Professional Documents
Culture Documents
수) LENTIGO
수) LENTIGO
수) LENTIGO
4, April 1996
REVIEW
LENTIGO
SIMEEN BER RAHMAN, M.D., AND
--
JAG BHAWAN, M.D.
다양한 용어들이 작고, 정확히 둥근 형태에 있고, 기관계 이상과 관련이 있을 수도, 또 없을 수도 있다.
황반변적 특성을 지닌 색소 침착성의 피부 점막에
있는 조직을 표현하기 위해 사용되어져 왔는데 이는 LENTIGINES WITHOUT ASSOCIATED SYSTEMIC
흑색점(lentigo), 점막 멜라닌반(melatonic macules), ANOMALIES (기관계 이상과 연관성이 없는 흑색증)
흑색증(melanosis) – 또는 멜라닌증 – 주근깨, 모반 해당 흑색증 그룹의 의학적, 조직학적 특성은 Tables
(ephelides), 경계 모반(junctional nevi) 등의 어휘를 2 와 3 에 요약되어 있음
포함한다. ‘흑색증’이라는 용어는 단수로는 lentigo, Juvenile Lentigo (Lentigo Simplex) – 청소년기
복수로는 lentigines 로 일컬어지는데 이러한 흑색증
조직장애를 묘사하기 위해 언급된 모든 어휘를 의학적 관찰. 이 조직 장애는 수가 적도 아동기에 주로
사용하는 것이 가능하다. 관련된 조직학적 특성에 발생하지만, 어떤 나이에서든지 나타날 수 있다. 조직
대한 묘사는 혼란을 야기하였는데, 그 이유는 장애는 햇빛에 노출된 부위에 편증되지 않고도 신체의
조직학적 특성을 자세히 다루는 연구가 이제껏 몇 모든 곳에 발생할 수 있다. 그 크기는 1mm 에서부터
없었기 때문이다. 더불어, 이러한 연구들은 아주 5mm 에 달하며 색으로는 갈색과 검은색의 색소가
제한된 수의 조직 장애에 대해서만 이루어지는 것이 고르게 침착되어 있다. 또한 이들은 손으로 만져지지
보편적이었다. 해부학적 변이를 고려한 정보의 부족, 않는다.
그리고 이러한 조직 장애의 조직학적 성격을 연구하기 현미경을 통한 발견. 조직학적으로, 망융선이 보통
위해 사용된 방법론과 기술들이 이 문제점을 더욱 정도의 신장을 보이게 된다. 멜라닌 세포는 기저
복잡하게 만들었다. 흑색증은 단독으로 존재하거나 세포층에서 그 수가 증가할 수도 있다. 증가된 색소는
복수로 존재할 수도 있는데, 많은 의학적 기형과 각질형성세포를 비롯한 멜라닌 세포에서도 발견될 수
연관된 형태로 보여질 수도 있다. 본고는 다양한 있으며 멜라닌 소포는 각질층을 포함한 표피의
흑색증의 형상과 그들의 조직학적인 특성을 다룬 상층에서 관찰될 수 있다. 상피에서, 멜라닌 포식
문헌에 대한 논평으로서 이 복잡한 색소 침착에 대한 세포는 단독으로 혹은 단핵 세포가 동반된 침투가
조직 장애를 보다 더 이해하고 헷갈릴 위험성이 있는 보여지기도 한다. 어떤 저자들에 의하면, 이러한
많은 용어들을 명료히 하는 데에 주안점을 두고 있다. 조직 장애는 경계 모반의 전구체가 될 수도
있는데 이는 모반 세포의 소포가 망융선의
DEFINITION (용어 정의) 끝부분에서 보이는 경우도 있기 때문이다. 우리의
흑색증은 1 에서 5mm 정도의 작은 색소가 들어간
의견에 의하면, 이러한 조직 장애는 흑색증이
반점을 의미하나 1cm 보다는 작고 주위에 정상적으로
보이는 피부가 둘러싸고 있는 것을 의미한다. 아니라 초기모반 (nevus incipiens)라고 불리는
조직학적으로, 기저층의 색소 침착 증가 및 변이형 것이 더 맞을 것 같다.
