Panniculitis in connective tissue disease

Abstract

Specific and nonspecific panniculitis occur in connective tissue diseases. Microscopic studies of the epidermis and dermis, immunofluorescent microscopy, and extensive serologic testing may aid in the diagnosis and classification of these unusual syndromes. Connective tissue panniculitis and focal lipoatrophy may be forms of immunoreactive panniculitis or connective tissue disease (or both) in which typical microscopic, serologic, or other events related to classic connective tissue disease have not occurred.

Update on management of connective tissue panniculitides

“…Direct immunoflourescence may show granular staining of IgG, IgM and C3 at the dermal-epidermal junctional about 50% of the time, particularly in patients with concomitant DLE, and sometimes there is deposition of immune complexes in small deep vessels. These findings may aid in diagnosis 1,12,15 . Although these criteria have not been universally adopted, most contributors to the literature agree that LEP has a distinctive histologic appearance.…”

“…Antimalarials have a long history of use with successful clearing of lesions of LEP and lupus profundus in adults and in children. They are considered first line therapy for most cases of LEP 1,6,10,12,13,15–17,27 . Hydroxychloroquine is typically given at a dose of < 6.5 mg/kg/day based on ideal body weight.…”

“…There are also reports of so-called “connective tissue panniculitides” that are associated with autoimmune phenomena, but are not attributable to a well defined connective tissue disease 1,2 .…”

Lipoatrophic panniculitis and chromosome 10 abnormality

“…2,3 Immunofluorescence microscopy is often positive, along with the presence of ANA in most cases. 4 The sclerosis syndromes include subcutaneous systemic and localized scleroderma, morphoea profunda, pansclerotic morphoea of children and eosinophilic fasciitis. Clinically, these conditions overlap, with plaque-like lesions and histology showing a lymphocytic panniculitis that develops to hyaline fibrosis of the dermis and connective tissue septae of the fat.…”

Polymyositis with antisynthetase syndrome presented with lobular panniculitis: A case report

“…However, the presence of myopathy, arthralgia, and interstitial lung disease favored the diagnosis of polymyositis and antisynthetase syndrome. 14,17 Symmetrical proximal muscle weakness. 16 Investigations ANA, anti-dsDNA, anti-RNP, leukopenia, hypocomplementemia and elevate ESR.…”