Acrokeratoelastoidosis in hansen's disease: A new insight

Vrutika Hasmukh Shah; Kinjal D Rambhia; Jayesh I Mukhi; Rajesh P Singh

doi:10.4103/ijdpdd.ijdpdd_6_20

CASE REPORT

Year : 2020 | Volume : 7 | Issue : 1 | Page : 30-32

Acrokeratoelastoidosis in hansen's disease: A new insight

Vrutika Hasmukh Shah¹, Kinjal D Rambhia², Jayesh I Mukhi³, Rajesh P Singh³
¹ Department of Dermatology, Venereology and Leprosy, Seth G. S. Medical College and KEM Hospital, Mumbai, Maharashtra, India
² Department of Dermatology, Venereology and Leprosy, H B T M C and Dr. R. N. Cooper Hospital, Mumbai, Maharashtra, India
³ Department of Dermatology, Venereology and Leprosy, Government Medical College, Nagpur, Maharashtra, India

Date of Submission	14-Jan-2020
Date of Decision	21-Mar-2020
Date of Acceptance	21-Mar-2020
Date of Web Publication	02-Jun-2020

Correspondence Address:
Kinjal D Rambhia
Department of Dermatology, Venereology and Leprosy, H B T M C and Dr. R. N. Cooper Hospital, Juhu, Mumbai, Maharashtra
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ijdpdd.ijdpdd_6_20

Abstract

Acrokeratoelastoidosis (AKE), first described by Costa in 1953, is characterized by small, firm, keratotic papules with central umbilication along the borders of the hands and feet. It is a type of palmoplantar marginal papular keratoderma and needs to be differentiated from other marginal papular keratodermas. AKE can be inherited or sporadic, with predisposing factors being excessive sun exposure, hyperhidrosis, and repetitive trauma. We here describe a case of AKE in a leprosy patient with probably secondary to repetitive chronic trauma due to loss of sensory function.

Keywords: Acquired marginal papular keratoderma, acrokeratoelastoidosis, leprosy, trauma

How to cite this article:
Shah VH, Rambhia KD, Mukhi JI, Singh RP. Acrokeratoelastoidosis in hansen's disease: A new insight. Indian J Dermatopathol Diagn Dermatol 2020;7:30-2

How to cite this URL:
Shah VH, Rambhia KD, Mukhi JI, Singh RP. Acrokeratoelastoidosis in hansen's disease: A new insight. Indian J Dermatopathol Diagn Dermatol [serial online] 2020 [cited 2021 Oct 24];7:30-2. Available from: https://www.ijdpdd.com/text.asp?2020/7/1/30/285806

Introduction

Hyperkeratotic papules along the margins of the hands and feet include various conditions, such as acrokeratoelastoidosis (AKE), focal acral hyperkeratosis (FAH), hereditary papulotranslucent acrokeratoderma, mosaic acral keratosis, degenerative collagenous plaques of the hands, keratoelastoidosis marginalis, and digital papular calcinosis.^[1],[2] Rongioletti et al. have encompassed these under the umbrella term”marginal papular acrokeratoderma (MPA),” and these diseases are distinguished based on hereditary presence and pathological findings.^[3] AKE is described as small, firm, umbilicated skin-colored and keratotic papules along the borders of the hands and feet by Costa and histologically is characterized classically by fragmentation of elastic fibers or elastorrhexis.^[4]

Case Report

A 73-year-old man, a known case of leprosy, farmer by occupation, presented with asymptomatic roughness of the palms for 30 years. He was released from treatment for leprosy 40 years back. A history of repetitive chronic trauma to the hands was present. A history of excessive sun exposure and hyperhidrosis was absent. He had no other symptoms, with other medical history being noncontributory. There was no history of similar lesions among the family members. On cutaneous examination, multiple skin-colored, depressed, umbilicated and few crateriform, hyperkeratotic papules with a glossy and translucent surface were seen on the lateral border of the index finger and medial border of the thumb bilaterally in a symmetrical distribution [Figure 1]. A single nonhealing ulcer was present on the right great toe plantar aspect. His nails, hair, teeth, and mucous membranes were normal. Hot and cold temperature sensations were decreased over bilateral hands. Histopathological examination of the papule from the lateral border of the right index finger revealed orthokeratotic hyperkeratosis, acanthosis, and thickened, curved basophilic elastic fibers in the papillary and reticular dermis [Figure 2] and [Figure 3]. Verhoeff–Van Gieson stain revealed thick, curved, coarse, fragmented elastic fibers (elastorrhexis) in the dermis [Figure 4]. On clinicopathological correlation, a diagnosis of AKE in leprosy was made. The patient was started on 12% salicylic acid and urea combination ointment and was counseled regarding the nature of condition and various treatment modalities, but the patient was lost to follow-up.

