Blistering disorders: diagnosis and treatment

Abstract

Blistering diseases are a heterogeneous group of disorders that can affect either skin and mucous membrane, or both, varying in presentation, clinical course, pathohistology, immunopathology and treatment. Not infrequently the diagnosis is delayed. This can result in severe, and sometimes fatal consequences. Although these diseases are rare, it is very important to make an accurate diagnosis based on a combination of clinical profile and laboratory observations. A brief review is presented of the following bullous diseases: pemphigus, paraneoplastic pemphigus, bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita, dermatitis herpetiformis, linear IgA bullous disease, porphyria cutanea tarda, and subcorneal pustular dermatitis. Their clinical, pathohistologic and immunopathologic features and recommendations for therapy are discussed.

Cicatricial changes in ocular pemphigus

“…9,10,[16][17][18][19] Ocular involvement has been reported as a signal of severe disease and tends to occur several months after the onset of skin or other mucosal lesions. 6 However, there is evidence to suggest that ocular symptoms can precede the onset of skin and mucus membrane lesions, 11,[20][21][22] suggesting that ocular involvement may be underdiagnosed. In their series of 11 patients with ocular PV, Hodak et al 9 reported that eight patients (72.7%) had symptoms of ocular irritation, tearing, and foreign body sensation before the development of oral or cutaneous lesions.…”

Chronic bullous disease of childhood – A case report / Hronična bulozna bolest Kod Dece – Prikaz Slučaja

“…Cases of linear IgA dermatosis associated with gastrointestinal diseases, autoimmune diseases, malignancy and infections have been reported (9). The significance of these associations has yet to be determined, but they may play a role in the initial stimulation of the IgA mucosal immune system.…”

Gluten related disorders: even a dermatological issue

“…In particular, two different patterns of DIF are possible: (a) granular deposits in the dermal papillae and (b) granular @ C I C E d i z i o n i I n t e r n a z i o n a l i deposits along the basement membrane. Sometimes, a combination of both patterns, consisting in granular IgA deposition along the basement membrane with accentuation at the tips of the dermal papillae, may be present (28,29). Recently Ko et al suggested the existence of a third different pattern of IgA deposition at DIF, the fibrillar pattern, that may be related to a clinical variant of DH (30).…”