China
Africa
Explore chapters and articles related to this topic
Vesiculobullous Diseases
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Snejina Vassileva, Kossara Drenovska
Other rare varieties include pemphigus herpetiformis, which combines features of dermatitis herpetiformis and pemphigus, and IgA pemphigus, which is mediated by IgA autoantibodies. There are also drug-induced pemphigus and contact pemphigus related to numerous medications (D-penicillamine, penicillin, angiotensin-converting-enzyme inhibitors—e.g., captopril, enalapril, nifedipine, beta-blockers) and skin contact with pesticides or other chemical substances, respectively.
Dapsone
Published in M. Lindsay Grayson, Sara E. Cosgrove, Suzanne M. Crowe, M. Lindsay Grayson, William Hope, James S. McCarthy, John Mills, Johan W. Mouton, David L. Paterson, Kucers’ The Use of Antibiotics, 2017
Subcorneal pustular dermatosis is a rare condition of the eye that is associated with the IgA form of pemphigus, a blistering skin disease (Huff et al., 1985). Often termed the “Sneddon–Wilkinson” syndrome after the clinicians who first reported that dapsone was active against this disease of the eye (Sneddon and Wilkinson, 1956), the disease responds well to dapsone therapy, although other forms of immunosuppression are sometimes required to control the manifestations of the disease outside the eye. The broad spectrum of IgA pemphigus disease in general responds well to dapsone and, accordingly, it remains the drug of first choice (Ongenae et al., 1999; Chaudhari and Marinkovich, 2007). Dapsone has been found to be a useful steroid-sparing agent in patients with pemphigus vulgaris (Rosenberg et al., 1976), although more modern approaches to steroid-sparing immunosuppression, such as co-administration with mycophenolate or the use of rituximab, are now used in preference to dapsone (Piette and Werth, 2012).
Microscopic findings
Published in M. Alan Menter, Caitriona Ryan, Psoriasis, 2017
Pustular psoriasis demonstrates findings quite distinct from other psoriatic subtypes, although if a pustular flare develops on a longstanding lesion of plaque psoriasis, much overlap between the two subtypes is noted. In early disease, neutrophils migrate from dilated capillaries in the dermal papillae into the spinous layer of the epidermis (Figure 3.9). The neutrophils collect in intercellular spaces as neutrophilic “macroabscesses” or large spongiform pustules. Subsequently, the pustule will migrate superficially into a subcorneal location (Figure 3.10). In cases of chronic pustular psoriasis as well as pustular psoriasis that develops on skin previously involved by plaque psoriasis, epidermal changes such as regular acanthosis and parakeratosis are present in addition to the vascular changes of psoriasis.10,11 If a biopsy is taken from a clinical pustule, rather than from erythematous skin without a clinical pustule, elongation of rete ridges is more commonly seen. The pustules contain neutrophils, acantholytic keratinocytes, and, rarely, eosinophils as well.11 Occasionally, the pustule is located even more superficially in a subcorneal location. These cases may be difficult to distinguish from subcorneal pustular dermatosis, pustular impetigo, and the subcorneal pustular dermatosis variant of IgA pemphigus and special studies such as gram staining and immunofluorescence testing may be necessary in addition to clinicopathologic correlation. The generalized form of pustular psoriasis may be difficult to distinguish from acute generalized exanthematous pustulosis (AGEP). Findings that favor AGEP include eosinophils located in the dermis and within pustules, dyskeratotic keratinocytes within the epidermis, a mixed neutrophil-rich dermal perivascular and interstitial infiltrate, and absence of dilated, tortuous blood vessels in the papillary dermis. Elongated rete ridges may be seen in both pustular psoriasis and AGEP.11
Autoimmune blistering diseases: promising agents in clinical trials
Published in Expert Opinion on Investigational Drugs, 2023
Henning Olbrich, Christian D. Sadik, Enno Schmidt
