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.     

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.     

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.     

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.     

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.     

Primary Sjögren's Syndrome Followed by Chronic Myelogenous Leukemia: A Case Report with a Ten Year History

We describe a female patient with primary Sjögren's syndrome who subsequently developed chronic myelogenous leukemia. The patient received a bone marrow transplant (BMT) from a genotypically HLA-identical brother after pre-transplant preparation with busulfan and cyclophosphamide. Eighteen weeks later, cutaneous and mucosal chronic graft-versus-host disease developed, followed by death 11 months after transplantation. Anti-Ro/SS-A antibodies disappeared within 6 months post-BMT, but antinuclear antibodies remained positive through the post-transplant course.     

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.     

Hypergammaglobulinemic Purpura Associated with Sjögren's Syndrome and Chronic C Type Hepatitis

Although hypergammaglobulinemic purpura usually occurs secondarily on the lower legs in several disorders, purpura has also recently been reported to be associated with chronic C type hepatitis (HCV). To define the differences in the clinical, histological and laboratory findings and the prognosis of hypergammaglobulinemic purpura associated with those two disorders, we examined ten patients with hypergammaglobulinemic purpura, 6 patients with hypergammaglobulinemic purpura associated with Sjögren's syndrome (SjS) and 4 patients with hypergammaglobulinemic purpura with chronic C type hepatitis. Five of 6 patients with SjS were female. Attacks of purpura occurred in the pretibial area in all cases. Triggering factors included long walks and prolonged standing. The mean duration of attacks was 6.4 days. No systemic manifestations were associated. Anti-Ro/SS-A and anti-La/SS-B antibodies were expressed in one case. Spontaneous regression was noted in all cases; however, recurrence was noted in one. On the other hand, all the 4 patients with hypergammaglobulinemic purpura associated with HCV were men. Purpura was indurated in a few cases. Involved sites included the knee, forearm, abdomen and thigh in addition to the lower leg. The mean duration of attacks was 12.6 days. Recurrent purpura was noted in one case. Cryoglobulin was positive in three cases. In one patient with severe recurrent purpura, attacks stopped with prednisolone 10 mg/day. Histologically, leukocytoclastic vasculitis was detected in three cases associated with SjS and two cases with HCV. In conclusion, hypergammaglobulinemic purpura associated with HCV appears to occur unilaterally with a sex predilection for men and the manifestations last longer than those associated with SjS. Severe palpable purpura was also noted in association with HCV; systemic prednisolone resulted in good control.     

Association of the Acral Type of Pustular Psoriasis,Sjögren's Syndrome,Systemic Lupus Erythematosus,and Hashimoto's Thyroiditis

We describe a case of a 53-year-old Japanese female suffering from Sjögren's syndrome, systemic lupus erythematosus, and Hashimoto's thyroiditis who developed pustules, erythema, and erosions on her fingers and toes. The histological specimen showed psoriatic changes. Indirect immunofluorescent study using anti-human IL (interleukin)-8 antibody produced positive staining patterns in the lesional epidermis. These findings suggested the diagnosis of acral pustular psoriasis. Diaminodiphenylsulfone at 75 mg orally daily for 20 days and the application of 0.12% betamethasone valerate ointment led to gradual improvement.