Chronic mucocutaneous candidiasis - Skin in Infant/Neonate
Most forms of this disease develop in infancy, when the infant is only a few months old, or in the first or second decade of life. The usual pattern of development is oral candidal infection, then nail, followed by skin involvement. Vaginal candidiasis may also develop in early childhood but is not common before puberty.
CMC presents as a spectrum of disorders, which have an immunologic, endocrinologic, or autoimmune disorder association, thus producing a variety of clinical syndromes. Onset of CMC is usually nonendocrinopathic within the first year of life, whereas onset between the ages of 1 and 9 years is associated with endocrinopathies.
These syndromes vary in their clinical pictures and associated underlying disorders, which are classified according to hereditary and clinical factors as follows:
- Patients with HIV have a variant syndrome of mucocutaneous Candida infection as one of the most common skin manifestations of their disease.
- Familial CMC is another reported syndrome in which there is both chronic and recurrent oral candidiasis with minimal skin and nail involvement and no accompanying endocrinopathies. It is both autosomal recessive and autosomal dominant.
- Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is characterized by an autosomal recessive genetic defect involving the autoimmune regulator gene (AIRE). The syndrome is characterized by recurrent oral candidiasis and cutaneous candidiasis, which manifests as diaper rash. Over time, the lesions become more widespread and include the scalp, extremities, and nails. This syndrome is associated with a number of disorders that include hypothyroidism, adrenal failure, gonadal failure, insulin-dependant diabetes mellitus, hypoparathyroidism, gastric parietal cell failure, keratopathy, vitiligo, and alopecia.
- Chronic localized candidiasis, also referred to as Candida granuloma, presents as thickened crust formation on the face and scalp, which can lead to scarring alopecia later on.
- Candidiasis with chronic keratitis has been seen in young children, with patients developing photophobia as an initial complaint followed by keratoconjunctivitis, alopecia, and oral and vaginal candidiasis by the age of 5 years. It is autosomal dominant.
This condition also predisposes to significant infections with other fungal agents, bacteria, and viruses, but the skin and mucous membrane infection with Candida is the predominant clinical picture. Most patients do not go on to develop disseminated Candida infection. The severity of disease is highly variable; some patients improve with age (particularly those with autosomal recessive disease), while others affected may not survive.
B37.2 – Candidiasis of skin and nail
234568006 – Chronic mucocutaneous candidiasis