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Year : 2022  |  Volume : 67  |  Issue : 6  |  Page : 792-794
Acrokeratosis verruciformis of hopf in an adult – A rare case report with review of literature

1 From the Department of Pathology, AIIMS, Mangalagiri, Andhra Pradesh, India
2 Department of Dermatology, AIIMS, Mangalagiri, Andhra Pradesh, India
3 Department of Pathology and Lab Medicine, AIIMS, Kalyani, West Bengal, India

Date of Web Publication23-Feb-2023

Correspondence Address:
Tummidi Santosh
Department of Pathology and Lab Medicine, AIIMS, Kalyani, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.ijd_649_22

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How to cite this article:
Verma J, Gopinath H, Josephain K, Bharti JN, Santosh T. Acrokeratosis verruciformis of hopf in an adult – A rare case report with review of literature. Indian J Dermatol 2022;67:792-4

How to cite this URL:
Verma J, Gopinath H, Josephain K, Bharti JN, Santosh T. Acrokeratosis verruciformis of hopf in an adult – A rare case report with review of literature. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 23];67:792-4. Available from:


Acrokeratosis verruciformis of Hopf (AKV) is a rare genodermatosis, described by Hopf in the year 1931.[1] It exhibits an autosomal dominant mode of inheritance with incomplete penetrance due to which family history may not always be present. It develops usually at birth or during early childhood, occasionally may occur in adults in the fifth decade, due to its late onset.[1] There is no gender predilection. It is characterised by the symmetric distribution of multiple hyperkeratotic, verrucous and papular lesions over the dorsum of hands, feet, knees, elbows and forearms.[2],[3] Patients may have a chronic disease with recurrence or rarely malignant transformation to squamous cell carcinoma. Histopathological examination (HPE) is the gold standard in the diagnosis of AKV; however, dermoscopy has its value in differentiating these clinical entities.[4]

A 58-year-old female presented to the dermatology outpatient department with skin lesions on the dorsum of hands and feet for the last 20 years progressively extending up to forearms and thighs over years. Dermatological examination showed two types of lesions. Multiple warts-like lesions varying in size from 0.2 to 2 cm and other one being symmetrically distributed asymptomatic macules [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d. Patient revealed a family history of similar lesions in her mother and father's sister, that is two generations were inflicted but with the limited site of involvement [Figure 2]. The patient did not have any children. Hence, the third generation cannot be commented upon.
Figure 1: (a, b) Wart-like lesions along with hypomelanotic lesions on dorsum of feet with extension into legs. (c, d) Macular lesion on trunk and wart-like lesions on bilateral lower extremity

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Figure 2: (a) Family tree showing the affected person (Orange circle). (b) Patients palmer ink print showing interrupted dermal ridges

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On examination, she had no complaints of itching, pain or burning sensation. There was no seasonal variation or any systemic complaints. The clinical differentials kept for wart-like lesions were AKV of Hopf, epidermodysplasia verruciformis, stucco keratosis and for depigmented macules were epidermodysplasia verruciformis and idiopathic guttate hypomelanosis.

A punch biopsy from a wart-like lesion over the right forearm and from the depigmented macule over the right thigh was performed and HPE was done. Biopsy from the wart-like lesion showed hyperkeratosis, acanthosis, mild spongiosis in the epidermis with papillomatosis and thickened granular cell layer. A typical circumscribed epidermal elevation 'church spire-like pattern' was noted. Occasional apoptotic keratinocytes were also present. No intracytoplasmic inclusions were noted. The papillary and mid-dermis showed mild perivascular and peri-adnexal lymphocytic infiltrate. The deeper dermis was unremarkable [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d.
Figure 3: (a) Biopsy from wart-like lesion is showing hyperkeratotic, acanthosis, mildly spongiotic epidermis with papillomatosis. (b) The typical circumscribed epidermal elevation 'church spire-like pattern'. (c) Thickened granular cell layer in AKVH. (d) Dermis showing peri-appendageal collection of mild mononuclear infiltrate (×20, ×40; H&E)

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HPE of depigmented macule showed hyperkeratotic-to-orthokeratotic epidermis with flattened rete ridges. In addition, the basal layer of the epidermis showed reduced melanin pigment [Figure 4]a and [Figure 4]b. Hence summing up both dermatological and histopathological findings, the wart-like lesion was histologically compatible with AKV, and for depigmented macule, the possibility of hypomelanotic condition was considered.
Figure 4: (a) Biopsy from hypomelanotic lesion showing atrophied epithelium with flattened rete ridges. (b) Hyperkeratotic-to-orthokeratotic epidermis with reduced melanin pigment in the basal layer (×20, ×40; H&E)

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The patient is currently under treatment and routine follow-up [Figure 5]. She has shown a partial response with topical tazarotene in the last 2 months.
Figure 5: Post-treatment pictures

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Given the rarity of cases of AKV in literature, we hereby report this case with the presentation at an adult age which considers it to be sporadic as described in literature, however, as it was hereditary also since it was present in mother and father's sister with a similar presentation in adulthood. Hence given several clinical differentials of AKV, this case report highlights the role played by both clinical examination and HPE in its diagnosis. Furthermore, AKV may also undergo malignant transformation to squamous cell carcinoma in rare circumstances. Therefore, these cases should be carefully monitored and followed by dermatologists.

   Declarations Top

Ethics approval and consent to participate

This case report was conducted by the fundamental principles of the Declaration of Helsinki.

Consent for publication

Written consent for the publication and any additional related information was taken from the patient involved in the study.

Availability of data and materials

All the data regarding the findings are available within the manuscript.

Authors' contributions

JV carried out concepts and designing, literature search and contributed to manuscript preparation and clinical study. HG helped in data acquisition, data analysis and clinical study. JK carried out data acquisition, data analysis and clinical study. JNB carried out concepts and design and literature search. TS carried out data acquisition, data analysis, and manuscript preparation and will stand as guarantor also. All the authors have read and approved the final manuscript.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Bang CH, Kim HS, Park YM, Kim HO, Lee JY. Nonfamilial acrokeratosis verruciformis of Hopf. Ann Dermatol 2011;23:61-3.  Back to cited text no. 1
Patel N, Diwan N, Nair PA. Nonfamilial acrokeratosis verruciformis of Hopf. Indian Dermatol Online J 2015;6:110-2.  Back to cited text no. 2
[PUBMED]  [Full text]  
Agrawal SN, Jane SD, Rawal AA. Acrokeratosis verruciformis of Hopf in the family. Indian Dermatol Online J 2014;5:17-9.  Back to cited text no. 3
  [Full text]  
Behera B, Prabhakaran N, Naveed S, Kumari R, Thappa DM, Gochhait D. Dermoscopy of acrokeratosis verruciformis of Hopf. J Am Acad Dermatol 2017;77:33-5.  Back to cited text no. 4


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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