Eeshaan Ranjan1, Sunmeet Sandhu2, Ajay S Sharma3
1 From the Department of Dermatology, Military Hospital, Jammu, Jammu and Kashmir, India
2 Department of Dermatology, 7 Air Force Hospital, Kanpur, Uttar Pradesh, India
3 Department of Pathology, Military Hospital, Jammu, Jammu and Kashmir, India

Date of Web Publication31-Aug-2023

Correspondence Address:
Eeshaan Ranjan
Department of Dermatology, Military Hospital, Jammu, Jammu and Kashmir
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ijd.ijd_57_23

How to cite this article:
Ranjan E, Sandhu S, Sharma AS. Acrokeratosis verruciformis of hopf: A series of 3 cases with clinical, histopathological and dermoscopic features. Indian J Dermatol 2023;68:489
How to cite this URL:
Ranjan E, Sandhu S, Sharma AS. Acrokeratosis verruciformis of hopf: A series of 3 cases with clinical, histopathological and dermoscopic features. Indian J Dermatol [serial online] 2023 [cited 2023 Aug 31];68:489. Available from: https://www.e-ijd.org/text.asp?2023/68/4/489/384856

Sir,

Acrokeratosis verruciformis of Hopf (AKV) is a keratinization disorder described by Hopf in 1931.[1] It is characterised by keratotic papules commonly observed on the dorsum of the hands and feet.[2] Clinical differential diagnoses include other flat-topped skin lesions like epidermodysplasia verruciformis, seborrhoeic keratosis, verruca plana, lichen planus and superficial actinic porokeratosis. The diagnosis is usually confirmed by histopathology. Dermoscopy may serve as a useful, non-invasive tool to diagnose and avoid the need for a skin biopsy. We present three cases of AKV with clinical, histopathological and characteristic dermoscopic features that differentiate it from its differential diagnosis.

The first patient was a 21-year-old male who had presented with multiple, raised, skin-coloured lesions over the dorsum of both hands since childhood. Cutaneous examination revealed multiple, skin-coloured to hyperpigmented, flat-topped, 2–5 mm-sized keratotic papules present over the dorsum and lateral aspect of both hands [Figure 1]a. The second patient was a 25-year-old female presenting with multiple, hyperpigmented, keratotic papules over the dorsum of both hands since eight years of age [Figure 1]b. The third patient was an 18-year-old male who had presented with multiple, hyperpigmented, keratotic papules over the dorsal aspect of the proximal and distal interphalangeal joints of both hands for the last 6 years [Figure 1]c. Dermoscopy (DermLite DL4, polarised, 10 ×) of cutaneous lesions in all these three cases showed white, homogenous areas with a central white scale and brown pigment in a cobblestone appearance [Figure 1]d, [Figure 1]e, [Figure 1]f. Histopathological examination in all cases revealed orthokeratosis, hypergranulosis, acanthosis, irregular elongation of rete ridges and a mild perivascular lymphocytic infiltrate consistent with AKV [Figure 2].

Figure 1: (a–c) Clinical pictures showing multiple, flat-topped, keratotic papules present over the dorsum of the hand. (d-f) Dermoscopy (DermLite DL4, polarised, 10 ×) showing white homogenous structureless areas (red circle) with a few of these with a central white scale (yellow circle) and brown coloured pigment in a cobblestone appearance (blue circle)

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Figure 2: Histopathology (H&E) (40X) showing orthokeratosis, hypergranulosis, acanthosis, irregular elongation of rete ridges and mild perivascular lymphocytic infiltrate

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AKV is a genodermatosis with autosomal dominant inheritance caused by a mutation in the ATP2A2 gene located on chromosome 12q24. This gene encodes the sarcoplasmic reticulum Ca2+ ATPase2 pump, which has a role in signalling pathways regulating cell-to-cell adhesion and differentiation of the epidermis.[1] It has a chronic course and does not resolve spontaneously. Lesions usually start in early childhood, though the late onset of lesions has also been reported.[3] Patients present with keratotic papules over the dorsum of hands (mainly over the proximal and distal interphalangeal joints) and feet, and less frequently over arms, legs, knees and elbows.[2] The diagnosis is confirmed by histopathology showing hyperkeratosis, papillomatosis with a 'church-spire' appearance, acanthosis and hypergranulosis. Previous reports have shown white, homogenous areas and a cobblestone pattern on dermoscopy.[4] However, the lesional scaling seen in all three of our cases has not yet been seen in the literature. Dermoscopy can differentiate AKV from its close differentials. Lichen planus has characteristic wickham striae and radial capillaries.[5] Verruca plana on dermoscopy reveals dots or globular vessels.[6] Seborrhoeic keratosis has features of a brain-like appearance, comedo-like openings and multiple milia-like cysts.[6] Hence, dermoscopy may obviate the need to do a skin biopsy in AKV.

To conclude, we report a series of three cases of AKV along with their dermoscopic features. Lesional white scaling as a dermoscopic feature is being reported here. With the background of clinical features, dermoscopy helps to differentiate it from other clinically similar entities.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 

   References 
1.Dhitavat J, Macfarlane S, Dode L, Leslie N, Sakuntabhai A, MacSween R, et al. Acrokeratosis verruciformis of Hopf is caused by mutation in ATP2A2: Evidence that it is allelic to Darier's disease. J Invest Dermatol 2003;120:229-32.  
    2.DeFelice T, Robinson M, Patel R, Kamino H. Acrokeratosis verruciformis. Dermatol Online J 2012;18:12.  
    3.Faina V, Magri F, Sernicola A, Chello C, Gagliostro N, Grieco T. Late-onset non-familial acrokeratosis verruciformis of Hopf: A case report. Clin Ter 2019;170:e418-20.  
    4.Behera B, Prabhakaran N, Naveed S, Kumari R, Thappa DM, Gochhait D. Dermoscopy of acrokeratosis verruciformis of Hopf. J Am Acad Dermatol 2017;77:e33-5.  
    5.Litaiem N, Mansour Y, Jones M, Zeglaoui F. Dermoscopic signs of lichen planus. BMJ Case Rep 2016;2016:bcr2015213923. doi: 10.1136/bcr-2015-213923.  
    6.Kim WJ, Lee WK, Song M, Kim HS, Ko HC, Kim BS, et al. Clinical clues for differential diagnosis between verruca plana and verruca plana-like seborrheic keratosis. J Dermatol 2015;42:373-7.  
    
  [Figure 1], [Figure 2]