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Acrokeratosis Verruciformis of Hopf (Hopf Disease) | OMICS International
ISSN: 2332-0877
Journal of Infectious Diseases & Therapy
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Acrokeratosis Verruciformis of Hopf (Hopf Disease)

Hussein Salman1* and Waiel Osman2
1Dermatology Clinic, Khartoum Teaching Hospital, Khartoum, Sudan
2Registrar of dermatology, Khartoum Teaching Hospital, Khartoum, Sudan
Corresponding Author : Hussein Salman
Dermatology Clinic
Khartoum Teaching Hospital
Khartoum, Sudan
Tel: +249123094485
Received December 12, 2013; Accepted January 25, 2014; Published January 30, 2014
Citation: Salman H, Osman W (2014) Acrokeratosis Verruciformis of Hopf (Hopf Disease). J Infect Dis Ther 2:128. doi:10.4172/2332-0877.1000128
Copyright: © 2014 Salman H, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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Acrokeratosis verruciformis of Hopf is an autosomal dominant genodermatosis usually presenting with multiple planar wart-like lesions, typically observed on the dorsum of the hands, feet, elbows, and knees. The disease is very rare and the pathogenesis remains unknown. Considerable controversy surrounds the nature and relationship of acrokeratosis and Darier disease and whether they are manifestations of one genetic abnormality. We describe the case of a 40-year-old man seen in our clinic with skin-coloured, flat, warty papules localized to the dorsum of the hands and feet. Both clinical and histological findings were compatible with acrokeratosis verruciformis. We also review the disease, particularly its relation with Darier Disease and therapeutical options.

Acrokeratosis verruciformis; Hopf disease; Church spire; Darier’s disease
Literature Review
Acrokeratosis verruciformis is a very rare, inheritable Hyperkeratotic Dermatosis that was originally described by Hopf [1]. It is characterized by multiple, localized, symmetrical, flat, skin-colored, wart-like lesions, typically observed on the dorsum of the hands and feet.
Definition & clinical picture
Two distinct entities have been described in the literature under this title: true ackrokeratosis verruciformis and acral Darier‘s disease (differentiation is made by histopathologically – clefts) [2,3]. Ackrokeratosis verrucifomis is a genodermatosis inherited as autosomal dominant trait disorder [1-4]. It affects both sexes and is usually present at birth or appears in early childhood. However, the onset may be delayed until the fifth decade [3,4]. Numerous flat, hyperkertotic occasionally verrucous papules are present on the distal part of the extremities, predominantly on the dorsa of the hands and feet [5]. Small groups or isolated papules may develop on the knees, elbows, forearms, and also on other parts of the body. The forehead, scalp, flexures, and the oral mucosa are never affected.
The papules show considerable hyperkeratosis, an increase in thickness of the granular layer, and acanthosis. In addition, there is frequent slight papillomatosis. There is elevation of epidermis resembling church spire appearance. The rete ridges are slightly elongated (Figure 1).
Course & prognosis [1-4]
• After treatment, recurrence is expected.
• Transformation to S.C.C has been reported.
Treatment [1,6-8]
• Superficial destruction using liquid nitrogen, CO2 laser or shave excision.
• Topical retinoic acid has been helpful in some individual.
The disease is inherited as autosomal dominant Hyperceratotic Dermatosis, was first described by Hopf [1]. Our patient was 40 years old with Lesions on hands and feet elbows and knees. Family history is negative for similar condition but parents are cousins
Clinical synopsis
In mid-July 2013 a middle aged (40 years) man stepped in O.P.D in KDVD hospital. The patient was anxious with a frown face. He was reluctant to shake hand & could hardly uttered (salam).He complained that he had itchy rough keratotic skin eruptions on hands, feet, elbows & knees for (1) year. The lesions are also disfiguring & embarrassing; the lesions are slow in course
• Rofa’a tribe (Aljazera state)
• Merchant
• PH of atopy, rhintis. This condition was diagnosed as eczema abroad, In the Sudan he was diagnosed as psoriasis
Family history
• Married (wife is a cousin), he has [3] kids who are not affected
Clinical examination
• There is no similar condition in his small family or grand family.
• The parents of the patient are also cousins.
• The lesions are distributed mostly on the dorsum of hands, feet & interdigital spaces in the form of papules, nodules having skin-color appearance with rough keratotic surface (Figure 2,3), the lesions on the knees & elbows are in the form of papules, nodules & others co-alesced to form plaques (Figure 4,5).
• The scalp, hair, nails & mucous membranes are free
• No specific hobbies or habits
• Differential Diagnosis [1-7].
Thought of
• Darier’s disease
• Ackrokeratosis verrucifomis of Hopf
• Common warts
• Ackrokerato-elastodoidosis
• Epedermodysplasia verruciformis
• Reiter”s disease
• Erythema elevatum diotinum
Histopathology (Biopsy)
• Epidermal findings: - Hyperkeratosis prominent granular layer, acanthosis and church spires papillomatosis elongated rete ridges (Figure 6).
• Other positive findings: High total immunoglobulin (IgE).
Final diagnosis & management [1,5,9-12]
• Ackrokeratosis verrucifomis of Hopf+- Atopic dermatitis (coincidence), (syndrome)
Management [5,8,12]
• Educational explanation to the patient of the disease nature and the outcome
• Topical applications (keratolytics), e mild improvement
• Systemic vitamin-A acid orally after doing required investigations.
• PUVA was irritant (stopped).
• The patient is to take other modalities (eg. Cryo ,laser)
Conclusions & Recommendations
Rare problems in dermatology but can be encountered. Dermatology is specialty of differential diagnosis. Since many diseases are not liable for radical therapy, approach should include management [11,13].Clinical examination should be thoroughly done, in a naked patient (full privacy) in a day light, or a day-lit room (keep it as personal motto) [11].



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