Keratosis follicularis spinulosa decalvans

Keratosis follicularis spinulosa decalvans: Description, Causes and Risk Factors: Keratosis follicularis spinulosa decalvans is a rare genetic disorder characterized by development of hyperkeratotic follicular papules on the scalp followed by progressive alopecia of the scalp, eyelashes, and eyebrows. Associated eye findings include photophobia in childhood and corneal dystrophy. Keratosis follicularis spinulosa decalvansManifestations of the disease start in childhood, frequently on the face, but it might be circumscribed to the face and extremities or generalized. Cicatricial alopecia on the scalp and supercilium is the marking characteristic of the disease. Some cases show association with corneal opacity, photophobia and palm-plantar hyperkeratosis that usually start in adolescence up to this moment a completely effective therapy is not. This disease starts in the early years of life, initially on the face and progressing towards the trunk and limbs and it might spread to other parts of the body. Palm-plantar hyperkeratosis, photophobia, corneal abnormalities and atopies can be associated. Men are more seriously affected by the disease. It has X-linked mode of inheritance mapped to a locus at Xp22.13-p22.2. However, the condition has also been seen in families in which the pattern of transmission suggests an autosomal dominant inheritance. Some authors used the appellation of folliculitis spinulosa decalvans to define the autosomal dominant form, which could be differentiated from the commoner X-linked one by more pronounced follicular inflammation, particularly evident on the scalp. In general men are more severely affected while female heterozygotes may exhibit a milder phenotype in families with an X-linked dominant inheritance. The process of lionization may explain expression of KFSD in women. The pathophysiology of the destruction of the pilar follicle is not well known yet. The first changes observed are hyperkeratosis and hypergranulosis of the infundibulum and isthmus, which cause inflammatory reaction (at the beginning acute and lately chronic, with mononuclear infiltrate) on the epidermis and on the papillary dermis. Chronic changes are followed by fibrosis and the destruction of the follicle. Symptoms: The clinical features and course of keratosis follicularis spinulosa decalvans are characteristic. During infancy, keratosis pilaris begins on the face and, by childhood, progresses to involve the trunk and extremities. Sometime during childhood or up to the early teenage years, a cicatricial alopecia of the scalp and eyebrows develops and is the hallmark of this disorder. Hyperkeratosis of the palms and soles is a frequently associated finding and is usually manifested during adolescence. Other features occurring with this syndrome include atopy, photophobia, and corneal abnormalities. Sex-linked inheritance has been proposed by several authors. Diagnosis: It is typically diagnosed based on an examination of your skin and a review of your medical history. Your doctor will ask questions about your signs and symptoms. The genetic testing will be needed in order to confirm the diagnosis. Apart from this other necessary skin tests will be ordered to rule out other conditions. Treatment: Different systemic treatments including isotretinoin, etretinate, dapsone and antibiotics have been tried with varied results. It is suggested that the use of retinoids in the early and more active phase of the disease, when histopathology shows perifollicular infiltrate, can bring some benefit. Treatment is even more disappointing when the disease predominantly shows cicatricial changes, as in this present study. Treatment in this circumstance is reduced only to the use of topical palliative medication. NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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