Chronic mucocutaneous candidiasis and systemic lupus erythematosus: a new variant of chronic mucocutaneous candidiasis?
Dewton de Moraes VasconcelosMaurício Domingues FerreiraPatrícia de Campos PieriAlberto José da Silva Duarte
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Abstract:
Chronic mucocutaneous candidiasis (CMC) is characterized by susceptibility to Candida infection of skin, nails, and mucous membranes. Autoimmune endocrinopathies are common in CMC patients, but there are no reports of the involvement of systemic autoimmune disorders. We present here the first case of this kind of association in a patient with an autosomal dominant variant of CMC. The individual had had this disorder since childhood and systemic lupus erythematosus with secondary antiphospholipid syndrome, as well as renal, articular and hepatic manifestations without thymoma.Keywords:
Chronic mucocutaneous candidiasis
Mucocutaneous zone
Systemic candidiasis
Chronic mucocutaneous candidiasis is a chronic infection of mucoses, nails and skin due to Candida sp., that does not respond to standard treatment. We report on three patients with chronic mucocutaneous candidiasis in association with iron deficiency, IgA deficiency in one patient and adult onset in another. We review the clinical manifestations, the classification and the treatment of this disorder. The immunologic defects associated with chronic mucocutaneous candidiasis provide a model to study the mechanisms of defense of the host against fungal infections.
Chronic mucocutaneous candidiasis
Mucocutaneous zone
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Mucocutaneous zone
Chronic mucocutaneous candidiasis
Thrush
Systemic candidiasis
Diaper Dermatitis
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Хронічний шкірно слизовий кандидоз -нозологічна форма первинного імунодефіциту у дітей. Випадок шкірно слизового кандидозу в дитини
Chronic mucocutaneous candidiasis
Mucocutaneous zone
Primary Immunodeficiency
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Chronic mucocutaneous candidiasis
Mucocutaneous zone
Systemic candidiasis
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Epidemiological assessment of mucocutaneous infections in patients with recurrent infection syndrome
Abstract Background Recurrent infection syndrome (RIS) results from repeated interactions between hosts and environmental infectious agents and is considered normal (NRIS) because of its benign evolution and positive effects in the development of normal immune responses. Abnormal RIS (ARIS) is characterized by the unusually high frequency of severe infections, either as a result of anatomical or functional abnormalities or due to primary or secondary immunodeficiencies (PIDs and SIDs, respectively). Recurrent mucocutaneous infections (MCIs) can be manifestations of RIS or ARIS and could be more frequent in primary immunodeficiencies. Similarly, etiologic agents might vary from what is observed in the general population. Methods We carried out a descriptive study to determine the prevalence of aerobic bacterial and fungal mucocutaneous infections in 452 patients with recurrent infections, using clinical records to establish immunological status associated with the presence and characteristics of the infections. Microbiological analyses from mucocutaneous lesions were used to confirm the etiology. Results We found mucocutaneous infections in 50 patients for a total of 62 episodes (bacterial or fungal infections in 38 vs. 12 patients, respectively). Mucocutaneous infections were more frequent (21.8% vs. 9.1%; OR = 2.8) and recurrent (8.7% vs. 0.2%; P = 0.000) in primary immunodeficient patients. Furthermore, those with defects in phagocytic cells presented more mucocutaneous infections (56.2%) than patients with other primary immunodeficiencies (11.3%; OR = 10.1). Conclusions Bacterial and fungal mucocutaneous infections are more frequent and severe in primary immunodeficient patients, particularly those with defective phagocytosis. Early and adequate assessment of the nature of mucocutaneous infections in ARIS should impact the ability of physicians to treat promptly, avoid complications and reduce the costs of medical assistance.
Mucocutaneous zone
Chronic mucocutaneous candidiasis
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Chronic mucocutaneous candidiasis (CMC) is a rare disease associated with immunodeficiency and characterized by persistent and refractory infections of the skin, appendages and mucous membranes caused by members of the genus Candida. Several different disorders are classified under this common denominator, including chronic and recurrent mucocutaneous infections due to Candida spp., which are sometimes linked to autoimmune endocrinopathies. These fungal infections are usually confined to the mucocutaneous surface, with little propensity for systemic disease or septicemia. We describe a patient with CMC who had an esophageal candidiasis refractory to treatment for decades and who developed an epidermoid esophageal cancer. No risk factors such as familiar susceptibility, smoking, alcohol drinking, or living in an endemic area were verified. This case report suggests the participation of nitrosamine compounds produced by chronic Candida infections as a risk factor for esophageal cancer in a patient with autosomal-dominant chronic mucocutaneous candidiasis.
Chronic mucocutaneous candidiasis
Mucocutaneous zone
Esophageal candidiasis
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Chronic mucocutaneous candidiasis (CMC) is characterized by susceptibility to Candida infection of skin, nails, and mucous membranes. Autoimmune endocrinopathies are common in CMC patients, but there are no reports of the involvement of systemic autoimmune disorders. We present here the first case of this kind of association in a patient with an autosomal dominant variant of CMC. The individual had had this disorder since childhood and systemic lupus erythematosus with secondary antiphospholipid syndrome, as well as renal, articular and hepatic manifestations without thymoma.
Chronic mucocutaneous candidiasis
Mucocutaneous zone
Systemic candidiasis
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Chronic mucocutaneous candidiasis
Mucocutaneous zone
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Key Clinical Message Even if initial immunologic screen is normal, a high index of suspicion for immunodeficiency should guide the evaluation and management of patients with recurrent episodes of mucocutaneous candidiasis. Although rare, a diagnosis of chronic mucocutaneous disease should always be considered in order to improve their outcome.
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