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Year : 2009  |  Volume : 54  |  Issue : 5  |  Page : 53-56
Epidermodysplasia verruciformis with Hansen's disease, herpes simplex labialis and multiple eccrine hidradenoma

1 Dermatologist, Urban Health Center, Division of Community Medicine, Annamalai University, Annamalai Nagar - 608002, Tamil Nadu, India
2 Division of Pathology, Annamalai University, Annamalai Nagar - 608002, Tamil Nadu, India
3 Division of Community Medicine, Annamalai University, Annamalai Nagar - 608002, Tamil Nadu, India
4 Professor of Pathology, Annamalai University, Annamalai Nagar - 608002, Tamil Nadu, India
5 Reader in Pathology, Rajah Muthiah Medical College, Annamalai University, Annamalai Nagar - 608002, Tamil Nadu, India

Correspondence Address:
L Padmavathy
B# RSA Complex, Annamalai Nagar, Chidambaram - 608002, Tamil Nadu
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Epidermodysplasia verruciformis (EV) - a rare, lifelong heritable disease due to a unique susceptibility to human papilloma virus. The disseminated verrucous lesions or pityriasis versicolor like lesions persist from early childhood and can transform into a cutaneous malignancy in a fourth of patients. The association of EV with multiple eccrine hidradenoma, herpes simplex labialis and Hansen's disease is a very rare occurrence and is reported in a 25-year-old woman.

Keywords: Eccrine hidradenoma, Epidermodysplasia verruciformis, Hansen′s disease, Herpes simplex labialis, human papilloma virus, Lewandowsky and Lutz syndrome

How to cite this article:
Padmavathy L, Rao L L, Ethirajan N, Krishnaswamy B, Manohar U. Epidermodysplasia verruciformis with Hansen's disease, herpes simplex labialis and multiple eccrine hidradenoma . Indian J Dermatol 2009;54, Suppl S1:53-6

How to cite this URL:
Padmavathy L, Rao L L, Ethirajan N, Krishnaswamy B, Manohar U. Epidermodysplasia verruciformis with Hansen's disease, herpes simplex labialis and multiple eccrine hidradenoma . Indian J Dermatol [serial online] 2009 [cited 2022 Jan 27];54, Suppl S1:53-6. Available from:

   Introduction Top

Lewandowsky and Lutz syndrome is a rare lifelong heritable disease due to a unique susceptibility to the human papilloma virus (HPV) often transmitted as an autososmal recessive trait. Eccrine hidradenoma originating in the epithelial matrix of the poral and ductal segment of the eccrine sweat gland is usually a solitary tumor, though rarely, lesions can be multiple. Our patient had a tuberculoid type of Hansen's disease and herpes simplex labialis in addition. A case of Epidermodysplasia verruciformis (EV) with multiple eccrine hidradenomas, tuberculoid Hansen's disease and Herpes simplex labialis in a 25-year-old female patient is being reported for this rare association.

   Case History Top

A 12-year-old girl, born of a consanguineous marriage, presented in 1991 with multiple asymptomatic skin lesions on the whole body, from early childhood. Her maternal uncle also had a similar complaint. The lesions began on the face as asymptomatic small, hypopigmented macules and gradually spread to involve the trunk and limbs over a period of 8 years. There was no history of spontaneous regression. Cutaneous examination revealed multiple, hypopigmented macules some of which have coalesced to form patches with a polycyclic appearance, resembling pityriasis versicolor on chest and face. There were many hyperpigmented flat-topped and warty papules and plaques of various sizes on legs [Figure 1].

She had a hypopigmented, erythematous, anesthetic infiltrated lesion, 3cm 4cm on the radial border of the right forearm [Figure 1]. A clinical diagnosis of tuberculoid Hansen's disease was corroborated by biopsy. She was treated with MDT PB (Rifampicin 600mg once a month and Dapsone 100mg daily for six months). She was lost for follow-up.

The same patient resurfaced 13 years later in 2004, with multiple, asymptomatic, bluish, hyperpigmented papules and nodules of various sizes all over the body, more on the front and back of chest and trunk, in addition to the preexistent hypopigmented lesions [Figure 2]. Her 1 years old daughter was clinically unaffected. The hypopigmented anesthetic patch on forearm had healed completely, with the patient regaining sensation in the patch. She was found to have grouped vesicles on an erythematous base, with a history of recurrence, suggestive of herpes simplex on the upper lip [Figure 3]. A Tzanck smear revealed multinucleated giant cells.

