Cutaneous Sarcoidosis with Nail Manifestations:

A Rare Finding

Firman Parrol

, Karin Rachmani

, Sri Linuwih Menaldi

, Sondang P. Sirait

, Eliza Miranda

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

National Central General Hospital, Indonesia

Keywords: Sarcoidosis, nail sarcoidosis, cutaneous sarcoidosis

Abstract: Sarcoidosis is a multisystem granulomatous disease, characterized by naked granuloma lesions with

multiorgan involvement such as the lung, skin, eye, liver, peripheral lymph nodes and, nail. Cutaneous

sarcoidosis is one of the most common findings, while nail sarcoidosis presents with a very low

incidence. We report a 46-years-old male patient, referred to the Department of Dermatology and

Venereology CiptoMangunkusumoHospital with enlarged erythematous plaque lesions on the ear and

nose since three months ago. Our patient also showed dystrophy and onycholysis of

toenails.Erythematous plaque lesions are a hallmark of chronic sarcoidosis, whilenail involvement is

closely linked with chronic and systemic sarcoidosis. Furthermore, skin and nail biopsies also showed

naked granuloma lesions. These findings strengthen our evidence of sarcoidosis, specifically chronic

sarcoidosis. Althoughnail sarcoidosis is found in our patients, there is internal organ involvement.

Therefore, a stepwise approach needs to be done to diagnose nail sarcoidosis.

1 INTRODUCTION

Sarcoidosis is a systemic granulomatous disease

with multiple organ involvement. Sarcoidosis may

manifest in organs such as the lung, skin, eye, liver,

cardiovascular, gastrointestinal, urogenital, kidney,

peripheral lymph nodes, and nail.(

Haimovic A

2012).

On the skin, sarcoidosis can be classified into

specific and non-specific lesions. If skin biopsy

revealed naked granulomas, it is classified as

specific lesion. Whereas, non-specific lesions

usually seen in erythema nodosum. (Mana J et al.,

2012).

Cutaneoussarcoidosis is one of the most

common form of sarcoidosis. Lesions most often are

found on the head and neck, both symmetrically or

asymmetrically on any part of the skin and mucosa.

Although incidences are very low, sarcoidosis can

also appear in other locations, one of them is nail.

Mana J et al., 2012).Despite its rarity, dermatologists

must consider nail sarcoidosis as a serious problem

because its chronic nature and related to systemic

disease.(Momen SE et al., 2013).

In this paper we

report cutaneous sarcoidosis with nail manifestation

which is a rare finding. However, systemic

involvement is not yet discovered.

2 CASE

A 46-years-old man was referred with enlarged

erythematous plaque lesions in the ear and nose

since three months ago. The lesion first appeared

two years agoon the right ear with nummular size.

Since then, the lesion spread to the whole auricle of

left ear. Three months later, erythematous plaque

lesion also appeared on the anterior nares. The

patient observed that the lesion size was progressing

over time. There was no history of pain, itching, loss

of sensation, or bleeding. The patient then came to

dermatologist and he was given mupirocin cream

and wet dressing of potassium permanganate for one

year. However, the condition did not resolve after

the treatment.

After one year of the initial lesion on the ear,

patient complained a disfiguration on the nail. Initial

changes were observed in both toes. Both of the

nails were damaged accompanied by swollen toes.

This was also observed on the fourth right toes and

the fifth left toes. There were no history of pain, itch,

bleeding or trauma before. The patient was referred

to dermatologists who diagnosed him with fungal

infection of the nail. He was given loprox® for six

months but there was no improvement. The patient

Parrol, F., Rachmani, K., Menaldi, S., Sirait, S. and Miranda, E.

Cutaneous Sarcoidosis with Nail Manifestations: A Rare Finding.

DOI: 10.5220/0009987403030306

In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease (ICTROMI 2019), pages 303-306

ISBN: 978-989-758-469-5

303

then referred to another dermatologist who

suspected him with Leprosy. The patient then

transferred to CiptoMangunkusumoCentral General

Hospital for slit skin smearexamination. There was

no history of chronic cough, dyspnea, heart disease,

fever, arthralgia, or ocular disorder. There were no

night sweats or significant weight reduction. There

was no history of any similar disease in patient’s

family. In 2013, patient was diagnosed with

astrocytoma at the spinal cord. According to patient,

the diagnosis was made based on MRI. The

astrocytoma paralyzed patient from the waist to the

feet. The sensory, motoric and autonom nerves were

damaged.

Physical examinations showed multiple

erythematous plaques with rough scales were seenin

both ears. The lesions werevary in size from

lenticular to nummular, arranged discreteand some

of them was confluent with well demarcated

borders. On the nosethere was a solitary nummular

circumscribed erythematous plaque with soft

consistency. (Figure 1) Dermoscopic examination

revealedorange color on skin lesion.

