Relapsing linear acantholytic dermatosis in a four-year-old boy

Abstract

Linear acantholytic dermatoses are a spectrum of cutaneous disorders that form a subset of linear dermatoses with distinct clinical features and histopathologically show acantholysis. The lesions may be zosteriform or follow the lines of Blaschko. This report describes a four-year-old boy who, on a follow up of two years, exhibited a relapsing acantholytic dermatosis along the lines of Blaschko. Histopathology of a representative lesion revealed epidermal acantholysis with multiple acantholytic keratinocytes with in the prickle cell layer and an absence of corp ronds and grains, consistent with features of Hailey-Hailey disease. This, to our knowledge, is the third case of relapsing linear acantholytic dermatosis reported.

An unusual Case of Segmental Hailey–Hailey Disease Developing in a 1.5-Year-Old Child

“…In the past, superimposed linear Hailey-Hailey disease has erroneously been taken as a distinct disorder called “relapsing linear acantholytic dermatosis”. [ 4 5 ] In one of these cases,[ 4 ] examination of the patient's mother revealed the classical non-segmental involvement of Hailey-Hailey disease, and the patient herself did also develop non-segmental lesions at 24 years of age. [ 2 ]…”

Type 2 segmental Hailey–Hailey disease with systematized bilateral arrangement

“…8 In earlier reports of segmental HHD, a unilateral distribution of one or more segmental lesions was observed. [3][4][5][6][7] In this report, we describe a patient with type 2 segmental HHD in the form of systematized bilateral lesions that are superimposed on a classical, non-segmental involvement.…”

“…Hailey–Hailey disease is caused by mutations in the calcium‐dependent adenosine triphosphatase ATP2C1 gene encoding an adenosine triphosphate‐powered Ca 2+ pump 2 . Rarely, a segmental arrangement of the disorder may occur 3–8 . Both type 1 and type 2 segmental presentations of HHD, reflecting mosaicism, have been reported 3–10 .…”

“…Rarely, a segmental arrangement of the disorder may occur 3–8 . Both type 1 and type 2 segmental presentations of HHD, reflecting mosaicism, have been reported 3–10 . Type 1 segmental HHD reflects heterozygosity for a de novo post‐zygotic mutation that occurs at an early stage of embryogenesis and leads to a segmental disease.…”