Hypereosinophilic syndrome as prodrome of bullous pemphigoid:report of a case

Persistent hypereosinophilia, cardiac involvement and a recurrent erythematous-papular pruritic eruption histologically characterized by eosinophilic spongiosis are described in a 77-year-old man. This condition, suggestive of "idiopathic" hypereosinophilic syndrome, represented the prodromic phase of atypical bullous pemphigoid, which manifested 7 months later.     

Esophageal involvement in cicatricial pemphigoid: a rare cause of dysphagia

Cicatricial pemphigoid is a rare, autoimmune, blistering disorder affecting the mucous membranes and skin. Esophageal involvement affects a small proportion of affected individuals and may present up to 10 years after the initial onset of the disease. We present a case of esophageal cicatricial pemphigoid that presented initially as linear IgA disease. We describe the successful treatment of dysphagia by graded esophageal dilatations.     

Cicatricial pemphigoid: immunopathogenesis and treatment

Cicatricial pemphigoid (CP) is a chronic, autoimmune, subepidermal blistering disease of mucous membranes and skin that has a tendency to scar and result in tissue destruction. Recent studies demonstrate that CP is a heterogeneous disease in which patients can resemble each other clinically, histologically, and immunopathologically, but have autoantibodies that target different autoantigens in the epidermal basement membrane. Accordingly CP is now considered to be a disease phenotype rather than a single nosologic entity. CP can be associated with substantial morbidity, and in rare instances, mortality. Currently the management of patients with CP is grounded in clinical experience rather than the results of large randomized trials. This article discusses recent advances in the understanding of this disorder's pathophysiology and treatment.     

Pemphigoid and Pemphigus Foliaceus Successfully Treated with Topical Corticosteroids

Six patients with pemphigoid and three patients with pemphigus foliaceus were successfully treated with topical corticosteroids. This was especially effective in cases of pretibial localized pemphigoid and in mild cases of bullous pemphigoid and pemphigus foliaceus with negative or low-titer circulating autoantibodies.     

Localized Pretibial Pemphigoid Associated with Trichilemmal Carcinoma

We report a case of localized pretibial pemphigoid with concurrent trichilemmal carcinoma of the face in a 93-year-old woman. Immunoblot analysis showed circulating autoantibodies against the 230 kDa antigen. After removal of the skin tumor, the bullous skin lesions were reduced and healed with only the topical application of corticosteroid.     

Drug-induced bullous pemphigoid with dermal fluorescence on salt-split skin

The immunological features of drug-induced bullous pemphigoid appear to be similar to those of idiopathic bullous pemphigoid (BP), with presence of circulating and tissue-bound antibodies showing anti-basement membrane zone specificity. We describe a 28-year-old woman who developed a widespread blistering eruption with marked involvement of the mucous membranes shortly after commencing treatment with oral flucloxacillin. The eruption gradually cleared following drug withdrawal and treatment with oral corticosteroids. Indirect immunofluorescence showed circulating IgG anti-basement membrane zone (BMZ) antibody and C3 which bound to the dermal aspect of salt-split skin, and direct immunofluorescence (IMF) of perilesional skin showed a linear band of C3 at the BMZ. Western immunoblotting of the patient's serum showed positive reactivity with a 180 kDa antigen in epidermal extracts and no reactivity with dermal extracts. The dermal-binding pattern on indirect IMF with salt-split skin only occurs in a minority of patients with BP and has not been described previously in a drug-induced case.