Dermal mucinosis in discoid lupus erythematosus. Report of two cases

Abstract

Two patients with discoid lupus erythematosus (DLE) had lesions exhibiting prominent atrophy and telangiectasia. At the time of biopsy, the skin specimens were grossly mucinous. Light and immunofluorescence microscopy findings confirmed the diagnosis of DLE in both cases. Abundant mucin deposition throughout the dermis was also seen microscopically in both cases. Although secondary mucinosis is known to occur in LE, cases with massive mucinous change, ie, those reported herein, have apparently not been recorded. Mucinosis is not considered important in the histologic diagnosis of LE but may create some confusion with other mucinous disorders.

Papulonodular Mucinosis Associated with Subacute Cutaneous Lupus erythematosus

“…In a recent article, Pandya et al [30]stated that papulonodular mucinosis is restricted to patients with SLE; however, our patients as well as other reports in the literature [8, 9, 14, 17, 22, 25]demonstrate also the association with cutaneous manifestations of LE such as DLE and SCLE (table 1). Interestingly, the male-female ratio of 18:13 in patients with SLE and papulonodular mucinosis indicates that this type of mucinosis tends to occur more frequently in males, in contrast to the common ratio of 1:9 in patients with SLE, indicating a possible role for sex-related factors, such as androgenic hormones, in the pathogenesis of this associated disease [3].…”

“…However, the characteristic alterations of LE, such as epidermal involvement and vacuolar degeneration of the dermoepidermal junction, are missing [3, 14]. …”

“…However, in most cases, mucin deposition is rarely present in sufficient quantity to produce lesions that are clinically detectable in these diseases [3, 4]. Although recognized as early as 1954 by Gold [5], fewer than 40 patients with papulonodular mucinosis associated with LE have been described in the literature [3, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32]. In 79% of the patients, such skin lesions were associated with systemic LE (SLE), and in 21% with discoid LE (DLE).…”

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

“…This is reminiscent of LE tumidus lesions which are, however, usually smaller in size and located in sun-exposed areas [1]. Strong mucin deposition in nodular or papular form is rarely seen in discoid lupus erythematosus and occurs more frequently in patients with SLE accounting for about 2% of these cases [14, 15]. In addition, Jessner’s lymphocytic infiltration of the skin has been recognized as a cutaneous form of lupus erythematosus that does not show epidermal involvement [16, 17].…”

Specificity of dermal mucin in the diagnosis of lupus erythematosus: comparison with other dermatitides and normal skin

“…Increased dermal mucin and superficial to deep perivascular and periadnexal lymphocytic infiltrate are fairly constant histopathologic features of cutaneous LE . Additional findings such as interface dermatitis, basement membrane thickening and follicular plugging are seen at varying frequencies depending on the specific variant and chronicity of cutaneous LE .…”