Sarcoidosis is a granulomatous systemic disease of undetermined origin. Its dermatological manifestations are present in 25%
of cases and they are very polymorphic. Nail abnormalities are unusual in sarcoidosis and are often an indication of systemic
involvement. Dermoscopy of the nail may be very useful in some isolated cases and can guide us to the right diagnosis. We report a
rare case of systemic sarcoidosis presenting as nail dystrophy with painful phalanges and underlying bony cysts, treated successfully
by oral corticosteroid with good evolution
Sarcoidosis is a multisystem granulomatous disease that can
affect all organs of the body. The disorder course can vary from a
self-limiting illness to a chronic disease with flares and relapses.
Nail changes are rare in sarcoidosis and they are frequently an
indication of systemic disease. We report a rare case of systemic
sarcoidosis presenting as nail dystrophy with painful phalanges
and underlying bony cysts.
A 57-year-old Moroccan woman, without medical history, was
admitted to the dermatology department with two-year history of
painful toe nail lesions. Physical examination showed a paronychia
of all toes affected with thickening of the plate, hyperkeratosis and
splinter hemorrhages, associated to a longitudinal nail fracture
with localized groove longitudinal nail plate deformity of the right
big toe and hyperkeratotic verrucous lesions of the nail body and
proximal nail fold of the second toes (Figure 1). Dermoscopic
evaluation of proximal nail fold revealed a pink coloration of the
background, change of the microvascular architecture, linear
vessels of different diameter, glomerular and serpiginous vessels,
large areas of white scar-like depigmentation, translucent orange
globular-like structures and white scale (Figure 2). Dermoscopy
of nail plate showed a nail thickening, subungual hyperkeratosis,
Splinter hemorrhages, dark dots, nonparallel lesion edg, yellow
or white nail plate and longitudinal parallel white lines (Figure
2). Mycological examination of the nail was negative. A toes X-ray
demonstrated bony cysts, trabecular pattern and osteolysis in distal
phalanx. CT showed multiples lymphadenopathy above and under
diaphragmatic. A punch biopsy taken from the nail matrix, the
lymphadenopathy and salivary gland showed sarcoid granulomas
in the dermis and a chronic inflammatory infiltrate (Figure 3). the
serum level of the angiotensin- converting enzyme was normal (10-
70μg/L). With all this finding, the diagnosis of systemic sarcoidosis
was making, and the patient receive a systemic treatment with
corticosteroid: 0.5 mg/kg/day. After 1 week of treatment, the
patient had a good response.
Figure 1: Nail sarcoidosis
Paronychia of all toes affected, thickening of the plate, hyperkeratosis and splinter hemorrhages, longitudinal nail fracture of
the right big toe, hyperkeratotic verrucous lesions of the nail body and proximal nail fold of the second toes.
Figure 2: Dermoscopy of nail sarcoidosis
a. Dermoscopy of proximal nail fold showing pink coloration of the background, linear vessels of different diameter with
splinter hemorrhages, large areas of white scar-like depigmentation, translucent orange globular-like structures and white
scale.
b. Dermoscopy of nail plate showing a nail thickening, hyperkeratosis and yellow nail plate with glomerular vessels in the
proximal nail fold.
c. Nail edge dermoscopy showing subungual hyperkeratosis.
Sarcoidosis is a granulomatous systemic disease of
undetermined etiology. Cutaneous manifestations are present in
25 to 35% of sarcoidosis and it is usually an early manifestation
of the disease. On the other hand, nail changes are more
uncommon and are often accompanied to systemic involvement.
In a study released by Veien and colleagues 1.6% of patients with
cutaneous sarcoidosis had a nail dystrophy [1]. Clinically, nail
abnormalities may be in the form of nail dystrophy, pachyonychia,
onycholysis, longitudinal striae, splinter hemorrhages, subungual
hyperkeratosis, onychorrhexis, pterygium or squarely an anonychia
[2,3]. In our case, we reported a new clinical appearances of nail
sarcoidosis which is the hyperkeratotic verrucous lesions of the
nail body and proximal nail fold and longitudinal nail fracture with
localized nail plate deformity.
Dermoscopic features of nail sarcoidosis had never been
described before. In our patient, there were dermoscopic signs in
the proximal nail fold (pink coloration of the background, change of
the microvascular architecture, linear vessels of different diameter,
glomerular and serpiginous vessels, large areas of white scarlike
depigmentation, translucent orange globular-like structures
and white scale) and the nail plate (nail thickening, subungual
hyperkeratosis, Splinter hemorrhages, dark dots, nonparallel lesion
edg, Yellow or white nail plate and longitudinal parallel white lines).
Ungueale involvement during sarcoidosis is often associated with
digital bone cysts, dactylitis and lupus pernio, and in most cases is
accompanied by cutaneous or systemic involvement (lymph node,
pulmonary, articular or sinus involvement) [4,5]. Nail sarcoidosis
treatment is based on oral, topical or intralesional corticosteroid
therapy and synthetic antimalarials [6].
Nail involvement in sarcoidosis is unusual and is often
associated with systemic involvement such as underlying bone
location. the clinical aspects are multiple and can confuse with
other nail diseases, hence the interest of the dermoscopy which can
guide us in some cases.