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Department of Dermatology STD & Abstract
Leprosy, and 2Department of Pathology, Telangiectasia, or dilated blood vessels, may represent a benign condition or a marker of a
P.G.I.M.E.R. and Dr Ram Manohar Lohia
much more serious internal or cutaneous malignancy (e.g. mycosis fungoides). They can
Hospital, New Delhi, India
be generalized or localized in distribution. ‘Localized telangiectasia’ can be macular or
Correspondence papular. Macular ones can be further divided into two major categories: primary and
Ruchi Gupta, MD secondary. They can occur on their own (primary), after skin injury (e.g. sun damage, post-
Senior Resident radiation therapy, after prolonged topical steroid application), or may arise due to an
Department of Dermatology STD & Leprosy
underlying condition (secondary). While telangiectases can spring from a multitude of
P.G.I.M.E.R. and Dr Ram Manohar Lohia
Hospital
possible causes, the location of the lesions, in combination with a careful history and
Baba Kharag Singh Marg, exmination, often helps in elucidating the correct diagnosis. A patient with localized
New Delhi 110001 telangiectasia as a manifestation of malignancy may be difficult to diagnose, however the
India diagnosis is one of exclusion, and a thorough evaluation should be performed before
E-mail: ruchi85mon@gmail.com
diagnosing this condition. This article reviews possible conditions to consider in these
patients and suggests an approach to their evaluation.
Conflicts of interest: None.
Funding support: None.
International Journal of Dermatology 2015, 54, e294–e301 ª 2015 The International Society of Dermatology
Gupta et al. Diagnosing patients with localized telangiectasia Report e295
(a)
(b)
ª 2015 The International Society of Dermatology International Journal of Dermatology 2015, 54, e294–e301
e296 Report Diagnosing patients with localized telangiectasia Gupta et al.
Figure 3 An approach to a patient with localized macular telangiectasia. CM, capillary malformation; CTCL, cutaneous T-cell
lymphoma; DLE, discoid lupus erythematosus; TMEP, telangiectasia macularis eruptiva perstans
eral dermatomal or linear distribution that can be con- tion. Occurrence in a dermatomal pattern has been
genital or acquired. The underlying mechanism is believed reported.6
to be somatic mosaicism, which is unmasked by a sys- The costal fringe consists of a band-like pattern of
temic increase in estrogen levels exhibited during preg- venous telangiectasias along the anterolateral costal mar-
nancy, puberty, and alcoholic cirrhosis.3 The literature gins (Fig. 4). It is an easily recognized entity, usually seen
includes reports of UNT associated with hyperthyroid- in elderly men. Association with an underlying disease is
ism,4 while other cases showed no associated abnormali- not well established. Sartori et al.7 examined 1523 young,
ties.5 Dermatomes C3–D1 are the most commonly seronegative, active duty air force personnel (1203 men
involved sites.3–5 Histologically, there are multiple and 320 women) between the ages of 17 and 34 years.
dilated, thin-walled vessels in the papillary and upper The costal fringe was present in only three men (0.2%).
reticular dermis with notable absence of mast cells. Our They concluded that the costal fringe is a rare finding in
patient fulfilled all clinical criteria of UNT, i.e., multiple healthy young adults. On the other hand, linear telangiec-
blanching telangiectasias primarily in a T2 dermatomal tasias in a broad, crescent-like distribution across the
distribution in a young female during the postpartum per- chest are a relatively common finding in homosexual men
iod. If fewer spindle-shaped mast cells were seen on tolui- and are significantly, although not exclusively, associated
dine blue staining, we could have diagnosed our case as with HIV infection.8 Leg veins encountered in the early
UNT. stage of chronic venous disease is an important differen-
Spider nevus is another common telangiectatic condi- tial of venous telangiectasias, i.e., C1 stage of venous dis-
tion frequently associated with a high estrogenic state. It ease of legs as per CEAP (Clinical severity, Etiology,
is usually seen in pregnancy and is frequently associated Anatomy, and Pathophysiology) classification. Superficial
with liver disease but may be encountered in up to 15% spider veins (reticular veins) are seen over the lower leg
of normal people. An arcade of vessels radiating out from in this condition. At this stage, the telangiectasias can be
a central arteriole is the most characteristic clinical fea- managed with non-surgical modalities such as the intense
ture. The upper half of the body, especially the face, pulsed light system (IPLS). A few studies have reported
neck, upper chest, and hands, is the usual site of predilec- the successful use of IPLS for the treatment of leg telangi-
International Journal of Dermatology 2015, 54, e294–e301 ª 2015 The International Society of Dermatology
Gupta et al. Diagnosing patients with localized telangiectasia Report e297
ª 2015 The International Society of Dermatology International Journal of Dermatology 2015, 54, e294–e301
e298 Report Diagnosing patients with localized telangiectasia Gupta et al.
