1.Subcorneal pustular dermatosis type of IgA Pemphigus in a 35-year-old female: A case report
Elaine R. Tabayoyong ; Cindy J. Tan ; Katrina C. Estrella ; Ruth B. Medel ; Camille B. Angeles
Journal of the Philippine Medical Association 2023;102(1):82-89
Introduction:
IgA pemphigus is a rare, distinct variant of Pemphigus characterized by vesiculopustular eruptions mediated by IgA autoantibodies targeting keratinocyte cell surface antigens, desmocollins 1-3 and sometimes desmogleins 1 and 3. Its classical features have been described in literature but atypical cases have also been documented. This report presents such case posing a diagnostic dilemma.
Case Report:
A 35-year-old female presented with a 16-year history of intermittent eruptions of multiple hyperpigmented, annular and circinate, desquamating plaques and coalescing flaccid pustules on erythematous bases on the scalp, neck, trunk, and extremities. Histopathologic examination revealed subcorneal pustular dermatitis, and direct immunofluorescence was positive for granular intercellular IgG and IgA deposits in the epidermis. Antinuclear antibody test was negative and C3 level was normal. Antibody tests against desmogleins 1 and 3 were both negative. Topical potent corticosteroid therapy resulted in complete resolution of all lesions in three weeks.
Conclusion
Diagnostic dilemmas arise when laboratory results do not correlate with clinical findings. Findings of IgA autoantibodies in patients with pemphigus-like skin eruptions led to the diagnosis of subcorneal pustular dermatosis type of IgA pemphigus. Dapsone is the treatment of choice although topical potent corticosteroid alone may provide complete remission in some cases, avoiding the potential adverse effects of systemic therapy
Skin Diseases, Vesiculobullous
2.A literature review and clinical consensus guidelines on the management of Bullous Pemphigoid
Clarisse G. Mendoza ; Josef Symon S. Concha ; Cybill Dianne C. Uy ; Bryan K. Guevara ; Evelyn R. Gonzaga ; Maria Jasmin J. Jamora ; Jamaine L. Cruz‑Regalado ; Katrina C. Estrella ; Melanie Joy D. Ruiz ; Rogelio A. Balagat ; Mae N. Ramirez‑Quizon ; Johanna Pauline L. Dizon ; Marie Eleanore O. Nicolas
Journal of the Philippine Dermatological Society 2023;32(2):63-76
Bullous pemphigoid (BP) is the most common autoimmune blistering disease primarily characterized by
tense blisters and occasionally with urticarial plaques, affecting the skin and mucous membranes. These are
caused by autoantibodies against BP180 and BP230 which target antigens on the basement membrane zone.
The diagnosis relies on the integration of clinical, histopathological, immunopathological, and serological
findings. The management depends on the clinical extent and severity. We present in this article a literature
review and the clinical consensus guidelines of the Immunodermatology Subspecialty Core Group of the
Philippine Dermatological Society in the management of BP.
Pemphigoid, Bullous