Indian Journal of Dermatology
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Year : 2006  |  Volume : 51  |  Issue : 3  |  Page : 223-224
Keratosis palmoplantaris punctata (Buschke-Fischer-Brauer) with keratosis pilaris

Department of Dermatology and STD, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry - 605 006, India

Correspondence Address:
Devinder Mohan Thappa
Department of Dermatology and STD, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry - 605 006
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.27997

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How to cite this article:
Kumari R, Thappa D. Keratosis palmoplantaris punctata (Buschke-Fischer-Brauer) with keratosis pilaris. Indian J Dermatol 2006;51:223-4

How to cite this URL:
Kumari R, Thappa D. Keratosis palmoplantaris punctata (Buschke-Fischer-Brauer) with keratosis pilaris. Indian J Dermatol [serial online] 2006 [cited 2022 Jan 27];51:223-4. Available from:

An 8-year-old male child visited us with numerous tiny lesions over both his palms and soles. The lesions first appeared at the age of 6 months as tiny rough papules at the lateral aspect of soles and gradually progressed to involve the center of both the soles and the palms. These lesions started as rough pinpoint papules which later left behind keratotic pits. He also noticed few spiny papules bilaterally over his elbows and knees after 2 years of age. There was a positive family history of similar lesions in five members of the family over four generations. There was no history of any malignancy in any of the affected family members. There was no evidence of toxic origin of these keratoses e.g., arsenic.

On examination, diffuse palmoplantar keratoderma topped by keratotic pits was seen [Figure - 1]. These keratotic pits were bilaterally distributed uniformly over palms and soles. Also, multiple filiform keratotic spiny rough papules (keratosis pilaris) were seen over the elbows and knees [Figure - 2]. Yellowish, discoloured, racquet nails were seen in both the feet.

The child's mother who had accompanied him was the second case in the family with similar lesions. She gave history of similar lesions in her father, aunt and grandmother.

Histology of the representative plantar lesion in the child showed orthokeratosis and parakeratosis but absence of cornoid lamellae or elastorrhexis, confirming the diagnosis of punctate keratoderma. Both the cases were put on topical retinoids (tretinoin 0.1%) with a mild initial response.

Keratosis palmoplantaris punctata (Buschke-Fischer-Brauer) is a rare genodermatosis with a reported incidence of 1.2/100,000.[1] Synonyms include keratoma dissipatum hereditarium palmare et plantare (Brauer), keratoma disseminatum, keratodermia maculosa disseminata symmetrica palmaris et plantaris (Buschke-Fischer), keratodermia palmoplantaris papulosa, and others.[2]

The lesions usually first develop in the second or third decade. Pinpoint, hard keratotic papules, initially translucent but later opaque and warty, appear on the palms and soles. Some cases show crateriform lesions or keratoses that can be picked out.[1] The signs of the affected family members of our case clearly matched the features of keratosis punctata palmoplantaris (Buschke-Fischer-Brauer) but the age of onset was earlier in the affected child. Interestingly, the history revealed five members affected in 4 generations with equal severity. Both male to female and vice versa transmission seen as shown in the pedigree study was highly suggestive of autosomal dominant mode of transmission. Recent fine mapping of a punctate palmoplantar keratoderma gene has located it on 8q24.13-8q24.21 and 15q22-15q24.[3]

The differential diagnosis of this condition (which were excluded in our case) are porokeratosis punctata palmaris et plantaris (punctate porokeratosis), acrokeratoelastoidosis, punctate keratoses of palmar creases, focal acral hyperkeratoses, arsenic keratoses, calluses, viral warts etc.[4] Porokeratosis punctata palmaris et plantaris (punctate porokeratosis) shows cornoid lamellae and parakeratosis - these were absent in the patient described herein. Disorganized elastic fibers typically seen in acrokeratoelastoidosis were not found in our case.

According to the literature, keratolytic ointments and topical retinoids and calcipotriol are not very effective although may cause slight improvement. Many authors have found good response to acitretin and etretinate but prolonged therapy is required. Mild response to topical retinoids was seen in our case after 2 months but patient was lost to follow up.

Stevens et al reported a 4-generation family in whom punctate palmoplantar keratosis was associated with the development of both early- and late-onset malignancies.[5] None of the members of this effected family had any concurrent malignancy but follow up was advised.

Hyperhidrosis, freckle-like hyperpigmentation, facial sebaceous hyperplasia are other associations recently reported with punctate keratoderma. This is the first case report of punctate keratoderma with an early age of onset unusually associated with keratotic papules on elbows and knees and racquet nails. The pedigree analysis of this family also supports autosomal dominant inheritance.

   References Top

1.Campion RH, Burton JL, Breathnach SM, editors. Rook/Wilkinson/Ebling: Textbook of Dermatology. Blackwell Science Ltd: Oxford; 1998. p. 1572-3.  Back to cited text no. 1      
2.Emmert S, Kuster W, Zutt M, Hanssle H, Hallermann C, Kretschmer L, et al . A new family with the rare genodermatosis keratosis punctata palmoplantaris Buschke-Fischer-Brauer. J Am Acad Dermatol 2003;49:1166-9.   Back to cited text no. 2      
3.Gao M, Yang S, Li M, Yan KL, Jiang YX, Cui Y, et al . Refined localization of a punctuate palmoplantar keratoderma gene to a 5.06-cM region at 15q22.2-15q22.31. Br J Dermatol 2005;152:874-8.  Back to cited text no. 3      
4.Lucker GP, Van de Kerkhof PC, Steijlen PM. The hereditary palmoplantar keratoses: An updated review and classification. Br J Dermatol 1994;131:1-14.  Back to cited text no. 4  [PUBMED]    
5.Stevens HP, Kelsell DP, Leigh IM, Ostlere LS, MacDermot KD, Rustin MH. Punctate palmoplantar keratoderma and malignancy in a four-generation family. Br J Dermatol 1996;134:720-6.  Back to cited text no. 5  [PUBMED]    


  [Figure - 1], [Figure - 2]


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