A Case of Majocchi Purpura: Clinical and Histopathological

Approaches for Diagnosis

Fadhli Aulia Mughni, Sarah Mahri, Teffy Nuary, Rahadi Rihatmadja, Kusmarinah Bramono

Department of Dermatology and Venereology, Faculty of Medicine University of Indonesia / Dr. Cipto Mangunkusumo

National General Hospital, Jakarta, Indonesia

Keywords: clinical patterns, diagnosis, histopathology, Majocchi purpura

Abstract: Majocchi Purpura is one of the morphological varieties of pigmented purpuric dermatoses. They are rare

disorders with unknown etiology, and characteristically presents as nonblanching petechiae, pigmentation,

and the presence or absence of telangiectasia with a predilection for the lower extremities. They are usually

asymptomatic and self-limiting, but tend to have a chronic course and are highly recurrent. The diagnostic

approach for patients suspected with pigmented purpuric dermatoses is quite straightforward, with clinical

observation and histopathological examination as the mainstay diagnostic modalities. However, this

disorder may initially show similar manifestations as other cutaneous diseases, such as contact dermatitis,

vasculitis, or mycosis fungoides, making it oftenly misdiagnosed and mistreated by dermatologists. We

present a case of a 28-year-old woman initially referred with polyarteritis nodosum. However, the lesions

were painless and consisted of several hyperpigmented annular patches that clinically resembled Majocchi

Purpura. We submitted the case for biopsy with vasculitis as the differential diagnosis. Histopathological

findings were consistent with pigmented purpuric dermatosis, that we commenced the treatment with

symptomatic measures supplemented with antioxidants. Recognizing distinctive clinical patterns of disease

was indispensable. Laboratory examinations had limitations, and their results needed to be interpreted

within the clinical context. The correct diagnosis will prevent overtreatment and unnecessary healthcare

visit by patient.

1 INTRODUCTION

Pigmented Purpuric Dermatoses (PPD) is a group of

disorders characterized by nonblanching purpuric

rash, leaving residual hyperpigmented patches

mainly on lower extremities.(Sardana, 2004) The

etiology is unknown, although several drugs and

other conditions have been documented. (Devere,

2012) Most patients are asymptomatic or presenting

with mild symptoms. PPD is usually chronic, highly

recurrent, and difficult to treat, although self-

limiting cases have been reported.(Devere, 2012)

This disorder is morphologically categorized into:

(1) Schamberg’s disease (SD), (2) Purpura annularis

telangiectodes of Majocchi (Majocchi Purpura), (3)

Pigmented purpuric lichenoid dermatosis of

Gougerot and Blum (PPLD), (4) Eczematid-like

purpura of Doucas and Kapetanakis, (5) Itching

purpura of Lowenthal, and (6) Lichen aureus

(LA).(Kim, 2015)

PPD is considered uncommon, despite lack of

sufficient data in Indonesia. A

clinicoepidemiological study by Sharma and

GuptaSharma, 2012 in 2012 found 0.18% PPD cases

from 55,323 patients. All races may be affected,

with more occurrence seen in males except in

Majocchi purpura. (Sardana, 2004,Hoesly, 2009)

The majority have history of routine activity that put

constant pressure to the limbs, such as prolonged

standing during work, high-intensity sport, or

repetitive tasks. Several factors is believed to

contribute to its pathogenesis, including capillary

fragility, humoral immunity, cell-mediated

immunity, gravitational forces, venous hypertension,

focal infection, and contact allergy. (Devere,

2012,Sharma, 2012)

Most cases were diagnosed clinically. The

presentations of PPD are usually characteristic, with

pigmented patches and petechiae as the most

consistent findings, without palpable purpura.

However, initial manifestations may easily be

interpreted as other cutaneous diseases, mainly

402

Mughni, F., Mahri, S., Nuary, T., Rihatmadja, R. and Bramono, K.

A Case of Majocchi Purpura: Clinical and Histopathological Approaches for Diagnosis.