멜라닌 세포의 수 뿐만 아니라 표피의 과다 형성 또한 전자 현미경을 통한 발견. 기저층의 각질 형성
관찰될 수 있다. 후자의 경우는 수적으로는 증가할 수 세포는 초핵 구간에 집중된 멜라닌 과립을
있겠으나, 고치 형태를 띄진 않는다. 흑색증의 다양한 함유하고 있다. 각질 형성 세포는 주로
의학적 변이가 묘사되어 있다. (Table 1). 정상적으로 나타나는 반면 멜라닌 세포는 고르지
않은 핵과 조밀한 세포질을 지니고 있다. 거대한
CLINICAL FEATURES (의학적 특성들) 멜라닌 과립 (macromelanosome) 멜라닌 색소,
흑색증은 몇 년의 기간을 걸쳐 천천히 발달하거나 각질 형성 세포, 그리고 대식세포에서 관찰될 수
갑작스럽게 발진할 수도 있다. 그 색은 갈색에서
있다.
검갈색, 검은색에 이르기까지 다양하고 그 색소화가
동종의 것일 수도, 잡색일 수도 있다. 의학적 변이의 Freckles (Ephelides) – 주근깨 (모반)
대다수 중에서는 그 크기는 1mm 부터 5mm 까지 의학적 관찰. 의학적으로, 주근깨는 얼굴, 손등,
존재한다. 양성 흑색증 (lentigo benigna)라고도 그리고 밝거나 (특히 붉은 계열의) 머리카락과
불리우는 그 하위 종은 주로 얼굴에 발현되며, 직경이 밝은 피부를 가진 사람들의 팔 앞 부분의 햇빛에
몇 센티미터에 이르기도 한다. 이러한 조직 장애는 노출된 부위에서 나타난다. 그러나, 주근깨는
의학적으로는 악성 흑색증과 혼동될 수 있으나 조직적 또한 금발이거나 갈색 머리칼을 가진
형태를 들여다 보면 비전형적 멜라닌 세포가 없다는 사람들에게서도 나타날 수 있으며 어두운
사실을 알 수 있다. 흑색종의 가장자리에는 선명히, 머리카락과 어두운 피부도 예외가 아니다. 이러한
혹은 부드럽거나 들쭉날쭉한 경계가 있다. 그 숫자는 사람들에게서 나타나는 주근깨는 더 작고 더
몇 개 정도에서 수 천 개까지도 가능한데, 인체의
적다. 주근깨는 주로 여름 달에 나타나지만
손바닥, 발바닥, 그리고 점막으로 이루어진 표면을
포함한 모든 부위에 발현될 수 있다. 흑색증의 표현은 일생동안 유지된다.
어떤 경우에는 벗겨져 나갈 수도 있다. 흑색증은
From the Dermatopathology Section, Department of
may be eruptive, appearing rather suddenly. The color
Dermatology, Boston University School of Medicine, Boston,
ranges from brown to brown-black to black and the
Massachusetts.
pigmentation may be homogeneous or variegated.1,2 In
Address for correspondence: Jag Bhawan, M.D., Boston the majority of the clinical variants, the size ranges
Uni versity School of Medicine, Department of from 1 to 5 mm. The subtype, sometimes referred to as
Dermatology, 609 Albany Street, Boston, MA 02118. lentigo benigna and often seen on the face, may spread
up to a few centimeters in diameter.1,3 These lesions
may be clinically confused with lentigo maligna, but
the histologic features lack melanocytic atypia. The
margins of the lentigines are sharply defined or may be
smooth or jagged. Their number varies from a few to
thousands and they can occur anywhere on the body
including the palms, soles, and the mucosa! surfaces.1
The surface of the lentigines can sometimes be scaly.2
The lentigines may or may not be associated with sys
temic anomalies.
