Abstract
BackgroundMacular lymphocytic arteritis most commonly presents as hyperpigmented macules on the lower limbs. The pathogenesis of this disease is still unclear and there is an ongoing debate regarding whether it represents a new form of cutaneous vasculitis or an indolent form of cutaneous polyarteritis nodosa. ObjectiveTo describe clinical, histopathological, and laboratory findings of patients with the diagnosis of macular lymphocytic arteritis. MethodsA retrospective search was conducted by reviewing cases followed at the Vasculitis Clinic of the Dermatology Department, School of Medicine, University of São Paulo, between 2005 and 2017. Seven patients were included. ResultsAll cases were female, aged 9–46 years, and had hyperpigmented macules mainly on the legs. Three patients reported symptoms. Skin biopsies evidencing a predominantly lymphocytic infiltrate affecting arterioles at the dermal subcutaneous junction were found, as well as a typical luminal fibrin ring. None of the patients developed necrotic ulcers, neurological damage, or systemic manifestations. The follow-up ranged from 18 to 151 months, with a mean duration of 79 months. Study limitationsThis study is subject to a number of limitations: small sample of patients, besides having a retrospective and uncontrolled study design. ConclusionsTo the best of the authors’ knowledge, this series presents the longest duration of follow-up reported to date. During this period, none of the patients showed resolution of the lesions despite treatment, nor did any progress to systemic vasculitis. Similarities between clinical and skin biopsy findings support the hypothesis that macular lymphocytic arteritis is a benign, incomplete, and less aggressive form of cutaneous polyarteritis nodosa.
Highlights
Background: Macular lymphocytic arteritis most commonly presents as hyperpigmented macules on the lower limbs. The pathogenesis of this disease is still unclear and there is an ongoing debate regarding whether it represents a new form of cutaneous vasculitis or an indolent form of cutaneous polyarteritis nodosa
Some authors prefer to refer to it as ‘‘lymphocytic thrombophilic arteritis’’ to highlight the presumed pathogenic mechanism behind this entity.[7]. It is questionable whether macular lymphocytic arteritis (MLA) has sufficient defining features to be considered a distinct vasculitis from cutaneous polyarteritis nodosa (C-PAN), a necrotizing arteritis of medium-sized vessels with a predominant polymorphonuclear cell infiltrate.[14]
Laboratory evaluation consisted of complete blood count, renal and liver profile, erythrocyte sedimentation rate, C-reactive protein, urinalysis, complement levels, serum protein electrophoresis, serologic studies for hepatitis B and C, antinuclear antibody (ANA) test, rheumatoid factor (RF), anti---double stranded DNA antibodies, SS-A antibody, SS-B antibody, and antineutrophil cytoplasmic antibodies
Summary
The first description of macular lymphocytic arteritis (MLA) was made by Fein et al in 2003.1 It is characterized by erythematous or hyperpigmented macules, which can be round, annular, linear, or most often reticulated, affecting mainly the lower limbs, and to a lesser extent the upper extremities and the trunk.2---4 Nodules are not a typical feature; in some cases subtle subcutaneous indurations have been observed.[3,5,6] Livedo racemosa may appear as an isolated clinical manifestation or along with other dermatologic findings.[5,7] The lesions follow an indolent course and do not evolve toward a systemic disease, there have been reports of cutaneous ulceration, testicular infarcts, and neuropathy.8---11Histologically, MLA shows a dense infiltrate of mononuclear cells in the muscular wall of arterioles at the dermo-subcutaneous junction, with variable narrowing of their lumen by a typical hyalinized fibrin ring.[12,13] Some authors prefer to refer to it as ‘‘lymphocytic thrombophilic arteritis’’ to highlight the presumed pathogenic mechanism behind this entity.[7]. Nodules are not a typical feature; in some cases subtle subcutaneous indurations have been observed.[3,5,6] Livedo racemosa may appear as an isolated clinical manifestation or along with other dermatologic findings.[5,7] The lesions follow an indolent course and do not evolve toward a systemic disease, there have been reports of cutaneous ulceration, testicular infarcts, and neuropathy.8---11. Macular lymphocytic arteritis most commonly presents as hyperpigmented macules on the lower limbs. The pathogenesis of this disease is still unclear and there is an ongoing debate regarding whether it represents a new form of cutaneous vasculitis or an indolent form of cutaneous polyarteritis nodosa. Study limitations: This study is subject to a number of limitations: small sample of patients, besides having a retrospective and uncontrolled study design
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