Amicrobial pustulosis associated with IgA nephropathy and Sjögren's syndrome

Amicrobial pustulosis is a rare clinical entity characterized by a relapsing pustular eruption, primarily involving the skin folds. We describe a case of amicrobial pustulosis associated with autoimmune diseases (APAD). The patient suffered from IgA nephropathy and Sj?gren's syndrome. Skin symptoms were alleviated dramatically after corticosteroid pulse therapy and tonsillectomy.     

Sjögren's syndrome in dermatology

Sjogren's Syndrome (SS) is a systemic autoimmune disease characterized by dry eyes (keratoconjunctivis sicca) and dry mouth (xerostomia). To fulfill diagnostic criteria, patients must have objective signs of dryness on examination and laboratory confirmation of an autoimmune process as evidenced by a positive autoantibody to SS-A antigen or a characteristic lip biopsy. SS may exist as a primary condition or in association with other systemic autoimmune disorders (termed secondary SS) such as rheumatoid arthritis, systemic lupus erythematous (SLE), progressive systemic sclerosis (scleroderma), or dermatomyositis. Exclusions to the diagnosis include pre-existing lymphoma, hepatitis C or HIV infection. Pathogenesis involves both genetic (especially HLA-DR) and environmental factors. Both T-cells and B-cells are involved in the generation of cytokines and chemokines within the glands. The epithelial cells of the glands also play a role in pathogenesis. The dermatologic manifestations range from drynessness (sicca) and its complications to vasculitis. There is a significant overlap in the clinical manifestations, as well as treatment, of SS and SLE. However, SS patients require special attention to the complications of ocular dryness (keratocojunctivitis sicca and blepharitis) and oral dryness (rapid tooth loss and oral candidiasis) SS patients have a markedly increased risk of lymphoma and enlarged lymph nodes or persistently enlarged parotid/submandibular glands that require further evaluation.     

Dyspareunia and Sjögren's syndrome

BACKGROUND: Dyspareunia is frequently associated with a psychiatric origin, particularly in patients with no obvious vulvovaginal or pelvic disease. The aim of this study was to assess the frequency of dry syndrome in patients with dyspareunia and to evaluate the main clinical and biological features and follow-up data for women with dyspareunia and dry syndrome. PATIENTS AND METHODS: Twenty-two patients presenting chronic idiopathic dyspareunia (without clear vulvovaginal dermatosis or infection) were included in this retrospective study. All patients underwent history-taking, gynecological examination, a Schirmer tear test, a sugar test, labial salivary gland biopsy assessment and immunological examination. A diagnosis of Sj?gren's syndrome was considered where histological examination of the salivary glands showed a lymphocyte infiltration corresponding to stage 3 or 4 in the Chisholm classification. Diagnosis of dry syndrome without Sj?gren's syndrome was made in patients with xerostomia and/or xerophthalmia without a specific histological picture of Sj?gren's syndrome or immunological abnormalities at salivary gland biopsy. RESULTS: Based on our criteria, 10 patients (45%) had dry syndrome, including 4 with Sj?gren's syndrome and 6 with dry syndrome without Sj?gren's syndrome. 9 of these 10 patients presented either xerostomia (7 cases) and/or xerophthalmia (7 cases). Vaginal dryness was reported by 3 of the 10 women with dry syndrome but also by 4 of 12 women without dry syndrome. Examination of the vulva showed no particular clinical features and treatment with an emollient was not effective in all cases. DISCUSSION: This study showed a high frequency of dry syndrome in patients with chronic "idiopathic" dyspareunia. The incidence of the condition was even greater in women with functional conditions evocative of dry syndrome. Women presenting dyspareunia with no clearly related clinical causes should thus be carefully assessed for dry syndrome.     

