An Update on the Pathogenesis of Skin Damage in Lupus.

PURPOSE OF REVIEW: Lupus erythematosus (LE) is characterized by broad and varied clinical forms ranging from a localized skin lesion to a life-threatening form with severe systemic manifestations. The overlapping between cutaneous LE (CLE) and systemic LE (SLE) brings difficulties to physicians for early accurate diagnosis and sometimes may lead to delayed treatment for patients. We comprehensively review recent progress about the similarities and differences of the main three subsets of LE in pathogenesis and immunological mechanisms, with a particular focus on the skin damage. RECENT FINDINGS: Recent studies on the mechanisms contributing to the skin damage in lupus have shown a close association of abnormal circulating inflammatory cells and abundant production of IgG autoantibodies with the skin damage of SLE, whereas few evidences if serum autoantibodies and circulating inflammatory cells are involved in the pathogenesis of CLE, especially for the discoid LE (DLE). Till now, the pathogenesis and molecular/cellular mechanism for the progress from CLE to SLE are far from clear. But more and more factors correlated with the differences among the subsets of LE and progression from CLE to SLE have been found, such as the mutation of IRF5, IFN regulatory factors and abnormalities of plasmacytoid dendritic cells (PDCs), Th1 cells, and B cells, which could be the potential biomarkers for the interventions in the development of LE. A further understanding in pathogenesis and immunological mechanisms for skin damage in different subsets of LE makes us think more about the differences and cross-links in the pathogenic mechanism of CLE and SLE, which will shed a light in predictive biomarkers and therapies in LE.

Lymphocytic infiltration of the skin (Jessner-Kanof) but not reticular erythematous mucinosis occasionally represents clinical manifestations of Borrelia-associated pseudolymphoma.

BACKGROUND: Lymphocytic infiltration of the skin (LIS) and reticular erythematous mucinosis (REM) are characterized histologically by an inflammatory cutaneous lymphocytic infiltrate similar to the histological appearance of pseudolymphoma. OBJECTIVES: To re-evaluate a large cohort of patients with the clinical and/or histological diagnosis or differential diagnosis of LIS and REM and to assess the evidence for infection with Borrelia. METHODS: Sixty-nine cases of LIS and 34 cases of REM were retrospectively investigated. Haematoxylin and eosin sections were re-examined, and histological diagnoses were specified and confirmed by clinicopathological correlation. Evidence for Borrelia infection was assessed by immunohistochemistry and focus-floating microscopy (FFM). RESULTS: LIS appeared to serve as a collective term for two main clinicopathological reaction patterns: (i) (tumid) lupus erythematosus (LE) (32 of 69, 46%) and (ii) pseudolymphoma (31 of 69, 45%). Other diagnoses (five of 69, 7%) included polymorphic light eruption, arthropod bite reaction, spongiotic dermatitis, drug eruption and urticaria. Spirochaetes were detected by FFM in 24 of 31 (77%) cases with a pseudolymphomatous reaction, while all nonpseudolymphomatous reactions were negative. Of the cases initially considered as REM, 21 of 34 (62%) were classified as LE, four of 34 (12%) as pseudolymphoma (three of four positive for Borrelia), and five of 34 (15%) as other diagnoses (folliculitis, morphoea, seborrhoeic dermatitis, prurigo and arthropod bite reaction). The diagnosis of Borrelia-associated pseudolymphoma was made significantly more often in those cases where LIS was considered as initial differential diagnosis than REM (P < 0.05). CONCLUSIONS: LIS and REM seem to represent clinicopathological reaction patterns. Our results confirm that, after accurate clinicopathological correlation, most cases of both conditions constitute hidden variants of LE. Furthermore, LIS, in contrast to REM, frequently comprises pseudolymphomatous reactions including borrelial lymphocytoma.

Múltiples placas eritematosas en una mujer de 70 años / Multiple erythematous plaques in a 70-year-old woman

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Cutis laxa adquirida localizada asociada a traquioniquia / Localized acquired cutis laxa associated with trachyonychia

La cutis laxa adquirida es una rara enfermedad de causa desconocida que afecta al metabolismo de la elastina. Clínicamente se caracteriza por piel redundante e hiperelasticidad, y el estudio histológico muestra reducción o ausencia de fibras elásticas en la dermis. Presentamos un caso de cutis laxa adquirida localizada asociado con traquioniquia

Acquired cutis laxa is a rare disease of unknown cause, which affects elastin metabolism. Clinically, it is characterized by redundant skin and hyperelasticity, while the histological study shows a reduction in or absence of elastic fibers in the dermis. We present a case of localized acquired cutis laxa associated with trachyonychia