DOI: 10.5220/0008158404020405

In Proceedings of the 23rd Regional Conference of Dermatology (RCD 2018), pages 402-405

ISBN: 978-989-758-494-7

because their rare occurrence entices clinicians to

weigh other more common disorders showing

similar features. Pigmented purpuric dermatoses

need to be distinguished from contact dermatitis,

stasis dermatitis, angioma serpiginosum, mycosis

fungoides, and most importantly, vasculitis.

(Sardana, 2004,Devere, 2012) Histopathology

examination most commonly shows perivascular

infiltrate of lymphocytes around superficial blood

vessels, endothelial cell swelling, erythrocyte

extravasation, and hemosiderin deposition. (Sardana,

2004-Kim, 2015) While crucial for confirming the

clinical diagnosis of PPD, skin biopsy is also

important to exclude cutaneous T-cell lymphoma,

which in its early stages closely resembles PPD.

(Sardana, 2004)

No satisfying therapy has been found for PPD.

Some lesions have been reported to subside

spontaneously, however numerous agents have been

used to alleviate the symptoms and skin lesions,

including topical corticosteroids, antihistamines,

bioflavonoids, ascorbic acid, griseofulvin,

pentoxifylline, cyclosporine, and phototherapy, with

variable but inconclusive outcomes. (Sardana, 2004)

Misdiagnosis may lead to overtreatment and

unnecessary healthcare visits, therefore it is crucial

for dermatologists to be able to recognize PPD

lesions and manage the patients accordingly.

2 CASE

A 28-year-old woman was referred to the

Dermatovenerology clinic, Cipto Mangunkusumo

National Central General Hospital with the diagnosis

of polyarteritis nodosum. She presented with

multiple brownish-red lesions on the legs that have

been present for four months. Initially,

asymptomatic red spots appeared on both feet. They

spread upward reaching up to the thighs.

Approximately two weeks later, some lesions faded

to brownish discoloration. The lesions were more

noticeable in relatively cold environment, such as in

air-conditioned room. She also occasionally

complained of ankle joints pain since two months

before presentation. She took oral B-complex

vitamins, but to no avail.

Physical examination was unremarkable except

the presence of multiple brownish-purpuric patches

bilaterally on the arms, lower legs, and feet. Some of

the macules were annular and reticular. No palpable

purpura was observed. (Figure 1).

Figure 1. Multiple petechial and hyperpigmented

patches in annular (arrow) and reticular (circle)

configurations.

Past medical history was unremarkable. Previous

use of medications was denied. The patient usually

wore high-heeled shoes for work, which require

repeated walking between offices during working

hours. Initial laboratory examinations showed mild

leukopenia, eosinophilia, and high erythrocyte

sedimentation rate. The results of hemostasis,

urinalysis, as well as blood chemistry examinations

were within normal ranges. At the Internal Medicine

clinic, she was also examined for anti-nuclear

antibodies (ANA), Hepatitis B surface antigen

(HbsAg), anti-hepatitis C virus (anti-HCV), humman

immunodeficiency virus (HIV), mixed activated

partial thromboplastin time (APTT), and lupus

anticoagulant to rule out other diagnostic

possibilities. All those results were negative, except

for ANA.

We diagnosed this patient with Majocchi

purpura. Schamberg disease, another type of PPD,

and vasculitis was also considered. The patient was

then sent for biopsy. Histopathology from two

different sites revealed superficial perivascular

lymphocytic infiltrates, endothelial cell swelling,

and erythrocytes extravasation, which are consistent

with PPD. (Kim, 2015) (Figure 2)

The patient has been followed up for two

months, and was treated symptomatically with

emollients and antioxidants, including ascorbic acid

500 mg daily. Topical corticosteroid twice daily was

started one month ago because the patient exhibited

mild occasional pruritus. There was slight

improvement of the patient’s skin lesions, without

worsening of symptoms or appearance of new

purpuric patches

A Case of Majocchi Purpura: Clinical and Histopathological Approaches for Diagnosis

403

Figure 2. (A) Perivascular lymphocytic infiltrates in the upper dermis (H&E, 50X); (B) Endothelial cell swelling,

superficial perivascular lymphocytes (H&E, 100X); (C) Erythrocytes extravasation around the superficial blood vessel

(H&E 400X).

