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Pemphigus vulgaris and foliaceus
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Unlike PV, PF typically does not affect mucosal sites; both endemic and sporadic PF share the same clinical, histological, and immunological findings. Patients with PF usually present with multiple pruritic, scaly, and crusted erosions with flaky circumscribed patches in mostly seborrheic areas (Figure 9.7) that can extend, merge, and progress to exfoliative dermatitis (erythema and scaling that covers >90% of the body surface area); blisters are rarely seen owing to their superficial localization and consequent rupture. Occasionally, patients may present with erythematous, scaly, and crusted plaques in the butterfly area of the face, resembling the malar rash of systemic lupus erythematosus (SLE). This type of presentation of PF is designated as pemphigus erythematosus (Senear-Usher syndrome). Approximately 50% of these patients may show circulating antinuclear antibodies (ANAs).
Connective tissue
Published in Brian J Pollard, Gareth Kitchen, Handbook of Clinical Anaesthesia, 2017
Pemphigus vulgaris is the most common form and occurs predominantly in patients of Mediterranean or Jewish origin, with a peak incidence between 30 and 50 years of age. It was uniformly fatal before the advent of steroid therapy. There is a familial tendency and an increased incidence of HLA-13. There may be an association with other autoimmune diseases such as thymoma, myasthenia gravis and systemic lupus erythematosus. Large, superficial, flaccid blisters occur spontaneously or in response to trauma and are found in the groin, axillae and over the trunk. Oral lesions may predate cutaneous bullae by several months. Pressure with torsion may result in blister formation in normal looking skin (Nikolsky’s sign). Bullae are fragile, rupturing easily with coincident loss of large areas of skin; healing occurs without scarring. Lesions may occur on the lips and in the mouth, nose, pharynx and larynx causing difficulty in eating and hoarseness. In some variants, e.g. pemphigus erythematosus and pemphigus foliaceous, bulla formation occurs more superficially within the epidermis. These forms tend to be less severe.
Photoaggravated Dermatoses
Published in Henry W. Lim, Herbert Hönigsmann, John L. M. Hawk, Photodermatology, 2007
Victoria P. Werth, Herbert Hönigsmann
Clinically, lesions can present with erosions or flaccid blisters (Fig. 9). One epidemiological study linked sunlight and air temperature to disease activity in pemphigus vulgaris (129). It has been noted for years that pemphigus erythematosus, pemphigus foliaceus, and pemphigus vulgaris can be induced with UV, including UVB and PUVA irradiation (121,124,130–135). In one patient, irradiation with two MEDs of UVB induced pemphigus lesions at 24 hours (132).
Successful treatment with etanercept in a case of seronegative rheumatoid arthritis with corticosteroid/methotrexate-resistant pemphigus erythematosus
Published in Modern Rheumatology Case Reports, 2018
Naoaki Ohkubo, Kazuhisa Nakano, Ippei Miyagawa, Shigeru Iwata, Shunsuke Fukuyo, Satoshi Kubo, Yasutaro Tamaki, Shingo Nakayamada, Yoshiya Tanaka
Regarding the diagnosis, there were no oral ulcer and alopecia suggesting systemic lupus erythematosus (SLE), and there was no dry mouth suggesting Sjögren’s syndrome (SjS). Anti-nuclear antibody was positive, but disease-specific antibodies such as anti-ds-DNA antibody, anti-Sm antibody and anti-SS-A antibody were negative. Thus, we could not diagnose SLE and SjS. The staining pattern of antinuclear antibody was homogeneous type, suggesting possibility that the presence of anti-histone antibodies. However, there were no drugs recently started, so we could not regard this case as drug-induced lupus. On the basis of the 2010 American College of Rheumatology/European League Against Rheumatism RA classification criteria, ≥11 affected joints (score 5), increased inflammation response (score 1), and ≥6 weeks’ symptom duration (score 1) made a total score of 7. Accordingly, we excluded differential diagnosis, and diagnosed the patient as having RA. Moreover, the patient had multiple flaccid bullae including erythema on the cheek, intraepidermal blisters due to epidermal intercellular adhesion disorder based on histological findings, and a positive blood test for anti-Dsg-1 antibody. Accordingly, we diagnosed the patient’s condition as pemphigus erythematosus. The activity of RA was evaluated as high disease activity based on a simplified disease activity index (SDAI) score of 30.2. The skin rash area of pemphigus erythematosus had a pemphigus disease area index (PDAI) score of 10, evaluated as moderate disease activity. We started to administer methotrexate (MTX) at 8 mg/week for both active diseases. The treatment was continued for 3 weeks, but as both the arthritis and rash did not improve. Because an SDAI score and a PDAI score increased, we introduced ETN (Figure 6). The tenderness and swelling of the joints disappeared. Moreover, the skin rash also disappeared. In expectation of steroid-sparing effect of MTX, as we increased a dose of MTX, we started decreasing a dose of BMZ. Two years after the introduction of ETN, BMZ was withdrawn and no joint symptoms or skin rash recurred. The anti-Dsg-1 antibody level remained high (551 U/mL); however, low disease activity was maintained at SDAI 7.4 and PDAI 1, and these levels have been successfully controlled during the last 5 years.