post-capillary venules, sometimes they may involve dilata- a case of PAV, the spectrum of investigations should
tion of dermal capillaries leading to diffuse erythematous include tests for any underlying systemic disease, lym-
appearance, and thence clinicians should be aware that phoma, or connective tissue disorder.23 Old age and
an apparent acquired PWS may be an early manifestation refractoriness to treatment should raise the suspicion of
of localized morphea.17 premalignant PAV. When no underlying condition is
In 1888, Radcliffe Crocker18 first described a telangiec- found on multiple skin biopsies, the condition is labeled
tatic form of lupus erythematosus characterized by sym- as sclerodermiform plaque-like essential telangiectasias or
metric persistent circumscribed patches on both cheeks, Brocq’s telangiectasias. Recently, an etiological classifica-
which on palpation were decidedly thickened and shot tion with the key differentiating features for each individ-
through with dilated blood vessels. The extensor surface ual cause, to help in making a precise diagnosis of
of the hands was the seat of patchy persistent erythema, different causes and a step-by-step approach to the man-
resembling chilblain, which after involution showed atro- agement of patients with acquired poikiloderma, has been
phic scarring. According to Crocker, the patches on the described in detail in the literature.24
cheek simulated the red paint on a clown’s face. Under Sarcoidosis is a multisystem granulomatous disorder
the term erysipelas perstans faciei, Kaposi,19 in 1872, also with protean manifestations ranging from self-limited skin
brought attention to the deep red patches on the face lesions to intractable organ failure. Among the protean
with purpura preceding the onset of lupus erythematosus manifestations of cutaneous sarcoidosis, including macu-
acutus disseminatus. Lupus erythematosus telangiectoides lopapules, nodules, plaques, subcutaneous nodules, infil-
is a variant of disseminated discoid lupus erythematosus, trative scars, and lupus pernio, the angiolupoid
characterized by a persistent, blotchy, reticulate telangiec- sarcoidosis is a rarely reported variant characterized by
tasia, which occurs on the face, neck, ears, dorsa of the erythematous plaques with superficial telangiectasia on
hands, breasts, heels, and on the sides of the feet.20 Cases the face, particularly in paranasal areas. It results in
with localized lesions are on record.21,22 Other disorders severe disfigurement and can cause profound social
with telangiectasia on the face such as rosacea, carcinoid, embarrassment. Since its first description in 1913, only a
seborrheic dermatitis, hereditary hemorrhagic telangiecta- few cases had been reported in the English language liter-
sia (history of oro-nasal bleed and involvement of ature.25 Tsai et al.26 reviewed the dermatological files in
mucosa), and bloom syndrome (history of photosensitivi- Chang Gung Memorial Hospital over 16 years and found
ty) may be differentiated by the absence of skin atrophy. only eight cases with consistent features of angiolupoid
Facial telangiectasia due to prolonged steroid application sarcoidosis. They suggested that angiolupoid sarcoidosis
(history of topical application of high potency steroid) is a rare but real variant of sarcoidosis. Interestingly, the
and angiolupoid sarcoidosis can be differentiated by clas- incidence of pulmonary and ocular involvement is report-
sical histologic findings. The histology of this type of edly low.26–28 However, more cases are needed to con-
lesion shows an atrophic epidermis, with dilatation of the firm the optimistic conclusions. In spite of being
superficial vessels of the skin and slight infiltration of the asymptomatic, treatment of angiolupoid sarcoidosis is
papillary part of the corium. Vacuolization of basal cells imperative due to the severe disfigurement disturbing the
and thickening of the basement membrane helps in mak- patients. Although diverse methods of treatment have
ing the diagnosis. been tried, including the systemic, topical, and intrale-
Poikiloderma is a morphologic and descriptive term sional glucocorticoids, doxycycline, hydroxychloroquine,
referring to a combination of cutaneous atrophy, telangi- and antituberculosis agents, none of them are definitive.
