Dermatology Online Journal

Combination therapy with intralesional triamcinolone and oral dapsone for management of palisaded neutrophilic and granulomatous dermatitis
Yong He, Emanual Maverakis MD, Dalila Ramirez-Maverakis, Sarah Fitzmaurice MD
Dermatology Online Journal 19 (2): 17

University of California, Davis, Department of Dermatology, Sacramento, California

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Abstract

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a rare granulomatous disease with limited therapeutic options. Herein we present a patient whose skin lesions recurred after surgery and oral steroids, but responded well to a combination of intralesional triamcinolone and oral dapsone.

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Clinical synopsis

Figure 1	Figure 2
Figure 1. Palisaded neutrophilic and granulomatous dermatitis Figure 1a) Before treatment: large exophytic nodules proximal to the metacarpalphalangeal joints and distal to the proximal interphalangeal joints that limited hand mobility Figure 1b) After dapsone with intralesional triamcinolone therapy: nodules have flattened almost completely with full recovery of hand mobility Figure 2. Punch biopsy specimen of metacarpalphalangeal nodule showing: Figure 2a) Numerous palisaded granulomas involving the superficial and deep dermis (H&E x40) Figure 2b) Palisaded histiocytes, neutrophils, and lymphocytes with altered collagen and central fibrin (H&E x100) Figure 2c) Inflammatory infiltrate composed of histiocytes, neutrophils, and lymphocytes (H&E x400)

A 77-year-old male with past medical history of polymyalgia rheumatica (PMR) was referred to dermatology for evaluation and treatment of multiple erythematous firm nodules distributed over the dorsal aspect of his hands. The initial lesion appeared approximately 9 years earlier and a biopsy at that time demonstrated palisading granulomas consistent with rheumatoid nodule. The nodule was treated with surgical excision but it recurred within a few years. The patient subsequently presented to rheumatology with numerous tender granulomatous nodules on the metacarpalphalangeal and proximal interphalangeal joints bilaterally (Figure 1a). Work-up for rheumatoid arthritis was negative including a negative rheumatoid factor and anti-CCP antibody titer. The nodules on his hands continued to increase in size in the setting of concomitant treatment for PMR with hydroxychloroquine (400 mg daily) and prednisone (up to 20 mg daily). Hand strength and mobility began to decrease. Dermatology was consulted. On exam, there were large erythematous and exophytic nodules at the metacarpalphalangeal, distal interphalangeal, and proximal interphalangeal joints bilaterally that were mobile and mildly tender to palpation. A punch biopsy of one of the nodules showed inflammatory infiltrates composed of histiocytes and lymphocytes arranged in a palisade around fibrin and fragmented neutrophils, a pattern consistent with palisaded neutrophilic and granulomatous dermatitis (Figure 2).

Given that the lesions were seemingly unresponsive to prednisone and hydroxychloroquine, the patient was started on intralesional triamcinolone 40 mg/mL to one of the lesions. On follow-up, the injected lesion appeared to be less erythematous, less tender, and more pliable than the non-injected lesions. The patient was then started on oral dapsone, which was titrated to 75mg twice daily over the course of 3 months. Intralesional triamcinolone was continued once monthly. Two months after initiating dapsone, all nodules had responded, with the injected nodule more improved than the non-injected nodules (Figure 1b). The patient’s hand grip also improved, returning to normal.

Discussion

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a rare heterogeneous entity that has been described to encompass Churg-Strauss granuloma, rheumatoid papules, superficial ulcerating rheumatoid necrobiosis, and interstitial granulomatous dermatitis with arthritis [1]. Clinically, the lesions range from fleshy pink to violaceous papules and nodules to cutaneous cords and bands [2]. Lesions are usually asymptomatic but can be pruritic and/or tender. Most often, PNGD occurs in the setting of connective tissue disease but it can also occur in the absence of systemic disease. There have also been several reports in the literature of PNGD developing secondary to drugs, specifically TNF-alpha inhibitors and methotrexate [3].

