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Summary

  1. Top of page
  2. Summary
  3. Introduction
  4. Case report
  5. Discussion
  6. Conclusion
  7. Learning points
  8. References
  9. CPD questions
  10. Instructions for answering questions

Nail involvement in sarcoidosis is rare, but its presence is often associated with chronic systemic disease. We have reviewed all 33 reported cases of nail involvement with sarcoidosis, including our own case. Nail changes in sarcoidosis, while rare, can include dystrophy (n = 10, 30%), onycholysis (n = 7, 21%), subungual hyperkeratosis (n = 7, 21%), nail hyperkeratosis (n = 2, 6%) and longitudinal ridging (n = 8, 24%). All reported cases of nail sarcoidosis occurred in the setting of systemic involvement. In almost half of the cases, bony cysts were noted on radiological examination. The presence of nail dystrophy in a patient with sarcoidosis should therefore prompt radiological examination and investigation for systemic involvement.


Introduction

  1. Top of page
  2. Summary
  3. Introduction
  4. Case report
  5. Discussion
  6. Conclusion
  7. Learning points
  8. References
  9. CPD questions
  10. Instructions for answering questions

Sarcoidosis is a multisystem granulomatous disorder that can affect all organs of the body. The disease course can vary from a self-limiting illness to a chronic disease with flares and relapses.1 Nail changes are rare in sarcoidosis, but can include clubbing, discolouration, dystrophy, subungual hyperkeratosis, splinter haemorrhages and longitudinal ridges.2 Such changes are frequently an indication of systemic disease, often with the presence of lupus pernio and sarcoid dactylitis.2,3 Asymptomatic bony cysts can often underlie nail dystrophy, thus radiological examination is a necessity in suspected cases, particularly in the presence of pain.3,4 We report a case of sarcoidosis presenting as nail dystrophy with painful phalanges and underlying bony cysts, and review the literature of nail involvement in sarcoidosis.

Case report

  1. Top of page
  2. Summary
  3. Introduction
  4. Case report
  5. Discussion
  6. Conclusion
  7. Learning points
  8. References
  9. CPD questions
  10. Instructions for answering questions

A 56-year-old man presented with a 5-year history of discolouration of the nails. There was no history of trauma, and the patient’s medical history was unremarkable. Repeated cultures from nail clippings showed no growth of organisms. A 3-month course of oral terbinafine (Lamisil; Novartis Pharmaceuticals UK Ltd, Camberley, Surrey, UK) prescribed by the patient’s general practitioner had no effect. Vascular (Doppler) studies were reported as normal.

On physical examination, red to purple periungual discolouration with associated nail dystrophy was seen (Fig. 1a,b). Several other nails had subungual hyperkeratosis. The fingers were neurovascularly intact. Examination of the skin did not reveal any other lesions suspicious for sarcoidosis, and there were no signs suggestive of underlying connective tissue disease. Subsequent reports of pain in the distal phalanges led to the discovery of bony cysts typical of sarcoidosis upon radiological examination (Fig. 2).

image

Figure 1.  (a,b) Periungual discolouration with associated nail dystrophy.

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image

Figure 2.  Bony cysts in the distal phalanx.

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Laboratory investigations showed that the patient had an increased level of serum angiotensin-converting enzyme (10–70 μg/L). Chest radiography showed bilateral hilar lymphadenopathy.

The patient was referred to the respiratory physicians, who confirmed the diagnosis of systemic sarcoidosis.

Discussion

  1. Top of page
  2. Summary
  3. Introduction
  4. Case report
  5. Discussion
  6. Conclusion
  7. Learning points
  8. References
  9. CPD questions
  10. Instructions for answering questions

Nail changes are rare in sarcoidosis, and are often associated with systemic involvement. In a review of 188 white patients with cutaneous sarcoid lesions, 3 patients (1.6%) were found to have nail dystrophy.5 A further study of 40 patients with sarcoidosis found nail changes in 1 patient (2.5%).6

We performed a PubMed search from 1981 to 2010. The search criteria included the words ‘sarcoidosis’, ‘nail’, ‘dystrophy’, ‘dactylitis’ and ‘cutaneous’. We identified 32 further cases of nail involvement in sarcoidosis. Of these 33 patients (including ours), 13 (39%) were female and 9 (27%) were male; the gender of the remaining 11 patients was not specified. The most common nail changes included nail dystrophy (n = 10, 30%), longitudinal ridges (n = 8, 24%), subungual hyperkeratosis (n = 7; 21%), onycholysis (n = 7; 21%), nail hyperkeratosis (n = 2, 6%) and two cases (6%) of complete nail loss (Table 1).

