Keratosis follicularis spinulosa decalvans is a rare, xlinked disorder of keratinization of the hair follicle with inflammation and atrophy associated with corneal dystrophy and other symptoms. Keratosis follicularis spinulosa decalvans kfsd is a rare, x linked disorder with skin and eye involvement mim 308800. It involves the scalp but also the eyebrows and the eyelashes. Keratosis follicularis spinulosa decalvans is a disorder affecting the hair follicles. Ulerythema ophryogenes and keratosis follicularis spinulosa decalvans are rare. Keratosis follicularis spinulosa decalvans is an uncommon genodermatosis chiefly characterized by widespread keratosis pilaris, progressive cicatricial alopecia of the scalp, eyebrows, and eyelashes, and an excess of affected males. It develops when hair follicles get plugged by dead skin. Definition background information keratosis follicularis spinulosa decalvans kfsd is a skin disorder that is believed to be an xlinked genetic condition, since a majority of the individuals affected are males. Keratosis follicularis spinulosa decalvans kfsd affects the trunk. A rare inherited skin disorder affecting mostly males and characterized by hardening of skin around hair follicles which leads to scarring and loss of hair. 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. A 10monthold male infant had keratosis follicularis spinulosa decalvans, an xlinked dominant disorder. Congenital atrichia is most prevalent and most affected. Keratosis follicularis spinulosa decalvans and acne.
Keratosis follicularis spinulosa decalvans is a disorder affecting the hair follicles characterized by scarring alopecia of the scalp, eyebrows, and axillae, sometimes associated with photophobia and keratoderma. Folliculitis decalvans fd is a rare, chronic cicatricial scarring alopecia that occurs in adults and classically presents as an expanding patch of alopecia with peripheral pustules on the scalp picture 1ab. An unusual case of folliculitis spinulosa decalvans. Substance p in keratosis follicularis spinulosa decalvans. Keratosis follicularis spinulosa decalvans1,2,12,15. Palmoplantar keratoderma, ocular abnormalities, and atopy can also be present. Keratosis follicularis spinulosa decalvans kfsd, is a rare follicular syndrome. Kfsd is characterized by hardening of the skin keratosis on several parts of the body. The skin on the face, neck, forearms and other areas can also be affected. Keratosis follicularis spinulosa decalvans is a rare type of scarring alopecia described initially by macleod, characterized by lymphocytic predominance in histological sections.
Pdf on may 5, 2015, khaled gharib and others published keratosis follicularis spinulosa decalvans. Keratosis pilaris atrophicans faciei face erythromelanosis follicularis faciei et colli face and neck ulerythema ophryogenes eyebrows keratosis follicularis spinulosa decalvans. Keratosis follicularis spinulosa decalvans kfsd is a keratinization disorder characterized by diffuse follicular hyperkeratosis, progressive cicatricial alopecia, corneal dystrophy, and photophobia. Keratosis pilaris atrophicans kpa is a condition characterized by inflammatory skin growths that may lead to alopecia and scarring. Porteous me, strain l, logie lj, herd rm, benton ec 1998 keratosis. Keratosis follicularis spinulosa decalvans kfsd is a rare, inherited, skin disorder. Keratosis follicularis spinulosa decalvans in a family. Keratosis follicularis spinulosa decalvans is a rare xlinked disorder described by siemens in 1926, a disease that begins in infancy with keratosis pilaris localized on the face, then evolves to more diffuse involvement 580,762. Kfsd is a form of ichthyoses, a group of inherited conditions of the skin in which the skin tends to be thick and rough, and to have a scaly appearance. Ichthyosis follicularis shows several features with keratosis follicularis spinulosa decalvans. Keratosis follicularis spinulosa decalvans medigoo.
We report herein two cases of keratosis follicularis spinulosa decalvans kfsd. The thickening of the skin is accompanied by the loss of. Most frequently, the face, neck, and forearms are involved. Alfadley a, al hawsawi k, hainau b, al aboud k 2002 two brothers with keratosis follicularis spinulosa decalvans. The entity is one of a group of related disorders that shows keratosis pilaris with inflammation followed by atrophy. What is keratosis follicularis spinulosa decalvans. Keratosis follicularis spinulosa decalvans symptoms. Article as pdf 144 kb article as epub print this article. Ifap syndrome is characterized by the triad of follicular ichthyosis, total or subtotal atrichia, and photophobia to variable degree. Abstract keratosis follicularis spinulosa decalvans kfsd is a rare condition. Pdf keratosis follicularis spinulosa decalvans in a female child. Histology from scalp revealed follicular plugging with perifollicular infiltrate of lymphocytes and. Keratosis follicularis spinulosa decalvans verma r.
Keratosis follicularis spinulosa decalvans kfsd is a rare condition characterized by diffuse keratosis pilaris with a scarring alopecia of the scalp and associated photophobia, facial erythema, and palmoplantar keratoderma. The most prominent features present in the male patients. The distinction from kfsd is made mainly on histopathology which reveals. If the inline pdf is not rendering correctly, you can download the pdf file here. Atrophic pillar keratosis comprehends three conditions that differ from each other according to the location of the lesions and level of inflammation and atrophy.
