Tree Man Syndrome , Lewandowsky-Lutz dysplasia or Epidermodysplasia verruciformis (EV)

Tree Man Syndrome

Lewandowsky-Lutz dysplasia or Epidermodysplasia verruciformis (EV)
This is an extremely rare autosomal recessive hereditary skin disorder associated with a high risk of skin cancer, characterised by marked susceptibility to Human Papilloma Virus HPV (type 5, 8) infection, which in the normal population is asymptomatic, but in these patients gives rise to scaly maculopapular lesions of the hands and feet, which have been fancifully likened to tree bark.

Tree Man Syndrome (Picture Credit; documentingreality)

It was First described by Felix Lewandowsky and Wilhelm Lutz in 1922, both dermatologists.

More than 200 cases have been reported in the literature so far.
Epidermodysplasia verruciformis is universal and affects persons of all races. No sexual preference is noted for epidermodysplasia verruciformis, although sex-linked and autosomal dominant inheritance have been described.

It has an onset of between the ages of 1–20, but can be seen in all age groups including children but the largest proportion is in patients aged 25-58 years (average 39 years).


 In people with a genetic predisposition, there is a mutation of chromosome 17 occurring within two genes – EVER2 or EVER1, which leads to lowering of the immunological potential of the skin, such that it becomes easily penetrable by HPV virus up to the deep layers of the skin and there is emergence of clinical symptoms, whereas this same Virus (in favorable genetic conditions) is tolerated by the immune system.

Risk factors include;

• immunosuppression, such as HIV infection, organ transplantation, or idiopathic lymphopenia,

• profound CD8+ T-cell lymphocytopenia

• Carcinogenic cofactors (e.g ultraviolet B and x-ray irradiation) are probably involved in the progression from benign warts (verrucae) to cancer.

Signs and Symptoms;

• progressive development of hyperpigmented or hypopigmented flat wart-like papules,

• irregular reddish brown plaques,

• seborrheic keratosis-like lesions and Tinea Versicolor like macules on the trunk and sun-exposed areas (neck, face, dorsum of hands and feet).

• Thick visible warts appear all over the body as well as on the inside of the skin.

• The skin becomes thick and hardened and as old skin dies new skin is created.

• The hands and feet, the extremities, are enlarged and it is difficult to use them.

• Hands and feet have been described as looking like contorted, yellow-brown branches extending up to 3 feet.

• Skin looks like tree bark or tree roots, hence the name Tree-Man Disease or Tree-Man Syndrome

Diagnosis of Tree man syndrome is suspected when this lesions are resistant to appropriate therapy. It is based on clinical and histological findings. Skin biopsy shows verruca plana-like lesions with mild hyperkeratosis, hypergranulosis and acanthosis of the epidermis. Keratinocytes of the upper epidermal layers are enlarged with perinuclear vacuolization and a typical blue-gray pallor.

Differential diagnosis include;
squamous Cell Carcinoma
Tinea Versicolor
Warts, Nongenital

Presently,there is no definitive treatment for Lewandowsky-Lutz dysplasia, however the following approach have been beneficial;

• Preventive measures like avoiding sunlight,UV-B exposure, UV-A exposure, and x-ray irradiation.

• Drugs like topical imiquimod and 5-fluorouracil, systemic retinoids, interferon, and 5-aminolevulinic acid photodynamic therapy. In advanced human papillomavirus (HPV)–related carcinomas, an experimental therapy involves treatment with a combination of 13-cis retinoic acid and interferon alpha or cholecalciferol analogues.

• Surgical and electrosurgical removal and cryotherapy are used in the treatment of benign and premalignant skin lesions. Surgery is also indicated for treatment of malignant lesions. If skin grafting is necessary, the graft should be from sun-protected skin.

Tree man syndrome (Picture Credit; pixshark)

NOTES;

1. Anadolu R, Oskay T, Erdem C, Boyvat A, Terzi E, Gurgey E. Treatment of epidermodysplasia verruciformis with a combination of acitretin and interferon alfa-2a. J Am Acad Dermatol. Aug 2001;45(2):296-9.

2. Bhawna Bhutoria, Kaushik Shome, Sulekha Ghosh, Koushik Bose, Chhanda Datta, and Subodh Bhattacharya. "LEWANDOWSKY AND LUTZ DYSPLASIA: REPORT OF TWO CASES IN A
FAMILY". Indian J Dermatol. 2011 Mar-Apr; 56(2): 190–193.

3. Gubinelli E, Posteraro P, Cocuroccia B, Girolomoni G. Epidermodysplasia verruciformis with multiple mucosal carcinomas treated with pegylated interferon alfa and acitretin. J Dermatolog Treat. Sep 2003;14(3):184-8.

4. Gül U, Kiliç A, Gönül M, Cakmak SK, Bayis SS (October 2007). "Clinical aspects of epidermodysplasia verruciformis and review of the literature". International Journal of Dermatology 46 (10): 1069–72.

5. Hoffner MV, Camacho FM. Surgical treatment of epidermodysplasia verruciformis. Dermatol Surg. Mar 2010;36(3):363-7

6. Majewski S, Jablonska S. Epidermodysplasia verruciformis as a model of human papillomavirus-induced genetic cancer of the skin. Arch Dermatol. Nov 1995;131 (11):1312-8.

7. Medscape | article; 1131981