keratoelastoidosis marginalis of hands ( kemh ) , is an acquired , marginal , acrokeratoderma that predominantly affects the radial side of the index finger , first web space , and ulnar side of the thumb . prolonged uv exposure , heavy manual work , and repeated trauma to the hands seem to be the etiologic factors . a 75-year - old farmer presented with asymptomatic lesions over the web spaces of the thumb and index finger of both hands and raised lesions over his face since 15 years . he denied any history of preceding trauma , drug intake , or contact with any products causing irritation . cutaneous examination revealed hyperkeratotic and scaly papules arranged in a linear fashion along the radial margins the index fingers [ figure 1a and b ] . skin over the face was thick , leathery , and inelastic with deep wrinkles over the forehead and cheeks . provisional diagnosis of kemh with colloid milium with cutis rhomboidalis nuchae and favre  racouchot syndrome was made . ( a ) hyperkeratotic and scaly papules arranged in a linear fashion along the radial margins of right and left index fingers . ( c and d ) thick , leathery , and inelastic skin over the face with deep wrinkles over the forehead and cheeks . ( e ) deep furrows were seen over the nape of neck ( cutis rhomboidalis nuchae ) a 67-year - old farmer presented with asymptomatic rough , scaly lesions over the web space of the thumb and index fingers of both the hands since 10 years . he denied history of preceding trauma , drug intake , or contact with any products causing irritation . cutaneous examination revealed keratotic , crateriform papules over the radial aspect of both index fingers arranged in a linear fashion extending on to the first web space . similar lesions were also present on ulnar aspect of left little finger [ figure 2a  c ] . examination of the face revealed yellowish discoloration of the skin with leathery texture with multiple deep wrinkles over the face . back of the neck showed deep wrinkling and furrowing of the skin associated with a leathery texture , suggesting the chronic actinic damage of the skin . ( a ) keratotic crateriform papules along the radial aspect of the right index fingers arranged in a linear fashion extending onto the first finger web space . ( b ) keratotic crateriform papules along the radial aspect of the left index fingers arranged in a linear fashion . ( c ) similar lesions were also present on ulnar aspect of left little finger skin biopsy from keratotic plaques on the fingers of both the patients revealed compact hyperkeratosis and marked acanthosis . there was evidence of thickened , haphazardly oriented collagen bundles with basophilic degeneration [ figure 3a  c ] , and haphazardly oriented elastic fibers throughout the dermis with dilated blood vessels in the papillary dermis . van gieson stain showed thickened , degenerated , haphazardly arranged collagen bundles throughout the dermis and fragmented bundles of elastin [ figure 3d ] . on clinicopathological correlation , a final diagnosis of kemh was made . ( a ) h and e stain , 40 : compact hyperkeratosis , marked acanthosis with thickened haphazardly oriented collagen bundles . ( b ) h and e stain , 100 : marked basophilic degeneration of collagen and haphazardly oriented elastic fibers were throughout the dermis . ( c ) h and e stain , 400 : degenerated collagen and haphazardly oriented elastic fibers in the upper throughout the dermis . ( d ) verhoeff  van gieson stain , 40 : thickened , degenerated haphazardly arranged collagen bundles throughout the dermis and fragmented bundles of elastin a 75-year - old farmer presented with asymptomatic lesions over the web spaces of the thumb and index finger of both hands and raised lesions over his face since 15 years . he denied any history of preceding trauma , drug intake , or contact with any products causing irritation . cutaneous examination revealed hyperkeratotic and scaly papules arranged in a linear fashion along the radial margins the index fingers [ figure 1a and b ] . skin over the face was thick , leathery , and inelastic with deep wrinkles over the forehead and cheeks . provisional diagnosis of kemh with colloid milium with cutis rhomboidalis nuchae and favre  racouchot syndrome was made . ( a ) hyperkeratotic and scaly papules arranged in a linear fashion along the radial margins of right and left index fingers . ( c and d ) thick , leathery , and inelastic skin over the face with deep wrinkles over the forehead and cheeks . ( e ) deep furrows were seen over the nape of neck ( cutis rhomboidalis nuchae ) a 67-year - old farmer presented with asymptomatic rough , scaly lesions over the web space of the thumb and index fingers of both the hands since 10 years . he denied history of preceding trauma , drug intake , or contact with any products causing irritation . cutaneous examination revealed keratotic , crateriform papules over the radial aspect of both index fingers arranged in a linear fashion extending on to the first web space . similar lesions were also present on ulnar aspect of left little finger [ figure 2a  c ] . examination of the face revealed yellowish discoloration of the skin with leathery texture with multiple deep wrinkles over the face . back of the neck showed