C Morruzzi1, B Cribier, D Lipsker. 1. Service de dermatologie, hôpital Civil, CHU de Strasbourg, 1, place de l'hôpital, 67091 Strasbourg, France.
Abstract
BACKGROUND: A new clinicopathological entity has recently been described under the term of "macular arteritis". It is characterized by erythematous and hyperpigmented macules on the lower limbs having a chronic and indolent course. Histopathological examination shows lymphocytic arteritis of varying severity with intense hyalinised necrosis of a vessel with a smooth muscle wall. We report a new case of this condition. PATIENTS AND METHODS: A healthy 39-year old woman consulted for a 3-year history of a painless, non-pruritic eruption. She had multiple erythematous-purplish macules on the lower limbs. A skin biopsy showed severe lymphocytic arteritis with fibrinoid necrosis of the vascular wall and endoluminal thrombus, associated with perivascular lymphocytic infiltrate. This inflammation concerned the arteries of the deep dermis and of the superficial hypodermis. There were no neutrophils or eosinophils. There was no clinical or laboratory evidence of any extracutaneous signs. DISCUSSION: The clinical peculiarity of macular arteritis is the non-infiltrated and painless character of the primary lesion, in contrast with the classic lesions described in vasculitis, which are palpable and sometimes painful. In all cases described, the course is indolent with no associated systemic signs. The distinguishing histopathological characteristic is the presence of an intense lymphocytic infiltrate all around the arterial wall, associated with hyalinised necrosis and thrombosis of the vascular lumen. The histological picture is unusual and does not correspond to classical arterial abnormalities, in particular periarteritis nodosa. This clinicopathological entity seems original and is probably somewhere between inflammatory vasculitis and thrombotic vasculopathy. CONCLUSION: Lymphocytic macular arteritis is a new and recently defined clinicopathological entity characterised clinically by an elementary macular lesion and histopathologically by a picture of lymphocytic arteritis. Copyright 2010 Elsevier Masson SAS. All rights reserved.
BACKGROUND: A new clinicopathological entity has recently been described under the term of "macular arteritis". It is characterized by erythematous and hyperpigmented macules on the lower limbs having a chronic and indolent course. Histopathological examination shows lymphocytic arteritis of varying severity with intense hyalinised necrosis of a vessel with a smooth muscle wall. We report a new case of this condition. PATIENTS AND METHODS: A healthy 39-year old woman consulted for a 3-year history of a painless, non-pruritic eruption. She had multiple erythematous-purplish macules on the lower limbs. A skin biopsy showed severe lymphocytic arteritis with fibrinoid necrosis of the vascular wall and endoluminal thrombus, associated with perivascular lymphocytic infiltrate. This inflammation concerned the arteries of the deep dermis and of the superficial hypodermis. There were no neutrophils or eosinophils. There was no clinical or laboratory evidence of any extracutaneous signs. DISCUSSION: The clinical peculiarity of macular arteritis is the non-infiltrated and painless character of the primary lesion, in contrast with the classic lesions described in vasculitis, which are palpable and sometimes painful. In all cases described, the course is indolent with no associated systemic signs. The distinguishing histopathological characteristic is the presence of an intense lymphocytic infiltrate all around the arterial wall, associated with hyalinised necrosis and thrombosis of the vascular lumen. The histological picture is unusual and does not correspond to classical arterial abnormalities, in particular periarteritis nodosa. This clinicopathological entity seems original and is probably somewhere between inflammatory vasculitis and thrombotic vasculopathy. CONCLUSION:Lymphocytic macular arteritis is a new and recently defined clinicopathological entity characterised clinically by an elementary macular lesion and histopathologically by a picture of lymphocytic arteritis. Copyright 2010 Elsevier Masson SAS. All rights reserved.