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Childhood actinic keratosis in an albino transforming into squamous cell carcinoma

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Abstract

Actinic keratosis is a scaly, cutaneous lesion that is often seen in elderly patients. It is almost never seen in children and young adults. It develops on areas such as the face, ears and dorsa of the hands with chronic sun exposure. Actinic keratosis is a premalignant lesion, since histological changes in the epidermis may progress to squamous cell carcinoma if left untreated. There are roughly 70,000 people with albinism in India. These patients are prone to develop actinic keratosis which may undergo spontaneous remission or develop into invasive squamous cell carcinoma, basal cell carcinoma and malignant melanoma.We present an unusual case of a 35 year old albino who developed actinic keratosis at the age 15 years which progressed to squamous cell carcinoma.
Indian Dermatology Online Journal -
September-December 2012
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Volume 3
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Issue 3
199
Address for
correspondence:
Dr. Shubhangi V. Agale,
Flat No. 6, Dhanvantari
Building, Sir JJ Hospital
Campus, Byculla,
Mumbai - 400008,
India.
E-mail: shubhagale@
hotmail.com
Case Report
Department of Pathology,
Grant Medical College,
Mumbai, India
ABSTRACT
Actinic keratosis is a scaly, cutaneous lesion that is often seen in elderly patients. It is almost never seen in
children and young adults. It develops on areas such as the face, ears and dorsa of the hands with chronic sun
exposure. Actinic keratosis is a premalignant lesion, since histological changes in the epidermis may progress
to squamous cell carcinoma if left untreated. There are roughly 70,000 people with albinism in India. These
patients are prone to develop actinic keratosis which may undergo spontaneous remission or develop into invasive
squamous cell carcinoma, basal cell carcinoma and malignant melanoma.We present an unusual case of a 35 year
old albino who developed actinic keratosis at the age 15 years which progressed to squamous cell carcinoma.
Key words: Actinic keratosis, albinism, children, squamous cell carcinoma
IDOJ_89_11R5
INTRODUCTION
Albinism is an autosomal recessive disorder
characterized by lack of melanin pigment
due to which these people are prone to sun
damage.
[1]
Albinism is of two types - ocular and
oculocutaneous. The incidence of oculocutaneous
albinism is estimated to be one in 20000, in most
populations in the world.
[1]
The prevalence in
regions of sub-Saharan Africa is estimated to be
much greater than these gures; for example, it
is estimated at about 1:4000 in Zimbabwe and
in Tanzania it is 1:1429.
[2]
Albinism most commonly arises from mutations
in genes encoding for proteins involved in the
synthesis or transport of melanin by melanocytes.
Patients may present with nystagmus, strabismus,
photophobia and decreased visual acuity. Patients
with oculocutaneous albinism present commonly
with cutaneous manifestations of actinic keratosis
and are also prone to develop squamous cell
carcinoma, basal cell carcinoma, malignant
melanoma and dysplastic nevus syndrome.
[1,3]
Actinic keratosis is a scaly, cutaneous lesion that
is often seen in elderly patients. It is almost never
seen in children and young adults. We present
an unusual case of a 35 year old albino who
developed actinic keratosis at the age 15 years
which progressed to squamous cell carcinoma.
CASE REPORT
A 35 year old male presented with a swelling on
the right shoulder since two months. He also gave
history of tiny growths on trunk and upper limbs,
since the age of 15 years. He has past history of
photosensitivity and decreased vision since birth.
Family history revealed history of photosensitivity
and fair skin in other family members.
On physical examination, he had depigmented
skin and white hairs. He had photophobia,
nystagmus and decreased visual acuity.
There were multiple, erythematous, papular
lesions of 0.5-2 cm over trunk and upper arms
[Figure 1a and b]. An ulceronodular growth on
right shoulder was 3×2×2 cm, erythematous,
rm and non-tender. Based on these ndings,
clinical diagnosis of oculocutaneous albinism
with multiple actinic keratoses with squamous cell
carcinoma was made. Laboratory investigations
showed hemoglobin of 10 gm% with normal
leukocyte and differential count. His liver and
kidney function tests were normal. He was non
reactive for HIV and HBsAg.