3 DISCUSSION

The clinical appearance of pigmented purpuric

dermatoses may be similar to cutaneous vasculitis.

However, the purpuric patches in cutaneous

vasculitis are palpable, unlike in PPD. (Devere,

2012) Our patient was initially assessed as

polyarteritis nodosa (PAN), a necrotizing vasculitis

of medium-sized vessels that commonly affected the

nerves, intestinal tract, heart, and joints. Indeed, the

initial manifestations of some cases of PAN are skin

lesions. (Kazandjieva, 2017) However, she did not

complained of constitutional symptoms, such as

fever, malaise, weight loss, or abdominal pain, and

did not exhibit characteristic subcutaneous nodules.

Therefore, it was important to rule out related

systemic diseases, since the patient also complained

of ankle pain. There were several case reports

associating persistent PPD with CTCL and mycosis

fungoides, which can all be excluded by

histopathologic examination. (Toro, 1997,Martinez,

2001)

The reticular pattern of the macules and

exacerbation by cold suggested the possibilities of

disturbance of blood flow to the skin in response to

low temperature exposure, such as seen in livedo

reticularis. Occlusions of vessels in livedo reticularis

may be found in vasculopathy disorders, such as

antiphospholipid syndrome (APS) and

cryoglobulinemia. (Gibbs, 2005) The negative

results of HBsAg, anti-HCV, HIV, mixed APTT,

and lupus anticoagulant examinations of this patient

ruled out those diseases. Anti-nuclear antibodies

were positive, however it does not entirely confirm

the presence of an autoimmune disease. (Pisetsky,

2011) Our patient’s history and clinical appearance

does not support an autoimmune disease, but

continuous observation is needed.

Typical histopathologic findings of PPD

mentioned in literatures are perivascular infiltrate of

lymphocytes in superficial dermis, endothelial cell

swelling, erythrocytes extravasation, and

hemosiderin deposition. (Devere, 2012,Kim, 2015)

Superficial perivascular lymphocytes were clearly

observed in our patient, as well as endothelial

swelling and extravasation of erythrocytes in the

dermis. However, in two biopsy specimens we could

not find deposition of hemosiderin. Although it is an

important feature for distinguishing PPD from other

disorder, hemosiderin deposition is not always

found. In a retrograde analysis of 113 patients with

PPD in Korea, hemosiderin deposition was found in

only 26,3% subjects, while perivascular lymphocyte

infiltration and erythrocyte extravasation were found

in 79% and 50% patients, respectively.(Kim, 2015)

It is advisable to do histochemical staining with

Perls stain to identify hemosiderin more easily, as

well as with Masson-Fontana stain to exclude

melanin pigment. (Kazandjieva, 2017) Focal

karyorrhectic nuclear dust may occasionally be

found, especially in active pronounced lesions,

alongside with narrowing of vessel lumens.

(Kazandjieva, 2017,Weedon, 2010) Both features

were found in this patient.

The patient was treated with antioxidants and

symptomatic therapies, which consist of emollient,

anti-inflammatory and antipruritic agents. Treatment

of PPD is difficult, and results in literatures are

inconsistent. (Hoesly, 2009) proposed a systematic

approach for the management of Majocchi Purpura.

Through review of available literatures, the scheme

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404

suggested treatment of this disorder according to the

symptoms severity and use of combination treatment

if needed.

4 CONCLUSION

We reported a case of Majocchi purpura in a patient

who was initially assessed as a polyarteritis

nodosum. Although uncommon, it is important for

dermatologists to be able to recognize the

characteristic PPD lesions, and perform the

necessary examinations to rule out other suspected

disorders. Histopathology examination is until now

the most important tool to confirm the diagnosis of

PPD. Pigmented purpuric dermatoses is highly

recurrent, although some lesions are self-limiting.

Treatment is seldom satisfactory, and remains a

challenge for clinicians.

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