Much like its clinical presentation, the histopathologic features of PNGD vary. In an analysis of 9 patients, Chu et al attempts to classify the spectrum of histopathologic findings into early and late lesions [4]. “Early lesions” show evidence of leukocytoclastic vasculitis with diffuse, pandermal infiltrates composed of neutrophils, nuclear debris, and strands of amorphous basophilic material. “Late lesions” lack leukocytoclasia, with palisaded granulomas and degenerated collagen being the predominant finding.

PNGD may persist for months to years before resolving on its own. Owing to its rarity, no therapies have been extensively studied but oral glucocorticoids have been the treatment of choice. Other treatments that have been met with variable success include colchicine, cyclosporine, cyclophosphamide, hydroxychloroquine, NSAIDS, and dapsone [5].

In our patient, the PNGD recurred several years after surgical excision and did not respond to prednisone up to 20 mg daily and hydroxychloroquine 400 mg daily that had been prescribed for management of his PMR. The nodules also did not resolve on their own with time but in fact, became more numerous, erythematous, and painful. With institution of intralesional triamcinolone and oral dapsone, the patient noted resolution of his nodules and recovery of hand function.

In a similar case, Jarrett and Martin describes a 84-year-old female with PNGD that did not respond to topical clobetasol and increasing doses of prednisone up to 20 mg daily. Dapsone 50 mg was started and they noted complete resolution of skin lesions within 6 weeks [6]. In another case, Al-Daraji et al, describes a 54-year-old female with PNGD treated successfully with dapsone [7]. However, in both reports lesions were less nodular than described in our case.

The etiology of PNGD has not been elucidated but it is hypothesized that deposition of immune complexes in dermal vessels lead to ischemic injury and subsequent fibrosis. Indeed direct immunofluorescence studies have demonstrated presence of immunoglobulins and C3 deposits in vessel walls [5, 8]. Current therapies are purely anecdotal; from our experience, intralesional triamcinolone in combination with dapsone can be an effective treatment of patients with PNGD.

References

1. Sangueza OP, Caudell MD, Mengesha YM, Davis LS, Barnes CJ, Griffin JE, Fleischer AB, Jorizzo JL. Palisaded neutrophilic granulomatous dermatitis in rheumatoid arthritis. J Am Acad Dermatol. 2002;47:251-257 [PubMed]

2. Gottlieb GJ, Duve RS, Ackerman AB. Interstitial granulomatous dermatitis with cutaneous cords and arthritis: linear subcutaneous bands in rheumatoid arthritis revisited. Dermatopathology: Pract Conceptual. 1995;1:3-6

3. Stephenson SR, Campbell SM, Drew GS, and Magro CM. Palisaded neutrophilic and granulomatous dermatitis in a patient with rheumatoid arthritis on adalimumab. J Cutan Pathol. 2011;38:644-648 [PubMed]

4. Chu P, Connolly MK, LeBoit P. The histopathologic spectrum of palisaded neutrophilic and granulomatous dermatitis in patients with collagen vascular disease. Arch Dermatol. 1994;130:1278 [PubMed]

5. Finan MC and Winkelmann RK. The Cutaneous Extravascular Necrotizing Granuloma(Churg-Strauss Granuloma) and Systemic Disease: A Review of 27 Cases. Med Baltimore. 1983;62:142-158 [PubMed]

6. Martin JA and Jarrett P. Rheumatoid papules treated with dapsone. Clin Exp Dermatol. 2004;29:387-389 [PubMed]

7. Al-Daraji WI, Coulson IH, Howat AJ. Palisaded neutrophilic and granulomatous dermatitis. Clin Exp Dermatol. 2005;30:578-602 [PubMed]

8. Higaki Y, Yamashita H, Sato K et al. Rheumatoid papules: a report on four patients with histopathologic analysis. J Am Acad Dermatol. 1993; 28: 406-411 [PubMed]

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