She gave a history of bleeding from some of the lesions especially those on the back. A malignant transformation in some of the lesions was suspected. An excision biopsy from a large nodule on back, revealed a circumscribed, benign tumor in dermis, composed of oval to spindle cells with elongated nuclei, arranged in groups and cords separated by tubular lumina containing eosinophilic secretions and dense pigment incontinence in and around the tumor [Figure 4]. Based on these features, a diagnosis of a benign skin adnexal tumor of eccrine gland origin with associated pigment incontinence, probably eccrine hidradenoma was made. HPE from a lesion on front of chest revealed dysplastic changes, while HPE from a leg lesion showed the characteristic features of EV with koilocytes [Figure 5].

Routine biochemical and hematological parameters and X-Ray chest were within normal limits. Her VDRL and HIV were negative. Due to lack of facilities, PCR study for the viral genomes and further investigations for the exact immunodeficiency state could not be undertaken.

In view of the innumerable lesions, our patient was advised frequent reviews, to detect any malignant transformation as early as possible. This is a rare case of EV associated with multiple eccrine hidradenoma and herpes simplex labialis and tuberculoid Hansen's disease.

   Discussion Top

Lewandowsky and Lutz syndrome or EV was first described in 1922, as an epidermal nevus. [1] This disorder represents a unique susceptibility to cutaneous HPV infection. [2] Ruiter and Van Mullen demonstrated human papilloma virus (HPV) particles in tissue sections in 1966, on electron microscopy. [3] EV virus is the first known human oncogenic virus, characterized in 1978. [4] More than 20 different HPV types have been incriminated, including HPV 3, 5, 8, 9, 10, 12, 14, 15, 17, 19-25, 36-38, 46, 47, 49, 51 and 59. [5] There may be more than one HPV type in the same patient.

Two forms of EV are recognized. One form is induced by HPV-3 and sometimes by HPV-10, both of which cause plane warts. Hence, in this type of EV, multiple plane warts, more widely distributed and with no tendency to malignant transformation occur.

The second form of EV is related to HPV-5 and sometimes to HPV 8, 9, 14, 20, 24 38, 47 and others. There is a familial history with an autosomal recessive inheritance. Two susceptibility loci have been mapped to chromosome regions 2p21-p24 and 17 q 25. This latter region also contains a locus for psoriasis. In addition to plane warts, lesions resembling pityriasis versicolor and seborrheic keratoses develop, with the eventual complication of Bowen's disease or invasive squamous cell carcinoma. Malignant transformation which occurs in 25% of patients with EV depends on the oncogenic potential of the virus, which is highest in HPV-5, followed by HPV-8. [5]

EV lesions start in childhood and persist lifelong as was observed in our patient. As in our patient, 25% of cases are familial with parental consanguinity, suggestive of autosomal recessive inheritance. Maternal uncle of our patient also had EV. An X linked inheritance too is reported. Lack of CMI to HPV can lead to persistence of infection. [6] There is a decrease in absolute number of T lymphocytes and helper T cells. [7] Most patients are unresponsive to DNCB sensitization. Humoral immunity may be only slightly impaired.

Malignant transformation of lesions, mostly on sun exposed or traumatized areas, especially over forehead, occurs in 25-30% of patients at a relatively young age (third or fourth decade). [8] Malignant transformation depends on the oncogenic potential of the virus. [5] In genital malignancies and a proportion of head and neck cancers, especially tonsillar cancers, DNA is present in large amounts and can be detected by Southern blot. [2] More importantly, in these tumors, E6 and E7 messenger RNA (mRNA) of oncogenic HPVs is also found, suggestive of the pathogenic role of these viruses. [4]

Recent studies have detected HPV strains usually associated with EV in some of the malignant and premalignant skin lesions in renal transplant recipients. Neurological manifestations and isolated IgM deficiency have been reported in patients with EV. An EV like eruption caused by HPV may complicate HIV infection, [5] our patient was HIV negative and did not have any clinical evidence of neurological deficit.

Classically individuals with EV do not suffer from frequent bacterial or other viral infections. [9] Our patient had tuberculoid Hansen's disease and recurrent herpes simplex labialis lesions. Whether these conditions are a chance co-incidence is a moot question. Unusual association of EV with Coeliac disease and porokeratosis of Mibelli has been reported. [10]

EV should be distinguished by the help of HPE, from Acrokeratosis verruciformis, generalized verrucosis and lichen planus. There is no effective treatment for EV, except frequent reviews to detect any malignant transformation. Isotretinoin has been claimed to be beneficial in retarding the development of malignancies. Interferon has been effective in short term studies in reducing warts in laryngeal papillomatosis and EV, but lesions recur, when therapy is stopped. [9] Various other modalities of treatment tried with varying results are 5% 5-flourouracil ointment, retinoic acid 0.05-1% and isotretinoin 2%.

