Gharib et al., J Clin Case Rep 2015, 5:5 DOI: 10.4172/2165-7920.1000532 Volume 5 • Issue 5 • 1000532 J Clin Case Rep ISSN: 2165-7920 JCCR, an open access journal Open Access Commentary Keratosis Follicularis Spinulosa Decalvans: Diagnosis and Therapeutic Evaluation Khaled Gharib*, Mohamed Khater, Mohamed Nasr, Mohamed Soliman and Ahmed Abdelshaf Department of Dermatology, Zagazig University, Egypt *Corresponding author: Khaled Gharib, Department of Dermatology, Zagazig University, Egypt, Tel: 44519-57487; E-mail: kh_gharib@hotmail.com Received April 24, 2015; Accepted May 22, 2015; Published May 25, 2015 Citation: Gharib K, Khater M, Nasr M, Soliman M, Abdelshaf A (2015) Keratosis Follicularis Spinulosa Decalvans: Diagnosis and Therapeutic Evaluation. J Clin Case Rep 5: 532. doi:10.4172/2165-7920.1000532 Copyright: © 2015 Gharib K, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Figure 1: Partial loss in the scalp hair in the girl. Introduction and Objectives Keratosis Follicularis Spinulosa Decalvans (KFSD) is an X-linked genodermatosis characterized by scarring alopecia and follicular hyperkeratosis. Tis condition mainly afects males with females being carriers and will have milder symptoms. We present a family of two siblings of KFSD, boy had nine years and girl had fve years old. Tis genodermatosis ofen starts at infancy or early childhood. Keratosis pilaris atrophicans (KPA) is the umbrella term for a group of three rare and distinct clinical entities representing the scarring types of keratosis pilaris [1]. Tree categories of KPA include: Keratosis pilaris atrophicans faciei (KPAF), Atrophoderma Vermiculatum (AV) and Keratosis Follicularis Spinulosa Decalvans (KFSD). Tey have the following features in common: keratotic follicular papules, nonpurulent infammation of variable degree, and atrophic end stages characterized by irreversible hair loss and/or atrophic depressions similar to pitted scars [2]. KFSD simulates the ichthyosis follicularis alopecia photophobia (IFAP) syndrome. Te latter is characterized by non-scarring alopecia, extensive keratosis piliaris, severe photophobia and corneal dystrophy.  Te presence of scarring alopecia in our patients favors the diagnosis of KFSD over the IFAP syndrome. Te other follicular conditions that it needs to be diferentiated from are lichen planopilaris and lichen spinulosus [3]. Material and Methods Tis paper reviews the diferent aspects of KFSD, including pathogenesis, clinical, histological, diferential diagnosis and diferent therapeutic modalities and their impact on the prognosis of the disease. Results A nine year old male and his sister fve years old who born from a frst-degree consanguineous marriage visited our outpatient department with complaints of rough skin over the scalp and over the body since fve years for the boy and two year for the girl in association with total loss of scalp and eyebrow hair in the boy and partial loss in the girl. At birth, their parents noted the absence of scalp and eyebrow hair, which gradually, over the next three to four years grew to some measure and eventually became scanty (Figure 1). Physical examination disclosed multiple follicular fesh-colored horny papules over the scalp, eyebrows, cheeks and both upper and lower limbs. A closer view of the scalp, cheek and eyebrow revealed fne scaling and areas of scarring alopecia, punctuate atrophy , hair loss of eyebrow in the boy and hair loss of the lateral half of eyebrows in the girl. Te teeth, nails, palms and soles were found to be normal. Te boy had history of photophobia but not in the girl (Figures 2 and 3). A punch biopsy specimen from the scalp showed follicular plugging in the epidermis with mild acanthosis, early perifollicular fbrosis with mild lymphocytic infltrate. Te dermis was decreased in thickness with ill-formed sebaceous units. Te hair shafs appeared to be normal. With all the above fndings in hand, a clinical diagnosis of keratosis follicularis spinulosa decalvans was made. Figure 2: Multiple follicular fesh-colored horny papules in chest. Figure 3: Multiple follicular fesh-colored horny papules in neck. Journal of Clinical Case Reports J o u r n a l o f C li n i c a l C a s e R e p o r t s ISSN: 2165-7920