deep wrinkling and furrowing of the skin associated with a leathery texture , suggesting the chronic actinic damage of the skin . ( a ) keratotic crateriform papules along the radial aspect of the right index fingers arranged in a linear fashion extending onto the first finger web space . ( b ) keratotic crateriform papules along the radial aspect of the left index fingers arranged in a linear fashion . ( c ) similar lesions were also present on ulnar aspect of left little finger skin biopsy from keratotic plaques on the fingers of both the patients revealed compact hyperkeratosis and marked acanthosis . there was evidence of thickened , haphazardly oriented collagen bundles with basophilic degeneration [ figure 3a  c ] , and haphazardly oriented elastic fibers throughout the dermis with dilated blood vessels in the papillary dermis . van gieson stain showed thickened , degenerated , haphazardly arranged collagen bundles throughout the dermis and fragmented bundles of elastin [ figure 3d ] . on clinicopathological correlation , a final diagnosis of kemh was made . ( a ) h and e stain , 40 : compact hyperkeratosis , marked acanthosis with thickened haphazardly oriented collagen bundles . ( b ) h and e stain , 100 : marked basophilic degeneration of collagen and haphazardly oriented elastic fibers were throughout the dermis . ( c ) h and e stain , 400 : degenerated collagen and haphazardly oriented elastic fibers in the upper throughout the dermis . ( d ) verhoeff  van gieson stain , 40 : thickened , degenerated haphazardly arranged collagen bundles throughout the dermis and fragmented bundles of elastin marginal papular acrokeratoderma are classified by rongioletti et al . as hereditary and acquired forms . hereditary form with elastorrhexis ( fragmentation of elastic fibers ) is termed acrokeratoelastoides , whereas that without elastorrhexis is termed focal acral hyperkeratosis ( fah ) . hereditary forms also include punctuate palmoplantar keratoderma ( ppk - pt ) , hereditary papulotransluscent keratoderma and mosaic acral keratosis , whereas the acquired forms are kemh or degenerative collageneous plaques of the hand and digital papular calcinosis . it is also known as marginal papular acrokeratoderma , collagenous and elastotic marginal plaques of the hands , and digital papular calcific elastosis . burks et al . in 1960 reported the first five cases of this acquired disorder and termed it as degenerative collagenous plaques of the hands . chronic sun exposure and manual labor that involves chronic pressure or repeated trauma may be contributing factors . the hyperkeratosis may be due to repeated episodes of hypoxia of the affected areas resulting from papillary dermal capillary occlusion during periods of pressure , in conjunction with compression from the elastotic material that accumulates in the dermis , although this hypothesis has not yet been proved . it has been reported in people ranging in age from 42 to 78 years , but it remains a disorder mostly affecting the elderly population . clinically it presents as asymptomatic , white to yellowish , waxy scaly crateriform keratotic papules along margins of the hands . the radial side of the index finger , first web space , and ulnar side of the thumb are characteristically involved . solar elastosis in the dermis may be associated with thickened , fragmented , often calcified elastic fibers between distorted , degenerated collagen bundles . differential diagnosis of kemh includes acrokeratoelastoidosis of costa , focal acral hyperkeratosis , and punctuate palmoplantar keratoderma . differential diagnosis of keratoelastoidosis marginalis of the hands keratoelastoidosis marginalis is a progressive disease of a benign nature . these include laser therapy , high - potency topical corticosteroids , topical tazarotene and tretinoin , oral isotretinoin , and cryotherapy . retinoic acid ( 50 mg / d ) has reportedly shown some response , although the results are unsatisfactory . topical keratolytics such as salicylic acid and tretinoin provide temporary relief with recurrences on stopping application . apart from lesions of kemh , both our patients also had features of chronic actinic damage , in the form of waxy , yellowish , deeply wrinkled skin over the forehead , cheeks , nape of the neck ( cutis rhomboidalis nuchae ) along with senile comedones and nodular elastosis ( favre  racouchot syndrome ) . the occurrence of seborrheic keratosis , solar elastosis , favre  racouchot syndrome and cutis rhomboidalis nuchae are commonly noted signs of chronic actinic damage . however , the occurrence of kemh in occupations involving chronic sun exposure , such as farming , has rarely been reported . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed .
keratoelastoidosis marginalis of the hands ( kemh ) is a distinct solar elastotic dermatosis belonging to the group of marginal keratoderma . 
 it is characterized by linear plaques on the hand and calcified dermal elastotic masses predominantly affecting the radial side of the index finger , first web space , and ulnar side of the thumb . 
 it is predominantly seen in the middle - aged and elderly age group . 
 we report the occurrence of kemh in two farmers with other cutaneous features of chronic actinic damage .