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Year : 2013  |  Volume : 5  |  Issue : 1  |  Page : 29-31  

Keratosis follicularis spinulosa decalvans: A rare cause of scarring alopecia in two young Indian girls

1 Department of Pathology, PSG Institute of Medical Sciences and Research, Peelamedu, Coimbatore, Tamil Nadu, India
2 Consultant Dermatologist, Skin Care Clinic, Pollachi, Coimbatore, Tamil Nadu, India

Date of Web Publication6-Jul-2013

Correspondence Address:
Uma G Maheswari
Department of Pathology, PSG Institute of Medical Sciences and Research, Peelamedu, Coimbatore - 641 004, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0974-7753.114713

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Keratosis follicularis spinulosa decalvans (KFSD) is an X-linked xenodermatosis characterized by scarring alopecia and follicular hyperkeratosis. This condition mainly affects males with females being carriers and will have milder symptoms. We present two sisters with severe form of KFSD, progressing to scarring alopecia.

Keywords: Female, keratosis follicularis spinulosa decalvans, scarring alopecia

How to cite this article:
Maheswari UG, Chaitra V, Mohan SS. Keratosis follicularis spinulosa decalvans: A rare cause of scarring alopecia in two young Indian girls. Int J Trichol 2013;5:29-31

How to cite this URL:
Maheswari UG, Chaitra V, Mohan SS. Keratosis follicularis spinulosa decalvans: A rare cause of scarring alopecia in two young Indian girls. Int J Trichol [serial online] 2013 [cited 2023 Mar 24];5:29-31. Available from: https://www.ijtrichology.com/text.asp?2013/5/1/29/114713

   Introduction Top

Keratosis follicularis spinulosa decalvans (KFSD) is a type of scarring alopecia which is grouped under a broader term keratosis pilaris atrophicans (KPAs). The other two clinical entities under KPAs include KPA faciei and atrophoderma. Common to these conditions are keratotic follicular papules, non-purulent inflammation of variable degree, irreversible hair loss, and/or atrophic depression similar to pitted scars. Very few cases of KFSD have been reported in Indian literature. [1],[2],[3] We here present a case report of severe form of KFSD, a rare cause of scarring alopecia in 2 young Indian girls.

   Case Report Top

A 8-year-old female came to the clinic with complaints of hair loss over the scalp since 6 years with sparse eye brows. Hair loss was progressive in spite of trying Ayurvedic medicine and homeopathy. On examination hair density was reduced over the scalp with vellus and terminal hairs. Multiple follicular papules were seen giving roughness to the scalp [Figure 1] and [Figure 2]. Similar lesions were seen all over the back, chest wall, extensor aspects of limbs. There was no history of photophobia and ophthalmic examination showed no evidence of conjunctival/corneal involvement. Punch biopsies were taken and submitted for histopathological examination. Her 5 year old sister had similar complaints. Both the siblings were normal at birth, the above symptoms started from the age of 2 years. However, biopsy was not taken from the younger sibling. Histopathologic examination revealed hyperkeratosis and mild acanthosis of epidermis with follicular plugging and dilated infundibulum [Figure 3]. The dermis revealed perifollicular lymphocytic infiltrate with eccentric epithelial atrophy, polytrichia, and perifollicular fibrosis [Figure 4]. Periodicacid Schiffs (PAS) stain done did not show any fungal organisms. Considering family history and clinical features, a diagnosis of KFSD was offered.
Figure 1: Scalp showing sparse hairs and keratotic papules and sparse eyebrow hairs

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Figure 2: Closer view of scalp showing sparse hairs and prominent keratotic papules (revised)

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Figure 3: Microscopy – Epidermis shows follicular plugging, acanthosis, and mild hyperkeratosis (H and E, ×4)

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Figure 4: Higher power showing perifollicular fibrosis and lymphocytic infiltrate (H and E, ×10)

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   Discussion Top

The evaluation of hair loss is a diagnostic challenge to both dermatologist and pathologist. A good clinico-pathological correlation is very much essential. Alopecia can broadly be classified as scarring and non-scarring. KFSD is a rare type of primary scarring alopecia with lymphocyte predominance. [4]

