Cytophagic histiocytic panniculitis: a rare catastrophic form of systemic panniculitis

Malaviya, Anand N.; Hussain, Mahreen; Abdeen, Suad; Cherian, Sujith V.; Rashid, Waqar

doi:10.1093/rheumatology/37.7.799

Cited by 7 publications

(9 citation statements)

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“…CHP, a rare form of lobular panniculitis with characteristic histopathological appearance with`beanbag' histiocytes containing ingested lymphocytes, red cells and platelets. Our group has recently published some of these as case reports' [15,18,23]. It is important to note that papulo-nodular ulcerating crusted lesions on lower legs and distal arms resembling other forms of panniculitides have been well described in necrotizing vasculitis of polyarteritis nodosa (including cutaneous-PAN), Wegener's granulomatosis and ChurgStrauss syndrome [1,24].…”

Section: Discussionmentioning

confidence: 99%

“…Kuwaiti man with the catastrophic form of this disease was reported recently [18]. Clinical features included appearance of papulo-nodular lesions on the thighs and the abdominal wall that evolved into extensive nonhealing big ulcers on the thighs, legs, and abdominal wall, associated with terminal liver and bone marrow failure (Fig.…”

Section: Cytophagic Histiocytic Panniculitis (Chp) a 26-year-oldmentioning

confidence: 99%

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Musculoskeletal manifestations with panniculitis - a hospital based study on 62 patients in Kuwait

Malaviya

Francis

Kaushik

et al. 1999

Rheumatology International

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The study describes 62 patients seen over a period of 4 years mainly presenting with an acute inflammatory ankle arthritis/periarthritis associated with panniculitis. Based upon the clinical characteristics of panniculitis two distinct categories could be defined. These included patients with characteristic lesions of erythema nodosum, seen in 51 (82%), the idiopathic form in 29 (57%), and secondary EN in 22 (43%). Associated conditions included Behçet's disease, oral contraceptive pills, pregnancy and penicillin treatment. Patients clinically not having typical EN was seen in 11 (18%). In this group the histopathology showed erythema induratum in 4, cutaneous-polyarteritis nodosa with minimal panniculitis in 3, Weber-Christian disease, cytophagic histiocytic panniculitis, and 'mixed' lobular and septal variety of panniculitis in 1 each. (In 1 patient the disease was not panniculitis but cutaneous variant of granuloma annulare). It is concluded that in a hospital setting EN associated with acute musculoskeletal symptoms, mainly ankle arthritis/periarthritis or an acute peripheral polyarthritis, was a common rheumatological problem in Kuwait. However, other forms of panniculitides also presented with musculoskeletal manifestations requiring accurate histopathological diagnosis for appropriate treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Cytophagic Histiocytic Panniculitis (Chp) a 26-year-oldmentioning

confidence: 99%

Musculoskeletal manifestations with panniculitis - a hospital based study on 62 patients in Kuwait

Malaviya

Francis

Kaushik

et al. 1999

Rheumatology International

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“…1 Before the recognition of this entity as a form of lymphoma, this condition was reported under the designations of histiocytic cytophagic panniculitis and Weber-Christian disease. [2][3][4][5][6][7] In the days before the advent of immunohistochemistry, this condition was assumed to be of histiocytic derivation because of the extent of histiocytic infiltration involving the subcutaneous fat and overlying dermis. Hence, a certain subset of cases reported as histiocytic cytophagic panniculitis and Weber-Christian disease likely represented forms of SPTCL.…”

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confidence: 99%

Indolent Primary Cutaneous γ/δ T-Cell Lymphoma Localized to the Subcutaneous Panniculus and Its Association With Atypical Lymphocytic Lobular Panniculitis

Magro

Wang

2012

Am J Clin Pathol

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The 2005 classification of lymphoma proposed the designation of subcutaneous panniculitis-like T-cell lymphoma exclusively for those tumors composed of α/β neoplastic cells. Subcutaneous lymphomas that comprised γ/δ T cells are termed primary cutaneous γ/δ T-cell lymphoma. The different clinical outcomes justified this separation; a more indolent course was characteristic of the α/β variants vs the poor 5-year survival of the γ/δ forms. We describe 5 patients with γ/δ T-cell lymphoma localized to the subcutis with a less aggressive clinical course. Two patients were alive 5 years after presentation, and in the remaining 3, the disease is in complete remission after simple intervention. Three patients had a waxing and waning phase, likely representing a prelymphomatous phase, which then progressed to an overt malignant tumor. Therefore, it is important to recognize that not all patients with γ/δ T-cell lymphomas have a poor prognosis.

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“…It might be triggered in response to an unknown T cell disorder [6]. Recently, CHP was suggested to be a cutaneous manifestation of hemophagocytic syndrome or a natural disease progression of SPTL likely associated with EBV infection [7, 8]. The pathological examination of subcutaneous nodules in this case showed no atypical lymphocytes, indicating that a diagnosis of SPTL was insufficient.…”

Section: Discussionmentioning

confidence: 91%

“…The differential diagnoses include infection (especially tuberculosis), tumor, histiocytosis, neurosarcoidosis and lupus panniculitis [8, 9]. A skin biopsy is therefore necessary to make the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Bilateral ptosis as first presentation of cytophagic histiocytic panniculitis: a case report

et al. 2017

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BackgroundCytophagic histiocytic panniculitis (CHP) is a rare form of nodular panniculitis that may progress to panniculitis-like T-cell lymphoma. We report a case of CHP that first manifested as bilateral ptosis, which is the first reported case of this presentation.Case presentationA 25-year-old woman without medical history was referred to the neurology department of our hospital for evaluation of bilateral ptosis. Three months previously, she suddenly complained of bilateral ptosis without apparent cause. Simultaneously, non-painful tender subcutaneous nodules and eschar-like skin lesions were observed on her extremities and trunk. A diagnosis of CHP was made based on skin biopsy from the left thigh showing lobular panniculitis, vasculitis, and adiponecrosis, with infiltration of inflammatory cells, including lymphocytes, histiocytes, and phagocytic histiocytes. Her condition continued to worsen with corticosteroid and immunosuppressive agent (thalidomide) treatment. Significant improvement was noticed after three cycles of chemotherapy of THP-COP (pirarubicin, cyclophosphamide, vincristine, and prednisolone).ConclusionsCHP is a rare condition whose clinical presentation may include bilateral ptosis and biopsy is required for diagnosis of CHP.

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Cytophagic histiocytic panniculitis: a rare catastrophic form of systemic panniculitis

Cited by 7 publications

References 0 publications

Musculoskeletal manifestations with panniculitis - a hospital based study on 62 patients in Kuwait

Musculoskeletal manifestations with panniculitis - a hospital based study on 62 patients in Kuwait

Indolent Primary Cutaneous γ/δ T-Cell Lymphoma Localized to the Subcutaneous Panniculus and Its Association With Atypical Lymphocytic Lobular Panniculitis

Bilateral ptosis as first presentation of cytophagic histiocytic panniculitis: a case report

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