229
International Journal of Dermatology
Vol. 35, No. 4, April 1996
13654632, 1996, 4, Downloaded from https://onlinelibrary.wiley.com/doi/10.1111/j.1365-4362.1996.tb02994.x by University Of Ulsan, Wiley Online Library on [27/05/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Table 1. Classification of Lentigines
Lentigines With Associated
Lentigines Without Associated Anomalies Ultra-violet Induced Lentigines Anomalies/Syndromes
Juvenile lentigo/lentigo simplex Solar lentigines/senile lentigines/senile freckle LEOPARD
Freckles/ephelides Reticulate black solar lentigo'' Moynahan''
Generalized lentigines/lentiginosis profusa Lentigo benigna '' Peutz-Jeghers
PUVA-induced Syndromes associated with myxomas
Unilateral/segmentalt Tanning bed LAMB
Mucosal/genital NAME
Labial CARNEY
Genital Langier-Hunziker
Atypical Penile'' Neurofibromatosis
Acral Leschke's''
Xeroderma pigmentosa
Bannayan-Riley-Ruvalcaba
Tay's
Centrofacial neurodysgraphic
*Subtype of the above.
t May also be associated with systemic disorders.
trated in the supranuclear area.7 The keratinocytes usu processes than the melanocytes of adjacent normal
ally appear normal, whereas the melanocytes may have
skin.5,9 Solar elastosis is not apparent.13
an indented nucleus and a dense cytoplasm.7 Giant
melanin granules (macromelanosomes) may be seen in
Lentigines Profusa (Generalized Lentigines)
the melanocytes, keratinocytes, and macrophages.5
Clinical Picture. Numerous lentigines can be a cuta
Freckles (Ephelides)
neous sign of a systemic disorder, but occasionally may
appear in the absence of any associated abnormalities or
Clinical Picture. Clinically, freckles appear as light
triggering factors.2 Lentiginosis profusa that persisted for
brown macules on sun-exposed areas of the face, back
up to 20 years following an attack of measles was docu
of the hands, and forearms in individuals who have
mented by Darier in 1902; a similar phenomenon was
light, especially red hair and light skin;1,4,s but, they
observed by Gaucher and Milian in 1897 in a patient fol
can also be seen in people with blonde or brown hair,
lowing an episode of typhoid fever.14 The exact incidence
dark hair, and dark skin. The freckles in such people
of lentiginosis profusa following infection or exanthem is
are smaller and fewer.9 Freckles usually appear in the
not known, but appears to be a rare phenomenon. The
summer months but may persist throughout life.8 Ex
clinical appearance of the individual lesions of lentigi
amination of the skin with Wood's light may uncover
nosis profusa is similar to freckles, but the distribution is
freckles that are not evident in ordinary light.10 The
widespread. The lesions appear as small pigmented mac
distribution of the lesions is symmetric and they may
ules that are present at birth or arise during childhood or
be sparse or dense.9 The color varies from pale yellow
early adulthood.15 The lesions may be numerous and
to brown and even black, and tends to deepen after
widespread, involving the trunk, extremities, palms, geni
sun exposure. The borders are well defined and irregu lar
talia, and mucosa! surfaces such as conjunctiva, but the
and the diameter may be up to 5 mm.4,8 Freckles can
buccal mucosa and soles may be spared.15 The size of the
be induced by a single exposure to UVB in patients with
macules vary from 1 mm to 2 cm and the color is from
natural freckles.11
dark brown to black. The texture of the skin remains
Microscopic Findings. Histologically, rete ridges
normal and lesions are not palpable.15 The differential
are not elongated but there is an increase in the
pigment content in the basal cell layer; however, an diagnosis should include LEOPARD, LAMB, and NAME syn
increase in the number of melanocytes is not dromes which have other associated systemic features.
