# Pupp syndrome



## PennyG (Oct 12, 2010)

Looking for the diagnosis code for PUPP Syndrome.  Can anyone help me?


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## preserene (Oct 12, 2010)

Is it in pregnancy or in a carcinoma status?
Since you posted the thread in OBGYN , I assume you are asking for PUPPP in Pregnancy.

 All these or some of them could be manifested in pregnancy-*Pruritic/urticarial/papules/plaques/pregnancy(PUPPP*

You would not be able to get a code with the same terminology. But this is more suitable and appropriate I believe : *698.9*
Give also the code for pregnancy Status . if any specified condition you have , then give *698.8*
This rules out  Herpes gestationis ; it has a separate code and it is not included in PUPPP.


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## gost (Oct 12, 2010)

For PUPPS, i would go with 646.8x and 708.1.


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## preserene (Oct 12, 2010)

when you say PUPP S, it is related to 2conditions -Pregnancy and Ca.

Pupps in Pregnancy specifically rules out the code for Herpes gestationis( Viral/infectious cause). So the code 646.8 is ruled out. 
The code for UTRECARIA  cannnot be given in this condition of pregnancy  why ? because the condition PUPPP in pregnancy, has only a component of the whole scenerio ie 'U' in PUPPP syndrome. It is a SYNDROME.
If the patient has liver disorders in pregnancy , I may consider because the  most of the liver disorders in pregnancy has many of the symptoms& signs meriting to PUPP.

However without the other info associated in this condition with the fact , that PUPPP as such do not show a definitive code in ICD-9, I would opt for what I said before


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## gost (Oct 13, 2010)

While 646.8x is used to report Herpes Gestationis, it is not exclusive to this dx.  646.8x is "Other unspecified complications of preganacy."  As there is no specific code for PUPPS and PUPPS is pregnancy related by definition, I stand by my assertion that 646.8x is correct in this case.

Prserene, maybe I am misunderstanding your terminology but did you say PUPPS is related to cancer?  (You said pregancy and "Ca" which, in my experience is used to abbreviate "cancer.")  Pruritic Urticarial Papules and Plaques of Preganacy (PUPPP or PUPPS) has no connection with cancer that I am aware of.  However, to report pruritic urticaria, I again stand by my choice of 708.1.


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## preserene (Oct 13, 2010)

It is a single entity  syndrome occurring in pregnancy;  not double diagnosis as to give 646.8 and 708.1.
646.8 is not exclusive for Herpes gestationis, but the other specified conditions are not correlated with pruritus, plagues or papules in that category, either.
(PUPPP Syndrome occurs in later months of preg)
Early PUPPP demonstrating urticarial papules and plaques on the abdomen with predilection for striae. More severe case of PUPPP with confluent urticarial plaque on abdomen  and lower extremities . 
 It occurs mostly during late 3rd trimester usually affects women with their first pregnancy. Most women that have PUPPP in their first pregnancy do not experience it again with other pregnancies.. 
There is no reason to worry about the rash harming mother or baby. The average rash will last around six weeks and it will all go away after delivery. The cause of PUPPP is not fully understood, although researchers believe it may have something to do with the fetal DNA of a male baby. The treatment is symptomatic with ointments and some times steroids The prognosis is exelent.
Pemphigoid gestationis (PG), also known as Herpes gestationis (HG), is an autoimmune bullous disease of pregnancy. Despite its name, this disease has no relationship to herpes simplex virus, but was so called because of the herpes-like nature of the blisters. This dermatosis classically develops in the second or third trimester (mean of 21 weeks).11 It occurs in 1 in 50,000 pregnancies, and has a strong association with HLA-DR3 and HLA-DR4, which might explain the greater prevalence of this condition in white women compared to black women.12 
Pemphigoid gestationis begins with the sudden onset of intensely itchy, urticarial lesions, which are found on the abdomen in 50% of cases. At this stage, it is very difficult to distinguish this disease from PUPPP. The lesions then progress to a generalized bullous eruption that usually spares the face, mucous membranes, palms and soles (Figure 4).9 The disease often resolves during the latter part of pregnancy, and flares at delivery or immediately postpartum in more than 60% of cases; 25% of cases appear for the first time after delivery
[Erythematous papules and plaques on abdomen with erosions at sites of previous vesicles in a patient with HG. Immunoglobulin G (IgG) autoantibodies are produced against the target antigen, known as bullous pemphigoid antigen 2 (BPAG2) or BP180 due to its 180-kD size. Bullous pemphigoid antigen 2 is a component of the hemidesmosome, and is critical in epidermal-dermal adhesion. Epitope mapping has demonstrated that PG autoantibodies bind an antigenic site (MCW-1) within the noncollagenous domain (NC16A) of the transmembrane 180-kD PG antigen.]
 The binding of IgG to the cutaneous basement membrane is believed to trigger an immune response that leads to the formation of subepidermal vesicles.14 Biopsy results reveal a subepidermal blister with an eosinophil-predominant infiltrate. 
The infiltrate is localized to the dermal-epidermal junction and perivascular areas. 
Direct Immunofluorescence (DIF) is the most sensitive and specific assay for differentiating PG from PUPPP. Performed on perilesional skin, DIF shows a linear band of C3 and/or IgG at the basement membrane. Salt-split skin studies demonstrate antibody binding to the roof of the vesicle.9 
Typically, PG regresses without scarring a few weeks after delivery. Affected women have an increased risk of subsequent development of autoimmune disorders, particularly Graves' disease. There is also a risk that PG will recur in subsequent pregnancies, often with increased severity, or in association with menses or OC use. Infants are not at higher risk for mortality, but may be born small for gestational age or premature. Up to 10% of infants will develop neonatal PG, but the disease abates without sequelae on clearance of maternal antibodies.
Urticaria, commonly known as hives, is a skin reaction pattern characterized by the appearance of itchy (pruritic), red (erythematous) raised welts or lumps called "wheals" on the skin. They usually occur in batches.  Chronic utrecaria can be immnnological/ hereditary.  Cause may be due  to allergy, infection, insect  or spider bite or immunological disorder associated. It  does not have predeliction to pregnancy  and no bearing  and waiting for the culmination of pregnancy. It has no affinity for some particular sites of the body too.
Whatever code you give, it is your choice.  But let us be fortunate enough to know the general guide acceptance/payers acceptance.
 Would the thread Poster/ Initiator make us to know which was the accepted code for the scenario?.
Well, if your code 646.8x IS OF the highest specific, then about hundreds of conditions can be reported into that category during pregnancy-other specific complications of pregnancy( not necessarily COMPLICATING PREGNANCY).