Onycodistrophy, onycholysis and digital clubbing

were observed on the fourth and first finger of the

right foot and the first and fifth finger of the left

foot. (Figure 2) On dermoscopic evaluation, there

were splinter hemorrhageon the nail bed. Multiple

atypical vesselswere found on the fourth nail of the

right foot. (Figure 3)

Figure 1. Multiple erythematous plaques were seen on

both ear and nose. (Arrows)

Figure 2. Nail dystrophy and onycholysis. (Arro.ws)

Based on history taking and physical examination, the

differential diagnosis were lupus vulgaris, cutaneous

sarcoidosis and cutaneous lymphoma. In order to

confirm our diagnosis, the Mycobacterium

tuberculosis (MTB),Mycobacterium Other Than

Tuberculosis (MOTT) culture and polymerase chain

reaction (PCR) obtained from skin tissue was done.

The results of MTB and MOTT culture were

negative, but PCR was positive for Mycobacterium

tuberculosis. Histopathological examination of the

nose revealed the epidermis of had flat rete ridges.

Epithelioid granuloma was observed in the dermis

with multiple giant cells surrounded by sparse

lymphocytes infiltrates. Nearby tissue was dominated

by inflammatory cells and areas of fibrosis. (Figure

4) On the other hand, histopathological examination

of the nail bed revealed acanthotic and irregular

elongation of the rete ridges. Epithelioid granuloma

was also observed in the dermis of the nailbed and the

proximal nail fold under the matrix. (Figure 4)

ZiehlNiehlsen staining showed no sign of acidfast

bacilli on the nose and nail specimen. Chest x-ray

examination revealed no sign of sarcoidosisor TB

infection in the lung, while x-ray examination on

patient's foot showed erosion on distal phalanx of

both thumb with osteolytic process on metatarsal and

phalanx of first finger, distal tibia and tarsal. No sign

of destruction was found.

Figure 3. Splinter hemorrhage and multiple atypical

vessels on the toes. (Arrows)

Figure 4. A, B. Epithelioid granuloma was observed in the

dermison plaque of the nose with multiple giant cells

surrounded by sparse lymphocytes infiltrates. (Arrows).C,

D. Epithelioid granuloma was also observed in the dermis of

the nailbed and the proximal nail fold under the matrix.

(Arrows)

C

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3 DISCUSSION

Cutaneous sarcoidosis are classified into specific

lesions with histopathological findings of naked

granulomas, and nonspecific lesion that appear from

a process that does not form granulomas.(

Haimovic

A,2012) Skin manifestation are present in one fifth

of sarcoidosis. Differentiating these lesions is

important because cutaneous lesions have a

prognostic significance. (Mana J et al., 2012).

Non-

specific lesion such as erythema nodosum typically

associated with good prognosis and spontaneous

resolution while maculopapular lesions and

subcutaneous lesions are linked with remission of

systemic disease in two years. (Mana J et al., 2012).

Whole plaques and lupus pernio are closely

associated with chronic disease. (Mana J et al.,

2012).

The last type of lesions are disfiguring and

can have cosmetical, social, and psychological

impact. (Mana J et al., 2012).

Cutaneous lesions are found on our patient’s

nose and both ears. These lesions are identified to be

erythematous plaque lesions. It is known that plaque

lesions are typically persistent and associated with

chronic forms of sarcoidosis. (Mana J et al., 2012).

is consistent with the findings in our patients, whose

lesions appeared since two years ago, therefore

indicating a chronic form of sarcoidosis. Relatively

symmetric plaques and nodules that occur on the

nose, earlobes, cheeks, and digits, are consistent

with lupus pernio, which has a tendency for systemic

involvement. Nail changes are hardly found in

sarcoidosis.(Momen SE et al., 2013).However, when

present, it is closely associated with systemic

sarcoidosis. The prevalence of nail sarcoidosis was

reported (1.6%) of 188 patients with cutaneous

sarcoidosis.(Velen NK et al., 1987) Despite its

rarity, dermatologists must consider nail

sarcoidosisas a sign of chronic systemic disease.

.(Momen SE et al., 2013).One paper reviewed 33

patients with nail sarcoidosis to describe the changes

of nail.

The most common findings included nail

dystrophy, longitudinal ridges, subungual

hyperkeratosis, onycholysis, nail hyperkeratosis and

nail loss consecutively. Confirmation of sarcoidosis

was made from histopathological findings from of

the nail biopsies, which were the presence of naked

granulomatous. Among 33 patients, seven patients

are noted to have granulomatous infiltrates of the

dermis.(Losada-Campa et al., 1995; Kalb RE et al

1985).

