Sézary syndrome, Kaposi sarcoma and generalized dermatophytosis 15 years after sulfur mustard gas exposure

Abstract

Background:The relationship between compromised immune system and the development of malignancy, generalized dermatitis, and infection after sulfur mustard gas exposure has been established. Main observation:We introduce a 58-year-old man with an abrupt, de novo and erythrodermic eruption in 2002 that was previously exposed to sulfur mustard during the Iran -Iraq war in 1987. Six weeks after the onset of diffuse eruption, he developed papules on the glans penis and generalized dermatophytosis. A biopsy of his eruption was consistent with cutaneous T-cell lymphoma/Sézary syndrome. A complete blood count demonstrated leukocytosis, eosinophilia and atypical lymphocytosis. Subsequently, Sézary syndrome was confirmed and T-cell count with increased CD4/CD8 in flow cytometry. The biopsy of his penile papules was consistent with Kaposi's sarcoma. Conclusion:These findings suggest a causative relationship between sulfur mustard gas exposure, cutaneous T cell lymphoma and immune compromised state with opportunistic infections. (J Dermatol Case Rep. 2012; 6(3): 86-89) Sézary syndrome, Kaposi sarcoma and generalized dermatophytosis 15 years after sulfur mustard gas exposure IntroductionCutaneous T-cell lymphoma (CTCL) comprises a group of clinicopathologic entities that are neoplastic proliferation of T lymphocytes that home to the skin. Eventually the T-cells may lose their dependence on an epidermal environment for growth, and spread to the dermis, lymph nodes, blood, and viscera. 1,2The cutaneous lesions evolve from patches to plaque and tumors (mycosis fungoides), and Sézary syndrome, where the neoplastic cells circulate in the peripheral blood. The patients present with generalized exfoliation erythroderma, intense pruritus, peripheral lymphadenophaty, and abnormal hyperchromatic mononuclear cells in the skin and peripheral blood. Decreased T-cell function may lead to subsequent immune compromised status and is followed by infection. 4 Kaposi sarcoma (KS) is a vascular tumor of intermediate malignancy, histologically characterized by proliferation of lymphatic and/or vascular endothelial cells caused by the Kaposi's sarcoma-associated herpesvirus (KSHV), human herpesvirus 8. KS is a systemic disease, which can present with cutaneous lesions with or without internal involvement. Four subtypes have been described: Classic KS, affecting middle aged men of Mediterranean descent, African endemic KS, KS in iatrogenically immunosuppressed patients, and AIDS-related KS. 5Sulfur mustard gas is a potent alkylating agent that has a long history of use as a chemical warfare agent, including recent use by Iraq against Iranian soldiers and civilians. The organs most commonly affected by sulfur mustard gas (SM) are skin, eyes, and airways. Skin lesions are seen in more than 90% of the patients exposed to SM. Although the acute DOI: http://dx.doi.org/10.3315/jdcr.2012.1109 86 systemic and cutaneous effects of SM are well known, few investigations have focused on reporting the long-term carcinogenic effects. 6,7Case Repor...

Mycosis fungoides two decades after exposure to sulphur mustard: a follow‐up of 1100 victims

“…Finally Emadi et al . reported a 58‐year‐old veteran with Sézary syndrome, Kaposi sarcoma and generalized dermatophytosis 15 years after short‐term exposure to SM …”

“…Finally Emadi et al reported a 58-year-old veteran with S ezary syndrome, Kaposi sarcoma and generalized dermatophytosis 15 years after short-term exposure to SM. 27 In spite of several limitations of this report including retrospective design, lack of comparison with unexposed subjects during the study period and unknown pathophysiology of MF development, our unique series can build a platform for other researchers to investigate the carcinogenicity impact of single exposure to SM. We also suggest molecular analysis such as TCR gene rearrangement or microRNA studies to probably help to differentiate early cutaneous T-cell lymphoma (CTCL) from other inflammatory skin disorders like dermatitis in those exposed to SM.…”

Association of Nitric Oxide With Delayed Skin Problems After Sulfur Mustard Exposure: Part of Sardasht-Iran Cohort Study

“…In Sardasht-Iran cohort study, it has been shown that (79.9%) of the Iranian SM-exposed individuals suffer from chronic skin disorders [2]. The most common nonsignificant chronic skin complications were xerosis, eczema, seborrheic dermatitis, cherry angioma, hyperpigmentation, urticaria, and vitiligo while mustard scar was the most significant finding among chemical victims in Sardasht City [2,3,14].…”

“…SM exposure leads to short-and long-term adverse effects on body organs, especially the skin, eyes, respiratory tract, and immune system [2]. The most common SMinduced chronic skin complications are mustard scars, xerosis, eczema, seborrheic dermatitis, cherry angioma, hyperpigmentation, and some rare skin cancers [2][3][4][5]. Despite various studies on the long-term clinical manifestations of skin toxicity in SM-exposed individuals, the pathogenesis of SM-induced skin disorders needs more investigations.…”

The injury progression of T lymphocytes in a mouse model with subcutaneous injection of a high dose of sulfur mustard

“…Research in humans showed that, 20 years after the SM exposure, the severely affected victims of the Iran-Iraq war had a lower CD3 + CD4 + level and higher CD3 + CD8 + level in plasma compared to normal controls [ 13 ]. Another study in humans, found that 15 years after sulfur mustard gas exposure, participants had Sezary syndrome and increased CD3 + CD4 + /CD3 + CD8 + cells in flow cytometry [ 14 ]. Other studies observed increased percentages of CD3 + CD4 + and CD3 + CD8 + T cells in tissues and body fluids in hairless guinea pigs [ 15 ].…”