Taiwan, Republic of China.


Am J Hematol. 1998 Jan;57(1):68-71. Abstract quote  

"PVP storage disease" is a disorder occurring in patients who have received high molecular weight polyvinylpyrrolidone (PVP), which cannot be excreted from the body.

These large polymers deposit in the histiocytes and cause proliferation and infiltration of histiocytes in the reticuloendothelial system. There was usually no significant damage to these organs except that prolonged administration might cause bone destruction, skin lesions, arthritis, and polyneuropathy.

We describe a patient who had received a large amount of PVP-containing solution for years. Severe bone marrow failure with extensive infiltration of bone marrow by foamy histiocytes occurred later. In addition, she suffered from multiple pathological fractures with spinal cord compression and arthritis of bilateral knee joints.
SKIN  
Cutaneous involvement in polyvinylpyrrolidone storage disease: a clinicopathologic study of five patients, including two patients with severe anemia.

Kuo TT, Hu S, Huang CL, Chan HL, Chang MJ, Dunn P, Chen YJ.

Department of Pathology, Chang Gung University and Chang Gung Memorial Hospital, Kwei San, Tao Yuan, Taiwan.


Am J Surg Pathol. 1997 Nov;21(11):1361-7. Abstract quote  

Polyvinylpyrrolidone (PVP), formerly a plasma expander, has continued to be inappropriately used in Taiwan for intravenous injection as a "blood tonic."

Five cases of PVP storage disease with cutaneous involvement were studied. Two patients presented with cutaneous eruptions mimicking collagen vascular disease and chronic pigmented purpuric dermatosis. Two other cases were found incidentally: one was with a metastatic tumor and the other in a pemphigus lesion. The fifth case was seen in a blind skin biopsy specimen taken to exclude Niemann-Pick disease after hematologic examination of a bone marrow smear. The latter patient and the patient with a collagen vascularlike disease also had severe anemia and serious orthopedic and neurologic complications due to massive infiltration of PVP-containing cells in the bone marrow with destruction of the bone. Severe irreversible anemia due to PVP storage disease has not been reported before. Three patients admitted having a history of receiving intravenous injection of PVP. The samples obtained from two of them indeed contained 5% PVP as determined by chemical analysis. PVP storage disease can be diagnosed by its histopathologic features. The skin biopsy specimens all showed a variable number of characteristic blue-gray vacuolated cells around blood vessels and adnexal structures with positive tinctorial reactions to mucicarmine, colloidal iron, and alkaline Congo red and negative to periodic acid-Schiff (PAS) and alcian blue. The PVP storage cells were shown to be CD68+ macrophages. The presence of PVP in the skin induced little or no inflammatory reaction. Only the pelvic mass in one patient had a foreign body granuloma formation. Our study showed that systemic parenteral administration of PVP preparation could result in the accumulation of PVP storage cells in the skin, with or without clinical eruptions.

The diagnosis of systemic PVP storage disease can be established by performing a skin biopsy for pathologic study. It is important for pathologists and clinicians to be aware of this iatrogenic storage disease to avoid misdiagnosis for hereditary storage disease, osteomyelitis, or signet-ring cell carcinoma.

Serious hematologic and orthopedic complications can be caused by repeated massive intravenous injection of PVP. Therefore, PVP preparations should be strictly prohibited for systemic administration.
Persistent perineal sinus following an abdominoperineal amputation: an unusual form of polyvinylpyrrolidone storage disease.

Balaton AJ, Vaury P, Bonan A.

Hopital Saint Joseph, Paris, France.

Pathology. 1988 Jan;20(1):83-4. Abstract quote  

A 58-year-old man had a persistent perineal sinus following an abdominoperineal resection. An excision of the sinus was performed.

Histology revealed that the sinus tract was bordered by a granulation tissue exhibiting numerous vacuolated cells containing polyvinylpyrrolidone.

The origin of polyvinylpyrrolidone was related to lengthy irrigation of the fistula with Betadine.

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL  
Polyvinylpyrrolidone-storage disease. Light microscopical, ultrastructural and chemical verification.

Reske-Nielsen E, Bojsen-Moller M, Vetner M, Hansen JC.

Acta Pathol Microbiol Scand [A]. 1976 Sep;84(5):397-405. Abstract quote  

The light and electron microscopical findings in the polyvinylpyrrolidine-storage disease are reported on the basis of biopsies of skin, striated muscle, bone marrow and liver from one patient and a subcutaneous nodule from another patient. Both patients suffer from diabetes insipidus and have been treated for several years with Insipidin retard, which contains polyvinylpyrrolidone (PVP) as the retarding agent.

Deposits of PVP have been demonstrated in all the tissues examined and can easily be recognized by certain staining qualities. The combination of haematoxylin-eosin, elastin (Weigert), alkaline Congo red and Sirius red for amyloid and PTAH is specially to be recommended. The ultrastructural findings consist of intracellular vacuoles containing a granular material and probably representing lysosomes.

The final identification of the nature of the deposits has been made by spectrophotometric analysis. The suspicion of a case of PVP-storage disease should result in a skin biopsy which usually is sufficient for the diagnosis.
VARIANTS  
CHEILITIS GRANULOMATOSIS  
Localized cutaneous polyvinylpyrrolidone storage disease mimicking cheilitis granulomatosa.

