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Chronic erythematous rash and lesions on trunk and limbs
Published in Richard Ashton, Barbara Leppard, Differential Diagnosis in Dermatology, 2021
Richard Ashton, Barbara Leppard
This is an autoimmune disease in which circulating IgG antibodies target desmosomal proteins (desmoglein 3). The epidermal cells come apart from one another (acantholysis) resulting in an intra-epidermal blister. These are always very superficial so are unable to stay intact for very long. You therefore see mainly erosions and crusts. The blisters are never haemorrhagic. The skin shears easily if rubbed producing an erosion (Nikolsky sign, Fig. 8.116). It most commonly begins with erosions in the mouth (seeFig. 6.09, p 108), and it may be weeks or months before the tell-tale blisters appear on the skin. It may spread very rapidly and be life-threatening.
Blistering skin disorders
Published in Rashmi Sarkar, Anupam Das, Sumit Sethi, Concise Dermatology, 2021
Pemphigus is a group of blistering disorders, which is characterized by acantholysis, i.e. loss of keratinocyte to keratinocyte adhesion, which results in the formation of epidermal blisters in mucosae and skin. Acantholysis is induced by autoantibodies against intercellular adhesion molecules. There are several types (Table 6.5), out of which pemphigus vulgaris (PV) is the most common. The average age of onset is 40 to 60 years. The lesions are thin-walled, delicate blisters that usually rapidly rupture and erode (Figure 6.3). They occur anywhere on the skin surface and very frequently occur within the mouth and throat, where they cause much discomfort and disability. The disorder is persistent, although fluctuating in intensity. Before adequate treatment became available, it was usually fatal. Pemphigus foliaceus is another variant where blistering occurs in the superficial layers of the epidermis. Clinically, blisters are seen rarely and erosions with crusting are seen mainly over the scalp, face, and seborrheic areas. It can sometimes become generalized and the patient may go into erythroderma. Though a chronic disease, its course is more benign than pemphigus vulgaris.
Skin diseases of the elderly
Published in Robert A. Norman, Geriatric Dermatology, 2020
Transient acantholytic dermatosis7 (Graver’s disease) (Figure 13) is common in men over 40 years of age; its cause is unknown. It can be persistent. Clinically there are scattered pruritic erythematous papules or papulovesicles with crusting on the neck, upper chest, back and trunk. Histologically there are acantholysis and dyskeratosis.
Vitamin D status in patients with autoimmune bullous dermatoses: a meta-analysis
Published in Journal of Dermatological Treatment, 2022
Miao Yang, Haijing Wu, Ming Zhao, Hai Long, Qianjin Lu
Vitamin D is synthesized in the skin with the presence of sunlight. However, UV radiation has been recognized as a triggering factor of AIBD (29,30). Exposure to UVB may induce acantholysis of uninvolved skin in pemphigus patients (31). Protection from sunlight is an important management strategy for AIBD patients. Moreover, the apparent skin lesions and pain resulted from the disease status may limit the outdoor activities. Hence, patients with AIBD are prone to develop hypovitaminosis D. Vitamin D inhibits the expression of autoantigen in pemphigus vulgaris and BP by keratinocytes regulatory mechanisms at the post-transcriptional level (32,33). Besides, calcitriol, the hormonally active vitamin D metabolite, was proved to decrease the BP-IgG induced releasing of IL-6 and IL-8 from keratinocytes in vitro and play an anti-inflammatory role in BP (34). Moreover, captopril, a recognized trigger factor of pemphigus, can induce keratinocytes detachment and apoptosis. This process was significantly attenuated by pretreatment with calcitriol (35). These evidences suggested that vitamin D exerts protective effects in AIBD. It probably forms a positive feedback mechanism in which inadequate vitamin D leads to severer disease status, and the disease status results in lower vitamin D level in reverse.
Treatment of pemphigus vulgaris: part 2 – emerging therapies
Published in Expert Review of Clinical Immunology, 2019
Rebecca L. Yanovsky, Michael McLeod, A.Razzaque Ahmed
A significant portion of studies on pathogenesis were focused on mechanisms of epidermal cell adhesion and the process of acantholysis. Limited if any attention was given to the loss of immune regulation and processes, during which B cell development occurs, leading to production of autoreactive B cells. This eventually produces autoantibodies that cause disease. Patients, who did achieve clinical remission, and subsequently developed relapse and resurgence of autoantibody, have not been studied to provide insights into mechanisms of relapse. It has been documented that conventional therapy does produce clinical remissions but has a high relapse rate. In 70-80% of patients treated with rituximab have relapses observed on long term follow up. Usually, clinical trials are funded for 24 months and occasionally 36 months. During this time remissions may be achieved by many patients. However, the time interval is too short to detect a true incidence of relapses.
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
In July X-4, bullous rash with desquamation appeared on the back. Erythema also developed on the cheek, and the anti-Dsg-1 antibody level was high. Intraepidermal blisters with acantholysis were found in the histological examination of the skin. Accordingly, pemphigus erythematosus was diagnosed. After the diagnosis, betamethasone (BMZ) was topically applied, without any clinical response. Thus, 2 mg BMZ was administered orally from December of the same year. Because no new blisters appeared, the dose of BMZ was gradually reduced from January X-3. When the dose of BMZ was reduced to 0.5 mg in April, new blisters recurred. Thus, the dose of BMZ was increased to 1 mg, and mizoribine was co-administered. When BMZ was reduced to 0.5 mg again, the recurrence of new blisters could not be controlled. Therefore, the co-administered drug was changed to minocycline. However, new blisters continued to appear and the anti-Dsg-1 antibody titer increased. Thus, tapering of corticosteroid from 0.5 mg BMZ was difficult. In December X-1, because the symptom aggravated, BMZ dose was increased to 1 mg. This resolved the appearance of new blisters. In February X, the dose of BMZ was reduced to 0.5 mg again, and new blisters recurred in March. In the same period, joint tenderness and swelling of both hands appeared. The arthritis gradually expanded to the joints of the fingers of both hands and knees, and swelling appeared in both knee joints. The symptoms were judged as the development of RA, and the patient was admitted to our hospital department for a thorough examination and treatment on May 6, X.