Pathological Findings
The received specimen of an ulceronodular
growth measured 3×2×2 cm [Figure 2a]. Cut
surface was grayish white, homogenous, rm
without hemorrhage and necrosis [Figure 2b].
Childhood actinic keratosis in an albino
transforming into squamous cell carcinoma
Shubhangi V. Agale, Grace F. D’Costa, Bhavana M. Bharambe, Varsha Bhatia
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DOI: 10.4103/2229-5178.101820
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Agale, et al.: Actinic keratosis in albino transforming into SCC
200 Indian Dermatology Online Journal -
September-December 2012
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Volume 3
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Issue 3
Microscopically, the biopsy from erythematous lesion on the
back revealed actinic keratosis characterized by dyskeratosis,
hyperkeratosis, irregular acanthosis, alteration of polarity
of basal cell layer and atypia in kertinocytes but no dermal
invasion [Figure 3a]. The sections from the nodular growth
revealed squamous cell carcinoma comprised of nests and
tongue-like processes of malignant squamous cells with
epithelial and keratin pearl formation [Figure 3b].
DISCUSSION
Actinic keratosis was rst described by Dubreuilh in 1904. The
factors implicated in development of actinic keratoses are UV
rays, fair skin, blue eyes, blond hair, immmunosuppression and
genetic diseases like albinism and xeroderma pigmentosum.
[3]
Prevalence of actinic keratosis depends upon the skin type and
geographical location. Males develop actinic keratosis more
frequently than females.
[1]
It develops on areas such as the
face, ears and dorsa of the hands with chronic sun exposure.
The rate of development of squamous cell carcinoma from
actinic keratosis is estimated to be 0.025 to 16% per year.
[3]
Among patients with multiple actinic keratoses, the cumulative
lifetime risk of having at least one invasive squamous cell
carcinoma is substantial, possibly 6-10%.
[4]
The cumulative
risk depends on the number of lesions and the length of time
they persist.
[5]
According to Foote et al,
[3]
patients with actinic
keratoses at highest risk for squamous cell carcinoma are older,
male, red haired and have prolonged residence as an adult
in a sunny region. Induration, erythema or erosion of actinic
keratoses may be indicative of progression to squamous cell
carcinoma. It is believed that several years can elapse before
this transformation occurs.
The lesions in actinic keratoses can be at or raised, single
or multiple, pigmented or pale and usually less than 1 cm
in diameter. Microscopically, actinic keratosis is defined
as epidermis having irregular hyperplasia or atrophy with
keratinocytes having a disorderly arrangement, accompanied by
Figure 1: (a) Multiple, papular, erythematous lesions of 0.5-2 cm over back, (b) Multiple, papular, erythematous lesions of 0.5-2 cm over
upper arm.
b
a
Figure 2: (a) A 3x2x2 cm, ulceronodular grayish white growth, (b) Cut surface showed grayish white, homogenous, rm tumor without hemorrhage
and necrosis.
a
b
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Agale, et al.: Actinic keratosis in albino transforming into SCC
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September-December 2012
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Volume 3
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Issue 3
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atypia.
[6]
Our case was a 35 year old male with oculocutaneous
albinism. He developed lesions of actinic keratosis at the age of
15 years, which is uncommon. One of the lesions increased in
size to attain the present size of 3×2×2 cm and was diagnosed
as squamous cell carcinoma.
There are different treatment options for actinic keratosis; of which
curettage and cryotherapy with liquid nitrogen, 5-ourouracil are
commonly used options.
[1,5]
Topical chemotherapeutic agents
such as those containing 5-ourouracil are used in patients
with multiple lesions,
[5]
which was also the treatment offered
in this case with excision of larger lesions.
Albinism is a disorder that affects individuals and their families,
socially and psychologically. Children with albinism have to
deal with issues such as photophobia, decreased visual acuity,
extreme sun sensitivity, hearing problems and skin cancer. They
also face social discrimination as a result of their appearance.
