Generalized Lupus Panniculitis and Antiphospholipid Syndrome in a Patient Without Complement Deficiency

Nousari, Hossein C.; Kimyai‐Asadi, Arash; Santana, Helen; Diglio, Gerardine; Tausk, Francisco; Cohen, Bernard A.

doi:10.1046/j.1525-1470.1999.00060.x

Cited by 19 publications

(6 citation statements)

References 22 publications

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“…Skin manifestations of antiphospholipid antibody syndrome in children reportedly include a high incidence of livedo reticularis and digital ischemia. 13,14 However, our patient did not develop these features throughout the observation period.…”

Section: Anticardiolipin Antibodies In Infancycontrasting

confidence: 52%

Autoantibody responses to cardiolipin and DNA in infancy: association with lymphocytic panniculitis

Miyagawa

Y²,

Taira

et al. 2000

Lupus

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We report an infant with anticardiolipin (aCL) antibodies who presented with erythematous nodular skin lesions and elevated liver enzyme levels. The cutaneous manifestation was histologically lymphocytic lobular panniculitis with vasculitic and hemorrhagic changes. The infant also had low levels of anti-double stranded DNA (dsDNA) and anti-single stranded DNA (ssDNA) antibodies. There were no detectable antibodies to small nuclear ribonucleoproteins including Ro/SSA and La/SSB. His mother was consistently seronegative for any of these antibodies. Without corticosteroid therapy, cutaneous lesions resolved and anticardiolipin antibodies, but not anti-dsDNA and ssDNA antibodies, normalized within 16 months after the onset of the disease. Our patient demonstrated an uncommon presentation of aCL related cutaneous manifestation, the presentation with panniculitis being only the third such patient reported in the literature. Of great interest was the appearance of aCL antibodies and skin lesions during very early infancy.

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Section: Anticardiolipin Antibodies In Infancycontrasting

confidence: 52%

Autoantibody responses to cardiolipin and DNA in infancy: association with lymphocytic panniculitis

Miyagawa

Y²,

Taira

et al. 2000

Lupus

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“… 134–139 A recent study indicates a relatively low frequency of association with SLE and a paucity of serologic abnormalities typically associated with a connective tissue disease diathesis; 140 50% of patients had no other clinical features of LE. 140 Pediatric patients with antiphospholipid antibody syndrome and/or hereditary complement deficiency 141 may present with LEP. Lupus erythematosus profundus affects approximately 1 to 2% of patients with LE.…”

mentioning

confidence: 99%

The cutaneous pathology of lupus erythematosus: a review

2001

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The presentation of lupus erythematosus (LE) ranges from a skin rash unaccompanied by extracutaneous stigmata to a rapidly progressive lethal multiorgan disease. The diagnosis and subclassification is traditionally based on the correlation of serological and clinical findings. The latter include a photoinduced skin rash, arthralgia, arthritis, fever, Raynaud’s phenomenon, anemia, leukopenia, serositis, nephritis and central nervous sysdtem disease. The conventional classification scheme includes systemic, subacute cutaneous and discoid LE. Recent advances in our understanding of the cutaneous histopathology which correlates with the traditional forms of LE, along with certain novel LE subtypes, are the focus of this review. In addition to the main subtypes of LE, we will discuss associated vasculopathic lesions and the contribution of immunofluorescence microscopy to the diagnosis of LE and related connective tissue disease syndromes. Consideration will be given to unusual variants of LE such as anti‐Ro/SSA‐positive systemic lupus erythematosus (SLE), bullous SLE, lymphomatoid LE, lupus erythematosus profundus, drug induced LE, linear cutaneous LE, chiblains LE and parvovirus B19‐associated LE.

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“…It occurs primarily in middle‐aged (40 to 50 years old) women and has a predilection for the buttock, arms, thighs and face (7). It is rare in children (3, 4). Clinically, lupus panniculitis is usually characterized by the appearance of persistent, firm, well‐defined nodules or plaques which are rarely as extensive as the involvement seen in our case.…”

Section: Discussionmentioning

confidence: 99%

“…Generalized forms of lupus panniculitis have been associated with hereditary complement deficiencies, particularly of C2 and C4 (8). Nousari et al (4) reported that excluding a case of lupus panniculitis associated with neonatal lupus and a case of pancreatic panniculitis in a patient with lupus erythematosus, only four cases of generalized lupus panniculitis have been reported in children under the age of 13. Our patient showed severe, extensive, deforming lupus panniculitis with no evidence of genetic complement deficiency.…”

Section: Discussionmentioning

confidence: 99%

“…Lupus panniculitis is a distinctive form of connective tissue panniculitis that commonly occurs in association with either systemic or another type of chronic cutaneous erythematosus (1, 2). Lupus panniculitis may rarely occur in children (3, 4), and generalized forms of lupus panniculitis are also rare without hereditary complement deficiencies (4, 5). Other connective tissue diseases, such as dermatomyositis and scleroderma, may involve the adipose tissue as may immunologic and inflammatory diseases of vessels and connective tissue (1).…”

Section: Introductionmentioning

confidence: 99%

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Lupus Panniculitis with Combined Features of Dermatomyositis Resulting in Severe Lipoatrophy

Yoo

Cho

2004

The Journal of Dermatology

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An 11-year-old girl presented with a one-year history of multiple, hard, slightly painful subcutaneous nodules on her right cheek, upper arms, and buttock. Histology of a skin biopsy specimen showed a lobular panniculitis. Laboratory studies revealed positive ANA, anti-double strand DNA, and elevated muscle enzymes. She was diagnosed as having lupus panniculitis. During hydroxychloroquine treatment, erythema over knuckle joints developed. These unusual clinical and laboratory findings of panniculitis associated connective tissue diseases made it difficult to make a precise diagnosis. We report this unusual case of lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy.

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Generalized Lupus Panniculitis and Antiphospholipid Syndrome in a Patient Without Complement Deficiency

Cited by 19 publications

References 22 publications

Autoantibody responses to cardiolipin and DNA in infancy: association with lymphocytic panniculitis

Autoantibody responses to cardiolipin and DNA in infancy: association with lymphocytic panniculitis

The cutaneous pathology of lupus erythematosus: a review

Lupus Panniculitis with Combined Features of Dermatomyositis Resulting in Severe Lipoatrophy

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