Vesiculobullous Hailey–Hailey’s disease with scarring mimicking a subepidermal autoimmune blistering disease and its management with retinoids

Abstract

epidermotropism with massive fibrosis, should be noticed carefully to prevent misdiagnosis.Primary cutaneous B-cell lymphomas rarely involve the subcutaneous tissue, but it may be secondarily infiltrated by systemic lymphomas. 2 Paulli et al. 2 reported primary CMZL, associated with HCV infection, presenting as subcutaneous nodules resembling lipomas. Chui et al. 3 suggested the possibility of an association between primary subcutaneous MZL and autoimmune diseases. Our first patient had a history of rheumatoid arthritis without HCV infection. Therefore, the patient with subcutaneous MZL should be investigated for HCV infection, autoimmune disease, and systemic lymphomas.Epidermotropism has been reported to be associated with several diseases including mycosis fungoides, S ezary syndrome, and Langerhans cell histiocytosis. 4 Epidermotropism is attributed to the local intraepidermal milieu attracting neoplastic immune cells. 4,5 It has been postulated that interferon-c-induced protein 10, expressed on the keratinocytes and stromal cells of the spleen and bone marrow, attracts neoplastic cells of CMZL with aberrant expression of CXCR3. Therefore, the risk of involvement of the spleen and bone marrow may increase with epidermotropic CMZL. 4 However, a case of epidermotropic CMZL without splenic involvement has been reported as well. 5 Although our second patient showed no evidence of splenic or bone marrow involvement, patients with epidermotropic CMZL should be examined thoroughly to rule out the systemic involvement of MZL.Massive fibrosis found in our second patient is another unusual pathological finding of primary CMZL. It is widely known that Th2 cytokines correlate with the extent of fibrosis in the later stages of scleroderma. 6 Primary CMZL with heavy chain class-switched immunoglobulins (IgG+) is known to be associated with Th2 cytokines contributing to fibrosis. Further, a distinct proportion of primary CMZL with plasmacytic differentiation has been reported to express IgG4. 7 IgG4-related diseases including autoimmune pancreatitis are chronic systemic inflammatory conditions characterized by polyclonal lymphoplasmacytic infiltration rich in IgG4-positive cells and various degree of fibrosis. They are known to be related to the Th2-dominant milieu as well. 8 Therefore, massive fibrosis in primary CMZL, seen in our second patient, might be attributable to Th2 cytokines.In conclusion, we report two cases of primary CMZL with unusual histopathology: subcutaneous involvement and epidermotropism with massive fibrosis.

Case Report: A Case of Hailey–Hailey Disease Mimicking Condyloma Acuminatum and a Novel Splice-Site Mutation of ATP2C1 Gene

“…Rarely, mucosal involvement was observed including conjunctival, oral, esophageal, and vaginal mucosa ( Burge, 1992 ; Oğuz et al, 1997 ; Fresco et al, 2020 ). There are some clinical variants in this disease, such as generalized, segmental, vesiculobullous, condylomatous, circinate or annular, lichenoid, and psoriasiform HHD ( Hwang et al, 2003 ; Vilmer and Dehen, 2004 ; Ghosh et al, 2017 ; Plaza et al, 2017 ; Ni et al, 2018 ; Leducq et al, 2020 ; Ting et al, 2021 ). HHD was concomitant with bullous pemphigoid, eczema herpeticum, and human papillomavirus infection in a few cases ( Chan et al, 2007 ; Shah et al, 2020 ; Li et al, 2021 ).…”