Table 1. Review of 33 cases of nail involvement in sarcoidosis.
AuthorPatient genderPatient age at diagnosis, yearsNail lesionsBony involvement?Cutaneous involvementSystemic InvolvementHistological findings
  1. NA, not available.

Aranegui et al.12NA38Trachyonychia of all fingersNoNilPulmonary sarcoidNA
Chang and Koopman15F41Longitudinal stripes, pits, hyperpigmentation at the base, subungual hyperkeratosisMultiple bone cysts of hands and feetErythema and scaling of skinRight hilar lymphadenopathy, lung fibrosisNA
Cohen and Lester16F45Dystrophy, onycholysis, hyperkeratosis of toenailLacy trabecular pattern, bony reabsorption of distal phalangesPeriungual violaceous discoloration and swelling of toesHilar lymphadenopathy, sinusitisPunch biopsy: granulomatous inflammation
Cox3F64Longitudinal ridges, thickening, fragility of thumb and some fingers,CystsLupus pernioPulmonary fibrosisNA
M46Longitudinal ridges, fragility, onycholysis, splinter haemorrhagesLucent areas in terminal phalanges, pathological fracturesLupus pernio; second toeFinger swellingNA
F64Longitudinal cracking and ridging of both thumbnailsAltered bony texture and cystsErythema nodosumHilar lymphadenopathy, uveitis, splenomegaly, impaired respiratory functionNA
M44Longitudinal ridges and cracking of toenailCystic changes and altered trabecular patternLupus pernio of nose and nasal cartilage destruction10-year history of hilar lymphadenopathy; fusiform swelling of hand and feet phalangesNA
M4212-year history of nail splitting and onycholysis of right index fingernail, painful and swollen nail fold, dystrophy and destruction of index fingernail and pterygium formation, hyperkeratotic hallux nailsCoarse trabeculation and cysts in terminal phalangesLupus pernio, sarcoid plaques8-year history of nasal sarcoidNA
Davies and McGavi13M41Distal onycholysis of finger and toe nails, subungual hyperkeratosisNALupus pernio of fingernailsNoneNA
Fujii et al.17F30Subungual hyperkeratotic lesions of toenailsNAErythema nodosumBilateral parotid gland enlargement and hilar lymphadenopathy,Discrete epithelioid granuloma in superficial dermis, nail plate was unaffected
Jayck18Eight patientsNAMild dystrophy to complete nail lossNAAll had cutaneous involvementAll had dactylitis,NA
Kalb and Grossman10F521-year history of nail dystrophy and pterygium formationBone cystsNABilateral hilar lymphadenopathyNoncaseating granuloma in dermis
Losada Campa et al.11F56Thickening, longitudinal ridges, pterygium, splinter haemorrhages, onycholysis, Subungual hyperkeratosisNANodular forearm lesion, lupus pernioPulmonary involvementNoncaseating granulomatous dermal infiltrate and papillomatosis in nail-bed epidermis, irregular focal acanthosis
F63Onychodystrophy, longitudinal grooves, subungual hyperkeratosis of toes, atrophic matrixNADisseminated subcutaneous nodules, lupus pernio of nose and toesBilateral hilar lymphadenopathyGranulomatous dermal infiltrate and papillomatosis in nail-bed epidermis, irregular focal acanthosis
M80Toenails: lateral deviation, thickening and discoloration. Thumb biopsy: distal onycholysis, splinter haemorrhages and infiltration of proximal foldsNAFinger and feet, lupus pernio, malar area of nose, helixNANA
Leibowitz19F60Nail dystrophy and ulceration and pain‘Punched-out’ lesions in metaphyses and periarticular sites, destruction of distal phalanges, expansion of phalange jointLupus pernio on noseDactylitisNA
NA55Nail dystrophy and ulceration, painBony cysts, loss of third and fifth phalanges on left handNADactylitis, pulmonary fibrosis, optic atrophy, axillary lymphadenopathy, arthritisNA
Mann and Allen20M65Dystrophy and longitudinal ridges,Lace-like reticulated trabeculated phalanges with cysts and thin sclerotic rimsLupus pernio on noseSwelling of interphalangeal joints, presence of axillary and inguinal lymphadenopathyNA
Micalizzi et al.14F53Longitudinal ridge, erosions of free edgesCystic lesions of phalangesEpitheloid granulomaJoint swellingNA
Momen and Al-Niaimi (present case)M56Nail dystrophy, red-purple periungual discoloration, subungual hyperkeratosisBony cystsNilBilateral hilar lymphadenopathy,NA
Patel and Sharma6F60Pitting and crackingBone cyst, coarse reticulationsErythematous macule on cheekJoint swelling, nasal crustingNA
Pitt21M24Dystrophic toenails and fingernails, painLytic lesionsNADactylitis in several fingers and toesNA
Santoro and Sloan9F33Nail dystrophy of all nails, onycholysis, leuconychia, erythematous subungual hyperkeratotic papulesBony cystsLupus pernio10-year history of pulmonary sarcoidPunch biopsy: epithelioid cells without central necrosis and sparse infiltrate of lymphocytes
Tosti et al.22F4714-year history of dystrophy, shortened and tapered terminal phalangesOsteolysis of terminal phalanx of index fingerHyperkeratotic and hemorrhagic cuticles, telangiectasia in proximal nail foldBilateral hilar lymphadenopathyLongitudinal nail biopsy: dermis of proximal nail fold, noncaseating granulomas with epithelial histiocytes
Wakelin & James4M43 NoCutaneous plaques over proximal nail fold and anklePulmonary, nasolacrimal, intracranial sarcoid and hepatic involvementProximal nail-fold biopsy: epithelioid granuloma in dermis

