Paniculitis en paciente con dermatomiositis

Abstract

Panniculitis is a rarely reported clinical finding in dermatomyositis. It may precede the other manifestations associated with dermatomyositis by as much as 14 months. In all cases, myositis and panniculitis improve simultaneously during treatment. The present report describes the case of a 30-year-old female patient with clinical and histopathological findings consistent with panniculitis two months after the onset of the muscle and cutaneous symptoms that permitted diagnosis of dermatomyositis. The skin lesions regressed following steroid treatment. Keywords: Dermatomyositis; Panniculitis; Steroids Resumen: La paniculitis es una manifestación cutánea infrecuente de la dermatomiositis. Puede preceder hasta en 14 meses a otras manifestaciones de la dermatomiositis. En todos los casos, la miositis y la paniculitis presentan mejoría simultánea durante el tratamiento. Describimos una paciente de sexo femenino de 30 años que presenta lesiones clínica e histológicamente compatibles con paniculitis luego de 2 meses de que el compromiso muscular y cutáneo permitió el diagnóstico de dermatomiositis. Las lesiones cutáneas remitieron con el tratamiento esteroideo.

Panniculitis as the initial manifestation of dermatomyositis with anti-MDA5 antibody

“…Histopathology shows lobular panniculitis with a lymphoplasmacytic infiltrate. In addition, there is usually overlying dermal–epidermal vacuolar changes and increased mucin deposition . In our case, the findings of the second biopsy prompted us to consider DM, even though dermal–epidermal vacuolar changes were not evident.…”

Update on management of connective tissue panniculitides

“…Childhood cases have been reported 42,43 . Only one malignancy was reported in association with dermatomyositis, a rhabdomyosarcoma in a 51 year-old, suggesting that panniculitis may characterize a clinical subset of dermatomyositis with less risk of malignancy 40,44,45 . The panniculitis of dermatomyositis does not appear to resolve spontaneously, unlike what is seen in LEP, but it has been noted to respond to treament directed at dermatomyositis 28 .…”

“…Like LEP, the panniculitis of dermatomyositis is characterized by a lobular lymphocytic infiltrate. There are reports of overlying dermal-epidermal vacuolar change and increased mucin deposition 42,45 . Distinguishing this entity from LEP relies on clinical information.…”

“…If panniculitis lesions appear in the setting of a prednisone taper, an increase in dose of corticosteroids may be beneficial 39,40,48 . Intravenous methylprednisilone is sometimes used in pulse dosing for the three consecutive days at 1 gram/daily for severe cases and has also been reported efficacious in oral formulation for the treatment of panniculitis in dermatomyositis 45 .…”

“…For example, a case of panniculitis developing in the setting of an increase in hydroxychloroquine dose suggests an distinctive feature from lupus associated panniculitides 42 . On the other hand, some reported cases describe positive antinuclear antibodies 40,42 , cytopenias 45 , increased mucin deposition 42,45 and positive direct immunofluorescence in vessels 44 pointing towards overlap with LEP. Yoo et al exemplify the overlap, and the possibility for confusion, with their case of panniculitis occurring in the setting of a markedly elevated creatine kinase and an elevated double-stranded DNA titer, which they characterized as a combination of LEP and panniculitis of dermatomyositis 55 .…”