Plum-colored tender Plaques, Palmar Xanthoma and IgM Gammapathy

presented by

Omid Zargari MD (1) and

Catherine Kiavash MD (2), Rasht, Iran

on December 19, 2004

(1) Assistant Professor of Dermatology (2) Resident, Department of Dermatology, Razi Hospital, Guilan University of Medical Sciences, Rasht, Iran
 
Abstract Sweet's syndrome is a condition characterized by the sudden onset of fever, leukocytosis, and tender, erythematous papules and plaques. This syndrome may be associated with hematologic and immunologic diseases. Here, we present a case of Sweet's syndrome with gammapathy.
Patient
O.A., 51-year-old woman
Duration
7 days
Distribution
Knees, legs, ankles, palms
History

Presented in May 2004 with one-week history for tender and pruritic plaques on the knees, legs, ankles and palms. Fever and bilateral arthralgia in knee joints were among the other complaints.
Past medical history was remarkable for diabetes mellitus (NIDDM) and hypertension from eight years ago and a history of transient ischemic attack.
Physical Examination

Oral temperature was 38.5ºC at the time of admission. Tender, dull-red plaques were noticeable on the knees, legs, and ankles in association with pitting edema. Similar lesions were present in both palms. Also, bilateral palmar xanthomas were seen. There was no sign of conjunctivitis or episcleritis.
Images

Laboratory Data

WBC: 14100 /mL with 84% PMN
Hb: 11.7 g/dL
CRP:+ ESR: 87mm
Cholesterol: 299mg% TG:991mg% LDL:213mg%
FBS:137mg%
Urinanalysis: proteinuria (+), glucosuria, trace hematuria, Bence Jones protein (negative)
Serum protein electrophoresis: raised IgM (1.5x more than normal)
Liver, kidney, and thyroid function tests: Normal
Histopathology

Skin biopsy taken from the lesion on the palm showed massive focal infiltration of mature polymorphonuclear cells in the dermis, vasodilatation and slight endothelial swelling.

Diagnosis Acute febrile neutrophilic dermatosis (Sweet’s syndrome)
+ Plane (palmar)xanthoma+ Gammapathy
Reasons Presented

Systemic steroid (30 mg/day prednisolone) was started for the patient and was associated with rapid improvement.

This syndrome was first described by Robert Douglas Sweet in 1964 as Acute Febrile Neutrophilic Dermatosis and is manifested by an acute onset of erythematous plaques, nodules, and occasionally vesicules and pustules, often accompanied with fever and neutrophilia.(1) The etiology of Sweet’s syndrome is unknown, though it is presumed to be a hypersensitivity reaction which leads to stimulation of a cascade of cytokines that precipitate neutrophil activation and infiltration. Von den Dreisch categorized this syndrome into four groups: idiopathic, parainflammatory, paraneoplastic, and pregnancy-related.(2)
Plane xanthomas may be associated with monoclonal gammapathy(3) and hyperlipidemia, particularly hypertriglyceridemia. This case had both of these conditions.
Questions

1. Is there any “association’’ between the Sweet’s syndrome and gammapathy or this is a “co-incidence”?
2. Do you recommend any further paraclinical investigations?

References

1. Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964;76:349
2. Von den Driesch P. Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994;31(4):557-60 (view abstract)
3. Modiano P. et al. [Normolipemic plane xanthoma, monoclonal gammopathy, anti-lipoprotein activity, hypocomplementemia]
Ann Dermatol Venereol. 1995;122(8):507-8.
Comments from Faculty and Members

Jerome Z Litt MD, Assistant Clinical Professor of Dermatology, Case Western Reserve University School of Medicine, Cleveland, OH, USA on Dec 19, 2004