In our patient, we also findonycodistrophy,

onycholysis and digital clubbing. Histopathological

examination revealed typical naked

granulomas.(Fernandez-Faith E et al., 2007).These

findings point us towards nail sarcoidosis.

Wakelin and James stated that the findings of

surrounding skin changes of erythematousplaques

over the proximal nail folds might direct towards a

diagnosis of sarcoidosis (Haimovic A et al 2012)

These skin changes are caused by the formation of

granulomas which have microcompressive effects in

the dermal compartment between the nail plate and

the phalanx.(Wakelin SH et al., 1995).Despite the

nail changes and granuloma lesion in the biopsy of

nail, one should have more evidence to confirm the

diagnosis of sarcoidosis in this patient. Nail

involvement in sarcoidosis is rare and is usually a

marker of chronic disease.Although most of the

cases reported are associated with digital bone cysts,

sarcoiddactylitis, and lupus pernio,cases without

these associations have also been described

proposed that most nail changes are secondary to

granulomatous compression of nail structures(Velen

NK et al., 1987).

Sarcoidosis is known to be “the great imitator”

because of its diverse manifestations and the ability

to resemble other cutaneous disease. .(

Haimovic

A,2012).Therefore, a stepwise approach of diagnosis

needs to be taken in every patient with

granulomatous inflammation.(Chopra S et al., 1999).

A stepwise approach to diagnose sarcoidosis in this

patient is divided into two parts. First, excluding

alternative cause of granuloma. Second, find at least

one additional organ involvement. Alternative

known cause of granuloma is very wide, including

tuberculosis, atypical mycobacteriosis, fungal

infections, reaction to foreign bodies, leishmaniasis,

rheumatoid nodules, and Melkersson-Rosenthal

syndrome. (Aranegui et al., 2010).

However, in this patient, another known cause

of granuloma are unlikely. Granulomatous lesion is

highly caused by sarcoidosis according to patient’s

history, negative culture of MTB, and MOTT, and

normal chestx-ray examination. Although PCR

showed positive for MTB,it will not affect the

diagnosis because positive result can still be found

in 80% of cutaneous sarcoidosis patient. Several

studies have shown the presence of Mycobacterium

DNA in sarcoid granuloma, suggesting previous

exposure to Mycobaterium, which might have

induced a granulomatous reaction. The common

problem raised during PCR assays is the high risk of

false positive results due to common laboratory

contamination or presence of killed or dormant

bacilli in the patient specimens. However, the key to

the diagnosis of tuberculosis is a positive result on

tissue culture which is the gold standard of

Cutaneous Sarcoidosis with Nail Manifestations: A Rare Finding

305

diagnosis. In our patient, tissue culture of MTB and

MOTT showed negative result. Therefore,

confirming cutaneous and nail sarcoidosis as our

diagnosis.(Makeshkumar V et al,2014;Brownell et al

2011).

Classic sarcoidal lesions in the small bones of the

hands and feet are well characterized and diagnosed

with conventional radiographs, on which they

demonstrate the familiar “lacy” lytic

appearance.(Moore SL et al., 2003). X-ray

examination on patient's foot showed erosion on

distal phalanx of both thumb with osteolytic process

on metatarsal and phalanx of first finger. This x-ray

finding is suggested as extracutaneous manifestation

of sarcoidosis. Another suspected systemic

manifestation in this patient was astrocytoma. This

finding raised question whether it is an astrocytoma

or granulomatous inflammation mimicking

astrocytoma. Neurosarcoidosis itself has a

predilection at the base of the brain or spinal cord

with cranial neuropathies are the most common

manifestation. Therefore, we plan to refer the patient

to Neurology Department to establish the diagnosis.

We also refer the patient to the Internal Medicine

Department to perform more diagnostic evaluation

to find involvement of other organs. We treat the

patients using clobetasol propionate cream 0.05%

twice a day for the lesion in the ear and nose. Lesion

in the nail is treated using clobetasol propionate

ointment 0.25%. Several literatures have reported

the efficacy of topical steroid to improve the

appearance of nail changes in 5 (15%) of the 33

patients. (Haimovic A et al., 2012; Aranegui et al.,

2010).

4 CONCLUSION

Cutaneoussarcoidosis is one of the most common

organs involved and appear in one third of

sarcoidosis cases. Cutaneoussarcoidosis lesion has

its prognostic significance. Different lesion could

have a different prognostic implication. In the other

hand, nail sarcoidosis is a rare manifestation of

sarcoidosis. Although it is rare, one must consider

the systemic involvement of sarcoidosis if the nail is

affected because its chronic and systemic nature. A

stepwise approach must be used to diagnose every

patient with nail involvement in sarcoidosis.

Documentation of additional organ involvement,

such as the lung, lymph nodes, eye, heart, and

nervous system is essential to diagnose nail

sarcoidosis which commonly observed with multi

organ involvement.

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