|Year : 2017 | Volume
| Issue : 3 | Page : 183-186
A study on palmoplantar keratodermas in childhood in a district hospital
Department of Dermatology, Punjab Health Systems Corporation, Punjab, India
|Date of Web Publication||7-Jun-2017|
House No. 626, Phase II, Urban Estate, Dugri Road, Ludhiana, Punjab
Source of Support: None, Conflict of Interest: None
Introduction: Palmoplantar keratodermas (PPKs) are a group of disorders in which there is marked thickening of the skin of palms and soles. The keratodermas can be diffuse, focal, or punctuate type.
Aims: The aim of this study was to study various types of PPKs, both hereditary and acquired in children up to 17 years of age.
Methods: A randomized controlled prospective study of twenty patients of PPK was taken up for the study. A detailed history, general physical examination, cutaneous examination with special examination of the oral mucosa, teeth, and nails along with routine investigations were done in all the patients.
Results: Most common causes of PPK were secondary to psoriasis and ichthyosis (25% each), followed by 15% cases secondary to pityriasis rubra pilaris. PPK due to pachyonychia congenita was seen in 5% patients and secondary to erythrokeratodermia variabilis in 5% patients. Monogenic PPKs including Vorners syndrome and Unna-Thost PPK were seen in 5% patients each and focal PPK seen in 15% patients.
Conclusion: It is important to note the age of onset of the keratodermas, severity of disease process, and the histopathological findings before reaching a diagnosis of PPKs.
Keywords: Acquired, childhood, hereditary, palmoplantar keratodermas, palms, soles
|How to cite this article:|
Puri N. A study on palmoplantar keratodermas in childhood in a district hospital. Indian J Paediatr Dermatol 2017;18:183-6
|How to cite this URL:|
Puri N. A study on palmoplantar keratodermas in childhood in a district hospital. Indian J Paediatr Dermatol [serial online] 2017 [cited 2017 Jun 23];18:183-6. Available from: http://www.ijpd.in/text.asp?2017/18/3/183/206091
| Introduction|| |
Palmoplantar keratodermas (PPKs) are a group of disorders in which there is gross thickening or hyperkeratosis of palmoplantar skin., Keratodermas can be both hereditary or acquired. Classification of keratodermas is as follows:
- Diffuse keratodermas affect most of the palms and soles
- Focal keratodermas mainly affect pressure areas
- Punctate-type keratodermas result in tiny bumps on the palms and soles
- Most often the abnormal skin involves only the palms and soles (nontransgradient) but sometimes it extends on to the top of the hands and feet as well (transgradient).
Hereditary PPKs are sometime symptoms of some generalized disorders such as ichthyosis or erythrokeratodermias. The keratodermas can be diffuse, focal, or punctuate type. It is important to see the presence or absence of associated features and pattern of inheritance., Furthermore, in some cases, the clinical features are not expressed in early childhood. One should follow the integrated approach so that a correct diagnosis can be reached at.
To study various types of PPKs, both hereditary and acquired in children up to 17 years of age.
| Methods|| |
A randomized controlled prospective study of twenty patients of PPK was taken up for the study in a district hospital of North India. Prior permission of hospital ethical committee was taken for the study. Written informed consent was taken from the parents of the patients for the study. A detailed history, general physical examination, cutaneous examination with special examination of the oral mucosa, teeth, and nails along with routine investigations were done in all the patients. History of hyperhidrosis and blistering was taken. Important points taken in the history were age of onset of keratoderma, family history of keratoderma, seasonal variation, and any other anomaly of hair and nails were noted. Other important points which were taken up in the history included deafness, muscle weakness, teeth loss, photosensitivity, and family history of PPK. History of consanguineous marriages was also taken. Specialized investigations including skin biopsy, X-rays of the digits, and audiometry were done wherever required.
| Results|| |
The data were collected, tabulated, and the results were analyzed statistically [Table 1],[Table 2],[Table 3],[Table 4].
| Discussion|| |
Regarding the age distribution of the patients, it was seen that maximum number (50%) of patients were between 10 and 17 years of age followed by 30% of patients were between 6 and 10 years of age and 20% patients were between 0 and 5 years of age. Males outnumbered females and male:female was 3:1. Family history of PPK was positive in 14 (70%) patients. Out of these 14 children, 7 patients had PPK either in mother or father and rest of the 7 patients had siblings with PPK. Most common causes of PPK were secondary to psoriasis [Figure 1] and ichthyosis (25% each), followed by 15% cases secondary to pityriasis rubra pilaris. PPK due to pachyonychia congenita [Figure 2] was seen in 5% patients and secondary to erythrokeratodermia variabilis in 5% patients. Monogenic PPKs including Vorners syndrome [Figure 3] and Unna-Thost PPK [Figure 4] were seen in 5% patients each and focal PPK [Figure 5] and [Figure 6] seen in 15% patients. Nail changes were seen in 60% patients, leukokeratosis and hyperhidrosis were seen in 1% patients each. Nail changes most commonly seen were subungual hyperkeratosis, pitting [Figure 7], nail plate thickening, and rough and brittle nails were seen in 25% patients each.
|Figure 1: Palmoplantar keratoderma due to plantar psoriasis in an 8-year-old male child|
Click here to view
|Figure 2: Thickened discolored nails in an 8-year-old male child with pachyonychia congenita|
Click here to view
Keratoderma can be both hereditary and acquired. Acquired keratoderma is a type of keratoderma which is nonhereditary and nonfrictional hyperkeratosis of palms and soles involving more than 50% of the acral areas. Patients with acquired PPKs need to be evaluated carefully to find out the cause. Various causes of acquired PPKs in childhood include: dermatoses-related (psoriasis, ichthyosis), PPK's associated with malnutrition, infections or systemic disease and idiopathic.
