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CASE REPORT
Year : 2016  |  Volume : 7  |  Issue : 3  |  Page : 195-197  

Keratoelastoidosis marginalis of the hands: A report in two farmers


Department of Dermatology, Venereology and Leprosy, Mahatma Gandhi Memorial (MGM) Medical College and Hospital, Navi Mumbai, Maharashtra, India

Date of Web Publication13-May-2016

Correspondence Address:
Swagata Tambe
19/558, Udyan Society, Nehru Nagar, Kurla, East Mumbai - 400 024, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.182364

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   Abstract 

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.

Keywords: Farmers, keratoelastoidosis marginalis, marginal keratoderma, sun exposure


How to cite this article:
Bhobe M, Tambe S, Jerajani H, Parulkar P. Keratoelastoidosis marginalis of the hands: A report in two farmers. Indian Dermatol Online J 2016;7:195-7

How to cite this URL:
Bhobe M, Tambe S, Jerajani H, Parulkar P. Keratoelastoidosis marginalis of the hands: A report in two farmers. Indian Dermatol Online J [serial online] 2016 [cited 2019 Oct 16];7:195-7. Available from: http://www.idoj.in/text.asp?2016/7/3/195/182364


   Introduction Top


Marginal keratoderma are broadly classified into acquired and familial forms. 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. It occurs in the middle-aged and elderly. Prolonged UV exposure, heavy manual work, and repeated trauma to the hands seem to be the etiologic factors.[1]


   Case Reports Top


Case 1

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. Skin lesions gradually increased in size and number over the years. He denied any history of preceding trauma, drug intake, or contact with any products causing irritation. There was no history of similar lesions among the family members. Cutaneous examination revealed hyperkeratotic and scaly papules arranged in a linear fashion along the radial margins the index fingers [Figure 1]a and [Figure 1]b. Skin over the face was thick, leathery, and inelastic with deep wrinkles over the forehead and cheeks. Multiple open comedones were present over the face [Figure 1]c and [Figure 1]d. Yellowish papules were seen near the outer canthus of the left eye. Deep furrows were seen over the nape of neck [Figure 1]e. Provisional diagnosis of KEMH with colloid milium with cutis rhomboidalis nuchae and Favre–Racouchot syndrome was made.
Figure 1: Cutaneous examination of case 1. (a) Hyperkeratotic and scaly papules arranged in a linear fashion along the radial margins of right and left index fingers. (b) Closeup view of skin lesions. (c and d) Thick, leathery, and inelastic skin over the face with deep wrinkles over the forehead and cheeks. Multiple open comedones over the cheeks, temples, and forehead. Yellowish papules near the outer canthus of left eye. (Favre–Racouchout syndrome). (e) Deep furrows were seen over the nape of neck (cutis rhomboidalis nuchae)

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Case 2

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. There was no history of similar lesions among the family members. 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 2]a,[Figure 2]b,[Figure 2]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 provisional diagnosis of KEMH with solar elastosis with cutis rhomboidalis nuchae was made.
Figure 2: Cutaneous examination of case 2. (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

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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 3]a,[Figure 3]b,[Figure 3]c, and haphazardly oriented elastic fibers throughout the dermis with dilated blood vessels in the papillary dermis. Verhoeff–Van Gieson stain showed thickened, degenerated, haphazardly arranged collagen bundles throughout the dermis and fragmented bundles of elastin [Figure 3]d. On clinicopathological correlation, a final diagnosis of KEMH was made.
Figure 3: Skin biopsy from keratotic plaques on the fingers in both the cases. (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

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   Discussion Top


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.[2]

KEMH belongs to a group of palmoplantar keratodermas of the marginal type. It is also known as marginal papular acrokeratoderma, collagenous and elastotic marginal plaques of the hands, and digital papular calcific elastosis.[1],[3],[4],[5] Burks et al. in 1960 reported the first five cases of this acquired disorder and termed it as degenerative collagenous plaques of the hands.[6]

Chronic sun exposure and manual labor that involves chronic pressure or repeated trauma may be contributing factors.[6] 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.[3]

It has been reported in people ranging in age from 42 to 78 years, but it remains a disorder mostly affecting the elderly population.[3] Men are more commonly affected than women in the white 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. The condition is slowly progressive and papules coalesce to form plaques.[6]

Histopathological examination reveals both epidermal and dermal changes. Epidermal changes include orthokeratosis and acanthosis. Solar elastosis in the dermis may be associated with thickened, fragmented, often calcified elastic fibers between distorted, degenerated collagen bundles. Inflammatory infiltrate may be present in the papillary dermis.

Differential diagnosis of KEMH includes acrokeratoelastoidosis of Costa, focal acral hyperkeratosis, and punctuate palmoplantar keratoderma. Differentiating features between these disorders are mentioned in [Table 1].[2],[6]
Table 1: Differential diagnosis of keratoelastoidosis marginalis of the hands

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Keratoelastoidosis marginalis is a progressive disease of a benign nature. Several treatment modalities have been tried with variable results. 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.[2] 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.

Declaration of patient consent

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 cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Mortimore RJ, Conrad RJ. Collagenous and elastotic marginal plaques of the hands. Australas J Dermatol 2001;42:211-3.  Back to cited text no. 1
    
2.
Rahbari H. Acrokeratoelastoidosis and keratoelastoidosis marginalis-any relation? J Am Acad Dermatol 1981;5:348-50.  Back to cited text no. 2
    
3.
Kocsard E. Keratoelastoidosis marginalis of the hands. Synonyms: Marginal keratoderma of palms; degenerative collagenous plaques of the hands. Dermatologica 1965;131:169-75.  Back to cited text no. 3
    
4.
Rahbari H. Collagenous and elastotic marginal plaques of the hands (CEMPH). J Cutan Pathol 1991;18:353-4.  Back to cited text no. 4
    
5.
Jordaan HF, Rossouw DJ. Digital papular calcific elastosis: A histopathological, histochemical and ultrastructural study of 20 patients. J Cutan Pathol 1990;17:358-70.  Back to cited text no. 5
    
6.
Burks JW, Wise LJ Jr, Clark WH Jr. Degenerative collagenous plaques of the hands. Arch Dermatol 1960;82:362-6.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]


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