Chronic mucocutaneous candidiasis - Skin in Child
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. Onset may occur after this age, and recurrent oral candidiasis is common in middle-aged women but without the skin and nail involvement. 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.
These syndromes vary in their clinical picture and associated underlying disorder, but, generally, infants present with either oral candidiasis (thrush) or diaper dermatitis. This is followed by onychomycosis and the possible later development of chronic disfiguring skin lesions, which appear as hyperkeratotic crusted areas on the face, scalp, and hands. These distinct syndromes are all referred to as CMC and present as follows.
Patients with HIV have a variant syndrome of mucocutaneous Candida infection as one of the most common skin manifestations of their disease. It presents in 1 of 4 ways:
- Pseudomembranous variety has the formation of white plaques.
- Erythematous candidiasis appears as smooth areas on the tongue due to loss of papilla.
- Hyperplastic candidiasis has the formation of white plaques that are firmly adherent to the mucosal surface.
- Angular cheilitis
Familial chronic mucocutaneous candidiasis is another reported syndrome in which there is both chronic and recurrent oral candidiasis with minimal skin and nail involvement and no accompanying endocrinopathies. This is commonly seen in children by the age of 2 years in both girls and boys, and a history will establish a similar condition among other family members but no endocrinopathy.
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. Approximately half of these patients have 4 or 5 of the above disorders. The most commonly associated endocrinopathy was hypoparathyroidism, followed by hypoadrenalism and ovarian failure.
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. It usually appears in early childhood before the child is aged 5 years. In some instances there is cutaneous horn formation with hyperkeratosis and acanthosis. There may be associated oral candidiasis.
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.
CMC is seen equally in both boys and girls.
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