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Year : 2012  |  Volume : 1  |  Issue : 2  |  Page : 115-117

Brauer-Buschke-Fischer keratoderma associated with two malignancies

Department of Dermatology, MVJ Medical College and Research Hospital, Hoskote, Bangalore, Karnataka, India

Date of Web Publication27-Sep-2012

Correspondence Address:
C Sujatha Vinod
Department of Dermatology, MVJ Medical College and Research Hospital, Hoskote, Bangalore - 562 114, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2278-344X.101715

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Hereditary punctuate palmoplantar keratoderma is a rare autosomal dominantly inherited condition with variable penetrance. We report a case of a 65-year-old manual laborer who presented with multiple asymptomatic hyperkeratotic lesions on both palms and soles of about 45-year duration, which was diagnosed as Brauer-Buschke-Fischer keratoderma and was associated with squamous cell carcinoma of chest wall and ethmoidal carcinoma. This case is being presented for its rarity and the association of Brauer-Buschke-Fischer keratoderma with two malignancies in a single individual.

Keywords: Brauer-Buschke-Fischer keratoderma, malignancy, squamous cell carcinoma, ethmoidal carcinoma

How to cite this article:
Vinod C S, Kishan Kumar Y H, Chawla S. Brauer-Buschke-Fischer keratoderma associated with two malignancies. Int J Health Allied Sci 2012;1:115-7

How to cite this URL:
Vinod C S, Kishan Kumar Y H, Chawla S. Brauer-Buschke-Fischer keratoderma associated with two malignancies. Int J Health Allied Sci [serial online] 2012 [cited 2024 Feb 24];1:115-7. Available from: https://www.ijhas.in/text.asp?2012/1/2/115/101715

  Introduction Top

Hereditary punctuate palmoplantar keratoderma is a rare autosomal dominantly inherited condition with variable penetrance. In 1910, this keratinization disorder was described by Buschke and Fischer as "keratoderma maculosa disseminata palmaris et plantaris." In 1912, Brauer demonstrated the hereditary nature of the disease with a similar presentation and named it as "keratoderma heriditarium dissipatum palmare et plantare." Later, this condition was named as Brauer-Buschke-Fischer keratoderma. [1]

  Case Report Top

A 65-year-old manual laborer presented with multiple asymptomatic hyperkeratotic lesions on both palms and soles of about 45-year duration. The lesions started over the lateral aspect of the palms when he was about 18 years of age and gradually over a period of few years, both palms and soles were affected with multiple punctate hard raised lesions. There was no history of itching, bleeding, exacerbation, or remission with climatic change. Patient was able to pick the lesions leaving small depressions but they used to reappear within a few weeks. Patient had undergone surgery and radiation for squamous cell carcinoma of anterior chest wall 5 years earlier. One month back, patient was diagnosed to have ethmoidal carcinoma and had undergone surgery. There was no history of smoking or occupational exposure to arsenic at any time and nobody in his locality had the same complaints. There was a strong family history with 7 members affected over 4 generations; none of these members were diagnosed to have any malignancy.

Cutaneous examination revealed multiple discrete and few coalescent punctuate, hyperkeratotic, crateriform papules and plaques of size varying from 3 to 15 mm over both palms and soles [Figure 1] and [Figure 2]. Nails showed thickening and longitudinal ridges of the nail plate.
Figure 1: Multiple discrete and coalescent punctuate, hyperkeratotic, crateriform papules and plaques of varying sizes over the palms

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Figure 2: Multiple discrete punctuate, hyperkeratotic, crateriform papules of varying sizes over the soles

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Complete hemogram and biochemical investigations were within normal limits. Skin biopsy revealed hyperkeratosis, hypergranulosis, acanthosis, and papillomatosis with dermal lymphocytic infiltrate [Figure 3].
Figure 3: HPE revealed hyperkeratosis, hypergranulosis, acanthosis, papillomatosis with dermal lymphocytic infiltrate (H and E, 100×)

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Brauer-Buschke-Fischer keratoderma, arsenical keratosis, porokeratosis punctata palmaris et plantaris, and acrokeratoelastoidosis lichenoides were the differentials considered. From the history, clinical findings of asymptomatic punctate hyperkeratotic papules over the palmoplantar surfaces and the histopathological findings showing absence of cornoid lamellae, diagnosis of Brauer-Buschke-Fischer Keratoderma was confirmed. As the patient was undergoing chemotherapy for ethmoidal carcinoma, no systemic treatment was started for him. His only son who was also suffering from the same condition was investigated in detail for any evidence of malignancy and was put on Tab. Acitretin 25 mg once daily as a treatment of punctate punctuate palmoplantar keratodermas (PPK); he showed significant improvement after 4 months of treatment.