Bone changes in sarcoidosis have often been incidental findings, and when terminal phalanges are affected, such changes are often associated with nail dystrophy.7 Radiological examinations were reported to have been performed on 20 patients (61%), with 17 (85%) of these showing bony changes in keeping with sarcoidosis, including bony cysts (n = 12; 60%), bony trabeculations (n = 4; 20%) and pathological fractures (n = 1; 5%). Pain was reported by 10 patients (30%), including ours; however, 9 of these patients presented with dactylitis, which usually manifests as finger swelling and pain. Pain is therefore not a reliable indicator of underlying bony involvement, but its presence should alert the clinician to possible bone involvement.

Nail involvement has often been associated with lupus pernio and dactylitis. Dactylitis has a prevalence of 0.2% in patients with sarcoidosis.8 Nail involvement was noted in three out of six patients (50%) with sarcoid dactylitis. Dactylitis can also present as a necrotizing form with ulceration and nail changes.9

All the reported cases were associated with systemic or cutaneous involvement, thus supporting the notion that nail changes may be an indicator of chronic disease. In our literature review, we found that 26 of the 33 patients (79%) were reported as having skin changes, with 12 (36%) exhibiting lupus pernio and 2 (6%) having erythema nodosum. Evidence of systemic involvement included lymphadenopathy [hilar (n = 8; 24%), axillary (n = 2; 6%), inguinal (n = 1; 3%)], pulmonary fibrosis (n = 6; 18%), arthritis (n = 2; 6%), sinusitis (n = 1; 3%) and splenomegaly (n = 1; 3%).