There are reports in the literature that the following drugs can cause Sweet's syndrome:
1. celecoxib (2001)
2. clofazimine (1994)
3. clozapine (2002)
4. co-trimoxazole (1996) (1989) (1986)
5. cytarabine (1993)
6. furosemide (1989)
7. gabapentin (2001) (1999) - ANECDOTES
8. glucagon (seven reports)
9. GCSF (twenty-eight reports!)
10. Hydralazine (five reports)
11. Hydroxyurea (2002)
12. Infliximab (2003)
13. Isotretinoin (2003)
14. Minocycline (three)
15. Nitrofurantoin (1999)
16. Oral Contraceptives (two)
17. Sulfamethoxazole (three)
18. Tretinoin (three)
19. Verapamil (1998)

Irwin Hametz MD, Freehold, NJ, USA on Dec 17, 2004

I am familiar with Sweets, having several cases over the years. There is likely to be recurrent episodes, and there is likely to be an underlying disease with the gammapathy. Please follow for malignant transformation of the gammapathy at some later date.

Khalifa Shaquie MD, PhD, Professor of Dermatology, College of Medicine, University of Baghdad, Baghdad, Iraq on Jan 7, 2005

I think all features of the case is in favour of erythema nodosum(EN) rather than Sweet's disease(SD) for the following reasons:
1-EN is relatively a common condition while SD is rare problem 2-The shins are the typical areas for EN 3-Tender indurated red plaques on shins in a symmetrical fashion is a classical presentation of EN 4-Fever & arthralgia are symptoms of EN 5-In early lesions of EN, neutrophilic infiltrate could be seen in skin biopsy.The fatty layer is unfortunately is not included in the biopsy of the present case 6-EN responds promptly to oral steroid 7-EN could be manifestation of Behcet's disease which is common in Iran & Iraq. Also SD could be a feature in Behcet's disease.
8-Ig gammapathy is not a rare coincidental finding in this age group. Still bone marrow aspiration is recommended.

Omid Zargari MD, Assistant Professor of Dermatology, Razi Hospital, Guilan University of Medical Sciences, Rasht, Iran on Jan 8, 2005

Thanks to Prof. Sharquie for his/her comments. It’s going to become a good discussion. Let’s see what the comments of our colleague from Baghdad are;
1- EN is relatively a common condition while SD is rare problem
It is correct, but I think using the rate of incidence of a disease for making a diagnosis is some sort of oversimplification.
2-The shins are the typical areas for EN 3-Tender indurated red plaques on shins in a symmetrical fashion is a classical presentation of EN It is also absolutely correct, but what about hands? The patient had significant involvement of both hands, which I think is not a typical area for EN but a common site for Sweet’s syndrome.
4-Fever & arthralgia are symptoms of EN
As same as Sweet’s syndrome. In addition, In Sweet’s syndrome, neutrophilia is another finding and fever is usually more prominent as in this case.
5-In early lesions of EN, neutrophilic infiltrate could be seen in skin biopsy.The fatty layer is unfotunately is not included in the biopsy of the present case 6-EN responds promptly to oral steroid Again, as like as Sweet’s syndrome. Which one responds more promptly? Indeed, good response to systemic corticosteroids is considered one of minor diagnostic criteria for Sweet’s syndrome.
7-EN could be manifestation of Behcets disease which is common in Iran & Iraq. Also SD could be a feature in Behcets disease.
These facts are not differentiating between EN and Sweet’s syndrome. Yes, Behcet’s disease (BD) is not rare in this area, but the patient had not any other sign (including aphthae) of BD.
8-Ig gammapathy is not a rare coincidental finding in this age group. Still bone marrow aspiration is recommeded.
This is the answer of one of my questions (to do or not to do further work up?) that unfortunately nobody responded!
The patient is currently under observing of an oncologist.

I think Prof. Sharquie denoted correctly to possible similarities between EN and Sweet’s syndrome, not to the differential points.
This patient had typical cutaneous lesions, a completely compatible histopathology (predominantly neutrophilic infiltration in dermis-I accept that quality of photography is not good) and three minor criteria; 1. Accompanying fever and arthralgia, 2. Leukocytosis (>8000), ESR>20, positive CRP, and 3. Excellent response to systemic steroids.

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