PREVALENCE OF EYELID DERMATITIS IN PRIMARY SJÖGREN'S SYNDROME

Background. The eyelid dermatitis seen in elderly patients is a relatively heterogenous and troublesome disease. Most cases seem to be idiopathic and show resistance to standard dermatologic therapy. Methods. Fifty-two patients with primary definite Sjögren's syndrome (4 men, 48 women, mean age 54 years) were enrolled in this study, and the prevalence of eyelid dermatitis was investigated. Diagnostic criteria for Sjögren's syndrome were based on the criteria proposed by the Japanese Ministry of Health and Welfare. Patch test was performed using ICDRG-European standard allergens and/or eye drops, hair dye, and cosmetics. Results. Of the 52 patients, 22 showed eyelid dermatitis. These changes were much more frequent in elderly patients and showed a good correlation with the presence of ocular dry sensation. No significant difference was observed in clinical and other laboratory findings between patients with or without eyelid dermatitis. Although 8 of the 13 patients showed positive patch test reaction to various allergens, no close relationship existed between the use of a suspected substance and the onset or severity of eyelid dermatitis. Conclusions. These results suggest that the presence of rubbing dermatitis of the eyelid may be one of the cutaneous manifestations of Sjögren's syndrome.     

A case of Sjögren's syndrome, sarcoidosis, previous ulcerative colitis and gastric autoantibodies

We describe a patient with sicca syndrome. Raynaud's phenomenon and fixed waxy skin lesions. Investigations conformed both Sjögren's syndrome and sarcoidosis. The patient had previously had ulcerative colitis and other antibody evidence of autoimmunity. The spectrum of disorders reported in association with Sjögren's syndrome and sarcoidosis is wider than suggested in the acronym TASS syndrome (thyroiditis, Addison's disease, Sjögren's syndrome and sarcoidosis), and we suggest that the acronym TOASSUC (thyroiditis. other autoimmunity, Sjögren's syndrome, sarcoidosis, ulcerative colitis) includes a wider range of disorders and may he more memorable.     

The Coexistence of Psoriasis Vulgaris,Sjögren's Syndrome,and Hashimoto's Thyroiditis

A 52-year-old woman presented with psoriasis vulgaris, Sjögren's syndrome, and Hashimoto's thyroiditis with a 5-year history. She had a number of immunological abnormalities and typical psoriatic plaques over her entire body. The relationship between psoriasis, Sjögren's syndrome, and Hashimoto's thyroiditis is discussed from the viewpoint of immunology, and similar cases in the literature are reviewed. This is the first report of a coexistence of psoriasis vulgaris, Sjögren's syndrome, and Hashimoto's thyroiditis.     

Three cases of pigmented cosmetic dermatitis‐like eruptions associated with primary Sjögren's syndrome or anti‐SSA antibody

Pigmented cosmetic dermatitis‐like (Riehl's melanosis‐like) pigmentation was reported in three of 27 patients with primary Sjögren's syndrome. But case reports of such eruptions are rare. We describe three cases of such eruptions associated with primary Sjögren's syndrome or anti‐SSA antibody and possible associations with specific types of human leukocyte antigen (HLA) and infiltrating lymphocytes. These middle‐aged Japanese women had reticular facial pigmentation and histopathological examination revealed interface dermatitis, melanophages, and dense lymphocytic infiltration around hair follicles and sweat ducts. HLA typing revealed common antigenic equivalents or genetic typing of HLA‐A2, DR52, DPA1(02:02) and DPB1(05:01). Immunohistochemical staining revealed major subsets of T cells to be CD8 and CD45RO. Some Foxp3‐ and few IL17‐positive cells were found in strong contrast to the major CD4 subset of infiltrated T cells in annular erythema associated with Sjögren's syndrome. Apparently, our patients' pigmentation represented a specific etiology associated with primary Sjögren's syndrome or anti‐SSA antibody.     

Acral Ichthyosiform Mucinosis in Association with Sjögren's Syndrome: A Peculiar Form of Pretibial Myxedema?