observed.4,5,u The density of dopa-reactive Microscopic Findings. The histologic picture is
melanocytes in split-epidermal sheets has been similar to that of a lentigo simplex; there is slight to
reported to be decreased in comparison to adjacent moderate elongation of the rete ridges and an increase
uninvolved skin or skin from the same site of different in the number of melanocytes as well as melanin in the
individuals.5,9 The melanocytes may be strongly dopa- melanocytes and kera tinocytes. Macromelanosomes
positive, larger, and have more dendritic may be present.2·15
230
Lentigo
Rahman and Bhawan
13654632, 1996, 4, Downloaded from https://onlinelibrary.wiley.com/doi/10.1111/j.1365-4362.1996.tb02994.x by University Of Ulsan, Wiley Online Library on [27/05/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Table 2. Clinical Features of Lentigines without Associated Systemic Anomalies
Variant Age Site Size Color Margins
Simplex Childhood
Any area 1-5 mm Brown-black Jagged/smooth
Freckles Childhood Sun-exposed Pale brown
1-5111111 Jagged
Generalized Birth to adult
Face, trunk, extremities 1111111-2 cm Brown-black Jagged/smooth
buttock/genitalia
Unilateral Early
Unilateral/segmental Variable Shades of Jagged
childhood Labial
Lips/oral mucosa Few mm brown Brown- Jagged
Adult black Tan to
Labia minora/majora, vulva Up to a few 15 111111 Jagged
Genital Women Penile glans corona/shaft Atypical penile lesions brown
Men up to 1 cm
Acral Childhood to adult Palms, soles 1-5111111 Brown Regular
Unilateral/Segmental Lentigines
multiple. Although the most common site is the lower
lip, lesions can also occur on the gingiva, buccal mu cosa,
Clinical Picture. Pigmented macules have been de palate, muco-buccal folds, and tongue.27 The age
scribed that are limited to one side of the body8 or of onset ranges from 25 to 71 years. A predominance of
arranged in a segmental, zosterifonn pattern.1•2•16 They women was observed by some authors;25•27 however, in
can be seen in people with dark hair and dark skin in another study men predominated.26 Differential diagno
the absence of exposure to the sun.8 The zosteriform sis can include the Laugier-Hunziker and the Peutz
pattern does not necessarily have a systemic manifesta Jeghers syndromes. Lack of involvement of other sites
tion;1•2 however, an association with diseases such as and associated systemic features should help in differen
sickle cell anemia,17 ipsilateral rigid cavus foot,18 men tiating labial melanotic macule from these syndromes.
tal deficiency,19 familial goiter,20 blue nevi,21 and Microscopic Findings. Histologically, hyperpara
plaque-type of blue nevus22 have been recorded. They keratosis and epithelial hyperplasia may be seen. There
have also been regarded as 'formes frustes' of neurofi is an increase in the pigment content of the basal
bromatosis.3•19 Lesions may be seen at birth or during layer.25-27 An increase in the number of dendritic
early childhood. In unilateral/segmental lentigines, cir melanocytes has been observed.27 Melanophages, acti
cumscribed dark macules appear on normal back vated fibroblasts, and scattered telangiectasias may
ground skin.8 This is important because it distinguishes also be seen.25•27
this entity from segmental nevus (nevus spilus), where
the background skin is hyperpigmented.1•2 The mac Genital Lentigines
ules may concentrate in the midline of the body form
ing a distinct border8 that is also seen in zosteriform Clinical Picture. Genital lentigines are seen in both
speckled lentiginous nevus. sexes. In men, the most common sites are the glans
Microscopic Findings. There is elongation of the penis, corona, corona sulcus, and the penile shaft. The
rete ridges with an increased number of melanocytes lesions have irregular borders and vary in color from
and melanin in the basal layer with melanophages in tan to brown to dark b_rown and skip areas are seen.
the upper dermis.17,19-21,23,24 The diameter of the individual lesions may be up to
Electron-Microscopic Findings. The melanocytes are
slightly increased in number and appear to contain
melanosomes at various stages of development. Large Table 3. Histologic Features of Lentigines
number of clumped melanosomes and membrane bound without Systemic Disorders
melanosome complexes are seen in the sur rounding
Le11tigo
basal keratinocytes, but not more than in the Finding Simplex Freckles Generalized Unilateral Mucosa/
uninvolved skin.17 EH + + + ± +
RR + + + + ±
Mucosal/Genital Lentigines: MEL + ± + ± +
Labial Melanotic Macule (Solitary Labial Lentigo)
PIG + + + + +
MP + + + +
Clinical Picture.Melanotic macules may be seen on
CI +
the lower lips in healthy adults without any systemic
SE
disorder.25 The term 'solitary labial lentigo' is used by
some,25 whereas others prefer the term 'labial melanotic EH = epidermal hyperplasia; RR = rete ridge elongation; MEL =
increased number of melanocytes; PIG = epidermal pigmentation;
macule'26,27 because these pigmented macules can be MP= melanophages; CI= cellular infiltrate; and SE= solar elastosis.
