Anti-alpha-fodrin antibodies in Sj?gren syndrome and lupus erythematosus.

OBJECTIVES: To investigate the prevalence of anti-alphafodrin antibody in patients with Sjogren syndrome (SS), lupus erythematosus (LE), or both and the association of this antibody with other clinical manifestations. DESIGN: A study of screening and diagnostic tests. Mean follow-up was 152 months (range, 4-572 months). SETTING: A university hospital associated with a research laboratory in Tokyo, Japan. PATIENTS: Nine patients with primary SS, 15 patients with SS secondary to LE, and 44 patients with LE alone. MAIN OUTCOME MEASURES: Frequencies of clinical and laboratory findings, including anti-alpha-fodrin antibody. RESULTS: Anti-alpha-fodrin antibody was more commonly detected in patients with primary (7/9; P<.001) and secondary (9/15; P<.001) SS than in those with LE alone (3/44). When patients with primary and secondary SS were combined and compared with those with LE alone, the sensitivity of anti-alpha-fodrin antibody was 67%, specificity was 93%, and both positive and negative predictive values were 84%. The presence of anti-alpha-fodrin antibody was associated with pernio, hyperglobulinemia, rheumatoid factor positivity, and the presence of anti-SS-B (La) antibody (P<.01) but not with annular erythema, photosensitivity, vasculitis, or renal disorder. CONCLUSIONS: Although anti-alpha-fodrin antibody was detected in patients with SS and in those with LE, it seemed to be more valuable for the diagnosis of SS than was anti-SS-A (Ro) because anti-alpha-fodrin was much less prevalent in patients with LE alone. It may be possible to consider this novel autoantibody as pathophysiologically associated with some extraglandular manifestations characteristically seen in patients with SS.     

Multiple dermatofibromas in a patient with systemic lupus erythematosus and Sjögren's syndrome

Multiple dermatofibromas (DFs) are rare and have been thought to be associated with altered immunity. In this report, we describe a 27‐year‐old Japanese woman with systemic lupus erythematosus (SLE) and Sjögren's syndrome in whom eight nodules appeared over a period of 4 years. Histopathological findings were consistent with DF. SLE rather than Sjögren's syndrome seemed to have induced the multiple DFs in this patient. We also reviewed the reported cases with multiple DFs associated with SLE and/or Sjögren's syndrome. Review of the previous reports indicates that SLE is the most frequent autoimmune disorder associated with multiple DFs, and that both SLE and immunosuppressive treatments play a part in induction of multiple DFs. Therefore, if multiple DFs are present it is important that the status of the patient be evaluated from the standpoint of autoimmune diseases, particularly SLE, or immunosuppression.     

Amicrobial pustulosis associated with autoimmune disease in a patient with Sjögren syndrome and IgA nephropathy

Amicrobial pustulosis associated with autoimmune disease (APAD) is a rare clinical condition, characterized by relapsing pustular eruption, affecting mainly the skin folds. Almost all previously described cases were young women with varying underlying autoimmune diseases. We report a 36‐year‐old woman with the interesting triad of APAD, Sjögren syndrome and IgA nephropathy. Her rashes responded to oral prednisolone and cyclophosphamide.     

Sjögren syndrome and systemic lupus erythematosus are distinct conditions

Sjogren syndrome (SS) and systemic lupus erythematosus (SLE) are both collagen vascular diseases that can be accompanied by Ro antibodies. Clinical evidence suggests that they are wholly distinct diseases. SS is strongly linked to lymphoma while lupus is not. SS patients do not commonly exhibit photosensitivity even though anti-Ro antibodies circulate in their blood; SLE patients generally exhibit photosensitivity. SS does not respond to hydroxychloroquine in a reproducible fashion whereas SLE does. SS has not been linked to parvovirus B19, but SLE has. However, SS and SLE do have similarities. Their autoantibody profiles are similar. They effect women more than men and have similar HLA haplotypes and autoantibodies; this is not likely coincidence but it may not clinically relevant.     

Prurigo pigmentosa in a patient with primary biliary cirrhosis and Sjögren syndrome

A 44‐year‐old Japanese woman suddenly developed severely pruritic erythematous papules on her trunk in a symmetrical distribution. Biopsy specimens showed the typical histopathological findings of prurigo pigmentosa. She had had recurrent episodes of high fever spikes for several years, and lost 10 kg in the last year. She was diagnosed as primary biliary cirrhosis (PBC) associated with subclinical Sjögren syndrome (SjS). Predonisolone (60 mg/day) for two weeks was effective for the PBC and fever, but not for the prurigo pigmentosa. PBC may be involved in the pathogenesis of this rare skin disease.     

