Bacterial and Atypical Mycobacterial Infections
Clay J. Cockerell, Antoanella Calame in Cutaneous Manifestations of HIV Disease, 2012
While there are several diseases causing intraepidermal bullae, such as bullous impetigo and pemphigous foliaceus, SSSS is distinct because of the marked absence of inflammatory cells either in the dermis or in the blister space due to the absence bacteria in the skin. There is acantholysis with a split between the stratum corneum and the granular layer which leads to blister formation. There is no significant epidermal necrosis and the basal cell layer is intact, two features that aid in distinguishing SSSS from TEN. Electron microscopy reveals widening of intracellular spaces and a loss of desmosomes. A split at the same location in the epidermis is also seen in pemphigus foliaceus, an autoimmune bullous disease, although the presence of inflammatory cells as well as positive immunofluorescence studies for deposits of IgG and C3 between keratinocytes distinguishes it from SSSS.22
Blistering skin disorders
Rashmi Sarkar, Anupam Das, Sumit Sethi in 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.
Chronic erythematous rash and lesions on trunk and limbs
Richard Ashton, Barbara Leppard in Differential Diagnosis in Dermatology, 2021
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.
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.
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.
Therapeutic outcomes of pemphigus in a referral service in Northern Brazil: a retrospective study of 32 patients
Published in Journal of Dermatological Treatment, 2021
Carla Andréa Avelar Pires, Yandra Sherring Einecke, Nathalia Gabay Pereira, Caio César Chaves Costa, Alyne Condurú dos Santos Cunha, Amanda Gabay Moreira, Fernanda do Nascimento Rodrigues, Marília Brasil Xavier
PV patients had average age of 47.6 ± 15.7 (range, 23–77 years). Disease duration ranged from 1 to 16 years (mean: 3.9 ± 3.6). Mucocutaneous PV was the most common clinical presentation (19 patients) followed by mucosal-dominant PV (3 patients). One patient presented pemphigus vegetans (a variant of PV) and another patient had gingival PV characterized by erosive stomatitis. Nikolsky’s sign was positive in 16 patients, negative in 2 patients and not described in medical records in another 2 patients. Histopathology showed suprabasal acantholysis in all skin biopsies of PV patients (Table 1). In this group, the predominant clinical features were the presence of exulcerated/hyperchromic lesions, crusts and, in some cases, flaccid blisters.
Related Knowledge Centers
- Bullous Pemphigoid
- Cell Junction
- Desmosome
- Differential Diagnosis
- Histology
- Keratinocyte
- Varicella Zoster Virus
- Pemphigus Vulgaris
- Skin Condition