ectasia, and varied macular pigmentary changes that The differential diagnosis of angiolupoid sarcoidosis
result in a mottled skin appearance. Its etiology includes includes lupus pernio, chilblain lupus erythematosus, and
both congenital and acquired causes. PAV is characterized mycobacterial (including leprosy) and fungal infections.
by one or a limited number of patches (composed of tel- Hitherto, the natural progression of lesion along with the
angiectasia, atrophy, and hyper/hypopigmentation) espe- histopathological features is of utmost importance in
cially over the breasts or buttocks (limb girdle area). It making one diagnosis over another. Lupus pernio charac-
may be an idiopathic disorder or a manifestation of con- teristically involves the tip of the nose, does not show any
nective tissue diseases (lupus, dermatomyositis, sclero- tendency of resolution, and is associated with severe pul-
derma), lymphomas (parapsoriasis en plaques and monary involvement. Angiolupoid variant, on the other
mycosis fungoides), and genodermatoses (Rothmund– hand, is distinctive with predominant involvement of the
Thomson syndrome, hereditary sclerosing poikiloderma, cheeks and relative absence of systemic involvement. In
dyskeratosis congenita). Other causes include physical addition, the presence of multiple dilated blood vessels
trauma (radiodermatitis, burns, freezing) and certain in the papillary dermis in addition to paucilymphocytic
ingested substances such as arsenic compounds. Hence, in non-caseating granulomas is enough to differentiate it
International Journal of Dermatology 2015, 54, e294–e301 ª 2015 The International Society of Dermatology
Gupta et al. Diagnosing patients with localized telangiectasia Report e299
from other granulomatous diseases. Similarly, chilblain skin, and mucous membranes often rupture and bleed
lupus and infectious causes can be easily differentiated by after slight trauma. The most common clinical manifesta-
the presence of hydropic degeneration of basal cells and tion is spontaneous and recurrent nosebleeds (epistaxis)
the infectious organisms, respectively. beginning on average at age 12 years.33
Topical corticosteroid-induced rosacea-like dermatitis
(TCIRD) is an entity that starts after discontinuing potent
Conclusion and recommendations
topical corticosteroid after its application for a consider-
able time. It is a rebound phenomenon, which usually As even benign-looking telangiectasias can be a marker of
appears on the face in a perioral, diffuse, and centrofacial underlying systemic disease and result in profound disfig-
distribution. It may or may not be associated with skin urement, picking the correct diagnosis out of the basket
atrophy. The majority presents with a history of exacer- requires a systematic approach. When associated with
bation of symptoms after sun exposure, most probably skin atrophy, most telangiectasias are secondary in origin.
due to atrophy and vasodilatation of the facial skin. Inter- Although history and location of lesion are usually help-
estingly, eye problems are reportedly absent.29 Treatment ful in elucidating the correct diagnosis in disorders with
involves a fine balance between gradual tapering to com- primary telangiectasia, most secondary telangiectasias
plete cessation of the topical steroid and addition of oral require a skin biopsy for final confirmation. Patients with
anti-inflammatory antibiotics and/or topical antibiotics. potentially more serious conditions such as PAV need
observation and multiple biopsies before the diagnosis of
Secondary telangiectasia without skin atrophy cutaneous T-cell lymphoma is ruled out.
Conditions such as TMEP, rosacea, carcinoid, seborrheic Although treatment results may not be permanent for most
dermatitis, and hereditary hemorrhagic telangiectasia of these cases, for cosmetically sensitive areas of the body,
(HHT) present with linear telangiectasia on the face with- gentle cautery can be used for a small number of lesions.
out any skin atrophy. TMEP is a rare form of cutaneous Laser therapy is also an effective treatment and can be useful
mastocytosis characterized by multiple symmetrically in those having a larger number of lesions. We treated our
scattered telangiectatic macules primarily involving the patient with pulsed dye laser (585 nm, at 2 maximum) for
trunk, extremities, or face. Histologically, minimal mono- three months, but it failed to improve her eruptions.