Erythematous swelling of the lip associated with Sjögren's syndrome and mimicking cheilitis granulomatosa

A 64-year-old Japanese woman developed therapy-resistant erythematous swelling of her upper lip. Our tentative clinical diagnosis of cheilitis granulomatosa was ruled out later by the laboratory findings including increased levels of anti-nuclear-antibody (ANA), anti-SSA/Ro antibody, and positive Schirmer test as well as by a histopathological picture showing a dense perivascular infiltration of plasma cells and mononuclear cells in the dermis instead of granulomatous changes. To the best of our knowledge, this is the first patient in whom annular erythema associated with S ogren's syndrome involved only the upper lip and produced clinical features simulating cheilitis granulomatosa.     

Sjögren's syndrome and granulomatous panniculitis

An 83‐year‐old Cambodian woman presented with multiple painful erythematous nodules on the lower legs, forearms and wrists. Subsequent history and investigations revealed a granulomatous lobular panniculitis associated with Sjögren’s syndrome. This is a rare cutaneous association of Sjögrens syndrome with only three similar case reports in the literature.     

Subcorneal pustular dermatosis and Sjögren's syndrome

A 41-year-old Japanese woman presented at a local outpatient clinic because of a slightly pruritic pustular eruption on the trunk and extremities. Her skin lesion had been treated using an antifungal cream, but the condition had continued to worsen. According to the patient, the same pustular eruption had occurred during pregnancy, but diminished after delivery. She has no family history of skin eruption. On physical examination, she presented with well-circumscribed erythematous macules with small pustules on the borders, involving the axilla, groin, upper and lower extremities and upper back ( Fig. 1 ). Bacterial and fungal cultures of the lesions were negative. A skin biopsy obtained from a lesion in the left forearm demonstrated a subcorneal pustule containing polymorphonuclear leukocytes ( Fig. 2 ). A perivascular dermal infiltrate was present, consisting predominantly of mononuclear cells. There was no spongiosis or acantholysis. On direct immunofluorescence, there were no immunoglobulin A (IgA) and complement 3 (C3) deposits in the intercellular space of the epidermis.

Figure 1 Open in figure viewer PowerPoint Clinical appearance of the annular erythema with small pustules on the forearm     

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.     

Photoprovocation test and immunohistochemical analysis of inducible nitric oxide synthase expression in patients with Sjögren's syndrome associated with photosensitivity

Summary Background Annular erythema (AE) in Sjögren's syndrome (SS) usually develops on areas of sun‐exposed skin and is exacerbated during summer. Objectives To evaluate photosensitivity in SS and to investigate the involvement of ultraviolet (UV) radiation in the development of AE in SS. Methods Phototesting with UVA and UVB was performed on 14 SS patients, including 10 with primary SS. Clinical and histological features as well as expression of inducible nitric oxide synthase (iNOS) in the evoked skin lesions were compared with those of lupus erythematosus (LE). Eleven SS patients had a history of photosensitive AE ( n = 4), papules ( n = 3) or other types ( n = 4) of lesions on their sun‐exposed skin that were induced or aggravated by sunlight exposure. Results Phototesting induced a prolonged erythematous response ( n = 8), infiltrated erythema (IE) ( n = 4) and/or papules ( n = 3) in 11 of 14 SS patients, including one with primary SS without a history of photosensitivity. Histologically, the induced IE and papules showed coat‐sleeve‐like or sparse perivascular infiltration of lymphocytes similar to that in primary skin lesions of AE in SS. No epidermal changes characteristic for LE were found except for partial and mild liquefaction degeneration in three cases. In contrast, two cases were indistinguishable from the papular type of polymorphic light eruption in several aspects, including their primary skin lesions and early response to a photoprovocation test. Immunohistochemistry revealed diffuse expression of iNOS throughout the epidermis, which is characteristic for LE, in the three SS patients with minimal liquefaction degeneration, while the remaining seven SS patients examined exhibited no iNOS staining or a normal expression pattern. Conclusions Our results indicate that photosensitivity exists in certain primary SS patients, and that UV is critical to the development of AE in SS, probably through a pathological mechanism distinct from that in LE.     

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.