Histological confirmation of sarcoidosis was obtained from nail biopsies in 7 (21%) of the 33 patients. Granulomatous infiltrates of the dermis were commonly noted.10,11 Wakelin and James4 reported the presence of surrounding skin changes of erythematous plaques over the proximal nail folds, and suggested that these skin changes migh point towards a diagnosis of sarcoid. In such cases, nail-fold biopsies may be taken instead of nail-matrix biopsies to avoid nail scarring and nail plate dystrophy. Losada-Campa et al.11 suggested that nail changes caused by sarcoidosis are all secondary to sarcoid granulomas having microcompressive effects in the dermal compartment between the phalanx and the nail plate, which are both rigid structures. They recommended a longitudinal biopsy be performed instead of punch biopsy, as they found all nail structures to be affected by sarcoidosis.

Treatment with systemic and high-potency topical steroids were reported to improve the appearance of nail changes in 5 (15%) of the 33 patients, but did not seem to affect the bony changes.3,4,12–14

Conclusion

  1. Top of page
  2. Summary
  3. Introduction
  4. Case report
  5. Discussion
  6. Conclusion
  7. Learning points
  8. References
  9. CPD questions
  10. Instructions for answering questions

Our review shows that although nail changes are rare in sarcoidosis, they are often associated with systemic involvement (commonly pulmonary involvement, dactylitis and lymphadenopathy) and underlying bony changes. Thus, in patients presenting with nail involvement, a search for possible systemic involvement should take place, and this should include radiological imaging of hands and feet alongside chest imaging.

Learning points

  1. Top of page
  2. Summary
  3. Introduction
  4. Case report
  5. Discussion
  6. Conclusion
  7. Learning points
  8. References
  9. CPD questions
  10. Instructions for answering questions

• To increase awareness of prevalence of nail involvement in sarcoidosis.

• To encourage clinicians to investigate for bony cysts and systemic involvement when patients present with nail sarcoidosis.

References

  1. Top of page
  2. Summary
  3. Introduction
  4. Case report
  5. Discussion
  6. Conclusion
  7. Learning points
  8. References
  9. CPD questions
  10. Instructions for answering questions

CPD questions

  1. Top of page
  2. Summary
  3. Introduction
  4. Case report
  5. Discussion
  6. Conclusion
  7. Learning points
  8. References
  9. CPD questions
  10. Instructions for answering questions

Learning objective

To highlight the prevalence of nail involvement in sarcoidosis, and that its presence should prompt investigations for possible underlying bony and systemic involvement.

Question 1

Which of the following was a common histological finding in patients with nail sarcoidosis?

a) Ampullar dilatations of the nail plate

b) Dilated nail-fold capillaries

c) Epidermal spongiosis

d) Fibroblastic proliferation

e) Noncaseating granuloma in the dermis

Question 2

Treatment with which of the following resulted in the improvement of sarcoid nail appearance in some cases?

a) Psoralen ultraviolet A (PUVA) therapy

b) Systemic antifungals

c) Systemic steroids

e) Topical emollients

d) Topical tar

Question 3

Of the patients reviewed with sarcoid nail involvement, which cutaneous feature of sarcoidosis was the most commonly seen?

a) Erythema nodosum

b) Hypopigmentation

c) Lupus pernio

d) Sarcoid plaques

e) Scar infiltration

Question 4

Of the patients reviewed with sarcoid nail involvement, which radiological finding was commonly present?

a) Bony cysts

b) Bifurcation of the distal phalanx

c) Osteophyte formation

d) Sclerosis

e) Pathological fractures

Question 5

In the patients reviewed with sarcoid dactylitis, which of the following was a common symptom?

a) Hyperalgesia

b) Pain

c) Paralysis

d) Parasthesia

e) Pruritis

Instructions for answering questions

  1. Top of page
  2. Summary
  3. Introduction
  4. Case report
  5. Discussion
  6. Conclusion
  7. Learning points
  8. References
  9. CPD questions
  10. Instructions for answering questions

This learning activity is freely available online at http://www.wileyhealthlearning.com/ced.

Users are encouraged to

• Read the article in print or online, paying particular attention to the learning points and any author conflict of interest disclosures

• Reflect on the article

• Register or login online at http://www.wileyhealthlearning.com/ced and answer the CPD questions

• Complete the required evaluation component of the activity

Once the test is passed, you will receive a certificate and the learning activity can be added to your RCP CPD diary as a self-certified entry.