There are three types of PPKs including diffuse, focal, and punctuate. In diffuse PPK, there is even, thick, symmetric hyperkeratosis over the whole of the palms and soles, which appears at birth or few months of life. Diffuse PPK is autosomal dominant which is confined to palms and soles. Out of the diffuse PPK, two main variants need to be discussed including:
- Epidermolytic diffuse PPK: Also known as Vorners PPK with symmetric thickening of palms and soles with dirty snake-like skin appearance ,,,
- Nonepidermolytic PPK: Also known as Unna-Thost PPK with well-demarcated waxy keratoderma. Clinically, it is very difficult to differentiate between the two types and both can be differentiated only histologically.
In focal PPK, keratin masses develop at the site of frequent friction, especially feet. It is seen commonly in pachyonychia congenita with prominent nail involvement. In punctuate PPK, multiple tiny punctuate keratosis over the entire palmoplantar surface starting from the lateral edge of the digits is seen. It is not essential that all the clinical features are expressed in early childhood. It is important to note the age of onset of the keratodermas, severity of disease process, and the histopathological findings. However, in most of the cases, the histopathological findings are rarely specific. Definitive diagnosis can be made only by demonstration of mutations in specific genes.
Evaluation of patients with PPK's is very important. The evaluation includes proper history, complete physical, and cutaneous examination. If no specific findings are seen, then laboratory and radiological investigations should be done. Regarding the treatment of these patients with PPK, conservative treatment options are given including topical keratolytics, retinoids, and corticosteroids. In recalcitrant cases, oral acitretin or etretinate can be given.
| Conclusions|| |
It is important to recognize the patients with the type of PPK and every PPK needs to be thoroughly investigated for any other systemic abnormalities and also family history of PPK should be taken.
Financial Support and Sponsorship
Conflicts of Interest
There are no conflicts of interest.
| References|| |
Stevens HP, Leigh IM, Fitzpatrick TB, Freeberg IM, Eisen AZ, Wolff K, et al
. Keratoderma of palms and soles. In: Dermatology in General Medicine. 5th
ed. New York: McGraw-Hill; 1999. p. 603-13.
Kimyai-Asadi A, Kotcher LB, Jih MH. The molecular basis of hereditary palmoplantar keratodermas. J Am Acad Dermatol 2002;47:327-43.
Lucker GP, Van de Kerkhof PC, Steijlen PM. The hereditary palmoplantar keratoses: An updated review and classification. Br J Dermatol 1994;131:1-14.
Sybert VP, Dale BA, Holbrook KA. Palmar-plantar keratoderma. A clinical, ultrastructural, and biochemical study. J Am Acad Dermatol 1988;18(1 Pt 1):75-86.
Patel S, Zirwas M, English JC 3rd
. Acquired palmoplantar keratoderma. Am J Clin Dermatol 2007;8:1-11.
Griffiths WA, Leigh IM, Marks R. Disorders of keratinization. In: Champion RH, Burton JL, Ebling FJ, editors. Textbook of Dermatology. Oxford, England: Blackwell Scientific Publications; 1992. p. 1325-90.
Yoshiike T, Hattori M, Ogawa H. Father and daughter cases of Vorner type hereditary palmoplantar keratosis: Biochemical analysis of stratum corneum and effect of oral retinoid therapy. Jpn J Dermatol 1982;92:751-6.
Hamm H, Happle R, Butterfass T, Traupe H. Epidermolytic palmoplantar keratoderma of Vörner: Is it the most frequent type of hereditary palmoplantar keratoderma? Dermatologica 1988;177:138-45.
Requena L, Schoendorff C, Sanchez Yus E. Hereditary epidermolytic palmo-plantar keratoderma (Vörner type) – Report of a family and review of the literature. Clin Exp Dermatol 1991;16:383-8.
Kanitakis J, Tsoitis G, Kanitakis C. Hereditary epidermolytic palmoplantar keratoderma (Vörner type). Report of a familial case and review of the literature. J Am Acad Dermatol 1987;17:414-22.
Kansky A, Arzensek J, Rode M, Strojan J. Keratodermia palmoplantaris of the Unna-Thost type in Slovenia. Acta Derm Venereol 1984;64:140-3.
Martinez-Mir A, Zlotogorski A, Londono D, Gordon D, Grunn A, Uribe E, et al.
Identification of a locus for type I punctate palmoplantar keratoderma on chromosome 15q22-q24. J Med Genet 2003;40:872-8.
Ackerman AB. Histopathologic concept of epidermolytic hyperkeratosis. Arch Dermatol 1970;102:253-9.
Reis A, Küster W, Eckardt R, Sperling K. Mapping of a gene for epidermolytic palmoplantar keratoderma to the region of the acidic keratin gene cluster at 17q12-q21. Hum Genet 1992;90:113-6.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
[Table 1], [Table 2], [Table 3], [Table 4]