Keratoelastoidosis marginalis of the hands: A report in two farmers.

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.

INTRODUCTION

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

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 1a and 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 1c and d]. Yellowish papules were seen near the outer canthus of the left eye. Deep furrows were seen over the nape of neck [Figure 1e]. 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)

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 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 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

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. Verhoeff–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.

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

DISCUSSION

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.[1345] 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.[26]

Table 1

Differential diagnosis of keratoelastoidosis marginalis of the hands

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.

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Conflicts of interest

There are no conflicts of interest.