Livedoid vasculopathy and hypercoagulability in a patient with primary Sjögren's syndrome

Background A 31‐year‐old woman presented with a 5‐year history of painful ulcerations, palpable purpura, porcelain‐white atrophic scars of the malleolar region and dorsal aspect of the feet, livedo reticularis on the limbs, arthralgia, xerophthalmia, and xerostomia. Methods Skin biopsy revealed vessel wall hyalinization and thrombosis of the microvasculature with a very scarce dermal inflammatory infiltrate. Biopsy of the oral mucosa showed mononuclear infiltration of an intralobular duct of a salivary gland. Results Laboratory studies, including autoantibodies and inflammation markers, were normal, except for a positive rheumatoid factor. Coagulation screening revealed C677T methylenetetrahydrofolate reductase (MTHFR) mutation, with a normal serum homocysteine. The patient was treated with oral methylprednisolone (32 mg/day with progressive reduction) and enoxaparin (20 mg/day subcutaneously), with complete ulcer healing within 4 months. Conclusion Livedoid vasculitis or vasculopathy has not been referred to previously in association with Sjögren's syndrome, but may be associated with other autoimmune disorders and anomalies of coagulation, namely factor V Leiden mutation, protein C deficiency, and MTHFR mutation, associated or not with hyperhomocysteinemia, a condition that seems to confer an increased risk of recurrent arterial and venous thrombosis. We stress the importance of anticoagulant therapy for ulcer healing and for the prevention of other thrombotic events.     

Primary localized cutaneous amyloidosis with unusual clinical features in a patient with Sjögren's syndrome

A 69‐year‐old woman presented with a 2‐year history of an eczematous lesion covering the genital area. Histopathological examination showed deposits of amorphous, eosinophilic material and an infiltrate of plasma cells through the entire dermis into the subcutaneous fatty tissue. Congo red‐stained deposits showed apple‐green birefringence with polarizing microscopy. On immunohistochemistry, the deposited material was positively stained with anti‐κ light chain antibodies but not with anti‐λ light chain. A diagnosis of primary localized cutaneous amyloidosis (PLCA) was made, and the patient was also diagnosed as having Sjögren's syndrome (SjS) based on clinical and laboratory findings. The lesion of PLCA has spontaneously regressed over a period of 18 months. We report a unique case of PLCA and SjS that clinically demonstrated genital eczematous features and spontaneous involution, and we also describe a possible association between PLCA and SjS.     

Ulcerative lichen planus associated with Sjögren's syndrome

Ulcerative lichen planus is a rare variant of lichen planus that is characterized by ulcerations of the feet and toes that are accompanied by toenail loss. However, the nail, oral mucosa, genital mucosa and the scalp are also sometimes affected by ulcerative lichen planus. Several authors have drawn attention to the association of ulcerative lichen planus and autoimmune diseases. We report a patient who had ulcerative lichen planus, with ulcerative erythema on the soles and palms, nail dystrophy and oral lesions, as well as Sjögren's syndrome; she was successfully treated with etretinate.     

Dermatologic manifestations of Sjögren syndrome

BACKGROUND: Sj?gren syndrome (SS) is a chronic autoimmune inflammatory disease that involves primarily the exocrine glands, resulting in their functional impairment. SS typically presents as dry eyes (xerophthalmia) and dry mouth (xerostomia). This process can manifest either as the independent phenomenon of primary SS or as secondary SS when found in the context of another autoimmune process, most commonly rheumatoid arthritis or systemic lupus erythematosus. Nearly half of the patients with SS develop cutaneous manifestations, which may include dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria-like lesions. These cutaneous manifestations have been underemphasized because they are often overshadowed by the more prominent sicca symptoms. However, certain skin findings are of paramount clinical and prognostic importance as they confer an increased risk for the development of life-threatening conditions, including multisystem vasculitis and non-Hodgkin lymphoma.OBJECTIVE AND CONCLUSIONS:In this review, the cutaneous manifestations of primary SS are discussed, with an emphasis on those findings that portend an increased risk of mortality.     

Nonnecrobiotic necrobiotic xanthogranuloma as an initial manifestation of paraproteinemia and small lymphocytic lymphoma in a patient with Sjögren syndrome

We report a unique case of periocular nonnecrobiotic necrobiotic xanthogranuloma in a 52-year-old white woman with Sj?gren syndrome who was subsequently found to have an immunoglobulin G paraproteinemia and coexisting small lymphocytic lymphoma. Therapy with fludarabine, cytoxan, and rituximab (FCR) resulted in a dramatic resolution of her sicca symptoms and periocular xanthogranulomas. This case further illustrates the association of hematolymphoid disorders with cutaneous xanthogranulomatous disease and the importance of additional appropriate laboratory and radiologic investigation for the accurate diagnosis of an underlying malignancy.