Mid-dermal elastolysis as a manifestation of immune reconstitution inflammatory syndrome in an HIV-infected patient
Authors: Carlo Cota, Alessandra Latini, Viviana Lora, Lorenzo Cerroni
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: The immune reconstitution inflammatory syndrome (IRIS) encompasses a group of disorders with different clinical manifestations occurring in HIV-infected patients after starting highly active antiretroviral therapy (HAART), resulting from the restored ability of the immune system to mount an antigen-specific inflammatory response. Previous studies have demonstrated that 10% to 25% of HIV-positive patients starting on antiretroviral therapy experience IRIS, and in more than half of them cutaneous involvement represents the first manifestation of the disease.1Lehloenya R. Meintjes G. Dermatologic manifestations of the immune reconstitution inflammatory syndrome.Dermatol Clin. 2006; 24: 549-570Abstract Full Text Full Text PDF PubMed Scopus (78) Google Scholar A 32-year-old Caucasian man with a 13-year history of HIV infection (CD4 cell count 269 cells/mm3, plasma HIV RNA load [VL] 16,315 copies/mL) started HAART in December 2012. Two months later his CD4 cell count increased to 465 cells/mm3, and the viral load was undetectable. At the same time, the patient reported a progressive, asymptomatic, erythematous, parchment-like appearance of the skin on his trunk (Fig 1). He denied prolonged ultraviolet light exposure. A skin biopsy specimen showed a sparse lymphohistiocytic infiltrate admixed with scattered, multinucleated histiocytic giant cells arranged between the collagen bundles in the mid dermis (Fig 2, A). Focal elastophagocytosis was also noted. Orcein staining highlighted a bandlike loss of elastic fibers in the mid dermis (Fig 2, B). A diagnosis of mid-dermal elastolysis was made. Four months later the patient experienced gastrointestinal symptoms associated with perianal fistulae. A diagnosis of Crohn's disease was confirmed by radiologic, endoscopic, and histopathological examination. Mid-dermal elastolysis is a rare condition characterized by localized, bandlike loss of elastic fibers in the mid dermis.2Gambichler T. Mid-dermal elastolysis revisited.Arch Dermatol Res. 2010; 302: 85-93Crossref PubMed Scopus (69) Google Scholar It presents clinically with variably large areas of fine wrinkling, tiny perifollicular papules, or both. The pathogenesis is unclear. Several cases seem to be related to ultraviolet radiation, whereas in others immunologic mechanisms may be involved. The association with different autoimmune disorders and the elevated antinuclear antibody found in some patients with mid-dermal elastolysis have supported the hypothesis of an autoimmune origin.2Gambichler T. Mid-dermal elastolysis revisited.Arch Dermatol Res. 2010; 302: 85-93Crossref PubMed Scopus (69) Google Scholar To our knowledge, mid-dermal elastolysis in the course of HIV infection has been reported only once.3Bannister M.J. Rubel D.M. Kossard S. Mid-dermal elastophagocytosis presenting as a persistent reticulated erythema.Austral J Dermatol. 2001; 42: 50-54Crossref PubMed Scopus (32) Google Scholar In contrast to our patient, the onset of skin lesions was unrelated to treatment, and an association with actinic damage was suggested as the cause of the skin disease. In our patient, skin manifestations of mid-dermal elastolysis appeared at the same time of the immune restoration after HAART, thus suggesting that they may be etiologically related. Indeed, our case fulfills diagnostic criteria of IRIS, namely: temporal association between initiation of HAART and development of symptoms, evidence of immune restoration with virologic and immunologic response, and clinical symptoms consistent with an inflammatory process.1Lehloenya R. Meintjes G. Dermatologic manifestations of the immune reconstitution inflammatory syndrome.Dermatol Clin. 2006; 24: 549-570Abstract Full Text Full Text PDF PubMed Scopus (78) Google Scholar The subsequent occurrence of Crohn's disease may support the intriguing hypothesis of a multiorgan autoimmune IRIS phenomenon. In fact, IRIS-induced tissue inflammation may result in an increase in local inflammatory cytokines and recognition of viral and/or self-antigens by the infiltrating T cells.4McLeod D.S.A. Woods M.L. Kandiah D.A. Immune reconstitution inflammatory syndrome manifesting as development of multiple autoimmune disorders and skin cancer progression.Intern Med J. 2011; 41: 699-703Crossref PubMed Scopus (7) Google Scholar The inflammatory environment induced by IRIS and the loss of immune self-tolerance to tissue-associated antigens may lead to increased susceptibility to develop local and/or systemic pathological autoimmune conditions.5Krupica Jr., T. Fry T.J. Mackall C.L. Autoimmunity during lymphopenia: a two-hit model.Clin Immunol. 2006; 120: 121-128Crossref PubMed Scopus (154) Google Scholar This mechanism could support the hypothesis that mid-dermal elastolysis is an autoimmune process against elastic fibers.