Keratosis follicularis spinulosa decalvans is a skin disease that stems from a genetic defect that occurs on the X chromosome. It is an inherited disorder that mainly affects Caucasian males. This rare condition affects only a few people, compared to the other causes of hair loss. German dermatologist, Hermann Werner Siemens, was credited with being the first medical professional to recognize the symptoms of this disease. Siemens conducted research on skin diseases that led him to isolate the disease, in 1926. As a result, the disease is also called Siemens’ Syndrome 1. Because of its effect on the patient’s hair, some scientists describe it as woolly hair.

Symptoms/Diagnosis
The underlying symptoms of woolly hair start in infancy. Due to the nature of the genetic defect, they initially develop keratosis pilaris, bumps or rashes in and around the body’s hair follicles. Keratosis is an excess of keratin, a protein in skin. When people have keratosis pilaris, hair follicles get trapped in their pores. These skin abnormalities generally affect the back, forearms, thighs, and even one’s butt. The skin condition resembles goose bumps (usually a temporary reaction to external stimuli). Some people refer to this condition as chicken skin.

Prognosis
In people with keratosis follicularis spinulosa decalvans, the skin around the hair follicles hardens and gets stiff. Hair is not able to grow through the affected follicles. Somtimes hair breaks off in the follicle (ingrown hairs). Most people have tufted (woolly) hair. This may result from the abrasion that the hair suffers when pushing through the keratin plugs in the hair follicles. The condition affects scalp hair, eyebrows, beards and eyelashes. Scarring alopecia is the primary cause of hair loss, often complete baldness. People who have the genetic defect also report sensitivity to light, eyelid thickening, and itching. Medical examinations also reveal that some patients have abnormal development of the lower jaw and corneal abnormalities.

Treatment
Keratosis follicularis spinulosa decalvans is treatable, but no cure is available at this time. Some people have successfully relieved symptoms with application of topical corticosteroids and injections of triamcinolone acetonide, when used for sustained periods. Retinoids taken orally seem to be most effective when hair loss is in progress. However, the medical community does not recommend prolonged use of retinoids for children. Recurring infections are often treated with antibacterial or anti staphylococcal drugs. Diagnosis and treatment requires testing by a competent dermatologist.