Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obervation Letter
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
Case Report
2003:69:5;338-339
PMID: 17642932

Pachyonychia congenita-like nail changes treated successfully with a combination of vitamins A and E: A case report

BB Mahajan, A Pall, G Garg, R RR Gupta
 Dermatology Department, Government Medical College & Hospital, Faridkot - 151203, India

Correspondence Address:
B B Mahajan
Dermatology Department, 224 Medical Campus, Faridkot - 151203
India
How to cite this article:
Mahajan B B, Pall A, Garg G, Gupta R R. Pachyonychia congenita-like nail changes treated successfully with a combination of vitamins A and E: A case report. Indian J Dermatol Venereol Leprol 2003;69:338-339
Copyright: (C)2003 Indian Journal of Dermatology, Venereology, and Leprology

Abstract

A 20-year-old man presented with thickening, subungual hyperkeratosis and discoloration of all the nails of both hands and both great toes since birth. No other ectodermal abnormalities were found. Treatment with vitamins A and E in high doses along with a moisturizing ointment topically for one year resulted in complete resolution of all nail abnormalities.
Keywords: Pachyonychia congenita, Vitamin A, Vitamin E

Introduction

Pachyonychia congenita is a rare genodermatosis in which hypertrophy of the nails occurs, in some cases associated with nail bed and hyponychial hyperkeratosis.[1] Erythema of the nail bed appears in the first year or two of life followed by thickening of zones of the palms and soles.[2] We report a case of pachyonychia congenita-like nail changes that was successfully treated with oral vitamin A and E.

CASE REPORT

A 20-year-old man presented with thickening, subungual hyperkeratosis and discoloration of all the nails of both hands and both great toes since birth. These nails thickened progressively with a beak like longitudinal hypercurvature [Figure - 1]. On examination, the palms and soles, scalp and body hairs, and sweating were normal. There was a history of normal dentition. There was no history of similar nail abnormalities in the family. There were no mucosal lesions. Eye checkup, including fundus examination, was normal.

As the patient could not afford acitretin, the drug of choice, he was put on vitamin A 200,000 units and vitamin E 800 units daily along with topical application of a moisturizing ointment (Cotaryl, which primarily contains urea and lactic acid) twice daily. Both vitamins were given intermittently for three weeks following a rest period of one week. Fundus examination was done after every three months. The patient showed improvement after 3 months of therapy. Treatment was continued for a year to achieve complete resolution [Figure - 2]. No side effects were observed. No nail abnormalities were observed even one year after stopping treatment.

Discussion

Two major subtypes of pachyonychia congenita are recognized. In the Jadassohn-Lewandowsky form, pachyonychia occurs together with palmoplantar hyperkeratosis, hyperhidrosis and follicular keratosis, while the Jackson-Lawler syndrome is characterized by the appearance of multiple epidermal cysts (steatocystoma multiplex), and hair (viz. alopecia and hypertrichosis) and teeth abnormalities. Chang et al and Pryce et al have described patients with thickening of the nails in the absence of other abnormalities and claimed that this could represent a forme fruste of pachyonychia congenita.[3],[4] Our patient could represent such a forme fruste of pachyonychia congenita.

In milder forms of pachyonychia congenita, local emollients and keratolytics have been used with considerable improvement. In severe cases of pachyonychia congenita, retinoids given for long periods produce a reasonable degree of flattening of the nails.[5] Surgical excision has also been tried but recurrence is frequent. In our patient, intermittent vitamin A 200,000 units and vitamin E 800 units along with topical application of Cotaryl ointment resulted in complete resolution of all the nail abnormalities. This therapy is both cost effective and well tolerated without any side effects.

References
1.
Feinstein A, Friedman J, Schwach-Millet M. Pachyonychia congenita. J Am Acad Dermatol 1988;19:705-1.
[Google Scholar]
2.
Su WP, Chun SI, Hammond DE, et al. Pachyonychia congenita: a clinical study of 12 cases and review of the literature. Pediatr Dermatol 1990;7:33-8.
[Google Scholar]
3.
Chang A, Lucker GPH, Van De Kerkhof PCM, et al. Pachyonychia congenita in the absence of other syndrome abnormalities. J Am Acad Dermatol 1994;30:1017-8.
[Google Scholar]
4.
Pryce DW, Verbov JL. A family with pachyonychia congenita affecting the nails only. Clin Exp Dermatol 1994;19:521-2.
[Google Scholar]
5.
Hoting E, Wassilew SW. Systemic retinoid therapy with etretinate in pachyonychia congenita. Hautarzt 1985;36:526-8.
[Google Scholar]
Show Sections