International Journal of Dermatology
Vol. 35, No. 4, April 1996
13654632, 1996, 4, Downloaded from https://onlinelibrary.wiley.com/doi/10.1111/j.1365-4362.1996.tb02994.x by University Of Ulsan, Wiley Online Library on [27/05/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
15 mm. They tend to increase in size and number and ULTRA VIOLET (UV)-INDUCED LENTIGINES
darken in color with psoralen-ultraviolet A (PUVA)
therapy.28,29 Penile lentigines should be differentiated The clinical and histologic features of various types of
from the atypical penile lentigines which are usually UV-induced lentigines are summarized in Tables 4 and 5.
larger than 1 cm in size.28,30,31 They may have irregular
borders with a variegated pigment pattern28,31 as well Solar Lentigines (Senile Lentigo, Senile Freckle,
as areas of depigmentation.29 Clinically, the atypical Liver Spots)
penile lesions have to be differentiated from melanoma.31
In women, these lentigines occur any where on the Clinical Picture. Solar lentigines occur as multiple
genital mucosa.28 They may appear as individual mac pigmented macular lesions on sun-exposed areas, and
ules or as a mottled, pigmented patch with skip areas, are commonly seen in middle-aged and elderly peo
the diameter can be up to S to 15 mm or larger. They ple.4,12 The age of onset is between the second and
may also appear in episiotomy scars and after child the
birth. Associated diabetes mellitus has been noted.28 ninth decade.34 The sites of predilection are the face,
Microscopic Findings. In genital lentigines the most arms, dorsa of the hands,35 and upper trunk.36 Solar
consistently recorded features in both sexes are epithelial lentigines increase slowly in number and individual le
hyperplasia (usually with elongated rete ridges), basal sions may increase in size.12 Ninety percent of the
cell hyperpigmentation, slight melanocytic hyperplasia, whites over 70 years are likely to have solar lentigines
and the presence of melanophages in the connective tis on the dorsa of the hands. Solar lentigines appear as
sue stroma. No cytologic atypia of the melanocytes is flat macules; the color varies from tan to brown with
seen.28,29 In contrast to the penile lesion, vulvar lentigines some variation.5,36 The margins may be smooth or ir
lack the prominent melanocytic dendrites.28 Occasional regular,36but are not as sharply defined as those of the
ly, a lymphoid infiltrate, stromal fibroplasia, and vascu junctional nevus.4 The size varies between 1 mm and 1
lar dilatation may be seen.28 Histologically, there is ep cm, but can also be greater than 1 cm. The lesions may
ithelial hyperpigmentation with slight increase in the coalesce to form larger plaques.12,36 They have a flat or
number of normal appearing melanocytes in penile lenti slightly depressed surface which may be cleaved by fine
go, whereas in the atypical penile lentigo the melanocytes wrinkles. Orifices of the eccrine gland and the pilary
may be normal,31 atypical,30 or dendritic with a large canal may be seen on the surface.35
number of melanophages in the upper dermis.30,31 Microscopic Findings. The epidermis is slightly
Electron-Microscopic Findings. Atypical nuclear acanthotic and the granular layer is preserved.12 The
features include an increase in size, hyperchromasia, rete ridges are elongated and may be either club
and indentation; markedly dendritic melanocytes have shaped36 or tortuous. Bud-like extensions can be seen
also been reported in penile lentigo.29 These findings between the rete ridges.12 The rete ridges may also ap
suggest a defect in melanosome transfer; however, pear crowded, show branching, and may fuse at the
these findings are difficult to interpret in view of lack base.35 The epidermis between the rete ridges may be at
of appropriate controls. rophic.5 There is a marked increase in pigmentation of
the basal cells in the proliferating rete ridges.12 The
Acral Lentigines melanocytes may be normal4 or increased in num
ber.2,12,35,36 In 1983, Rhodes et al. did a quantitative
Clinical Picture. In blacks, the paler skin of the study of dopa-incubated, paraffin-embedded sections of
palms and soles may show a variety of pigmentary solar lentigines from the backs of patients between the
changes, including mottled pigmentation, lentigines, ages of 24 to 71 years. They observed an increased num
nevi, and melanomas.32 Acral lentigines are pigmented
ber of melanocytes, concentrated along the club-shaped
macules, 1 to 5 mm in size, well circumscribed32 and
epidermal rete ridges with a density of 25 ± 7% as com
are seen on the palms and soles. They are more com
pared to adjacent normal skin (14 ± 3%). No signific nt
mon and more numerous in blacks and dark-skinned
difference was seen between the mean diameter of the
people, but may also be seen in whites. They may be
dopa-reactive melanocytes in solar lentigines as com
seen in children as well as adults.33 Some authors have
pared to the adjacent normal skin. Cellular atypia is not
claimed the evolution of malignant melanoma in acral
seen.37 The dermis contains elastotic material with
lentigines after 40 years,32 however, these reported
telangiectasias.4,34 There may be a mild dermal fibrosis
findings lack confirmation or regular follow-up and
and mjld to moderate inflammatory infiltrate with a few
may be regarded as accidental occurrences.