Benign nodular hidradenoma is a skin appendage tumor that is characterized by sweat gland differentiation and classified as eccrine in origin. Other synonyms for this condition are solid/cystic hidradenoma, eccrine acrospiroma, clear cell hidradenoma and eccrine sweat gland adenoma. It is an uncommon tumor with a male: female ratio of 1:2. [11],[12],[13]

Eccrine hidradenoma originating in the epithelial matrix of the poral and ductal segment of the eccrine sweat gland usually pursues a benign clinical course. Recurrences occur following incomplete excision. Malignant eccrine hidradenoma is rare and can arise from benign tumors or may be malignant at the outset. [14] Eccrine hidradenoma presents as a solitary tumor in most instances. However, rarely, several lesions are also described as in our patient. Unlike the changes seen in HPE, clinically the tumor rarely gives the impression of being cystic, a clinical pitfall, as had been our experience in this patient. Seen on the scalp, face and trunk, the surface may be skin colored, red or bluish. [14] Occasionally, they may ulcerate and discharge serous fluid as was observed in some lesions on the back in our patient.

The co-existence of eccrine hidradenoma and EV has not been described hitherto in the literature to the best of our knowledge. The present case is being reported for the rare association of EV with multiple eccrine hidradenomas, tuberculoid Hansen's disease and Herpes simplex labialis.

   Acknowledgment Top

Authors thank the authorities for facilities provided for carrying out the work and for permission to publish the article.

   References Top

1.Sehgal VN, Luthra A, Bajaj P. Epidermodysplasia verruciformis: 14 members of a pedigree with an intriguing Squamous cell carcinoma transformation report. Int J Dermatol 2002;41:500-3.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Majewski S, Jab?oρska S, Orth G. Epidermodysplasia verruciformis : Immunological and non immunological surveillance mechanisms: Role in tumor progression. Clin Dermatol 1997;15:321-34.  Back to cited text no. 2    
3.Ruiter M, Van Mullem PJ. Demonstration of electron microscopy of an intranuclear virus in Epidermodysplasia verruciformis . JInvest Dermatol 1966;47:247-52.  Back to cited text no. 3    
4.Orth G. Epidermodysplasia verruciformis . In : The Papovaviridae papilloma viruses. In : Salzmann NP, Howley PM, eds. NewYork: Plenum Press; 1987. p. 199-235.  Back to cited text no. 4    
5.Weedon D, Strutton G. Viral Diseases. In : Weedon D, StruttonG, eds. Skin pathology. 2 nd ed. Churchill Livingstone; 2002. p.701-5.  Back to cited text no. 5    
6.Beutner KR. Human papilloma virus infection. J Am Acad Dermatol 1989;20:114-23.  Back to cited text no. 6  [PUBMED]  
7.Sait MA, Garg BR. Treatment of warts: A study of 106 cases. Indian J Dermatol Venereol Leprol 1985;51:96-8.  Back to cited text no. 7    
8.Sharma VK, Kaur S, Kumar B, Sehgal S. Epidermodysplasia Verruciformis with squamous cell carcinoma in a family. Indian J Dermatol Venereol Leprol 1985;51:80-6.  Back to cited text no. 8    
9.Douglas LR, Androphy EJ. Warts. In : Freedberg IM, Eisen AZ, Wolff K, et al , eds. Fitzpatrick's dermatology in general medicine. 5th ed. New York: McGraw Hill; 1999. p. 2490.  Back to cited text no. 9    
10.Khatri ML, Shafi M. Epidermodysplasia verruciformis . Indian J Dermatol Venereol Leprol 1993;59:207-13.  Back to cited text no. 10    Medknow Journal
11.Hashimato K, DiBela RJ, Lever WF. Clear cell hidradenoma: Histological, histochemical and electron microscopic studies. Arch Dermatol 1967;96:18-38.  Back to cited text no. 11    
12.Johnson BL, Helwig EB. Eccrine acrospiroma. Cancer 1969:23:641-57.  Back to cited text no. 12    
13.Hernandez-Perez E, Cestoni-Parducci A. Nodular hidradenoma and hidradenocarcinoma. J Am Acad Dermatol 1985;12:15-20.  Back to cited text no. 13    
14.Jerajani HR, Amladi ST. Skin tumours and lympho proliferative disorders. In : Valia RG, Valia AR, eds. IADVL text book and atlas of dermatology. 2 nd ed. Mumbai: Bhalani Publishers; 2001. p. 1168-9.  Back to cited text no. 14    


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

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