Two Japanese women developed well-dermarcated ichthyosiform plaques on the lateral aspect of their lower legs. Deposition of mucin was demonstrated throughout the papillary dermis, unlike the site of mucin deposition seen in pretibial myxedema. Their thyroid function was normal. The condition of both women was complicated by Sjögren's syndrome. One of them who presented with positive anti-microsomal and anti-thyroglobulin antibodies had goiter, suggesting that her malady was also complicated by Hashimoto's thyroiditis. Their skin manifestations differed from those described in cutaneous mucinosis including pretibial myxedema, specifically with regard to the well-demarcated ichthyosiform appearance, the mucin deposition in the papillary dermis, and the association with Sjögren's syndrome. To the best of our knowledge, our cases may thus be considered to be a previously undescribed form of cutaneous mucinosis associated with Sjögren's syndrome.     

SKIN LESIONS ASSOCIATED WITH SJÖGREN'S SYNDROME AND ANTICYTOPLASMIC ANTIBODIES IN SLE PATIENTS

Among forty-two systemic lupus erythematosus patients, three had antibodies against cytoplasmic antigens SSA and SSB. All three patients showed annular nonscarring erythema as a sign of their illness. The most predominant site of skin lesions was the face, but trunk and extremities were involved as well. All of the patients had clinically distinct Sjögren's syndrome following the onset of their skin lesions. These findings suggest that systemic lupus erythematosus patients with anticytoplasmic antibodies might often have accompanying clinical features of nonscarring erythema and Sjögren's syndrome.     

Primary Sjögren's Syndrome and Psoriasis Vulgaris in a Case of OKT4 Epitope Deficiency

We report a 29-year-old female OKT4 epitope deficiency patient with primary Sjögren's syndrome and psoriasis vulgaris. Immunological investigations during the prolonged clinical course of her herpes zoster revealed that she has OKT4 epitope deficiency and primary Sjögren's syndrome. She had been treated for psoriasis vulgaris for 17 years without systemic immunosuppressive therapy. Flow cytometric study revealed that her OKT4 deficiency is heterogeneous and excluded interference with the OKT4 epitope by anti OKT4 autoantibodies. The rare coexistence of primary Sjögren's syndrome and psoriasis implicates an immune disturbance due to an unusual phenotype of CD4.     

Urticated annular erythema: a new manifestation of Sjögren's syndrome

A patient with a unique urticated annular erythema associated with Sjögren's syndrome and some features of systemic lupus erythematosus (SLE) is reported. There has been one previous report of a similar eruption occurring in a patient with Sjögren's syndrome.¹     

Anti‐signal recognition particle antibody‐positive polymyositis in a patient with Sjögren's syndrome showing various types of annular erythema: Positive correlation between the activities of annular erythema and myositis

Annular erythema with Sjögren's syndrome (AESS) is occasionally found, especially in Asian patients, which is classified into three types. We present a case of Sjögren's syndrome showing various types of AESS with anti‐signal recognition particle antibody‐positive polymyositis. We successfully treated the eruption and myositis with a low dose of prednisolone. Every onset of annular erythema coincided with elevation of serum creatine kinase levels, which suggests the correlation between the activities of annular erythema and polymyositis.     

Hypergammaglobulinaemic purpura of Waldenström and Ro/SSA autoantibodies

Summary Seven out of nine patients (78%). seen over a 13-year period with hypergammaglobulinaemic purpura of Waldenström were found to have antibodies to Ro/SSA. Over this period we saw 175 patients who had antibodies to Ro/SSA. In six of the seven patients. associated diseases were recognized. Five had Sjögren's syndrome, and one had systemic lupus erythematosus with Sjögren's syndrome and thyroiditis (and died 19 years after initial presentation from cerebral infarction). Screening for antibodies to Ro/SSA is important in the diagnosis of patients with hypergammaglobulinaemic purpura. and is helpful in predicting prognosis.     