Pemphigus diseases are characterized by intraepidermal cleft formation induced by autoantibodies against desmosomal structures. The main subtypes of pemphigus are pemphigus vulgaris (PV) and pemphigus foliaceus (PF); paraneoplastic pemphigus (PNP) and IgA pemphigus are extremely rare [1,2]. PV is the most frequent subtype at an incidence of 1–10 per million per year with middle-aged persons affected most commonly [25]. It presents with painful erosions and ulcers of mucous membranes, mostly in the oral cavity, as well as flaccid blisters and erosions of the skin. PF exclusively involves the skin and shows puff-pastry-like scaling and erosions mainly in seborrheic areas, whilst PNP is characterized by severe mucositis affecting oral, ocular, anogenital, bronchopulmonary, and gastrointestinal mucosae besides skin [26]. DIF in PV, PF, and PNP shows intercellular deposits of IgG, complement C3 and more rarely IgA within the epidermis. Target antigens are desmoglein (Dsg)-3 and -1 in PV, Dsg-1 in PF and various desmosomal and plakin molecules in PNP including Dsg-1 and -3, desmocollins, envo-, peri-, and desmoplakin, plectin, BP230 [27–29] and α2-macroglobulin-like 1 protein [30].
A review of disease burden and clinical management for generalized pustular psoriasis in China
Published in Expert Review of Clinical Immunology, 2022
Based on literature and our clinical experience to date, the differential diagnoses of GPP include acute generalized exanthematous pustulosis, superficial candidiasis, subcorneal pustular dermatosis, Stevens-Johnson syndrome – a severe mucocutaneous disease characterized by severe purulent conjunctivitis, severe stomatitis with extensive mucosal necrosis, and purpuric macule, and is caused by drug exposure or infections; with a pustular eruption as an unusual form of the disease [29,30], amicrobial pustulosis of the folds, pemphigus foliaceous, IgA pemphigus subcorneal pustular dermatosis type, transient neonatal pustular melanosis, acropustulosis of infancy and SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis), adding to the challenge of correctly diagnosing GPP [1,31–34]. Moreover, diagnostic criteria for GPP vary between research entities. ERASPEN criteria stipulate that a diagnosis of GPP can be confirmed only upon disease relapse >1 episode or persistence >3 months [26]. However, the Japanese Dermatological Association (JDA) guidelines state that a definitive diagnosis of GPP can be made if patients show the following four features: 1) systemic symptoms, including fever and fatigue; 2) systemic or extensive flush accompanied by multiple sterile pustules that can sometimes merge as lakes of pus; 3) neutrophilic subcorneal pustules histopathologically characterized by Kogoj’s spongiform pustules; and 4) the above clinical and histological features occur repeatedly [35]. GPP should be suspected in patients with the second and third features [35].
A brief guide to pustular psoriasis for primary care providers
Published in Postgraduate Medicine, 2021
Jeffrey J. Crowley, David M. Pariser, Paul S. Yamauchi
For GPP, the major diagnosis to exclude is AGEP [9]. Clinically, it may be almost impossible to distinguish AGEP from GPP. The main clue would be a history of recent drug ingestion, as 90% of AGEP cases are associated with medication (commonly certain types of antibiotic, and calcium channel blocker agents) [35]. The main features of AGEP include [35]: acute widespread appearance of pinhead-sized sterile pustules, with erythema, edema, fever, and leukocytosis (the illness is usually mild, <5% mortality). Furthermore, AGEP has a more abrupt onset and a shorter duration than GPP – AGEP signs/symptoms occur within 48 hours and resolve within 1 to 2 weeks – AGEP does not recur, and has no association with plaque psoriasis. Skin biopsy (including at least one intact pustule) should be taken for histopathological examination to distinguish AGEP from other pustular skin conditions [35,36]. Other differential diagnoses for GPP include localized forms of pustular psoriasis, IgA pemphigus [37], and subcorneal pustular dermatosis (also called Sneddon-Wilkinson disease) [38].