KFSD is a rare genetic disorder with X-linked and autosomal dominant pattern of inheritance. [5] This condition was first described by Macleod, [6] but the term KFSD was used by Siemen in the year 1926. [7] The gene has been mapped to Xp21.2-p22.2. [8] Sporadic cases have also been described. [9] Clinically the condition is characterized by follicular hyperkeratosis and scarring alopecia. Follicular hyperkeratosis begins during infancy or early childhood, first on the face, affected the eyebrows, cheeks, forehead, and nose. Scarring alopecia of scalp, eyebrows begins in early childhood and progresses further. Other associated features include palmoplantar keratoderma, corneal dystrophy with photophobia, high periungal cuticles. [9] Occasional cases have been associated with Downs syndrome. [10]

In X-linked dominant inheritance pattern, men present with full blown disease, whereas female act as carriers and have milder form of disease. [11] Females with severe form of disease have also been reported. [3],[11] These cases either had autosomal dominant pattern of inheritance and few cases were sporadic in onset. A theory of non-random X inactivation (process of lyonization) was proposed in cases with sporadic onset. [3] In present case report, both the sisters were affected without any other family members being involved. They may either have autosomal dominant pattern of inheritance or process of lyonization.

The differential diagnosis of KFSD include icthyosis follicularis alopecia photophobia (IFAP) syndrome (which is characterized by non-scarring alopecia), lichen planopilaris, and lichen spinulosus. IFAP syndrome is characterized by non-scarring alopecia in contrast to KFSD. The other two conditions can be also be differentiated by thorough clinic-pathological examination. [4],[9],[11]

There is no specific treatment for KFSD. Various drugs have been tried to delay scarring alopecia such as isotretinoin and dapsone. [10],[11] For symptomatic relief emollients, topical steroid, and keratolytic agents can be used.

   References Top

1.Reddy BS, Thadeus J, Garg BR, Rathnakar C. Keratosis follicularis spinulosa decalvans. Indian J Dermatol Venereol Leprol 1995;61:106-8.  Back to cited text no. 1
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2.Puri N, Malhotra SK. Keratosis follicularis spinulosa decalvans: A rare entity. Egypt Dermatol Online J 2008;4:1-6.  Back to cited text no. 2
3.Sequeira FF, Jayaseelan E. Keratosis follicularis spinulosa decalvans in a female. Indian J Dermatol Venereol Leprol 2011;77:325-7.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.Sellheyer K, Bergfeld WF. Histopathologic evaluation of alopecias. Am J Dermatopathol 2006;28:236-59.  Back to cited text no. 4
5.Porteous ME, Strain L, Logie LJ, Herd RM, Benton EC. Keratosis follicularis spinulosa decalvans: Confirmation of linkage to Xp22.13-p22.2. J Med Genet 1998;35:336-7.  Back to cited text no. 5
6.Macleod JM. Three cases of ichthyosis follicularis associated with baldness. Br J Dermatol 1909;21:165-89.  Back to cited text no. 6
7.Rand R, Baden HP. Keratosis follicularis spinulosa decalvans. Report of two cases and literature review. Arch Dermatol 1983;119:22-6.  Back to cited text no. 7
8.Oosterwijk JC, Nelen M, van Zandvoort PM, van Osch LD, Oranje AP, Wittebol-Post D, et al. Linkage analysis of keratosis follicularis spinulosa decalvans, and regional assignment to human chromosome Xp21.2-p22.2. Am J Hum Genet 1992;50:801-7.  Back to cited text no. 8
9.Ross EK, Tan E, Shapiro J. Update on primary cicatricial alopecias. J Am Acad Dermatol 2005;53:1-37.  Back to cited text no. 9
10.Alfadley A, Al Hawsawi K, Hainau B, Al Aboud K. Two brothers with keratosis follicularis spinulosa decalvans. J Am Acad Dermatol 2002;47:S275-8.  Back to cited text no. 10
11.Bellet JS, Kaplan AL, Selim MA, Olsen EA. Keratosis follicularis spinulosa decalvans in a family. J Am Acad Dermatol 2008;58:499-502.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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