Palisaded neutrophilic and granulomatous dermatitis: An unusual cutaneous manifestation of immune-mediated disorders (2022)

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Seminars in Arthritis and Rheumatism

Volume 34, Issue 3,

December 2004

, Pages 610-616

Objective

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is an uncommon skin eruption most often associated with rheumatoid arthritis and other immune-mediated diseases. We present 4 cases to familiarize rheumatologists with the clinical presentation and histopathology of PNGD.

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Methods

We report 4 cases to illustrate the clinical and histologic spectrum of this rare skin disease found in rheumatologic patients. The disease pathogenesis and treatment options are discussed.

Results

All 4 patients presented with symmetric erythematous-to-violaceous papules and plaques that, upon biopsy, revealed a dermatitis composed of variable numbers of histiocytes and neutrophils. Some cases responded to topical corticosteroid treatment or to dapsone, whereas others resolved spontaneously.

Conclusions

PNGD is a rare cutaneous finding in patients with a variety of immune-mediated systemic diseases, most often rheumatoid arthritis. It is a benign condition that may spontaneously remit or may respond favorably to topical corticosteroids or dapsone.

Section snippets

Case 1

A 57-year-old woman with seronegative rheumatoid arthritis of 6 years’ duration presented with a 3-week history of a slightly pruritic eruption on her medial thighs, back, and shoulders. She also complained of worsening arthritis, with development of effusions in her knees. She had been treated with methotrexate and started infliximab therapy 3 months before the eruption. She was referred to the dermatology department because of concerns that her skin eruption might represent a drug reaction,

Discussion

Very likely, the first cases of PNGD were described in 1965 by Dykman et al. They reported 2 cases of patients with rheumatoid arthritis who developed linear cords on the lateral trunk (7). Biopsy of these cords showed an interstitial granulomatous dermatitis with lymphocytes and neutrophils, as well as areas of degenerated collagen. They depicted this as an “unusual form of rheumatoid granuloma.” Gottlieb et al later described the histopathologic characteristics of similar linear cords in

References (17)

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Cited by (53)

  • Palisaded neutrophilic granulomatous dermatitis: Spectrum of histologic findings in a single patient

    2017, JAAD Case Reports

    Clinically, PNGD presents with tender, erythematous-to-violaceous papules, plaques, or nodules affecting the extensor surfaces. Acral, including palmar, involvement is characteristic, and umbilicated papules overlying bony prominences are also described.1,2,5 Histologically, early lesions of PNGD present with neutrophilic infiltrates and leukocytoclastic vasculitis; fully developed lesions feature palisaded granulomas with collagen trapping and neutrophil remnants.1

  • Reactive Granulomatous Dermatitis: A Review of Palisaded Neutrophilic and Granulomatous Dermatitis, Interstitial Granulomatous Dermatitis, Interstitial Granulomatous Drug Reaction, and a Proposed Reclassification

    2015, Dermatologic Clinics

    As detailed, there exists substantial overlap between PNGD, IGD, and IGDR. The terms are at times used interchangeably in the literature,1,23 and some authors view drug-induced IGD and IGDR as the same entity with authors viewing IGD as a subset of PNGD, whereas other authors draw strict distinctions. The confusing nomenclature dates back to the earliest report, by Dykman and colleagues32 in 1965, who are credited with the first report of PNGD while describing patients with rheumatoid arthritis with linear subcutaneous bands, although that report was later the basis for further reports by Ackerman in 1993,30 which led to the term Ackerman dermatitis, which is felt by many to represent what is currently known as IGD.1,11

  • Emerging Adverse Cutaneous Drug Reactions

    2012, Dermatologic Clinics

  • Palisaded neutrophilic and granulomatous dermatitis associated with the initiation of etanercept in rheumatoid arthritis: A case report

    2011, Dermatologica Sinica

    Although TNF-α antagonists suppress only a specific area of the immune system and are considered safe and well tolerated, various adverse cutaneous reactions have been confronted. In addition to the formation of leukocytoclastic vasculitis, urticaria, lichenoid drug eruptions and lupus erythematosus-like eruptions reported during TNF-α antagonist therapy,1 it has been suggested that anti-TNF-α therapy is associated with the onset of PNGD.2,3 It is well known that RA is the most common disease associated with PNGD; however, the correlation between the use of TNF-α antagonists and the development of PNGD in patients with RA remains controversial.

    Palisaded neutrophilic and granulomatous dermatitis (PNGD) is an unusual entity with variable clinical manifestations and histopathological features. It is associated with a variety of immune-mediated systemic diseases, most commonly in rheumatoid arthritis. We report a 42-year-old female with a long-standing history of rheumatoid arthritis, presenting with multiple pruritic erythematous papules and nodules on the lower legs 1 month after beginning treatment with etanercept. Microscopic examination of a fully developed lesion showed a diffuse dense interstitial lymphohistiocytic infiltrate interspersed with palisaded granulomas consisting of epithelioid histiocytes and multinucleated giant cells surrounding central zones of degenerated collagen, neutrophils and leukocytoclastic debris. A diagnosis of PNGD was made on the basis of typical histopathologic features. Withdrawal of etanercept led to gradual resolution of the skin lesions, with no new skin lesions appearing afterwards. Although the correlation between the use of tumor necrosis factor-α (TNF-α) antagonists and the development of PNGD remains controversial and warrants further investigation, PNGD should be considered in the differential diagnosis of skin eruptions within a setting of anti-TNF-α therapy.