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## gost (Oct 15, 2010)

OK.  I don't want to get into an endless debate on this so I will tell you why I disagree with you and tell you why I think I am correct and then I will let this drop.  Before I do, let me add this caveat:  I admit that I have not closely read your last post because of it's length.  It appears to be an in-depth commentary on herpes gestationis.  I've ignored this because this condition has no association with HG except that the same code is used to report both conditions.

1.  You state that this is a "single entity syndrome" and does not justify using 2 ICD-9 codes.  Yet you suggest using 698.9 and a pregancy status code.

2.  PUPPS is by definition a pregnancy related condition.  A condition caused by pregancy is definitely a complication of pregnancy.  If the skin rash occurs without pregnancy being present, it is no longer PUPPS.

3.  Syndrome or not, if there is no specific ICD-9 code for the condition, the condition is coded either to the broad "NOS," "NEC," or "other..." or is coded to symptoms, whichever is appropriate.  Since there is no code for PUPPS 646.8x is the only viable choice.  I would also add 708.1 to clarify what the complication is but I suppose that is optional.

4.  Finally, just to satisfy my curiosity, I did a Google search for PUPPS ICD-9 codes.  I didn't find much but I did find that several coding forums, payer policies, and provider web sites seem to agree on the primary dx being 646.8x.

I hope you can see my reasoning.  It is not my intention to be argumentative.  I just don't want to see anyone coding incorrectly if it can be avoided.


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## preserene (Oct 15, 2010)

Niether I agree with you  or I don't have to even others say. Because  this 646.8 particularly deals with Herpes gestionis and conditions which are not at all in anyway related to those symptoms and signs occuring in PUPPP syndrome. Yes I am aware there is no cod efor PUPPP. 
Why I said the pregancy status becausejust to suppor the the last letter "P" has to be addressed to  which is the mainstay. it is actually PUPPP. That is not the point for arguement anyway.
Having said that any unrelated conditions to the presenting syptoms cannot be accepted with, which does not address the criteria we need there( meaning having just only pregnancy state)
Yet another point is this can be taken as an occurence of pregnancy not in the true sense of complication. That is why the last P is added to it!  By and large it does not complicate the pregnancy; it is with excellent prognosis and limited by culminating delivery.
So, definitely it is not a condition complicating pregnancy.
By the way can you furnish those webs or threads you said just for my analysis,if you wish to. .
 Thank you and Bye Gost


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## DoDCoder4You (Oct 18, 2010)

PUPPP Syndrome, also known as Pruritic Urticarial Papules and Plaques of Pregnancy is a rare (approximately 160-250 women world-wide; 15-25% in multiparous women) but tremendously uncomfortable condition that affects expectant mothers in the latter weeks of pregnancy. The severe itching of PUPPP significantly reduces sleep in the weeks prior to birth. Skin eruptions are generally located on the lower abdomen, below the umbilicus and on the extremities ~ especially on areas stretched thin with stretch marks. PUPPP usually does not affect the face, palms, or soles of the feet. Generally, expectant women with PUPPP have greater weight gain, babies that are heavier than normal and an increased chance of having twins.


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