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Original Article

Clinicoepidemiological study of pigmented

purpuric dermatoses
Lata Sharma, S. Gupta
Department of
Dermatology and
Venereology, Institute
of Medical Sciences,
Banaras Hindu University,
Varanasi, Uttar Pradesh,
India

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ABSTRACT
Background: Pigmented purpuric dermatoses (PPD) are a group of vascular disorders with varied manifestations
which cause concern and are resistant to treatment. The literature is still lacking in clinicoepidemiological
studies. Aim: To study the epidemiology, etiological, host and environmental factors, clinical manifestations,
its variations, and the type prevalent in this part of the world. Materials and Methods: All cases of PPD were
selected for the study from Skin and Venereal Disease, Out Patient Department between January 2008 and June
2009. Their history, examination, hematological investigations, and, in a few, histopathology findings were also
recorded and data obtained were evaluated statistically. Results: There were 100 cases of PPD of total 55 323
patients (0.18%). There were 79 males and 21 females between 11 and 66 years. They were working as police
men, security guards, barber, chemist, teachers, students, farmers, businessmen, and housewives. In a majority,
there was a history of prolonged standing in day-to-day work. Purpuric, brownish pigmented, lichenoid or
atrophic lesions were seen depending upon the type of PPD on lower parts of one or both lower limbs. Blood
investigations were normal. Schambergs disease was seen in ninety five, Lichen aureus in three, lichenoid
dermatosis and Majocchis disease in one case each. Discussion: Three clinical types of PPD were diagnosed
which may represent different features of the same disease. Cell-mediated immunity, immune complexes, capillary
fragility, gravitational forces, venous hypertension, focal infection, clothing, contact allergy to dyes, and drug
intake have been incriminating factors in the past. Patients occupation and environmental factors may also be
considered contributory in precipitating the disease. Conclusions: The study revealed the problem of PPD in
this geographical area, its magnitude, clinical presentation, the type prevalent, and possible aggravating factors
to be kept in mind while managing the disease.
Key words: Aggravating factors, clinicoepidemiological study, pigmented purpuric dermatoses

DOI: 10.4103/2229-5178.93486
Quick Response Code:

Address for
correspondence:
Dr. Lata Sharma,
Department of
Dermatology and
Venereology,
Institute of Medical
Sciences, Banaras Hindu
University,
Varanasi 221 005,
Uttar Pradesh, India.
E-mail: lataims@
rediffmail.com

INTRODUCTION
Pigmented purpuric dermatoses (PPD) are
a group of vascular disorders with varied
manifestations. They are chronic, progressive,
cause concern, and are resistant to treatment.
They include (1) Schambergs disease (SD),
(2) Itching purpura (Eczematid like purpura
of Doucas and Kapetanakis), (3) Pigmented
purpuric lichenoid dermatosis (PPLD) of
Gougerot and Blum, (4) Lichen aureus (LA),
and (5) Purpura annularis telengiectoides or
Majocchis disease (MD). Though PPD is known
since 1901 when Schamberg first published a
report,[1,2] the literature is still lacking in large
clinicoepidemiological studies.

MATERIALS AND METHODS

The cases of PPD were selected from Skin

Indian Dermatology Online Journal - January-April 2012 - Volume 3 - Issue 1

and Venereal Disease Out Patient Department

between January 2008 and June 2009. There
were 100 (0.18%) cases of PPD which were
selected of 55 323 patients. History of cases
was recorded on a pretested proforma which
included place of residence, occupation, chief
complaints with duration, site of initial lesions,
associated symptoms, progress of the disease,
history of local application type of clothing,
bleeding tendency, photosensitivity, heat or cold
intolerance, abdominal pain, polyuria, polyphagia,
polydipsia, joint pain and urine discoloration,
and occupation involving prolonged standing..
History of any other disease or drug intake
was also recorded. History of hematological
disorders, diabetes, hypertension, hepatitis,
hyperlipidemia, rheumatoid arthritis, lupus
erythematosus or thyroid dysfunction, joint pain,
and photosensitivity in the past along with family
history were recorded.

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Sharma and Gupta: Clinicoepidemiological study of PPD

A general, systemic, and dermatological examination of the

subjects was done after proper exposure in good daylight.
The site, distribution and type of skin lesions, their color,
discrete or confluent, palpable or non-palpable, blanching,
telangiectasia, lichenoid eruptions, or excoriations were
recorded along with examination finding of mucosa, nails,
hairs, and scalp for the presence of any other disease. Other
diseases like stasis dermatitis, Henoch-Schonlein purpura,
leukocytoclastic vasculitis, purpuric clothing dermatitis, purpuric
generalized lichen nitidus, hyperglobulinemic purpura, drug
hypersensitivity reaction, non-accidental injury, self-inflicting
hematoma, or suction cup, where lesions may resemble LA,
were differentiated.
Hematological investigations, hemoglobin, total and differential
leukocyte count, platelet count, erythrocyte sedimentation rate,
and bleeding and clotting time, were advised in every case and
blood sugar, fasting and postprandial, in patients with personal
or family history of diabetes. Biopsy was also done in few cases
to confirm the diagnosis.