melanophages.2,5,36 Some authors believe that solar
Microscopic Findings. Acral lentigines are histo
lentigo is the same as a large-cell acanthoma, because
logically similar to lentigo simplex.32 The lesion shows the histologic features are similar, the only difference
elongation of rete ridges and diff se melanocytic hy
being that, in large-cell acanthoma, the keratinocytes are
perplasia in the basal layer. The melanocytes are elon larger in the lower half of the rete ridges.34 Others dis
gated and show pigmented, well-developed dendrites.32
agree pointing out that large-cell acanthomas are clini
cally more keratotic38 and paler in color than solar
231
232
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Rahman and Bhawan
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Table 4. Clinical Features of UV-Induced Lentigines
Variants Age Site Size Color Margins
Solar 2nd to 9th decade Sun-exposed 1-5111111 Tan-brown black Jagged
PUVA
Any Buttock/groin, chest, genitalia Pin head to 3 cm Shades of brown Jagged
Tanning-bed
Adults''· Acral, anterior legs, arms, chest 2-5111111 Brown-black Jagged
'' = cases reported only in adults.
lentigines and they differ histologically by the absence of appearance as in an 'ink spot.' They are seen in associa
the club-shaped proliferation of the rete ridges. These tion with numerous solar lentigines in individuals of
two lesions also have a different nuclear DNA content as Celtic origin and fair skin. Usually one black reticulate
measured by computerized microscopic image analysis. lentigo is seen amongst a sea of solar lentigines.40
The DNA index was found to be 1 in solar lentigo (simi Microscopic Findings. There is hyperplasia and
lar to the normal diploid lymphocyte control) and nor mal mild elongation of the epidermal rete ridges without
adjacent epidermis, whereas large-cell acanthomas any budding or tortuous appearance. There is marked
measured between 1.28 and 1.5.39 hyperpigmentation of the basal layer with skip areas
Electron-Microscopic Findings. The melanocytes involving rete ridges. Additionally, there may be a
are increased in number and occur as single cells or as min imal increase in the number of melanocytes as
small aggregates above the dermoepidermal junction. com pared to the normal adjacent skin. Mild solar
The keratinocytes are laden with melanosomal com elastosis in the upper dermis is observed in addition to
plexes and the size of these may vary, but they are a mild perivascular lymphocytic infiltrate. Dopa-
larger than those of the surrounding keratinocytes. In incubated, paraffin-embedded sections show prominent
the upper layers of the epidermis, numerous melano and thick er dendritic melanocytic processes compared
somes are seen, dispersed and not as complexes, indi
to adja cent normal skin.40
cating that not only is there an increase in melanin syn Electron-Microscopic Findings. The keratinocytes
thesis, but also a probable delay in the lysosomal of the involved area show an increased number of
destruction of the melanosomes. 5 In 1980, using a melanosomes within complexes as compared to the
scanning microscope on epidermal sheets, Montagna et normal uninvolved skin. The melanosomal complexes
al. 35 observed that the underside of a lentigo had contain 2 to 8 melanosomes as compared to 2 to 5 in
crowded epidermal rete ridges and columns fusing to the normal pale skin. The number of melanocytes ap
form a plate, pock-marked by craters, the resultant pear to be unchanged.40
patterns resembled chain-links from which columns
descended downward. Light oval bodies measuring 10 PUVA-Induced Lentigines
to 15 p with dendritic processes were seen at the tips
of the rete ridges. These bodies were lighter in color in
Clinical Picture. Individuals undergoing prolonged
the lentiginous areas as compared to the surrounding
PUVA-therapy for psoriasis may develop prominent, dis
noninvolved epidermis, where they appear like plates.