Keratosis follicularis spinulosa decalvans in a female. The scarring alopecia in keratosis follicularis spinulosa decalvans occurs in patients in their teens. Histology from the vertex showed scarring with a mixed perifollicular inflammatory infiltrate and foci of acute suppurative folliculitis. Histopathologic examination revealed hyperkeratosis inflammation and atrophy. Pdf keratosis follicularis spinulosa decalvans case report. Ichthyosis follicularis with atrichia and photophobia. Additionally, there was keratosis pilaris on the cheeks, eyebrows and thighs.
Keratosis follicularis spinulosa decalvans springerlink. Keratosis follicularis spinulosa decalvans kfsd is a genetic disorder within the family of keratosis pilaris atrophicans. Keratosis follicularis spinulosa decalvans kfsd is a keratinization disorder. Histopathology from a keratotic papule over the scalp showed follicular plugging, basket weave orthokeratosis, mild perivascular inflammatory. Keratosis follicularis spinulosa decalvans in a female sequeira ff. Keratosis follicularis spinulosa decalvans with associated mental. A family with several affected members is reported. Keratosis follicularis spinulosa decalvans kfsd is a rare, inherited, skin. Keratosis follicularis spinulosa decalvans wikipedia. He reported dryness and flaking of his scalp at the age of 10 years, which progressed to lesions and permanent hair loss. Keratosis follicularis spinulosa decalvans is an xlinked inherited genodermatosis was has recently been mapped to the xp22 region. Keratosis follicularis spinulosa decalvans kfsd is an xlinked xenodermatosis characterized by scarring alopecia and follicular hyperkeratosis. Av, and c keratosis follicularis spinulosa decalvans kfsd. Keratosis follicularis spinulosa decalvans is a rare disease, with genetic transmission either xlinked or sporadic, characterized by follicular hyperkeratosis and cicatricial alopecia.
Keratosis follicularis spinulosa decalvans kfsd is an inherited rare disorder characterized by diffuse keratosis pilaris and scarring alopecia. Keratosis follicularis is usually worse in the summer and improves in the winter. This sporadic or xlinked recessive dominant disorder xp21. His cutaneous abnormalities consisted of generalized hyperkeratosis, spiny follicular papular lesions, universal alopecia, and hypoplastic nails.
Variations of keratosis pilaris include keratosis pilaris rubra red, inflamed bumps which can be on arms, head, legs, keratosis pilaris alba rough, bumpy skin with no irritation, keratosis pilaris rubra faceii reddish rash on the cheeks, keratosis pilaris atrophicans, keratosis follicularis spinulosa decalvans, atrophoderma vermiculatum. Keratosis pilaris atrophicans refers to uncommon forms of keratosis pilaris in which there are scarlike follicular depressions and loss of hair. Clinical findings, cutaneous pathology, and response to therapy in 21 patients with keratosis pilaris atrophicans. We present two sisters with severe form of kfsd, progressing to scarring alopecia.
Picture of skin problems keratosis pilaris atrophicans. Seven male patients and six female carriers are described. The face, neck, and forearms are frequently involved. Pdf pcongenital alopecia universalis is a very rare presentation. Keratosis follicularis spinulosa decalvans kfsd is a rare, inherited, skin condition. Although initially described as a sexlinked disorder, several different inheritance patterns have been observed. Diagnosis and therapeutic evaluation find, read and. Keratosis follicularis spinulosa decalvans, a relatively rare disorder, was first described by macleod, however, it was siemens who first used this descriptive term in 1926. Overlap between ulerythema ophryogenes and keratosis. Keratosis follicularis nord national organization for. This condition mainly affects males with females being carriers and will have milder symptoms. We have studied a large british family with kfsd using polymorphic markers from xp21p23 and obtained a lod score of 2. Keratosis pilaris atrophicans faciei kpaf and atrophoderma vermiculatum av occur mostly on the face. Special attention has been paid to the dermatological and ophthalmic markers of kfsd in patients and carriers.
Ulerythema acneiforme, in histopathology of diseases of the skin. Kpaf and av present mainly on the face with kfsd often appearing on the eyebrow and av most commonly seen on the cheeks, sparing the eyebrows and scalp. Keratosis follicularis spinulosa decalvans dovemed. Keratosis follicularis spinulosa decalvans is a rare disease, with genetic transmission. Keratosis follicularis spinulosa decalvans is a rare disease, with genetic transmission either xlinked. The case was diagnosed as keratosis follicularis spinulosa decalvans. Another common finding associated with keratosis follicularis is the development of multiple, small, yellowbrown, flattened wartlike verrucous bumps. Keratosis follicularis spinulosa decalvans kfsd is a rare x linked disease which is characterised by follicular hyperkeratosis of the skin and corneal dystrophy. Keratosis follicularis spinulosa decalvans genetic and. With clinical correlation, the diagnosis of keratosis follicularis spinulosa decalvans and concurrent acne keloidalis nuchae was made. Successful treatment of keratosis follicularis spinulosa. We report the case of a 24yearold man who presented with pustules, atrophic scars, and alopecia on the scalp, along with follicular keratotic papules on the cheeks, chest, abdomen, back, lateral upper arms, thighs, and axillae, of 6 years duration.
Histopathology of keratosis pilaris shows modest thickening of the outer layer of skin. Patients may experience associated pruritus or pain. Associated eye findings include photophobia in childhood and corneal dystrophy. Home published aheadofprint successful treatment of keratosis follicularis spinulosa dec. Successful treatment of keratosis follicularis spinulosa decalvans with an 800nm diode laser.
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