  • Granulomatous diseases: Kids are not just little people

    2017, Clinics in Dermatology

    Both IGD and PNGD can have either spontaneous resolution or clearance after treatment of the underlying condition. Several treatments have been reported to result in variable improvement; these include oral or topical steroids, dapsone, and colchicine.82,90 Xanthogranulomatous inflammation is characterized histologically by the presence of foamy histiocytes, Touton giant cells, lymphocytes, and varying degrees of necrosis or necrobiosis and fibrosis.91,92

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Copyright © 2004 Elsevier Inc. All rights reserved.

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FAQs

What is Palisaded neutrophilic granulomatous dermatitis? ›

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is an inflammatory cutaneous disorder of unknown etiology that usually manifests as skin-colored to erythematous papules or plaques on the extremities (picture 1A-B). PNGD typically occurs in association with systemic disease.

What is a granulomatous dermatitis? ›

Background. Interstitial granulomatous dermatitis (IGD) is a rare disease that clinically presents with a pruritic and painful rash revealing symmetric, erythematous, and violaceous plaques over the lateral trunk, buttocks, and thighs [1]. Fewer than 70 cases have been documented in the literature [2].

What is a Palisading granuloma? ›

Palisading granuloma (known also as necrotizing granuloma) is a histological variety of a granuloma, characterized by the presence of macrophages and giant cells arranged in a tier-like fashion on the border of collagen degeneration (necrobiosis) [4, 5].

How is granulomatous dermatitis treated? ›

Corticosteroid injections. If the skin lesions aren't clearing up with topical treatment, your doctor may suggest a corticosteroid injection. Repeat injections may be needed every 6 to 8 weeks until the condition clears up.

Is granulomatous dermatitis benign? ›

Granulomatous periorificial dermatitis (GPD), also known as childhood granulomatous periorificial dermatitis (CGPD), is an uncommon, benign inflammatory skin disease that affects primarily dark-skinned prepubertal children; few cases have been reported in adults.

Is granulomatous dermatitis an autoimmune disease? ›

Aim. Both interstitial granulomatous dermatitis (IGD) and palisaded neutrophilic granulomatous dermatitis (PNGD) are rare disorders typically associated with systemic autoimmune conditions.

What autoimmune disease causes granuloma annulare? ›

Granuloma annulare is occasionally associated with diabetes or thyroid disease, most often when lesions are numerous or widespread. It may, rarely, be related to cancer, especially in older people whose granuloma annulare is severe, doesn't respond to treatment or returns after cancer treatment.

What medications cause granulomas? ›

Other drugs that have been reliably reported to cause granulomatous hepatitis include quinidine, phenytoin (usually with vasculitis), sulfonamides (usually with vasculitis), nitrofurantoin, aspirin, papaverine, procainamide, sulfasalazine, mesalamine, and glyburide.

What is granuloma? ›

A granuloma is a small area of inflammation. Granulomas are often found incidentally on an X-ray or other imaging test done for a different reason. Typically, granulomas are noncancerous (benign). Granulomas frequently occur in the lungs, but can occur in other parts of the body and head as well.

What is an eosinophilic granuloma? ›

An eosinophilic granuloma is a benign bone tumor. It's the mildest form of Langerhans cell histiocytosis, a rare disease that causes your body to produce too many immune cells. Eosinophilic granulomas usually affect kids under 10. The growths are always benign. This means they're not a symptom — or cause — of cancer.

What is Necrobiotic granuloma? ›

A collagenolytic or necrobiotic non-infectious granuloma is one in which a granulomatous infiltrate develops around a central area of altered collagen and elastic fibers. The altered fibers lose their distinct boundaries and exhibit new staining patterns, becoming either more basophilic or eosinophilic.

What causes granulomatous inflammation? ›

Granulomatous inflammation is caused by a variety of conditions including infection, autoimmune, toxic, allergic, drug, and neoplastic conditions. The tissue reaction pattern narrows the pathologic and clinical differential diagnosis and subsequent clinical management.

What does granulomatous mean? ›

What does that mean? A granuloma is a small area of inflammation. Granulomas are often found incidentally on an X-ray or other imaging test done for a different reason. Typically, granulomas are noncancerous (benign). Granulomas frequently occur in the lungs, but can occur in other parts of the body and head as well.

What does granulomatous disease mean? ›

Chronic granulomatous disease (CGD) is a genetic disorder in which white blood cells called phagocytes are unable to kill certain types of bacteria and fungi. People with CGD are highly susceptible to frequent and sometimes life-threatening bacterial and fungal infections.

What causes suppurative granulomatous dermatitis? ›

The most familiar atypical mycobacterial infection which reveals suppurative granuloma histopathologically is swimming pool granuloma or fish tank granuloma. It is caused by Mycobacterium marinum which is the most common variant of atypical mycobacterial infection affecting human.

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