  Discussion Top

The PPK are classified into acquired and hereditary forms. Acquired forms of punctuate keratodermas can be associated with arsenical keratosis, various malignant conditions like angiosarcoma of liver, bronchial adenocarcinoma. Acquired punctuate keratoderma of the palmar creases occur predominantly in Afro-Caribbeans, in association with an atopic diathesis. [1]

The hereditary forms of punctate PPK are classified into 3 types. Type 1 (Brauer-Buschke-Fischer keratoderma), Type 2 (porokeratosis punctata palmaris et plantaris), and Type 3 (acrokeratoelastoidosis lichenoides). Type 1 is an autosomal dominant condition with onset in the later teens, twenties, or later. Type 2 presents with numerous tiny keratotic spines which resemble the spines of a music box. Histologically, spines correspond to columnar parakeratosis that resemble cornoid lamellae. Type 3 presents with oval or polygonal crateriform papules on the borders of hands, feet, and wrist. [2] Unilateral linear punctate PPK also has been reported recently. [3]

Brauer-Buschke-Fischer keratoderma is a rare condition with a reported prevalence rate as 1.17/100 000 population. It presents with numerous hyperkeratotic papules that are irregularly distributed on the palms and soles. The papules may vary greatly in size and tend to coalesce on pressure points. The majority of cases are diagnosed incidentally, since most patients are asymptomatic. Sometimes, walking may be painful due to larger hyperkeratotic plaques over the pressure points. [4]

Although to date no specific genes have been identified for punctate PPK, Zhang et al. identified the chromosome 8q24.13-8q24.21 as the responsible gene in two Chinese families. [5] Steffen Emmert et al. have studied 47 patients in 14 families with Brauer-Buschke-Fischer keratoderma and calculated a frequency of 45.4% affected children from known-affected individuals. [6] Males are predominantly affected with this condition, with a relative sparing of females. Its association with atopy and nail changes have been reported. [7] The common nail changes reported are half moon distal nail plate dystrophy, subungual hyperkeratosis, longitudinal ridges, thickened nail plate, etc. [8]

The association of Brauer-Buschke-Fischer keratoderma and various malignancies has been reported. [9] Punctate PPK and malignancy in a family over four generations was extensively studied by H P Stevens et al. In this study, 23% of patients with PPK developed malignancies, whereas only 2% of unaffected individuals developed malignancy. It is not a chance occurrence and could be explained as both the keratoderma and malignancies may be caused by a defect in a gene which has a role in epithelial development and regulation of keratin expression. [10]

Association of Brauer-Buschke-Fischer keratoderma with two different malignancies is a rarity, which is not reported in literature to the best of our knowledge.

  References Top

1.Stevens HP, David PK, Stephen P, Bryant, Timothy BD, Spurr NK, et al. Linkage of an American pedigree with palmoplantar keratoderma and malignancy (Palmoplantar ectodermal dysplasia type III) to 17q 24: Literature survey and proposed updated classifi cation of the keratodermas. Arch Dermatol 1996;32:640-51.  Back to cited text no. 1
2.Arash KA. Type 1 hereditary punctate keratoderma. Dermatol Online J 2003;9:38.  Back to cited text no. 2
3.Sharma S, Barman KD, Garg VK, Jain S. Unilateral linear punctate palmoplantar keratoderma. Indian J Dermatol Venereol Leprol 2012;78:85-8.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.Zentsov A, Veitschegger M. Keratodermas. Int J Dermatol 1993;32:493-8.  Back to cited text no. 4
5.Zhang XJ, Li M, Gao TW, He PP, Wei SC, Liu JB, et al. Identification of a locus for punctate palmoplantar keratoderma at chromosome 8q 24.13-8q 24.21. J Invest Dermatol 2004;122:1121-5.  Back to cited text no. 5
6.Emmert S, Wolfgang KG, Hans CH. 47 patients in 14 families with the rare genodermatosis keratosis punctata palmoplantaris Buschke-Fischer-Brauer. Eur J Dermatol 2003;13:16-20.   Back to cited text no. 6
7.Gupta R, Mehta S, Pandhi D, Singal A. Hereditary punctate palmoplantar keratoderma (PPK) (Brauer - Buschke - Fischer syndrome). J Dermatol 2004;31:398-402.  Back to cited text no. 7
8.Mittal RR, Jha A. Hereditary punctate palmoplantar keratoderma. A clinical study. Indian J Dermatol Venereol Leprol 2003;69:90-1.  Back to cited text no. 8
[PUBMED]  Medknow Journal  
9.Bennion SD, Patterson JW. Keratosis punctate palmaris et plantaris and adenocarcinoma of colon. J Am Acad Dermatol 1984;10:587-91.  Back to cited text no. 9
10.Stevens HP, Kelsell DP, Leigh IM, Ostlere LS, MacDermot KD, Rustin MH. Punctate plamoplantar keratoderma and malignancy in a 4 generation family. Br J Dermatol 1996;134:720-6.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]

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