Large annular purpura and paraneoplastic purpura in a patient with Sjögren's syndrome and cervical cancer

We report a 79‐year‐old female with anaphylactoid purpura on her legs and unusual large annular purpura on the trunk. Histopathological characteristics of leukocytoclastic vasculitis were observed in the upper and middle dermis of both types of skin lesions. She was complicated by Sjögren's syndrome and advanced cervical cancer. The annular purpura spontaneously resolved in a week and did not recur. However, the anaphylactoid purpura relapsed more frequently and spread more widely following the elevation of her serum SCC antigen levels from the onset of purpura until her death. We consider that the characteristic annular configuration was caused by the complication of Sjögren's syndrome and that the recurrent anaphylactoid purpura indicated paraneoplastic vasculitis primarily caused by the tumor specific protein immune complexes. Complication by Sjögren's syndrome many also play a role in the development of allergic vasculitis.     

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.     

Comorbid autoimmune diseases in patients with sarcoidosis: A nationwide case–control study in Taiwan

The association between sarcoidosis and autoimmune comorbidities has been reported, however, it has seldom been confirmed by a large nationwide study. Our study aimed to clarify the association between sarcoidosis and autoimmune comorbidities in the Taiwanese. A total of 1237 patients with sarcoidosis and 4948 age‐ and sex‐matched control subjects were selected from the National Health Insurance Research Database of Taiwan from 1997 to 2010. Multiple logistic regressions were performed to calculate the odds of comorbidities between the two groups. The prevalence of sarcoidosis was 2.17/100 000 individuals in Taiwan. Sarcoidosis patients tended to run a higher risk of autoimmune comorbidities than the control group (17.6% vs 9.4%, P < 0.05). Autoimmune thyroid disease (adjusted odd ratio [aOR], 1.32; 95% confidence interval [CI], 1.05–1.64), Sjögren's syndrome (aOR, 11.6; 95% CI, 4.36–31.0) and ankylosing spondylitis (aOR, 3.80; 95% CI, 2.42–5.97) were significantly associated with sarcoidosis. The sex‐stratified analyses were carried out to demonstrate a significant association of sarcoidosis with ankylosing spondylitis in both sexes, but with autoimmune thyroid disease in male patients and with Sjögren's syndrome female patients, respectively. Besides, the diagnosis of the autoimmune comorbidities strongly associated with sarcoidosis tended to be established after that of sarcoidosis. This study demonstrated that patients with sarcoidosis tended to have autoimmune thyroid disease, Sjögren's syndrome and ankylosing spondylitis, and the diagnosis of sarcoidosis usually preceded that of associated comorbidities. Clinicians should be alert to autoimmune comorbidities in patients with sarcoidosis.     

Asymptomatic Primary Sjögren's Syndrome in a Patient with Penicillin Drug Eruption

A 20-year-old woman visited our clinic because of acral numbness and skin eruptions after administration of oral penicillin. Serological tests revealed an increase of immunoglobulins (G & M), positive rheumatoid factor, anti-nuclear factor, anti-SSA and -SSB antibodies. Ophthalmologic and otolaryngological studies were consistent with those of Sjögren's syndrome. However, a skin biopsy specimen failed to show any specific changes for Sjögren's syndrome and the diagnosis of penicillin drug eruption was considered to be more preferable. We briefly discussed the coexistence of a drug allergy and asymptomatic connective tissue disease.     

ETIOLOGIES OF THE SICCA SYNDROME: PRIMARY SYSTEMIC AMLOIDOSIS AND OTHERS

Background. The sicca syndrome has been defined as the occurrence of xerostomia and xerophthalmia. Sjögren's syndrome is the most common cause of the sicca syndrome; however, these two syndromes are not synonymous and there are many potential etiologies of the sicca syndrome. A less known cause of sicca syndrome is amyloidosis that to date has only been reported in the nondermatology literature. Observations. A 79-year-old man with known amyloidosis presented with persistent xerostomia. He had the classic cutaneous findings of periorbital and “pinch” purpura. A labial biopsy showed diffuse deposition of amorphous eosinophilic material surrounding salivary acini. Apple-green birefringence was noted with Congo red staining and the diagnosis was made of amyloidosis in the minor salivary glands causing xerostomia. Conclusions. The sicca syndrome can be caused by systemic amyloidosis. Because this fact is not in the dermatologic literature, many dermatologists are not aware of this uncommon presentation. The knowledge of the many causes of the sicca syndrome and an understanding of the differences between this and Sjögren's syndromes are important for any dermatologist.     

