|Year : 1992 | Volume
| Issue : 2 | Page : 95-98
Acrokeratosis verruciformis of HOPF
VL Rege, RV Hede, NS Nadkarni
V L Rege
Source of Support: None, Conflict of Interest: None
A case of acrokeratosis verruciformis of Hopf with generalized hyperkeratotic, hyperpigmented lesions on unusual sites like face, trunk and extremities is reported. Similar changes in the milder form were noted in patient's sister.
Keywords: Acrokeratosis Verruciformis of Hopf
|How to cite this article:|
Rege V L, Hede R V, Nadkarni N S. Acrokeratosis verruciformis of HOPF. Indian J Dermatol Venereol Leprol 1992;58:95-8
|How to cite this URL:|
Rege V L, Hede R V, Nadkarni N S. Acrokeratosis verruciformis of HOPF. Indian J Dermatol Venereol Leprol [serial online] 1992 [cited 2020 Feb 19];58:95-8. Available from: http://www.ijdvl.com/text.asp?1992/58/2/95/3760
| Introduction|| |
Acrokeratosis verruciformis of Hopf (AKV) is a rare autosomal dominant cutaneous disorder first described by Hopf in 1931. ,, It is present at birth or appears in early childhood, but the onset may be delayed upto the fifth decade. , It affects both sexes but is more common in males as compared to females with a ratio of 5:1.3 The lesions are basically seen on the dorsal aspect of hands and feet but may extend on to knees, elbows, forearms and other parts of the body. ,,,,, Classically, there are verruciform papules resembling flat warts varying from fleshy, dull red, to brown in colour. ,,,,, The lesions may be finely granular to lichenified polygonal papules.  Friction of the lesions may cause blister formation.  Palms may show thickening with punctate pits. ,,,, Nails may be thickened and may show whitish discolouration. ,,, Transformation of skin lesions to squamous carcinoma has been reported. ,,
We are reporting 2 cases of AKV.
| Case Reports|| |
Case 1: A 25-year-old male patient presented with generalised eruptions for last 22 years. Patient first noticed firm papular lesions on the extremities, which spread bilaterally symmetrically to the trunk and the face over the years [Figure - 1].
The individual lesions were starting as skin coloured papules slowly enlarging to form hyperpigmented hyperkeratotic plaques. Some of the lesions had healed leaving hyperpigmentation. Patient also had noticed development of crops of blisters at the age of 12 years which would heal up without any scarring. By the age of 18 years, the blistering stopped but patient noticed progressive diffuse alopecia of scalp. The lesions were asymptomatic throughout, however the itching which had come up in the last few months brought the patient to the hospital for treatment.
The lesions were warty, hyperpigmented, hyperkeratotic papules and plaques varying in size from 0.5 cms. to 2 cm. inter spread with hyperpigmented macules. The skin in between the lesions was poikilodermic showing diffuse atrophy and reticulate pigmentation [Figure - 2].
Palms and soles showed diffuse hyperkeratosis with pitting. There was total alopecia on the scalp. Hair over the other parts of the body were normal. Nails showed varying degree of involvement from mild pitting, thickening to complete destruction. There were hyperpigmented macules over the tongue.
Systemic examination did not reveal any abnormality.
Laboratory investigations were within normal limits. Biopsy of the skin lesion showed hyperkeratosis and thickening of granular layer. There was acanthosis and papillomatosis with circumscribed elevation of epidermis resembling church spires [Figure - 3]. The rete ridges were slightly elongated and extended to a uniform level.
There was no dyskeratosis, no basaloid cells nor pseudohorn cysts. The papillary dermis showed moderate to marked chronic inflammatory infiltrate. Biopsies were done from more than one site to rule out associated Darier's disease and / or malignant changes.
Case 2: A 22-year-old female patient, sister of the patient 1, had hyperkeratotic, hyperpigmented papules of 0.5 to 1 cm. in size. The lesions had started at the age of 5 years and spread to all parts of the body within 1 year. Some of the nails showed thinning of nail plate and pitting. She refused biopsy.
The other family members did not have any evidence of similar disease.
| Comments|| |
The clinical features of generalised verrucous, hyperpigmented lesions, positive family history, early onset of the disease and the classical histopathological findings in case 1 established the diagnosis of AKV.
. The generalised distribution, involvement of forehead, scalp, flexural aspects and oral mucosa were the unusual findings in case 1. Usually the lesions of AKV are noted on the extremities. ,,,, Other sites have been occasionally reported. ,, However Panja is of the opinion that the lesions are never seen on the forehead, scalp, flexures or oral mucosa. 
Amongst the family members, only 1 younger sister had similar lesions spread all over the body. However, they were smaller in size and less in number.
Seborrheic keratosis and Darier's disease were considered as a differential diagnosis. There was superficial resemblance in the morphology of lesions to Seborrhoeic keratosis.
However, the absence of squamous and basaloid cells and the presence of church spires in the histopathology ruled out the possibility of seborrhoeic keratosis.  Further, the whole tumour also extended below the line drawn from normal epidermis at one end of the tumour to the normal epidermis at the other end. 
There are different views regarding the relationship between AKV and Darier's disease. Herndon et al  considered AKV to be a forme fruste of Darier's disease and that AKV and Darier's disease were variable expressions of a single underlying genetic defect, based on clinical, pathological and familial analysis of the pedigree. On the other hand, Panja , on comparative familial, clinical and histopathological analysis of AKV and Darier's disease, has suggested that they are separate entities. There is yet another view saying that AKV and Darier's disease coexist. ,
Malignant transformation of the lesions in AKV has been reported. ,, In our case (case 1) there was no suggestion of any malignancy in any one of the lesions morphologically nor we could find any change suggestive of malignancy in the histopathological sections studied from various sites. However, the patient is being followed up.
| References|| |
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|2.||Baden HP. Darier- White disease (Keratosis follicularis) and miscellaneous hyperkeratotic disorders. In : Dermatology in General Medicine (FitzpatrikTB, Eisen AZ, Wolff K, et al, eds), 3rd edn. New York : Mc Graw-Hill Book Company, 1987; 525. |
|3.||Panja RK. Acrokeratosis verruciformis (Hopf)a clinical entity? Br J Dermatol 1977; 96 643-52. |
|4.||Ebling FJG, Marks A, Rook A. Disorders of keratinization. In : Text Book of Dermatology, (Rook A, Wilkinson DS,' Ebling FJG, et al, eds),4th edn. Bombay : Oxford University Press, 1987; 1445. |
|5.||Herndon JH Jr, Wilson JD. Acrokeratosis verruciformis (Hopf) and Darier's Disease, genetic evidence for a unitary origin. Arch Dermatol 1966; 93 : 305-10. |
|6.||Dogliotii M, Schmaman A. Acrokeratosis verruciformis : Malignant transformation. Dermatologica 1971; 143: 95-9. |
|7.||Lever WF, Schamberg-Lever G. Histopathology of the skin. 7th edn Philadelphia: JB Lippincott, 1990; 83-4. |
[Figure - 1], [Figure - 2], [Figure - 3]