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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
Pemphigus foliaceus. Here the split is just below the granular layer so the blisters are very superficial. The antigen is desmoglein 1. Clinically widespread crusted erosions on the face, scalp and upper trunk are seen. It is often misdiagnosed as seborrhoeic eczema as no blisters are seen. It is less common than pemphigus vulgaris in Europe and USA, but commoner in Africa and South America. Diagnosis is confirmed by histology, immuno-fluorescence, and high titres of serum antibodies.
Blistering diseases in the elderly
Published in Robert A. Norman, Geriatric Dermatology, 2020
Blisters are flaccid and easily rupture, resulting in large painful erosions3. These lesions can heal or spread further at their periphery. Healing usually occurs without scarring and is often accompanied by post-inflammatory hyperpigmentation4. A Nikolsky’s sign can be exhibited by applying pressure on the periphery of the bullae and extending these lesions laterally3. Initial presentation in the majority of patients with pemphigus vulgaris is in the oral cavity (Figures 1 and 2)4. In contrast patients with pemphigus foliaceus initially present with lesions affecting the skin of the face, scalp and trunk (Figures 3 and 4)5. Other mucosal areas commonly affected by pemphigus vulgaris include the larynx, pharynx, esophagus, anus and genitalia1,3. When the skin is involved in pemphigus vulgaris it usually presents on the face, trunk, back, breast, groin and axillae (Figures 3 and 4)1. Pemphigus vulgaris must be differentiated from paraneoplastic pemphigus, which is not a true pemphigus but a complex of symptoms with a clinical heterogeneous phenotype with some lesions that resemble pemphigus vulgaris and erythema multiforme. It affects the skin and mucosal tissues, and is associated with tumors such as lymphomas, chronic lymphocytic leukemia and bronchogenic carcinoma.
Major Histocompatibility Complex and Autoimmune Disease
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Ursula Holzer, Gerald T. Nepom
In pemphigus, a chronic blistering disease of the skin mediated by autoantibodies, two forms can be distinguished: pemphigus vulgaris (PV) and pemphigus foliaceus (PF). The autoantibodies recognize epitopes of desmoglein 3 in PV or desmoglein 1 in PF. An association with the MHC II molecules DRB1*0402-DQB1*0302 and DRB1*14*-DQB1*0503 in PV has been shown.58-60 The association with DRB1*14 was concluded as a secondary phenomenon due to linkage disequilibrium between the DQB1*0503 and DR14-related alleles as patients with PV carrying DQB1 *0503 carried either DRB1*1401, 1405 or 1408, but all patients with DRB1*14 carried DQB1*0503.60 This observation is particularly noteworthy, since it indicates that a singular clinical entity, such as PV, can have two completely different HLA associations. A possible explanation is that different antigenic epitopes of the desmoglien 3 protein, for example, are presented by each of the two different HLA molecules associated with the disease. In PF, a strong association was found with DRB1*0102, DRB1*0404, DRB1* 1402 and DRB1*1406.58,61,62
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
Bullous skin diseases such as pemphigus erythematosus are autoimmune diseases, in which acantholysis is caused by autoantibodies targeting desmoglein (Dsg), one of the desmosome components that bind epidermal keratinocytes to each other, and intraepidermal blisters are formed. The pathological conditions vary depending on which Dsg the autoantibodies target. It is known that anti-Dsg-1 antibody causes fragile blisters and pemphigus foliaceus that forms crust with desquamation, and anti-Dsg-3 antibody causes pemphigus vulgaris that forms flaccid blisters on the entire body including mucous membranes. Pemphigus erythematosus is considered to be a subtype of pemphigus foliaceus, characterised by intraepidermal blisters that develop on seborrheic sites such as the face, chest and back, and butterfly rashes on the face. It is treated with oral corticosteroids or immunosuppressive drugs targeting anti-Dsg antibodies. Severe or intractable cases are treated with high-dose intravenous immunoglobulin therapy, intravenous cyclophosphamide pulse therapy, plasma exchange or rituximab. Although some case reports show that anti-tumor necrosis factor α (anti-TNF-α) inhibitors successfully treat pemphigus, others show that administration of anti-TNF-α inhibitors cause the appearance of blisters on the skin in patients with rheumatoid arthritis (RA). Thus, the reported findings on the effect of anti-TNF-α inhibitors on pemphigus have been inconsistent. In this study, we report on the case of a patient who was successfully treated with etanercept (ETN) in both the short and long terms after RA developed during treatment of pemphigus erythematosus.
Ultra-low dose rituximab for refractory pemghigus vulgaris: a pilot study
Published in Expert Opinion on Biological Therapy, 2020
Irene Russo, Serena Miotto, Andrea Saponeri, Mauro Alaibac
Eight patients were eligible for the trial (5 males and 3 females, aged 34–73 years) (Table 1). All patients were diagnosed with pemphigus vulgaris (PV), none with pemphigus foliaceus (PF), with a 2–7 years of history disease. All patients had severe long-lasting disease and before rituximab they were treated with several systemic agents including prednisone, azathioprine, dapsone, intravenous immunoglobulins, plasmapheresis, nicotinamide in combination with tetracyclines (Table 1). Two patients (patients 4 and 6) had received rituximab prior to the infusion in our Center, at a higher dosage (500 mg at 2-week intervals), but they experienced a relapse of the disease (Table 1).
Two cases of annular erythema without bullous lesions by autoimmune blistering diseases
Published in Modern Rheumatology, 2018
Miho Kabuto, Noriki Fujimoto, Toshihiro Tanaka
A 70-year-old Japanese female noticed pruritic erythematous lesions mainly on her trunk eight months previously. She was treated with topical corticosteroids and transient low-dose oral prednisolone by a local doctor, which led to temporary improvement. Since the eruptions relapsed repeatedly, she was referred to our hospital in 2014. Physical findings demonstrated multiple annular erythemas with marginal scale and central pigmentation on her trunk (Figure 1a). No bullous or mucosal lesion was observed. Laboratory investigations showed slightly elevated levels of lactate dehydrogenase (269 U/l) and C-reactive protein (0.32 mg/dl). The anti-nuclear antibody (ANA) titer was 1:40 showing homogeneous pattern. Autoantibodies against desmoglein-1 was detected with high titer (over 1000 U/ml), and no other autoantibodies against ss-DNA, ds-DNA, Sm, RNP, SS-A/Ro, SS-B/La, BP180-NC16A and desmoglein-3 were detected using enzyme-linked immunosorbent assay (ELISA). Histopathological findings of a cutaneous biopsy specimen from the annular erythema on the abdomen revealed intraepidermal blister formation, acantholytic cells, epidermal neutrophilic infiltration, and dermal perivascular lymphocytic and eosinophilic infiltration (Figure 1b) without vacuolar degeneration of the basement membrane zone (BMZ). Direct immunofluorescence (IF) examination demonstrated intercellular (IC) deposition of IgG (Figure 1c) and C3 in the epidermis. Based on these findings, we diagnosed the patient with pemphigus foliaceus. Since bullae developed few weeks later, we initiated treatment with oral prednisolone at 10 mg a day. Relapse of the annular erythema around the abdomen was observed at 7.5 mg a day, and the dosage of prednisolone was increased up to 20 mg a day. The serum autoantibodies against desmoglein-1 level decreased to 279 U/ml and no recurrence of eruption had been observed.