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Year : 2016  |  Volume : 5  |  Issue : 4  |  Page : 303-305

Acrokeratosis verruciformis: An unusual presentation

Department of DVL, Gandhi Medical College and Hospital, Hyderabad, Telangana, India

Date of Web Publication23-Dec-2016

Correspondence Address:
Sudha V Damarla
Department of DVL, Gandhi Medical College and Hospital, Hyderabad, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2277-8632.196596

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How to cite this article:
Damarla SV, Arakkal GK, Chintagunta SR, Vasavi Latha C H. Acrokeratosis verruciformis: An unusual presentation. J NTR Univ Health Sci 2016;5:303-5

How to cite this URL:
Damarla SV, Arakkal GK, Chintagunta SR, Vasavi Latha C H. Acrokeratosis verruciformis: An unusual presentation. J NTR Univ Health Sci [serial online] 2016 [cited 2020 Mar 28];5:303-5. Available from: http://www.jdrntruhs.org/text.asp?2016/5/4/303/196596


Acrokeratosis verruciformis (AKV) is a rare autosomal dominant genodermatoses characterized by multiple keratotic papules on the hands and feet, with punctate keratoses on the palms and soles with varying degrees of nail involvement. [1] We report an unusual case of familial AKV with hypopigmented macules in a 48-year-old male along with his three daughters who presented with multiple, hyperkeratotic papules and plaques all over the body since childhood. All his three daughters also presented with similar lesions without any hypopigmented macules.

A 48-year-old male presented to our outpatient department along with his three daughters, all having multiple verrucous lesions over the trunk and extremities. Lesions started over the dorsum of the hands and feet when he was 10 years old, gradually spreading to involve the trunk. He started developing multiple, tiny, hypopigmented macules over the last 6 years on the trunk. His three daughters aged 19, 17, and 14 years also developed similar kind of lesions over the extremities and trunk at the age of approximately 10 years. There was no history of consanguinity.

Cutaneous examination revealed multiple, pigmented verrucous papules on the face, trunk, and extremities [Figure 1]. Father had multiple, small hypopigmented macules on the trunk [Figure 2]. There were no hypopigmented macules in case of his daughters. Hair, nails, and oral mucosa were normal in all the four patients. Differential diagnosis considered were AKV, epidermodysplasia verruciformis (EDV), and Darier's disease. Routine investigations were normal. Biopsy of verrucous papule from trunk showed marked hyperkeratosis, acanthosis, and papillomatosis, with "church spire" appearance, which is a typical finding of AKV [Figure 3]. There were no signs of parakeratosis, acantholysis, dyskeratosis, or vacuolization of keratinocytes. Biopsy taken from the hypopigmented macule showed decreased pigment in the basal layer [Figure 4]. Polymerase chain reaction (PCR) for human papilloma virus (HPV) was negative, ruling out EDV. We could not perform genetic studies for AKV and EDV because the patient could not afford them. Based on the clinical and histological findings, we made a diagnosis of AKV. However, we are unable to explain the cause of hypopigmented macules in AKV.
Figure 1: Multiple verrucous papules over the extremities

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Figure 2: Multiple hypopigmented macules on the back

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Figure 3: (Hematoxylin and eosin, 10×) showing typical "church spire" appearance of epidermis

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Figure 4: (Hematoxylin and eosin, 40×) iopsy from hypopigmented macule showing decreased pigment in the basal layer

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AKV of Hopf is a disorder of keratinization affecting the distal extremities with early onset in life. AKV and Darier's disease are considered to be allelic disorders. Some authors consider that AKV of Hopf and Darier's disease are separate entities, while others believe that they are variable presentations of the same disease. [2],[3] Both the diseases have seen in different members of the same family in one case. [4]

AKV and Darier's disease, though clinically similar, histologically, AKV may remain nondyskeratotic and nonacantholytic throughout life, whereas acral lesions of Darier disease show various stages of acantholytic dyskeratosis. Basically, the keratinization process in Darier's disease is accentuated, altered, and faulty, whereas in acrokeratosis verruciformis, it is exaggerated but normal. [2]

Almost 50% of Darier's patients have acral warty papules, [5] which may be similar to AKV; there are case reports of AKV patients who later developed clinical features of Darier's disease including histopathologic features. [6]

According to Jittima et al., AKV and Darier's could be considered as two different diseases caused by different mutations in the same gene [ATP2A2 gene encoding the sarco (endo) plasmic reticulum Ca ATPase2 pump (SERCA2)], although their histopathological features are different. [1]

Our patient presented with multiple hypopigmented macules on the trunk, which led us to consider Darier's disease and EDV as differential diagnosis. However, histopathology of the biopsied lesion showed typical church spire appearance, with no dyskeratosis and vacuolated cells. Viral PCR was negative for HPV.

Small hypopigmented macules in Darier's disease were first described by Goddal and Richmond in 1965. These may be misdiagnosed as tinea versicolor, follicular vitiligo, or idiopathic guttate hypomelanosis.

Sornakumar and Srinivas reported a case of Darier's with perifollicular hypopigmentation. They gave an explanation that a defective keratinization interfering with melanosome transfer and an overall disruption of the "epidermal melanin unit" may be a probable cause of hypopigmentation in Darier's disease. Because AKV and Darier's disease are considered to be allelic disorders, we assume that the same phenomenon is occurring in AKV resulting in hypopigmented macules.

There is no curative treatment for AKV. The verrucous lesions on exposed parts were cauterized in all three daughters and the lesions responded well.

A literature survey did not reveal any case reports of AKV with hypopigmentation. We report this case to highlight the association of AKV with hypopigmented macules, which could be a variant. Further case reports of this type may help us to categorize genodermatoses.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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. Invest J Dermatol 2003;120:229-32.  Back to cited text no. 1
Panja RK. Acrokeratosis verruciformis: (Hopf)--A clinical entity? Br J Dermatol 1977;96:643-52.  Back to cited text no. 2
Waisman M. Verruciform manifestations of keratosis follicularis. Arch Dermatol 1960;81:1-14.  Back to cited text no. 3
Herndon J, Wilson J. Acrokeratosis verruciformis (Hopf) and Darier's disease, genetic evidence for a unitary origin. Arch Derm 1966;93:305-10.  Back to cited text no. 4
Penrod J, Everett M, McCreight W. Observations on keratosis follicularis. Arch Der matol 1960;82:367-70.  Back to cited text no. 5
Waisman M. Verruciform manifestations of keratosis follicularis. Arch Dermatol 1960;81:1-14.  Back to cited text no. 6


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


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