ABSTRACT
Pemphigus is a group of blistering autoimmune diseases that affect the skin and mucous membranes with autoantibodies against desmoglein. Among the pemphigus subtypes, pemphigus erythematosus is one of the rare lupus-like variant subtypes of pemphigus that shares common clinical and histopathological manifestations with systemic lupus erythematosus. The lesions appear clinically as erythematous erosive patches on the face in a butterfly distribution, and they manifest subcorneal acantholysis histopathologically. There is concomitant basement membrane zone deposition of immunoglobulin and complement in lesional skin in addition to intercellular space staining in the epidermis. Two cases of pemphigus erythematosus have been reported domestically. Here, we report a case of pemphigus erythematosus in an 18-year old woman.
Subject(s)
Female , Humans , Acantholysis , Autoantibodies , Autoimmune Diseases , Basement Membrane , Blister , Butterflies , Complement System Proteins , Desmogleins , Epidermis , Extracellular Space , Immunoglobulins , Lupus Erythematosus, Systemic , Mucous Membrane , Pemphigus , SkinABSTRACT
Pemphigus erythematosus (PE) is a superficial type of pemphigus, which can be aggravated by sunlight (espicially UV light). Because of the known side effects of corticosteroids, we evaluated the efficacy of hydroxychloroquine as a corticosteroid-sparing agent and/or the effect of a single-drug regimen in two patients with PE with photosensitivity. We obtained a good therapeutic response with hydroxychloroquine in these two patients with PE. This drug could be used in selected patients with pemphigus who are prednisolone/ immunosuppressive-resistant or who have certain degrees of photosensitivity.
Subject(s)
Humans , Adrenal Cortex Hormones , Hydroxychloroquine , Pemphigus , SunlightABSTRACT
We present here, a case of pemphigus erythematosus developed in a 31-year-old woman, who had been suffering from repeated episode, of remission and exacerbation with several erythematous, and somewhat oozing patche accompanying a mild pruritus or her face, upper part of the back and anterior chest. In serological studies, she had antinuclear antiboc@lies at. a titer of 1:160. Histopathological finding showed subcorneal bullae. Immunofluorescent study revealed IgG and C, deposition in the intercellular space of the epidermis and along the de rmoepidermal junction.