crete brown macules that have been termed freckles,41
These bodies represent melanocytes.35
stellate hyperpigmented freckles,42 or star-like hyper
pigmented lentigines.43 These are commonly seen on
Reticulated Black Solar (Ink Spot) Lentigo the upper part of the back and chest,42 buttocks, groin,
glans penis, and penile shaft with sparing of the palms,
Clinical Picture. Black reticulated solar lentigo is a soles, axillae and gluteal cleft.37 The color varies be
variant of the solar lentigo. They are seen on the sun-ex
tween light and dark brown.37•42 The size of the lesion
posed areas of the body and appear as very dark brown
varies between 3 and 8 mm,37 but, the stellate lesions
to black macules. They have an irregular outline and may measure up to 3 cm in diameter.42 Although these
may have a reticulate pattern giving a wiry or beaded lentigines may persist for 3 to 6 months after therapy
233
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International Journal of Dermatology
Vol. 35, No. 4, April 1996
235
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Rahman and Bhawan
number of melanocytes. This increased number of SYNDROMES ASSOCIATED WITH LENTIGINES AND MYXOMAS
melanocytes is primarily in the lower epidermis and to a
lesser extent in the upper epidermis; they are distributed A number of syndromes are associated with lentigines
singly or in the form of micro-nests.2 The melanocytes and with cardiac and mucocutaneous myxomas.48
are filled with melanosomes.2 Macro-melanosomes have These include the LAMB, NAME, and Carney's syn
been observed in keratinocytes as well as in melano dromes. They probably are variants of one syndrome
cytes.47 The histologic appearance of the pigmented that encompasses a variety of systemic anomalies.
macules in the Moynahan syndrome is similar to that of
the lentigines in the LEOPARD syndrome. LAMB Syndrome
Electron-Microscopic Findings. Ellipsoid melano
somes are the most commonly seen in the melanocytes Clinical Picture. A combination of Lentigines, Atrial
and keratinocytes with a size varying between 0.2 and Myxomas, Mucocutaneous myxomas, and Blue nevi
0.6 µm. The melanosomes are mostly single, but com constitute the LAMB syndrome.53 The lentigines are up
plexes may also be seen. Macro-melanosomes are seen to 10 111111 in diameter, brown in color, and favor the
in both keratinocytes and melanocytes. They are spheri face, lips, sclerae, and vulva.53 The mucocutaneous
cal and measure up to 2 µm in diameter and may or myxomas appear as papules or dermal nodules and can
may not have a membrane. Vesicles (30 to 40 µm in di appear at various sites of the body, including the oral
ameter) can be seen and are characterized by three mucosa, tongue, genitalia, breast, and shoulders.53 Car
zones: a central core of dense amorphous material, diac myxomas usually occur in the form of atrial myxo
translucent vesicles, and a thin outer layer having vesi mas. They are rarely seen in childhood. They may man
cles within a clear matrix. In addition, complex melanin ifest as intermittent valvular obstructions and embolic
granules and spherical granular melanosomes have been episodes. Benign thyroid nodules may be associated.
observed in keratinocytes and melanocytes.47 Microscopic Findings. Elongated epidermal rete
ridges are seen associated with marked
Peutz Jeghers Syndrome hyperpigmenta tion of the basal cell layer. The
melanocytes may have large dendritic processes and
Clinical Picture. This is an autosomal dominant dis
melanophages may be
order with a high degree of penetrance, characterized
seen in the upper dermis.53
by gastrointestinal polyps and pigmented macules
Electron-Microscopic Findings. An increased con
commonly seen on the oral mucosa, lips, face, palms,
centration of stage IV melanosomes, either singly or in
soles, and dorsa of the hands.3 The pigmented macules
complexes, may be seen in the melanocytes.53
are present at birth or appear in early childhood and
the oral mucosa is always involved.