Chi CC, Wang SH, Kuo TT.

Department of Dermatology, Chang Gung Memorial Hospital-Chiayi, Chiayi, Taiwan.

J Cutan Pathol. 2006 Jun;33(6):454-7. Abstract quote  

Polyvinylpyrrolidone (PVP), a polymer of the monomer N-vinylpyrrolidone with various molecular weights, was originally developed as a plasma expander.

Currently, it is widely used in hair sprays, skin care products, fruit juices, and as a retarding agent in drugs such as procaine and hormones. PVP polymers with a molecular weight greater than 20,000 cannot be excreted by the kidneys and therefore are phagocytosed and permanently stored in the reticular endothelial system, leading to the so-called PVP storage disease.

We report a case of localized cutaneous PVP storage disease presenting with persistent upper lip swelling and mimicking cheilitis granulomatosa, which has never been reported before.
SIGNET RING  
Mucicarminophilic histiocytosis. A polyvinylpyrrolidone (PVP) storage disease simulating signet-ring cell carcinoma.

Kuo TT, Hsueh S.

Am J Surg Pathol. 1984 Jun;8(6):419-28. Abstract quote  

In Taiwan, a peculiar type of cell with mucicarmine-positive bubbly or vacuolated cytoplasm was sporadically observed in a variety of surgical specimens removed for neoplastic or non-neoplastic conditions. They closely mimicked signet-ring cell carcinoma.

Study of 14 such cases and review of the related literature led to the conclusion that the peculiar cells were storage histiocytes containing polyvinylpyrrolidone (PVP). Because they were shown to be mucicarmine-positive, we designated them " mucicarminophilic histiocytes." Particulate bodies sometimes observed in association with mucicarminophilic histiocytes were believed to be the extracellular counterpart of the cytoplasmic vacuoles.

Our survey also revealed that PVP-containing intravenous injection fluids had been used in Taiwan as recently as 5 years ago. For surgical pathologists, PVP-containing mucicarminophilic histiocytes should not be mistaken for signet-ring cell carcinoma or signet-ring cell lymphoma.

Mucicarminophilic histiocytes can be identified by the positive mucicarmine, colloidal iron, Gomori methenamine silver, Congo red, Sudan black B, Fontana-Masson argentaffin, and Victoria blue; but negative periodic acid-Schiff, alcian blue, and Giemsa stains.

 

SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHER
CHARACTERIZATION
SPECIAL STAINS Histiocytes are positive for:
Mucicarmine
Colloidal iron
G omori methenamine silver
Congo red
Sudan black B
Fontana-Masson argentaffin
Victoria blue

Negative for periodic acid-Schiff, alcian blue, and Giemsa stains
IMMUNOPEROXIDASE  
ELECTRON MICROSCOPY  

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
GASTRIC ADENOCARCINOMA, SIGNET RING TYPE  
Polyvinylpyrrolidone storage disease: a source of error in the diagnosis of signet ring cell gastric adenocarcinoma.

Hewan-Lowe K, Hammers Y, Lyons JM, Wilcox CM.

Department of Pathology, Grady Memorial Hospital, Atlanta, Georgia 30335.

Ultrastruct Pathol. 1994 Jan-Apr;18(1-2):271-8. Abstract quote  

False-positive and false-negative diagnoses of cancer seldom occur during evaluation of gastric mucosal biopsy specimens obtained during flexible fiberoptic endoscopy of the stomach. When they do occur, false-negative diagnoses are usually the result of undersampling of the lesion, whereas false-positive diagnoses are the result of overinterpretation of benign histologic lesions.

The diagnosis of diffuse signet ring cell adenocarcinoma is associated with both false-positive and false-negative diagnoses of cancer. The signet ring cell can be easily overlooked. Mucicarminophilic cells can be overinterpreted as diffuse signet ring cell adenocarcinoma.

Polyvinylpyrrolidone is an unusual cause of this type of false-positive diagnosis of gastric adenocarcinoma. Judicious use of histochemical stains, immunohistochemistry, and electron microscopy plays a significant role in avoiding the false-positive diagnosis of diffuse signet ring cell adenocarcinoma.
MUCOPOLYSACCHARIDOSES  
Storage of polyvinylpyrrolidone mimicking a congenital mucolipid storage disease in a patient with Munchausen's syndrome.

Bubis JJ, Cohen S, Dinbar J, Hirschhorn B, Szeinberg A, Wolman M.

Isr J Med Sci. 1975 Oct;11(10):999-1004. Abstract quote  

A 24-year-old woman from the Sinai peninsula had, since the age of 15 years, repeatedly undergone surgery because of gastrointestinal bleeding. The bleeding was found to have been caused by self-inflicted injuries.

Histiocytes in the patient's liver and lymph nodes (the spleen had been removed nine years previously) contained a peculiar material which was, in part, basophilic and had puzzling histochemical characteristics.

The material is believed to be polyvinylpyrrolidone (PVP), which had been administered to the patient during one of her operations. The importance of recognizing this change, especially in countries where PVP is administered as a plasma expander, is stressed.

 

PROGNOSIS CHARACTERIZATION
GENERAL Variable depending upon extent of tissue deposit and which organ is affected

 

TREATMENT CHARACTERIZATION
GENERAL No effective treatment

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.


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Last Updated July 7, 2006

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