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.     

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.     

Neonatal lupus erythematosus presenting as papules on the feet

We describe a case of neonatal lupus erythematosus in a 4‐week‐old male baby presenting with nodules/papules on the plantar surface of both feet. Skin histology of the right foot papule was consistent with lupus erythematosus. Annular lesions more typical of this condition then appeared later and maternal antibodies were positive for speckled antinuclear antibodies (1:5120), anti‐Ro/SSA and anti‐La/SSB(+), rheumatoid factor 1380.0 IU/mL. The patient had no evidence of cardiac, haematological or hepatobilary involvement. Although the mother was asymptomatic, the maternal grandmother had recently been diagnosed with Sjögren's syndrome.     

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.     

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.     

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.     

Neonatal lupus erythematosus: a review of the racial differences and similarities in clinical, serological and immunogenetic features of Japanese versus Caucasian patients

There has been tremendous interest in neonatal lupus erythematosus (NLE) since the reports of anti‐Ro/SSA antibodies as a diagnostic marker. Recent studies, including ours, have revealed racial differences as well as similarities in the clinical features and immunogenetic backgrounds of Japanese and Caucasian patients with NLE. The frequency of photosensitivity and subacute cutaneous LE lesions is not high in Japanese infants with NLE, which is in sharp contrast to their Caucasian American counterparts. The majority of Japanese infants with NLE develop annular, erythematous or edematous lesions which have also been reported in association with Sjögren's syndrome. The frequency of isolated congenital heart block (CHB) is about 50% in Japanese anti‐Ro/SSA positive neonatal lupus infants; this is similar to the frequency among Caucasians. The HLA‐DR3 phenotype, which is found in the great majority of Caucasian mothers of NLE infants, is absent in Japanese mothers. Finally, both Japanese and Caucasian children with CHB are often identical to their mothers in their alleles of HLA‐DRB1, DQA1 and DQB1 loci.     

Autoimmune thyroid disease in anti-Ro/SS-A-positive children with annular erythema: report of two cases

Anti‐Ro/SS‐A‐associated recurrent annular erythema is a rare disorder, and represents a cutaneous manifestation of primary Sjögren's syndrome (SS). We report two childhood cases complicated with autoimmune thyroid disease, one with Graves' disease and the other with autoimmune thyroiditis. Both children were positive for anti‐Ro/SS‐A and anti‐La/SS‐B antibodies. One patient was lacking clinical SS with objective evidence of salivary gland involvement, while the other was diagnosed with primary SS. Our observation suggests that autoimmune thyroid disease in the subset of anti‐Ro/SS‐A‐positive children with annular erythema might occur with similar frequency to that in adult primary SS.     

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.     

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.     

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.     

Stimulated eccrine gland function in primary Sjögren''s syndrome

Sweat secretion rate, stimulated by iontophoresis of pilocarpine, was measured in 22 patients with primary Sjögren's syndrome and 22 age- and sex-matched normal control subjects. There was no significant difference in measured sweat rates (P=0·45). We conclude that the complaint of dryness of the skin in patients with Sjögren's syndrome is not due to decreased eccrine gland secretion.     

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.     

Anticentromere antibodies (ACA): clinical distribution and disease specificity

Sera from 3528 patients with autoimmune disease, and non-autoimmune disease, and 500 normal individuals were studied For the presence of anticentromere antibodies (ACA) by indirect immunofluorescence on HEP-2 cells. Sixty-seven specimens were identified showing discrete speckled staining: 55 (82.1%), 11 (16.4%), and one (1.5%), were from patients with autoimmune disease, non-autoimmune disease and normal control subjects, respectively. These ACA were present frequently in CREST syndrome (55%), Raynaud's disease (29.6%) and primary biliary cirrhosis (30%). Only 16.4% of the antibody positive patients carried a clinical diagnosis of CREST, which means that ACA are not specific for CREST syndrome. High antibody titre persisted irrespective of whether or not the patients had active disease. The ACA were present infrequently in Sjögren's syndrome, systemic lupus erythematosus, rheumatoid arthritis, immune thrombocytopenic purpura, Graves' disease, immune haemolytic anaemia, and vitiligo. Sera from 107 patients with various other autoimmune diseases were negative for ACA.     

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.     

The relationship between facial annular erythema and anti-SS-A/Ro antibodies in three East Asian women

A distinct annular erythema developed on the cheeks of three East Asian women who had anti-SS-A/Ro (SSA) antibodies. The erythema was characterized by a wide, elevated border and central pallor. Histologically, there was a coat-sleeve-like infiltration of lymphocytes around the blood vessels, appendages, and secretory gland cells in the dermis. Immunohistological analysis clarified that the majority of infiltrating lymphocytes were CD4-positive T cells. Abnormal expression of HLA-DR antigens in the perivascular, appendage, and secretory gland cells in the dermis was also observed. The differential diagnosis of the three patients lay between Sjögren syndrome (SjS), Sjögren/systemic lupus erythematosus overlap syndrome and an asymptomatic clinical state. These results are consistent with recent findings of major histocompatibility complex class II expression on target organs in various autoimmune diseases. Based on these findings, erythema appears to represent a broad cutaneous manifestation of these diseases. Furthermore, the presence of SSA antibodies, aberrant HLA-DR expression, and sun exposure may be responsible for the development of erythema.     

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     

Recurrent Annular Erythema in a Case of Seronegative Sjögren's Syndrome

We describe a case of Sjögren's syndrome who repeatedly developed annular erythema on her extremities. Her anti-nuclear antibody, anti-SSA/Ro antibody, and anti-SSB/La antibody were all negative. Characteristics of the annular erythema included a tendency to appear on the extremities especially in summer, spontaneous regression after 1–2 weeks, and residual slight pigmentation. The histological findings revealed dermal perivascular lymphocytic infiltration admixed with some neutrophils. Slight exsudative changes were found in the upper dermis. There were no epidermal changes. This case suggests the existence of annular erythema which may not be related to the anti-SSA/Ro or anti-SSB/La antibody. Unknown factors other than those antibodies may be involved in the pathogenesis of the annular erythema.     

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.