NAME Syndrome
The pigmented lesions are usually round to oval in
shape but can form irregular patches. The color varies
Clinical Picture. This syndrome may be a variant of
from brown to black. The size varies between 1 and 5
the LAMB syndrome which is composed of Nevi, Atrial
mm. They may be distributed on the gums, hard
myxoma, Myxoid neurofibroma, and Ephelides.s4,ss
palate, lips, palms, and soles. Rarely the nails may be
The pigmentary lesions are in the form of multiple flat
pigmented. The macules may disappear over time, but
macules beginning at bii;th and showing an accentua
the oral pigmentation does not.1-3 The associated
tion in the summer. The sites involved are neck, trunk,
polyps are found throughout the intestine, more
and thighs with the color varying from pale brown to
often in the jejunum and ileum and less frequently
dark brown. Pigmented macules may be seen on the
in the
palms and soles.55
colon, rectum, stomach, and duodenum. The polyps
Microscopic Findings. There is increased pigmen
are benign hamartomas and result in recurrent abdom
tation of the basal and spinous layers of the epidermis.
inal pain and perirectal bleeding. The risk of an adeno
Whether hyperplasia of the epidermal rete ridges exists
carcinoma is slight.2 Associated features include club is debated in the reported cases.54,55
bing of the fingers and ovarian tumors, especially
granulosa theca cell tumors.1•2 Carney's Syndrome
Microscopic Findings. The histopathologic ap
pearance of the lentigines show marked hyperpigmen Clinical Picture. This syndrome comprises cardiac,
tation of the basal cell layer, especially of lentigines on cutaneous, and mammary myxomatous masses; lentig
the lips; however, there may be no increase in the num ines and blue nevi; endocrine disorders and testicular
ber of melanocytes. 5 In the pigmented areas, the tumors.56,57 The involvement of organs is often multi
melanosomes are seen within the keratinocytes rather centric and bilateral and usually occurs in young pa
than within melanocytes. Pigmented macules on the tients.57 The cardiac myxomas may be single or multi
fingers show many melanosomes within the dendrites ple and may result in fibrosis or calcification. Cuta
of the melanocytes and a few in the keratinocytes.L,5 neous myxomas are seen commonly on the eyelids;
236
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International Journal of Dermatology
Vol. 35, No. 4, April 1996
other sites include nipple, scalp, face, ears, neck, are of various sizes. They may be solitary or grouped.59
trunk, limbs, and perineum. Oral lesions may be
seen.56 En
docrine involvement include adrenocortical disease
leading to Cushing's syndrome, pituitary adenomas
with acromegaly and gigantism, and calcifying pig
mented neuroectodermal tumors.56•57Testicular tumors
include Sertoli cell tumors, Leydig cell tumors, and
adrenocortical rest tumors. Features of mammary in
volvement include gynecomastia, generalized enlarge
ment of the breast, and myxoinatous alteration of the
stroma. Two types of pigmented lesions are seen. They
may appear as blue nevi or lentigines. Lentigines mea
sure 0.2 to 2 mm in diameter, are brown to dark
brown to black in color, round, with irregularly
shaped or jagged margins. The distribution is wide
spread, primarily on the face, eyelids, ears, vermilion
borders of the lips, trunk, conjunctiva or sclera, vulva,
extremities, and back of the hands. Other sites include
the scalp, finger tips, the anal verge, and glans penis.
The lesions may coalesce to form brown patches.56
Microscopic Findings. Histologically, the lentig ines
show hyperpigmentation of the basal layer and
melanocytic hyperplasia with or without elongation of
the rete ridges.56
Laugier-Hunziker Syndrome
Xeroderma Pigmentosa
Bannayan-Riley-Ruvalcaba Syndrome
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Vol. 35, No. 4, April 1996
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