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Familial keratosis follicularis spinulosa decalvans associated with woolly hair.
Int J Dermatol 2007; 46(8):840-3IJ

Abstract

Keratosis follicularis spinulosa decalvans (KFSD) is a rare inherited disorder of keratinization clinically characterized by diffuse follicular hyperkeratosis, progressive scarring alopecia of scalp, eyebrows and eyelashes, corneal dystrophy and photophobia. Woolly hair is a hereditary condition, transmitted as an autosomal dominant or recessive trait, usually seen in Caucasians at birth or shortly after, in which there are curly, thick, often heavily pigmented hairs. We report two cases, a son and his mother, in whom KFSD occurred in association with woolly hair. In addition, various dental anomalies, including agenesis, inclusions and teeth malformations, were present in the child. Interestingly, both patients simultaneously developed an inflammatory tinea capitis caused by Microsporum canis. To our knowledge, the association of KFSD with woolly hair has not been described. The dental anomalies found in the child are intriguing, as they have never been reported previously in patients with KFSD. Finally, the concomitant onset of inflammatory tinea capitis in both patients may be explained by the enhanced susceptibility to fungal infection in keratinizing disorders.

Authors+Show Affiliations

Dermatology Clinic, University of Catania, Catania, Italy.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

17651168

Citation

Lacarrubba, Francesco, et al. "Familial Keratosis Follicularis Spinulosa Decalvans Associated With Woolly Hair." International Journal of Dermatology, vol. 46, no. 8, 2007, pp. 840-3.
Lacarrubba F, Dall'Oglio F, Rossi A, et al. Familial keratosis follicularis spinulosa decalvans associated with woolly hair. Int J Dermatol. 2007;46(8):840-3.
Lacarrubba, F., Dall'Oglio, F., Rossi, A., Schwartz, R. A., & Micali, G. (2007). Familial keratosis follicularis spinulosa decalvans associated with woolly hair. International Journal of Dermatology, 46(8), pp. 840-3.
Lacarrubba F, et al. Familial Keratosis Follicularis Spinulosa Decalvans Associated With Woolly Hair. Int J Dermatol. 2007;46(8):840-3. PubMed PMID: 17651168.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Familial keratosis follicularis spinulosa decalvans associated with woolly hair. AU - Lacarrubba,Francesco, AU - Dall'Oglio,Federica, AU - Rossi,Alfredo, AU - Schwartz,Robert A, AU - Micali,Giuseppe, PY - 2007/7/27/pubmed PY - 2007/10/18/medline PY - 2007/7/27/entrez SP - 840 EP - 3 JF - International journal of dermatology JO - Int. J. Dermatol. VL - 46 IS - 8 N2 - Keratosis follicularis spinulosa decalvans (KFSD) is a rare inherited disorder of keratinization clinically characterized by diffuse follicular hyperkeratosis, progressive scarring alopecia of scalp, eyebrows and eyelashes, corneal dystrophy and photophobia. Woolly hair is a hereditary condition, transmitted as an autosomal dominant or recessive trait, usually seen in Caucasians at birth or shortly after, in which there are curly, thick, often heavily pigmented hairs. We report two cases, a son and his mother, in whom KFSD occurred in association with woolly hair. In addition, various dental anomalies, including agenesis, inclusions and teeth malformations, were present in the child. Interestingly, both patients simultaneously developed an inflammatory tinea capitis caused by Microsporum canis. To our knowledge, the association of KFSD with woolly hair has not been described. The dental anomalies found in the child are intriguing, as they have never been reported previously in patients with KFSD. Finally, the concomitant onset of inflammatory tinea capitis in both patients may be explained by the enhanced susceptibility to fungal infection in keratinizing disorders. SN - 0011-9059 UR - https://www.unboundmedicine.com/medline/citation/17651168/Familial_keratosis_follicularis_spinulosa_decalvans_associated_with_woolly_hair_ L2 - https://doi.org/10.1111/j.1365-4632.2007.03162.x DB - PRIME DP - Unbound Medicine ER -