[2]
Children with albinism need protection from sun that can be
achieved with the use of sunscreens, protective clothing,
and indoor activities.
[1]
They may be clearly well aware of
the short-term consequences of sun exposure but may be
less informed about the long-term risks of developing skin
cancer. The children themselves must be convinced that
staying out of the sun is a sensible and worthwhile choice. The
involvement of the parents is also crucial to a sustained health
intervention plan, if mothers can be educated and informed
about albinism they will have more condence to deal with
their child’s condition.
[7]
The Regional Dermatological Training
Center (RDTC) in Moshi, Tanzania runs a mobile skin care
clinic where a doctor and a nurse regularly visit villages
to examine the skin of albinos and provide education on
protection from UV light exposure.
[8,9]
It is very important for
children with albinism to be accepted socially so that they
can lead a normal life.
CONCLUSION
The high incidence of malignancies among children with
albinism suggests that periodic monitoring and treatment are
important in these patients.
REFERENCES
1. Ramalingam VS, Sinnakirouchenan R, Thappa DM. Malignant
transformation of actinic keratoses to Squamous cell carcinoma in an
albino. Indian J Dermatol 2009;54:46-8.
2. Hong ES, Zeeb H, Repacholi MH. Albinism in Africa as a public health
issue. BMC Public Health 2006;6:212.
3. Foote JA, Harris RB, Giuliano AR, Roe DJ, Moon TE, Cartmel B, et al.
Predictors for cutaneous basal and Squamous cell carcinoma among
actinically damaged adults. Int J Cancer 2001;95:7-11.
4. Alam M, Ratner D. Cutaneous Squamous cell carcinoma. N Engl J Med
2001;344:975-83.
5. Salasche SJ. Epidemiology of actinic keratoses and squamous cell
carcinoma. J Am Acad Dermatol 2000;42:4-7.
6. Dinehart SM, Nelson-Adesokan P, Cockerell C, Russell S, Brown R.
Metastatic cutaneous squamous cell carcinoma derived from actinic
keratosis. Cancer 1997;79:920-3.
7. Ackerman AB. Solar keratosis is squamous cell carcinoma. Arch
Dermatol 2003;139:1216-7.
8. Lund PM, Gaigher RA.A health intervention program for children
with albinism at a special school in South Africa. Health Educ Res
2002;17:365-75.
9. Simona B. Albinos in black, Africa: Problems and solutions. Int J
Dermatol 2004;43:616-21.
Cite this article as: Agale SV, D'Costa GF, Bharambe BM, Bhatia V.
Childhood actinic keratosis in an albino transforming into squamous cell
carcinoma. Indian Dermatol Online J 2012;3:199-201.
Source of Support: Nil, Conict of Interest: No.
Figure 3: (a) Actinic keratosis comprised of disorderly arranged atypical keratinocytes with loss of polarity in basal cell layer (H and E, ×200),
(b) Squamous cell carcinoma comprised of malignant epithelial cells with epithelial pearl formation (H and E, ×400).
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I read with dismay the article titled "The Actinic (Solar) Keratosis: A 21st-Century Perspective," published last January in the ARCHIVES.1 Not only does it misrepresent my thinking about the subject, but it reflects a mentality more like that of the 19th century than the 21st. In it, Wendy Fu and Clay J. Cockerell cite an article by me2 as verification for their statement that "another name proposed is solar keratotic intraepidermal squamous-cell carcinoma." Never did I propose such a term; on the contrary, I have decried both the term itself and the concept that animates it in these words: "In conclusion, the terms apocrine carcinoma in situ, sebaceous carcinoma in situ, and melanoma in situ have legitimacy; the designation squamous-cell carcinoma in situ [intraepidermal squamous-cell carcinoma] does not."3
Albinos in black, Africa: Problems and solutions
  • B Simona
Simona B. Albinos in black, Africa: Problems and solutions. Int J Dermatol 2004;43:616-21.