nuclear infiltrate, containing mast cells around the capil-
laries, are characteristic. Special stains, such as Giemsa or
Multiple choice questions
toluidine blue, highlight the presence of mast cells, reveal-
ing their metachromatic cytoplasmic granules. Unilateral 1 Macular telangiectasias most commonly arise from
TMEP over the face30,31 and breast32 have been reported which part of the cutaneous vasculature
previously, and to our knowledge, the illustrated case of a Capillary
linear unilateral TMEP involving the lateral wall of the b Postcapillary venules
axilla (T2 dermatome) in this review has never been c Small collecting veins
reported. In such unusual cases, it is all the more important d Precapillary arterioles
to have a systematic approach to avoid misdiagnosis. 2 All of the following conditions mostly involve above
Association with flushing and systemic symptoms such the waist area except
as diarrhea should raise the suspicion of carcinoid syn- a Acquired nevoid telangiectasia
drome, which is seen in 5% of cases with carcinoid b Stork bite
tumors of the gastrointestinal tract. Rosacea usually has a c Angioma serpinginosum
typical unmistaken distribution and morphology. Associa- d Costal fringe
tion with dandruff and yellow greasy scales are a pointer 3 In which of the following conditions are telangiectasias
towards seborrheic dermatitis. Similarly, telangiectasia are associated with skin atrophy?
mainly over the lips and oral mucosa and sometimes on a Purpura annularis telangiectoides
the face and dorsum of the hands in association with a b Rosacea
nosebleed are seen in HHT. It is characterized by the c Brocq’s telangiectasia
presence of multiple arteriovenous malformations. d Spider nevi
Although HHT is a developmental disorder, caused due 4 All of the following are true about telangiectasia mac-
to mutation in germline genes, endoglin, and ALK-1, ularis eruptiva perstans except
infants are occasionally affected. In most people, the fea- a Most common form of cutaneous mastocytosis
tures are age-dependent, and the diagnosis is not sus- b Characterized by red tan macules less than 0.5 cm
pected until adolescence or later. Small arteriovenous in diameter
malformations (telangiectasias) close to the surface of the c Most common site of involvement is upper trunk
ª 2015 The International Society of Dermatology International Journal of Dermatology 2015, 54, e294–e301
e300 Report Diagnosing patients with localized telangiectasia Gupta et al.
International Journal of Dermatology 2015, 54, e294–e301 ª 2015 The International Society of Dermatology
Gupta et al. Diagnosing patients with localized telangiectasia Report e301
25 Rongioletti F, Bellisomi A, Rebora A. Disseminated 33 Begbie ME, Wallace GMF, Shovlin CL. Hereditary
angiolupoid sarcoidosis. Cutis 1987; 40: 341–343. haemorrhagic telangiectasia (Osler-Weber-Rendu
26 Tsai H-J, Kuan Y-Z, Wong W-R, et al. Angiolupoid syndrome): a view from the 21st century. Postgrad Med J
sarcoidosis: a clinicopathological study of a distinct 2003; 79: 18–24.
variant of cutaneous sarcoidosis. Dermatol Sinica 2003;
21: 113–118.
27 Heinemann B. Uveitis with Brocq-Pautrier angiolupoid
(Boeck disease of the skin) [German]. Klin Monatsbl Answers
Augenheilkd 1990; 196: 415–416.
1b
28 Mana J, Marcoval J, Graells J, et al. Cutaneous
2c
involvement in sarcoidosis. Relationship to systemic
disease. Arch Dermatol 1997; 133: 882–888.
3c
29 Rathi SK, Kumrah L. Topical corticosteroid-induced 4a
rosacea-like dermatitis: a clinical study of 110 cases. 5c
Indian J Dermatol Venereol Leprol 2011; 77: 42–46. 6d
30 Fried SZ, Lynfield YL. Unilateral facial telangiectasia 7d
macularis eruptiva perstans. J Am Acad Dermatol 1987; 8a
16: 250–252. 9c
31 Gonzalez-Castro U, Luelmo-Aguilar J, Castells- 10 a
Rodellas A. Unilateral facial telangiectasia macularis
eruptive perstans. Int J Dermatol 1993; 32: 123–124.
32 Patel N, Stranahan D, Fenske NA. Unilateral
telangiectasia macularis eruptiva perstans of the breast.
Cutis 2012; 90: 26–28.
ª 2015 The International Society of Dermatology International Journal of Dermatology 2015, 54, e294–e301