Hailey Hailey disease: a case report

Abstract

Hailey Hailey disease or Familial chronic benign pemphigus is a rare autosomal dominant acantholytic disease, clinically characterized by flaccid bullae and erosions in the intertriginous areas, mainly the axillary and inguinal region. We herein report a case of a forty year old female belonging to ethnic Kashmiri population with clinical and histopathological features suggestive of Hailey Hailey disease. StreszczenieChoroba Hailey Hailey lub przewlekła rodzinna łagodna pęcherzyca jest rzadką autosomalnie dominującą akantholityczną chorobą, klinicznie charakteryzującą się wiotkimi pęcherzami i nadżerkami w obszarach wyprzeniowych, głównie pach i pachwin. W niniejszym raporcie opisujemy przypadek czterdziestoletniej kobiety należącej do etnicznej ludności Kaszmiru z klinicznymi i histopatologicznymi cechami wskazującymi na chorobę Hailey Hailey.

Multiple seborrheic keratosis in a patient with familial benign pemphigus

“…We described clinical case which demonstrates once again the difficulty of diagnosis of chronic benign familial pemphigus Hailey-Hailey and the need of the confirmation morphologically. Pronounced positive effect of systemic and topical antibiotics and the ineffective of oral corticosteroid therapy is consistent with the data of other researchers about individual response to therapy of patients with PHH [3,4].…”

“…Their effectiveness, however, individual. Medical therapies generally do not induce prolonged remissions [3,4].…”

Hailey-Hailey Disease – A Case Report

“…Genetic defect results in altered cellular connections within desmosomes and adherens junctions of epidermis, secondary to high cytosolic calcium levels. Other manifestations include oxidative stress and microRNAs [4,5].…”

“…Histopathology has a key role in diagnosis of HHD with characteristic dilapidated brick wall appearance, intracellular immunoglobulin G is not detected in epidermis by direct immunofluorescence staining in contrast to autoimmune pemphigus [5]. Treatment of HHD is very challenging; there is no specific therapy for HHD.…”

Hailey-Hailey disease presenting at unusual site

“…Normally this gene plays a part in ensuring that the cells in the outer layer of the skin (the epidermis) stick together. In HHD this adhesion of cells is weakened, resulting in acantholysis in areas of skin prone to friction [2,3]. Thereby, heat, sweating, infection and contact irritants can exacerbate skin lesions, for which reasons the disease has a relapsing and remitting course.…”