mller made one of the first documented observations of pachyonychia congenita in 1904 followed by reports published in 1905 by wilson and in 1906 by jadassohn and lewandowsky . two main types of pachyonychia congenita are recognized : ( 1 ) pachyonychia congenita type - 1 ( jadassohn - lewandowsky type ) and , ( 2 ) pachyonychia congenita type - 2 ( jackson - lawler type ) . pc type 2 is distinguished by the development of natal teeth , widespread steatocystomas , and occasionally pili torti . a third variant , pachyonychia congenita tarda has also been described and is characterized by a later onset that ranges from late childhood to middle age . the patient was a 22 yr female , born of a consanguineous marriage who came to our opd with chief complaint of hypertrophic brittle nails since childhood . her finger and toe nails both were affected and the nail beds were hypertrophied with [ figures 1 and 2 ] discoloration and longitudinal fissuring . she also had palmoplantar keratoderma [ figure 3 ] since childhood with thick plaques , hypertrophic keratosis , deep fissuring with oozing , crusting and bleeding points [ figures 4 and 5 ] . brittle , hypertrophic nails , longitudinal fissuring , and hypertrophic nail beds brittle , hypertrophic nails , longitudinal fissuring , and hypertrophic nail beds hypertrophic keratinization over soles . painful hypertrophic keratinization at finger tips a closeup view of palmar keratoderma koh microscopy and culture of nail clippings was done to rule out candidal or dermatophytic onychomycosis . on skin biopsy , paring of nails was done to reduce the thickness of nails and to provide symptomatic relief . after one month of therapy , a reduction was seen in the thickness of hyperkeratotic lesions of palms and soles and desquamation had started in these lesions . acanthosis , parakeratosis , and ballooning of epithelial cells [ h & e , 10 ] pachyonychia congenita ( pc ) is a rare type of ppkd , which is characterized by subungual hyperkeratosis of the distal nails and focal palmoplantar hyperkeratosis . it is classified as pachyonychia congenita type 1 ( jadassohn  lewandowsky ) and pachyonychia congenital type 2(jackson  lawler ) syndromes . acanthosis is present with patchy hypergranulosis , in which large keratohyalin granules are present without gross epidermolysis . in pc-2 , the cysts may be keratinous epidermoid cysts , eruptive vellus hair cysts or true steatocysts . it is characterized by hypertrophic nail dystrophy ( pachyonychia ) which is a characteristic feature in 90 - 98% cases , present at birth or developing within the first few months of life . other features include symmetric focal palmoplantar keratoderma , oral leukokeratosis ( not premalignant ) , palmoplantar hyperhidrosis , follicular keratoses , and laryngeal involvement . pc type 2 is characterized by almost all clinical features of pc type 1 but less severe keratodermas along with natal or prenatal teeth , numerous steatocystomas , although a variety of cysts , including epidermal inclusion cysts , pilosebaceous cysts , and vellus hair cysts , may be seen . in other clinical variants involvement of the nails alone has been reported . emollients and keratolytics ( e.g. salicylic acid , corn plasters , benzoic acid , propylene glycol ) may help mild keratodermas ; a comfortable footwear may reduce blistering and callosities . physical debridement of nails can also be done to reduce the thickness of nails . antifungal creams and systemic antifungal agents can be used to provide some relief but may need repeated intermittent courses . acitretin 2535 mg / day may be effective but causes increased tenderness in the lesions . a prolonged course of retinoids may produce a degree of flattening of the nails and other complications such as periosteal hyperostosis , increased sensitivity and fragility of the underlying epidermis , and this limits their usefulness . treatment of hyperhidrosis ( aluminum chloride lotion , and iontophoresis ) may reduce blistering and the use of botulinum toxin under regional analgesia may provide pain relief for several months . the mechanisms of manipulating gene expression by small molecules or gene therapy has become possible . genetic therapies for such diseases must either suppress the production of the toxic proteins or correct the genetic defect in the chromosome . utilizing the technological by - products of the human genome project , such as rna interference ( rnai ) and quantitative rt - pcr ( qrt - pcr ) , physicians and scientists have collaborated to create a candidate sirna therapeutic that selectively inhibits a mutant allele of krt6a , the most commonly affected pc keratin . in vitro investigation of this sirna
pachyonychia congenita ( pc ) is a rare genodermatosis with only 450 cases reported since 1906 . 
 it is of two types , type i due to mutation in genes 6a and 16 , and 6b and 17 in type ii with an autosomal dominant inheritance in both types . a 22 yr old female patient presented in our opd with hypertrophy of finger and toe nails , palmoplantar keratoderma , oral punctuate leukokeratosis , hyperhidrosis in palms and soles with maceration and malodour since childhood . 
 she had a positive family history with father and grandfather affected but less severely . 
 microscopy and culture of nail clippings and scrapping were done to rule out fungal infection . on biopsy 
 acanthotic epidermis , parakeratosis , orthokeratosis were seen . 
 no evidence of any associated malignancy was found after thorough workup . 
 she was diagnosed as pc type 1 . 
 she was put on topical steroids and orally on acetretin 25 mg od . 
 paring of the nails was done too reduce the thickness of nails & to provide symptomatic relief . 
 she was on a regular treatment for 3 - 4 months and showed some improvement in the form of reduced palmoplantar hyperkeratosis and reduced oral punctate keratosis but was later lost on followup . 
 she showed no adverse effect to therapy during this period . 
 this case is being reported because of its rarity .