RESULTS
Cases of PPD were 0.18% of total patients (55323). Of 100
cases, 79 were male and 21 were female; ratio was 3.8:1
(X2 9.691, P value = 0.08, not significant). Age of the patients
varied from 11 to 66 years, mean was 34.11 12.24 years. The
mean age for males was 35.05 12.68 years and for females
was 32.47 12.18 years.
Eighteen patients had onset of lesions before 20 years (four
cases from 11 to 15), 54 between 21 to 40 years, and 28 above
40 years. Age-sex distribution of PPD patients (X2 9.691, P
value = 0.08) was not significant. There were 24 students,7
teachers, 28 servicemen, 26 businessmen, and 15 house
wives. Patients from Uttar Pradesh were 88 (Varanasi 35), 11
from Bihar, and one from Jharkhand.
Patients complained of brown pigmented spots, two on one
and 98 on both lower limbs. Itching was associated in 30 cases
without any diurnal variation. It was intermittent in 27 and
severe and continuous in 3. Lesions were distributed on legs,
ankles, and feet in 66, ankles and feet in 21, legs and ankles
in 7, only ankles in, and feet in 2. Age of onset was less than
20 years in 18, 21 to 40 years in 54, and more than 40 years
in 28 patients. Duration of lesions was up to 1 year in 52, 1 to
2 years in 26, and more than 2 years in 22 (X2 16.208, P value
= 0.039, significant).
Cases were not aware of any aggravating factors for PPD,
though 54 cases who had white collar occupation were of
the opinion that their prolonged standing occupation wearing
socks and shoes during exercise or work all through the year
could be a contributory factor.

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One case of SD gave history of taking glipizide for diabetes for

3 months before he developed light brown spots with itching
over ankle and feet. None of the patients gave history of local
application of any medication or oil over lower limbs. Three
patients between 38 to 46 years of age were hypertensive and
were on irregular treatment with antihypertensive drugs. Family
history of diabetes was obtained in 2 and of PPD in 3 patients.
Pallor, icterus, cyanosis, clubbing, edema, and lymphadenopathy
were not evident in any of the patients. One serviceman and two
businessmen had hypertension. One case had onychomycosis
of toenails and one had scalp psoriasis. In 74 males and 21
females, light or dark brown pinpoint macules and purpuric
lesions over anterior aspect of lower limb were non-blanching,
bilateral and symmetrical, SD was diagnosed [Figure 1].
Itching was present in 27. In three males, rust-colored patches
along the medial side of lower limb, bilaterally symmetrical,
nonpalpable, and non-blanching LA was diagnosed [Figure 2].
One patient had itching. In 42-year-old male, tiny dark brown
lichenoid papules with purpura, coalescing to form plaques
with mild itching over lower limb PPLD was diagnosed. In
a 32-year- policeman, itchy, annular erythematous to dark
brown plaques and patches with central areas of atrophy,
symmetrically distributed over leg, ankle, and feet MD was
diagnosed [Figure 3].
Only 42 patients reported with investigations. No significant
abnormality was noted. Three patients between 34 to 46 years
of age were found to be diabetic. Punch biopsy was done in
three cases which showed perivascular lymphocytic infiltrate
over superficial blood vessels, swelling of endothelial cells,
narrowing of the lumen, and mild extravasations of red blood
cells with hemosiderin deposition in upper dermis [Figure 4].
Patients were advised vitamin C, topical corticosteroids,
and antihistaminic if itching and in few cases, griseofulvin or
photochemotherapy with 8-methoxypsoralen. They were also
asked to elevate legs during rest and avoid prolonged standing,
wearing tight shoes and shocks in hot humid climate. Of the 42
cases who reported with investigations, 36 were on follow-up
for 4 months; in few, purpuric lesions disappeared or became
pigmented and they observed slight improvement for short time.
Of 6 patients who were on follow-up for 6 months, the disease
was progressive in 2 and in4 there were no noticeable changes.

DISCUSSION
PPD is known to occur in all races.[2] In this study, there were
0.18% PPD patients of total Skin and Venereal Disease
outpatients of Sir Sunderlal Hospital which is a tertiary
care hospital providing comprehensive medical care to the
neighboring states and Nepal. It is a referral center situated in
Varanasi city which has a humid subtropical climate with high
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Sharma and Gupta: Clinicoepidemiological study of PPD

Figure 1: Schambergs disease in 21-year-old female showing

pigmented and purpuric lesions involving both ankles and discrete
lesions on left leg

Figure 2: Lichen aureus in 34-year-old male showing rust-colored

patches over lower legs bilaterally

Figure 3: Majocchis disease involving both legs symmetrically in

32-year-old male

Figure 4: Histopathology, H and E, 200 showing normal epidermis.

