Macular Lymphocytic Arteritis: Three Cases Questioning Its Classification as Primary Lymphocytic Vasculitis

Abstract

Background: Macular arteritis, macular lymphocytic arteritis (MLA) or lymphocytic thrombophilic arteritis all correspond to an identical new clinicopathological entity. Its individualization as a primary cutaneous lymphocytic arteritis is still controversial for certain authors as it could represent a latent form of cutaneous polyarteritis nodosa. Materials and Methods: We report here 3 additional cases of MLA, present a review of the literature and discuss the disease's nosology. Results: MLA is characterized clinically by a benign skin eruption consisting in bilateral asymptomatic erythematous/hyperpigmented macules mainly located on the lower legs and histologically by a medium-sized cutaneous lymphocytic prominent arteritis present in early cutaneous lesions. Conclusion: These findings support that MLA may be considered as a chronic and indolent primary lymphocytic cutaneous arteritis. Nevertheless, in some cases the objective obliteration of cutaneous vessels underlines the need for continuous monitoring in MLA patients.

Reappraisal of histopathology of cutaneous polyarteritis nodosa

“…We have reviewed the literature of 34 LTA/MA cases between 2003 and 2015 . The clinical features consisted of hyperpigmented patches, macules and livedo racemosa (reticular patchy hyperpigmentation).…”

“…The histopathology of all the documented LTA/MA cases in the literature identify small vessel arteritis with a fibrin ring and with infiltration of lymphocytes in and around the vessel wall. Reports included cases with partial internal elastic lamina destruction . Since less than half of the documented LTA/MA cases had numerous biopsies performed, it is possible that the biopsied lesions were of a later stage, which could explain the lack neutrophil infiltrate.…”

“…Histopathologically, C‐PAN is divided into four stages (acute, subacute, reparative or healing and healed stage), and multiple stages coexist in the same or in different biopsy specimens from the same patient . Lymphocytic thrombophilic arteritis (LTA) and macular arteritis (MA) are reported to be a new small vessel arteritis of the skin . Clinically, LTA/MA presents as asymptomatic hyperpigmented macules and livedo racemosa.…”

Reply to “Reappraisal of histopathology of cutaneous polyarteritis nodosa”

“…("starburst") livedo racemosa and/or palpable small erythematous to hyperpigmented nodules. [2][3][4][5] Therefore, we consider our 2 cases show clinical features of previously reported LTA/MA, and clinically it is difficult to distinguish from mild forms of C-PAN. 6 Overall clinical and pathological features of LTA/MA overlap with C-PAN, so we feel it is reasonable to conclude they are in the same spectrum.…”

Macular lymphocytic arteritis: Clinical-pathologic correlation of a rare vasculitis

“…Pathologically, this condition is characterized by medium-vessel vasculitis composed primarily of lymphocytes and notably lacking neutrophils, thus distinguishing it from cutaneous polyarteritis nodosa 2, 3. Clinically, published cases have typically presented with macules, papules, livedoid patches, and ulcerations on the lower extremities, and to date, no cases have described progression to systemic vasculitis 4, 5…”