Figure 1: Multiple skin-colored, depressed, umbilicated crateriform, hyperkeratotic papules over the bilateral medial aspect of index fingers

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Figure 2: Epidermis showing orthokeratosis, hyperkeratosis, and hypergranulosis with basophilic, thick curled elastic fibers and dilated capillaries in the reticular dermis (yellow arrow) (H and E, ×10)

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Figure 3: Thickened, curved basophilic elastic fibers in the dermis (H and E, ×40) (yellow arrow)

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Figure 4: Thick, curved, coarse, fragmented elastic fibers in the dermis (Verhoeff–Van Gieson stain, ×40)

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Discussion

AKE, a rare disease, was first described by Costa in 1953.^[2] AKE can be inherited or sporadic, and most cases of AKEs reported so far have occurred before the second to third decades of life.^[5] The inherited type, i.e., autosomal dominant form, manifests in children, adolescents, or young adults.^[4] The sporadic form manifests later in life, with predisposing factors being excessive sun exposure, hyperhidrosis, or repeated trauma, which may be present.^[5] Clinically, it is characterized by small, firm, grouped, clustered symmetrically located keratotic papules with central umbilication and glossy translucent surface along the borders of palms and soles. It can extend to the dorsal and palmar surfaces in some cases. In few cases, the papules may coalesce to form plaques.^[4] [Table 1] distinguishes between various MPA.^[3]

Table 1: Differential diagnosis of marginal papular acrokeratodermas

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Histologically, in AKE, epidermal changes seen are orthokeratotic hyperkeratosis, acanthosis, and hypergranulosis with or without epidermal hyperplasia.^[6] Dermal changes seen are reduced number of elastic fibers which are thickened, curved, fragmented, and appear as basophilic fibers in the papillary and reticular dermis.^[6] Verhoeff–Van Gieson stain, acid orcein stain, or Weigert stain may be used to demonstrate the changes in the elastic fibers. Histopathology helps to differentiate this rare genodermatosis from other clinically similar marginal papular keratodermas. The reticular dermis in degenerative collagenous plaques of the hand shows a dense deposit of collagen fibers.^[7] FAH is characterized by the absence of either of the above dermal changes.^[8] Fibroblasts in the dermis are seen to contain abnormal dense granules in or near their plasma membrane which suggests that the pathology in AKE may be that of abnormality in the secretion of elastic fibers rather than that of fiber degradation.^[9]

The disease which is described to be localized to the acral parts of the body can be seen in apparently normal skin in a patient with AKE, suggesting that the disorder could be regarded as a generalized defect of elastic tissue that is somehow limited in location.^[10] Chronic and repeated bouts of unnoticed trauma which was much more common over the acral parts of the body due to reduced sensations must have resulted in the predominance of clinical expression of the disorder over the palms. However, there is no establishment of a direct causal relationship between AKE and chronic trauma.

Treatment for AKE is mainly cosmetic and is deferred as the lesions are asymptomatic. Counseling of the patient regarding the benign nature of this lesion and limited treatment modalities is essential. Topical treatment includes keratolytics such as salicylic acid, tretinoin, corticosteroids, calcipotriol, and urea, all of which provide temporary relief with recurrences on stopping application.^[11] Topical treatments do not improve dermal elastolytic change and have to be continued regularly.^[11] Systemic treatment with prednisolone, dapsone, methotrexate, and acitretin has also been tried.^[4] Er: YAG laser surgery has been tried but with limited clinical improvement.^[12]

Conclusion

Thus, in leprosy, palms and soles which are often looked for trophic ulcer should also be looked for AKE lesions, which suggest a sign of repetitive chronic trauma. We report this case for its rare occurrence in leprosy.

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.

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