Palisaded neutrophilic granulomatous dermatitis in a case of seronegative arthritis
Year: 2023
Type: article
Abstract: Dear Editor, A 34-year-old woman presented with itchy lesions on the hands, legs, and back for 2 weeks and itchy lesions on the palms and soles for 10 years. She had excruciating joint pain and swelling of the small joints of the little finger 10 days before the onset of the rash. There was no history of prior drug intake. There was no history of photosensitivity, Raynaud’s phenomenon, breathlessness, or other systemic complaints. On examination, multiple erythematous to skin-colored papules measuring 0.5 cm × 0.5 cm and a few annular, nonscaly plaques measuring 1 × 1 cm were found on the extensors of the hands [Figures 1A], palms [Figure 1B], forearms, and dorsa of the forefoot distributed symmetrically. Joint movement was normal. There was no significant lymphadenopathy. Differential diagnoses of sarcoidosis, granuloma annulare, and autoimmune connective tissue disorders were considered. All routine investigations such as complete blood count, liver function tests, and renal function tests were within normal limits. Antinuclear antibody, double-stranded DNA, rheamatoid arthritis facto, and anti -cyclic citrullinated peptide (anti-CCP) antibodies were negative. ACE enzyme level was normal. Radiographic examination of the joints did not reveal any abnormality. Skin biopsy from the plaque on the dorsum of the hand showed palisading granulomas of histiocytes surrounding degenerating collagen and neutrophils in the upper dermis [Figure 2A]. Dermis also showed mixed inflammatory infiltrate with abundant neutrophils and eosinophilic altered collagen material surrounded by palisade of histiocytes, neutrophils, and nuclear debris [Figures 2B and C]. On clinicopathologic correlation, diagnosis of palisaded neutrophilic granulomatous dermatitis (PNGD) was made. Patient was started on tablet methotrexate15 mg/week and topical corticosteroids. She reported complete improvement in joint pain and skin lesions fover 6 months [Figure 3A and B].Figure 1:: (A) Multiple skin-colored papules arranged in an annular pattern on the dorsum of the left hand. (B) Multiple nontender papules on palmsFigure 2:: (A) Scanner view showing palisading granulomas and neutrophils in the upper dermis. (B) Dermis showing mixed inflammatory infiltrate and neutrophils. A palisade of histiocytes (black arrows) and neutrophil debris around eosinophilic degenerated collagen can be seen. (C) Nuclear debris (black arrows) surrounded by altered collagenFigure 3:: (A) Resolution of papules on the dorsum of hands at 6-month follow-up. (B) Complete clearance of lesions on palmsPNGD was first reported by Dykman et al. in patients with rheumatoid arthritis, where linear bands developed on the trunk.[1] PNGD presents as tender or asymptomatic multiple umbilicated papules with central perforation or crusting or as erythematous to violaceous papules, plaques, nodules, and annular plaques.[2] Rarely petechiae, livedo reticularis, urticaria have also been reported.[3] PNGD usually appears on extensor surfaces, especially elbows and fingers. The pathogenesis is unknown and suggested to be due to the immune complex deposition of IgM and C3 in the dermal vessels.[2] Various associations like systemic sclerosis,[4] Sjogren syndrome, autoimmune thyroiditis, hepatitis, inflammatory bowel disease, myelodysplastic syndrome, Wegener granulomatosis, Churg-Strauss syndrome, Takayasu arteritis, diabetes, and infections such as Streptococcus, HIV, Epstein–Barr virus, and Parvovirus have been described. It has also been associated with certain drugs such as allopurinol, TNF alpha inhibitors, ledipasvir, and sofosbuvir.[5] Early lesions in PNGD show cutaneous small-vessel leukocytoclastic vasculitis and dense neutrophils throughout the dermis. Early lesions differ from frank vasculitis in that they have broader fibrin cuffs, fewer extravasated red blood cells, and more neutrophils. Established lesions have palisaded granulomas and neutrophils involving the full thickness of the dermis and sometimes the subcutis. Late lesions show fibrosis and granulomas surrounded by collagen and mucin similar to granuloma annulare.[1] Immunofluorescence studies have demonstrated C3, IgM, and fibrinogen in dermal blood vessels.[2] The histopathological differential diagnoses include granuloma annulare and interstitial granulomatous dermatitis (IGD). PNGD and IGD represent two clinicopathologic patterns of granulomatous dermatitis.[2] The dermal infiltrates of PNGD often assume a band-like configuration and may be “bottom heavy” whereas the infiltration of IGD is not band-like and is usually “top heavy.”[3] PNGD occurs along a spectrum of cutaneous granulomatous reaction and there is frequently overlap between these entities, which further complicates the diagnosis. Due to the broad spectrum clinically and histologically, different names as linear subcutaneous bands, rheumatoid papules, Churg-Strauss granuloma, and cutaneous extravascular necrotizing granuloma of Winkelmann have been suggested. Chu et al.[4]proposed the name “palisaded neutrophilic and granulomatous dermatitis” to summarize the clinical and histologic spectra of this entity. In our case, granuloma annulare and rheumatoid neutrophilic dermatitis were considered as close histopathological differentials to PNGD. Rheumatoid neutrophilic dermatitis has dense neutrophilic infiltrate without vasculitis and any granulomatous reaction, whereas granuloma annulare has abundant mucin and abundant nuclear dust.[4,5] The histopathology of our case typically shows palisade of intact neutrophils with thick altered collagen bundles which helped to rule out other neutrophil granulomatous dermatitis disorders. Various treatment modalities have been tried including topical, intralesional, systemic corticosteroids, dapsone, colchicine, hydroxychloroquine, NSAID, cyclosporine, methotrexate, cyclophosphamide, infliximab, and oral tacrolimus.[5,6] In our case, the presence of palisading granulomas with neutrophils on histopathology and arthritis with negative rheumatoid factor and anti-CCP antibody point to a connection of PNGD with seronegative arthritis which is rarely reported. Oral methotrexate can be considered as an effective treatment modality. Dermatologists and dermatopathologists should recognize these different morphologies and identify a significant association with underlying systemic disease. Financial support and sponsorship Not applicable. Conflicts of interest There are no conflicts of interest.
Language: English
Subfield: Dermatology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4378907668