Acquired ichthyosis associated with systemic lupus erythematosus

Abstract

Acquired ichthyosis is an uncommon disease, it is characterized by symmetric scaling of the skin, which ranges from minor roughness and dryness to the desquamation of large plaques. It has been associated with various systemic diseases, although malignant conditions, especially Hodgkin's lymphoma are the most commonly cited. Drugs, HIV infection and autoimmune diseases such as dermatomyositis and mixed connective tissue disease have also been associated, and it has only rarely been found among patients with systemic lupus erythematosus (SLE). Herein, we report a patient with active SLE who developed a generalized acquired ichthyosis corroborated with skin biopsy, which completely disappeared after treatment. The association of autoimmune conditions with acquired ichthyosis could indicate that an abnormal host immune response, probably against components of the granular cell layer in particular the keratohyalin granules, may have a role in the pathogenesis. Thus, this finding may be a relevant cutaneous marker for an autoimmune origin.

Acquired ichthyosis associated with an overlap syndrome of systemic sclerosis and systemic lupus erythematosus

“…Other associated conditions include leprosy, sarcoidosis, thyroid disease, hyperparathyroidism, chronic renal failure, nutritional disorders, human immunodeficiency virus infection, bone marrow transplant, SLE, dermatomyositis, and therapy with certain medications, namely nicotinic acid, triparanol, butyrophenones, dixyrazinem cimetidine and clofazimine 1,3 . To our knowledge, our case is the first to describe AI associated with the overlap syndrome of SSc and SLE, although an association with SLE has been noted in rare cases 2,3,5,6 …”

“…AI may represent abnormal host immune responses, impaired lipogenesis or paraneoplastic consequences. In addition, abnormal circulating anti‐profilaggrin antibodies may result in autoimmune‐associated AI 2 …”

“…Acquired ichthyosis (AI) is an uncommon disease that usually appears in adulthood and is clinically and histologically indistinguishable from ichthyosis vulgaris 1 . AI has been associated with various conditions, including Hodgkin's lymphoma, sarcoidosis and autoimmune disorders such as systemic lupus erythematosus (SLE) and dermatomyositis 1,2 . Herein, we report a patient with an overlap syndrome of systemic sclerosis (SSc) and SLE who developed AI on her extremities.…”

Generalized morphea in a child with harlequin ichthyosis: a rare association

“…3 Congenital ichthyosis is often associated with a variety of typical neonatal phenotypes with scaling and erythema. The main subtypes are congenital ichthyosiform erythroderma, lamellar and harlequin ichthyosis, including overlapping phenotypes.…”

“…3 Harlequin ichthyosis is a congenital form, transmitted in an autosomal recessive mode. It is the most severe and often fatal form of ichthyosis.…”