Cutaneous lesions of the digits in systemic lupus erythematosus: 50 cases

Abstract

The objective of this study was to observe the clinical and pathologic features of digital lesions in a cohort of 50 patients with systemic lupus erythematosus (SLE). Biopsy and pictures of digital lesions were performed in 50 consecutive patients with SLE and digital lesions. A clinical diagnosis of vasculitis was previously suggested in 36% of cases. Pictures were reviewed by three dermatologists and all the tissue sections were analysed by the same pathologist. Files of patients were reviewed retrospectively. Activity of SLE was established according to the lupus activity index (LAI). Digital lesions in SLE were frequently painful (60%) with a finger-pulp inflammation (70%). According to clinical and pathological correlation, five patients had acute cutaneous lupus, five subacute cutaneous lupus, 21 discoid lupus and 15 chilblain lupus. Two patients presented vasculitis: one had an urticarial vasculitis concomitantly to a lupus flare, the other had an erythema elevatum diutinum, independent of SLE evolution. Thrombosis of dermal vessels was present in two patients with SLE-associated antiphospholipid syndrome and in two patients with chilblain lupus. LAI was >1.5 in only seven patients. These results highlight the tendency to clinically overestimate the prevalence of cutaneous vasculitis of the fingers in patients without active SLE. Clinical features of cutaneous lupus of the digits are polymorphous. So, a pathological examination of the lesions is often necessary for diagnosis and proper management.

Erythema elevatum diutinum in systemic lupus erythematosus

“…In a study involving 50 SLE patients with digital cutaneous lesions, only one was diagnosed to be EED [16]. This patient presented with symmetrical acral erythemato-violaceous papulo-nodular lesions, fever and arthalgia.…”

Familial Chilblain Lupus – A Monogenic Form of Cutaneous Lupus Erythematosus due to a Heterozygous Mutation in <i>TREX1</i>

“…Topical steroids may be of benefit in some patients [27]. Hydroxychloroquine has been used with inconsistent success [3] and usually requires continued treatment throughout the warm season [2, 27]. The therapeutic effect of other immunosuppressive drugs such as cyclophosphamide or mycophenolate mofetil has been reported in single cases only [21].…”

“…Chilblain lupus typically manifests itself during cold and damp times of the year or after a critical drop in temperature and is often difficult to distinguish clinically and histologically from true cold-induced chilblains [1, 3]. The presence of antinuclear antibodies and in few cases anti-Ro/SSA antibodies or rheumatoid factor as well as a positive lesional direct immunofluorescence are helpful to establish the diagnosis of chilblain lupus [4, 5] and to distinguish this form of lupus erythematosus from lupus pernio (cutaneous sarcoidosis) and acral vasculitis/vasculopathy due to cryoglobulinaemia.…”

Lupus erythematosus

“…Eine therapieresistente Akne bei SLE oder CCLE kann auch als mögliche Manifestation des LE gesehen werden[10]. Chilblain Lupus)[11]. In 60% waren die digitalen Läsionen schmerzhaft.…”

“…Differenzialdiagnostisch sind Perniones abzugrenzen, deren Unterscheidung klinisch und histologisch schwierig ist. Nach Bouaziz et al[11] ist der Chilblain Lupus ein nichtatropher CCLE, der wie Perniones ("Frostbeulen") aussieht. Viguier et al[12] untersuchten 33 Patienten mit Perniones; bei 8 Patienten wurde die Diagnose eines LE gestellt.…”