EPIDERMODYSPLASIA VERRUCIFORMIS – TREE MAN DISEASE A RARE DEBILITATING SKIN DISEASE – THE FACTS

EPIDERMODYSPLASIA VERRUCIFORMIS – TREE MAN DISEASE A RARE DEBILITATING SKIN DISEASE – THE FACTS

Epidermodysplasia verruciformis (EV), also known as treeman syndrome, is an extremely rare autosomal recessive. hereditary skin disorder associated with a high risk of skin cancer. It is characterized by abnormal susceptibility to human papillomaviruses (HPVs) of the skin. The resulting uncontrolled HPV infections result in the growth of scaly macules and papules, particularly on the hands and feet. It is typically associated with HPV types 5 and 8, which are found in about 80% of the normal population as asymptomatic infections, although other types may also contribute.

The condition usually has an onset of between the ages of one and 20, but can occasionally present in middle age. The condition is also known as Lewandowsky–Lutz dysplasia, named after the physicians who first documented it, Felix Lewandowsky and Wilhelm Lutz.

Signs and symptoms

Clinical diagnostic features are lifelong eruptions of pityriasis versicolor-like macules, flat wart-like papules, one to many cutaneous horn-like lesions, and development of cutaneous carcinomas.

Patients present with flat, slightly scaly, red-brown macules on the face, neck, and body, recurring especially around the penial area, or verruca-like papillomatous lesions, seborrheic keratosis-like lesions, and pinkish-red plane papules on the hands, upper and lower extremities, and face. The initial form of EV presents with only flat, wart-like lesions over the body, whereas the malignant form shows a higher rate of polymorphic skin lesions and development of multiple cutaneous tumors.

Generally, cutaneous lesions are spread over the body, but some cases have only a few lesions which are limited to one extremity.

Genetics

The cause of the condition is an inactivating PH mutation in either the EVER1 or EVER2 genes, which are located adjacent to one another on chromosome 17. These genes play a role in regulating the distribution of zinc in the cell nuclei. Zinc is a necessary co-factor for many viral proteins, and the activity of EVER1/EVER2 complex appears to restrict the access of viral proteins to cellular zinc stores, limiting their growth.

Other genes have also rarely been associated with this condition. These include the ras homolog gene family member H.

Treatment

No curative treatment against EV has been found yet. Several treatments have been suggested, and acitretin 0.5–1 mg/day for 6 months’ duration is the most effective treatment owing to anti-proliferative and differentiation-inducing effects.

Interferons can also be used effectively together with retinoids.

Cimetidine was reported to be effective because of its depressing mitogen-induced lymphocyte proliferation and regulatory T cell activity features. A report by Oliveira et al.showed that cimetidine was ineffective. Hayashi et al. applied topical calcipoTriol to a patient with a successful result.

As mentioned, various treatment methods are offered against EV; however, most importantly, education of the patient, early diagnosis, and excision of the tumoral lesions take preference to prevent the development of cutaneous tumors.

SUMMARY: 

  1. DEBILITATING ILLNESS
  2. NO KNOWN CURE
  3. CONTROLLED THROUGH MINOR SURGERY AT EARLY ONSET
  4. HEREDITARY
  5. PH MUTATION OF GENES

 

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