Tumoral calcinosis in scleroderma

A 36-year-old female patient with scleroderma/Sjögren's syndrome developed multiple cystic tumors on the dorsal aspect of her left hand, right elbow, and left shoulder joint two years after the onset of scleroderma. Histologically, amorphous eosinophilic substances located in subcutaneous tissue showed a strong positive reaction to PTAH and rosindole stain, and focal positive reaction to Von Kossa stain. Rheumatoid rice body like substances with chalky fluid were discharged from tumoral lesions. From these results, this case was diagnosed as tumoral calcinosis secondary to connective tissue degeneration due to the pathogenetic mechanism underlying scleroderma/Sjögren's syndrome.     

Scleredema of Buschke associated with rheumatoid arthritis and Sjögren''s syndrome

A case of scleredema of Buschke associated with rheumatoid arthritis and Sjögren's syndrome is described. The onset of the skin changes and rheumatoid arthritis was almost simultaneous and the sicca syndrome developed 4 years later.     

Clinical Analysis of Recurrent Hypergammaglobulinemic Purpura Associated with Sjögren Syndrome

Recurrent purpuric lesions are occasionally seen in patients with Sjögren syndrome. Hypergammaglobulinemia is one of the underlying precipitating factors of this condition. Clinical and histopathological analyses were performed on 5 cases of hypergammaglobulinemic purpura associated with Sjögren's syndrome, and the effects of immunomodulatory therapy were evaluated with regards to these conditions. Three out of 5 cases were successfully treated with oral gold compound (Auranofin) and one case with a low dose of cyclophosphamide. Episodic purpura subsided two months after initiation of therapy with improved serum IgG levels. Salivary flow and serum amylase levels also improved in some cases. Immunomodulatory therapy may be useful in managing recurrent purpura based on hypergammaglobulinemia associated with Sjögren syndrome.     

Riehl's Melanosis-like Eruption Associated with Sjögren's Syndrome

A 58-year-old Japanese female complained of facial erythema and Riehl's melanosis-like pigmentation. Histological examination showed liquefaction degeneration of the basal cells and pigment incontinence. Direct immunofluorescence revealed no deposition of immunoglobulins or complements at the basement membrane zone. Patch test and photopatch test using cosmetics and detergents she had been using were negative. She was diagnosed with Sjögren's syndrome based upon dryness of the mouth, dense periductal infiltration of lymphocytes and plasma cells in the labial salivary gland, positive Shirmer's test, hyper-γ-globulinemia, positive antinuclear antibody, and positive anti-SSA (Ro) antibody. The pigmentation gradually faded away when she protected her face from ultraviolet irradiation while continuing use of the cosmetics and detergents. Riehl's melanosis-like eruption could be a cutaneous manifestation of Sjögren's syndrome closely related with anti-SSA (Ro) antibody.     

Postmenopausal frontal fibrosing alopecia in a Japanese woman with Sjögren's syndrome

Postmenopausal frontal fibrosing alopecia (PFFA) is a rare alopecia that develops in the frontoparietal scalp of postmenopausal women. Etiology of PFFA is unknown. Most of cases of PFFA have been reported in European and North American countries. Herein, we report a Japanese case of PFFA associated with Sjögren's syndrome. A 66‐year‐old woman had had slowly progressive, band‐like, scarring alopecia on her frontoparietal scalp. Hair follicles on the margin showed follicular keratosis. Histologically, fibrosis and lymphocytic infiltration were mild. This case suggests that PFFA may show mild inflammatory reaction and mild fibrosis in Japanese women. The association with immunological disorders including Sjögren's syndrome should be studied further.