Upper dermis shows mild sclerosis (a) perivascular lymphocytic infiltrate
with (b) hemosiderin deposition and few intact RBCs around the blood
vessels in the upper dermis

variation between summer and winter (average temperature,

32 to 46oC in summers). There are no data available on any
effect of environmental factors in this disease.
Age of patients varied from 11 to 66 years. SD was the
commonest in males and number was highest between 21
to 40 years. Though LA and MD may occur in children and
young adults, PPLD is said to present in adults.[2,3] Family
history of SD was present in three cases which has also been
reported earlier.[4] In this study, most common prolonged
standing occupation were servicemen and businessmen, which
constituted 54% of cases.
Most of the PPD patients were asymptomatic but 30 patients
had pigmented spots (give a more scientific description.
I presume most were macules) with itching. Itching was
Indian Dermatology Online Journal - January-April 2012 - Volume 3 - Issue 1

intermittent in 27, severe and continuous in three patients

with no diurnal variation. Skin lesions were confined to lower
extremities only.[2] Drugs have been frequently reported to be
provocating factors in SD and diabetes is said to be associated.
In present study, three patients were hypertensive, three
diabetic, and there was history of taking glipizide in one case.[5]
As suggested by the authors, PPD commonly manifests as a
pigmented spots(do not use the word spot) bilaterally. In most
cases, the rash is symmetrical, petechial, and macular and
occasionally, telangiectasias, brown, red, or yellow patchy
pigmentation are seen. Unilateral presentation was found in
two cases, which is said to be rare.[6]
Various morphological patterns of PPD are said to represent

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Sharma and Gupta: Clinicoepidemiological study of PPD

different morphological patterns of the same disorder with

a similar histopathology in which there is disturbance in the
cutaneous blood vessels or humoral or cellular immunity.[7]
Gravity and increased venous pressure are important localizing
factors. There is extravasation of erythrocytes in the skin of lower
limb, mostly with marked deposition of hemosiderin in absence
of associated venous insufficiency or hematologic disorders.[1,8]
Exercise, capillary fragility gravitational dependency, focal
infections, clothing, contact allergy to dyes, and chemical
ingestion are said to influence disease presentation.[1,2] LA
has been found to be associated with trauma and hepatitis.[9]
In a study done in this hospital on lichen planus where 119
cases and equal number of controls were studied for hepatic
functions, 78 cases and 38 controls were found to have hepatic
dysfunction, but only two cases were positive for hepatitis B
surface antigen and all other cases and controls were negative
for hepatitis C.[10]
Spontaneous improvement after few months is said to be usual,
but recurrences have been found to occur.[1] It is rather resistant
to treatment. Though the diagnosis is quite straightforward, the
disease entity remains an enigma and a therapeutic challenge.[2]
There was lack of such detailed clinicoepidemiological study
of PPD with host, environmental factors, and hematological
parameters. Large-scale prospective and retrospective studies
are needed with a longer follow-up to find out the incidence,
prevalence, etiology, aggravating factors, and associations.
This study may provide an initiative for creating awareness for
diagnosing PPD cases and to find effective treatment.

CONCLUSION
The study revealed the problem of PPD in this geographical

area, its magnitude, clinical presentation, the type prevalent,

and possible aggravating factors to be kept in mind while
managing the disease.

REFERENCES
1.

Cox NH, Piette WW. Purpura and microvascular occlusion. In: Burns
DA, Breathnach SM, Cox NH, Griffiths CE, editors. Rooks Textbook of
Dermatology. 8th ed. Oxford: Blackwell Publishing Ltd.; 2010. p. 49.22.
2. Sardana K, Sarkar R, Sehgal VN. Pigmented purpuric dermatoses: An
overview. Int J Dermatol 2004;43:482-8.
3. Tristani-Firouzi P, Meadows KP, Vanderhooft S. Pigmented purpuric
eruptions of childhood: A series of cases and review of literature. Pediatr
Dermatol 2001;18:299-304.
4. Sethuraman G, Sugandhan S, Bansal A, Das AK, Sharma VK. Familial
pigmented purpuric dermatoses. J Dermatol 2006;33:639-41.
5. Adams BB, Gadenne AS. Glipizide-induced pigmented purpuric
dermatosis. J Am Acad Dermatol 1999;41:827-9.
6. Taketuchi Y, Chinen T, Ichikawa Y, Ito M. Two cases of unilateral
pigmented purpuric dermatosis. J Dermatol 2001;28:493-8.
7. Schroeder-Devere T. Pigmented Purpuric dermatoses. In: Wolf K,
Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffel DA, editors.
Fitzpatricks Dermatology in General Medicine. 7th ed. McGraw-Hill;
2008. p. 1633-6.
8. Ratnam KV, Su WP, Peters MS. Purpura simplex (inflammatory purpura
without vasculitis). A clinico-pathologic study of 174 cases. J Am Acad
Dermatol 1991;25:642-7.
9. Torrelo A, Requena C, Mediero IG, Zambrano A. Schambergs purpura
in children: A review of 13 cases. J Am Acad Dermatol 2003;48:31-3.
10. Sharma L, Singh SP, Dixit VK. Lichen planus and hepatic dysfunction.
Ind Med Gaz 2010;CXLIV:463-6.

Cite this article as: Sharma L, Gupta S. Clinicoepidemiological study of

pigmented purpuric dermatoses. Indian Dermatol Online J 2012;3:17-20.
Source of Support: Nil, Conflict of Interest: None declared.

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