|Year : 2017 | Volume
| Issue : 2 | Page : 125-127
Palisaded granulomatous neutrophilic dermatosis in systemic lupus erythematosus
Prachi Srivastava1, Anand Kumar Vaggu1, K Sujatha2, Jaideep Khare3
1 Department of Dermatology, Krishna Institute of Medical Sciences, Secunderabad, Telangana, India
2 Department of Pathology, Krishna Institute of Medical Sciences, Secunderabad, Telangana, India
3 Department of Endocrinology, Krishna Institute of Medical Sciences, Secunderabad, Telangana, India
|Date of Web Publication||27-Mar-2017|
Department of Dermatology, Krishna Institute of Medical Sciences, Ministers Road, Secunderabad - 500 003, Telangana
Source of Support: None, Conflict of Interest: None
Palisaded granulomatous neutrophilic dermatosis (PGND) is a cutaneous reaction pattern seen in association with some systemic diseases. It is mostly seen in adult patients with only three case reports of these lesions in children. We hereby report a 12-year-old female child with systemic lupus erythematosus flare having PGND, showing two different morphologies clinically which were also confirmed on histology depicting the much-speculated evolution of the disease. We are reporting the case for the rarity in pediatric patients, the presentation of different morphological variants in the same patient as well as the uncommon site of involvement.
Keywords: Child, flexor aspect involvement, palisaded granulomatous neutrophilic dermatosis, systemic lupus erythematosus flare
|How to cite this article:|
Srivastava P, Vaggu AK, Sujatha K, Khare J. Palisaded granulomatous neutrophilic dermatosis in systemic lupus erythematosus. Indian J Paediatr Dermatol 2017;18:125-7
|How to cite this URL:|
Srivastava P, Vaggu AK, Sujatha K, Khare J. Palisaded granulomatous neutrophilic dermatosis in systemic lupus erythematosus. Indian J Paediatr Dermatol [serial online] 2017 [cited 2020 Sep 20];18:125-7. Available from: http://www.ijpd.in/text.asp?2017/18/2/125/188450
| Introduction|| |
Palisaded granulomatous neutrophilic dermatosis (PGND) is now an umbrella term which includes interstitial granulomatous dermatitis, Churg–Strauss granuloma, and interstitial granulomatous drug reaction described previously. It is triggered by systemic disorders like connective tissue diseases (systemic lupus erythematosus [SLE], Sjogren's syndrome), vasculitis, arthritides such as rheumatoid and other arthritis and malignancy. It has varied clinical presentation ranging from leukocytoclastic vasculitis such as purpura, papules, and nodules to annular lesions.
| Case Report|| |
A 12-year-old female child, diagnosed case of SLE and on treatment since 18 months with azathioprine and hydroxychloroquine presented with red raised lesions on the flexor aspect of the thighs [Figure 1] and purpuric lesions on the shin of 10 days duration [Figure 2]. They were static since then and were accompanied with fever, polyarthralgia, and generalized weakness.
|Figure 1: Papular lesions on the flexor aspect of the thighs and buttocks|
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Cutaneous examination revealed bilaterally symmetrically distributed, well-defined erythematous papules on the flexor aspect of the lower legs, thighs extending up to the buttocks. The surface of a few papules showed central crusting and the surrounding skin was normal. Furthermore, the shin showed multiple nonblanching, purpuric macules up to the mid shin level. All the lesions were nontender. There were no lesions elsewhere on the body including the oral and genital mucosa. We kept a differential diagnosis of palisaded granulomatous neutrophilic dermatosis, vasculitis, and granuloma annulare and performed two biopsies, one from the nodular lesion and other from purpuric macule and sent it for histopathological examination as well as direct immunoflurosence.
To our surprise, both the biopsies revealed a similar picture showing small vessel vasculitis [Figure 3] with nuclear dust and mild fibrin deposition in mid and lower dermis and also granulomatous dermatitis in the same region. The granulomas consisted of degenerated collagen surrounded by neutrophils and a few lymphocytes [Figure 4]. The vasculitic picture dominated in the biopsy from the purpuric macule whereas the granulomatous one in the biopsy from the nodular lesion. Her laboratory investigations showed antinuclear antibody positivity with a titer of 1:100 and homogenous pattern, dsDNA positive, anemia with Hb of 9.3, and anti-histone antibodies positive. With these clinical and histopathologic findings, she was diagnosed as PGND in a case of flare of SLE. Topical Mometasone was added along with oral prednisolone for SLE flare management which was given at the dose of 1 mg/kg that is, 35 mg/day for 2 weeks which was then tapered to 0.5 mg/kg per day at the time of discharge. Subsequently, she was continued on 5 mg of Prednisolone daily by tapering 0.5 mg/week. She had an excellent response to the above line of therapy with complete resolution of lesions in 2 weeks.
|Figure 3: Histopathology from purpuric macule showing leukocytoclastic vasculitic picture with transmural infiltrate of neutrophils and nuclear debris|
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|Figure 4: Histopathology from papule showing degenerated collagen surrounded by neutrophils and occasional lymphocytes|
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| Discussion|| |
PGND is cutaneous reaction pattern seen in association with a few systemic diseases. The term was coined by Chu et al. in 1994; however, Finan and Winkelman first described this condition under the term Churg–Strauss granuloma in 1983., Typically, the lesions appear bilaterally symmetrically on the extensor aspect of the extremities and sometimes on the lateral aspect of the trunk. Clinically, the presentation may vary from papules and nodules to annular plaques.,, Few authors feel that the clinical spectrum may represent lesions of the same process seen at different stages of evolution. It is associated with various systemic diseases, the most common being rheumatoid arthritis (26.8%) followed by SLE (11.3%). The other associations being ANCA-associated vasculitis, Sjogren's syndrome, ankylosing spondylosis, acute myeloid leukemia, sarcoidosis, and ulcerative colitis.
The pathogenesis is suspected to be a reaction pattern owing to underlying inflammation. It is thought to evolve, beginning as leukocytoclastic vasculitis and then progressing through collagen degeneration followed by chronic inflammation and fibrosis causing the spectrum of clinical and histologic features. This evolution is represented histologically by three patterns depending upon the chronology of lesions with early lesions showing leukocytoclastic vasculitis, fully developed lesions resembling granuloma annulare and at the extreme end necrobiosis lipoidica like histology., There are also reports of PGND developing secondary to drugs like adalimumab.,
To the best of our knowledge, there are only three prior reports of PGND in children, first in a 10-year-old girl with Type 1 diabetes mellitus and celiac disease and the other two in SLE with one having annular morphology and the other having papulonodular presentation. Our patient showed leukocytoclastic vasculitis-like purpura as well as papules thereby depicting the clinical evolution of the disease which was also seen histologically with both vasculitic and granulomatous changes being seen in the same biopsy. Furthermore, these lesions are commonly present in the upper extremities (51%) whereas our patient had them only on the lower extremity which is seen only in 27% of the cases.
In addition, the lesions were present in the flexor aspect in contrast to the more common extensor site of involvement.,,, This has not been reported previously. PGND in SLE has been reported with flare of the disease, which was again confirmed in our case.,
About 20% of the cases may resolve spontaneously, however, the main target of therapy is to identify the underlying disease and control it. The various agents reported to be useful are NSAID's, colchicine, cyclosporine, hydroxychloroquine, and systemic steroids.,
| Conclusion|| |
We would like to emphasize on the consideration of PGND as a differential diagnosis in all patients with a history of collagen vascular disease or autoimmune disorders who present with vasculitic or papulonodular lesions on extremities.
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Conflicts of Interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]