Dermatology Clinic

CASE #1

Inflammation and Redness of the Fingernail Folds

DINA ZAMIL, BS; TARA L. BRAUN, MD; CHRISTOPHER RIZK, MD

A 35-year-old woman presents to the dermatology clinic with inflammation of her fingernails that first appeared 5 months ago and recently worsened. She reports wearing acrylic nails for the last year, removing them just before the clinic visit. On examination, the patient has erythema and swelling of the lateral nail folds and hyperpigmentation of the proximal nail folds of all fingers on her right hand. Examination also reveals nail dystrophy, onycholysis, and an absence of cuticles. She has not tried any treatments for this condition.

What is your diagnosis? Turn to page 22

CASE #2

Flat-Topped Papule With Wickham Line

DINA ZAMIL, BS; TARA L. BRAUN, MD; CHRISTOPHER RIZK, MD

A 32-year-old woman presents with an itchy rash that began 3 months ago. She has a history of anxiety but otherwise no known medical conditions. She has tried over-the-counter hydrocortisone cream to treat the rash, finding some relief from the itchiness, but the rash continues to spread. She has no known allergies and has not changed her skin care products or medications. On examination, the patient has dark purple and gray flat-topped papules with fine gray-white streaks on her flexural wrists, dorsal hands, elbows, ankles, and dorsal feet.

What is your diagnosis? Turn to page 23

Dermatology Clinic

CASE #1 Chronic Paronychia

Chronic paronychia is an inflammatory condition of the nail folds lasting longer than 6 weeks.1 Descriptions of paronychia in the medical literature date back to 1891.2 Chronic paronychia primarily results from environmental exposures, irritants, and allergens.1,3,4 More common in women, chronic paronychia typically affects multiple digits, especially the second and third digits of the dominant hand.4,5 The condition commonly occurs in patients aged 40 to 49 years, with a mean age at diagnosis of 43 years.6,7

Etiology and Risk Factors

Chronic paronychia results from inflammation of the perionychium of the nail. It is usually caused by damage to the cuticle that disrupts the barrier between the nail plate and the skin, allowing invasion of fungus or bacteria. Generally, any activity that subjects the nail bed to trauma increases the risk for paronychia. For example, frequent manicures and wearing artificial nails, nail biting, hangnail manipulation, and ingrown nails are risk factors for chronic paronychia.1

Chronic paronychia also is associated with continuous exposure to environmental irritants and, thus, is thought to be a form of hand dermatitis.3,5 Chefs, nurses, swimmers, house cleaners, dishwashers, and barbers are more likely to develop chronic paronychia due to repeated exposure to chemicals, acids, or alkalis.1,3,5,6 People who handle food may develop chronic paronychia due to food hypersensitivity reactions.3

Other populations predisposed to chronic paronychia include patients with diabetes, patients on antiretroviral therapy for HIV, and patients who are immunosuppressed.3,5 Less common causes of chronic paronychia include Raynaud disease, papulosquamous disorders, cancers, and drug toxicity.3

Candida albicans commonly is implicated in the etiology of chronic paronychia and is found in 40% to 95% of fungal cultures of patients with chronic paronychia.5 However, research suggests that topical steroids are more effective than systemic antifungal therapy at treating chronic paronychia.3,5,8 It is thought that Candida albicans infects the nail bed secondary to an existing inflammatory condition.3,5

Diagnosis of Chronic Paronychia

Clinically, chronic paronychia presents with cuticle damage, pain, and swelling and erythema of the nail fold. The condition often affects multiple digits.1,5 Nails often are thick and discolored. Damage to the nail matrix can lead to nail plate abnormalities, such as pitting, onychomadesis, longitudinal ridging, and Beau lines, whereas damage to the nail bed can lead to onycholysis. Lateral margins of the nail plate may even become green, suggesting secondary infection with Pseudomonas aeruginosa. 3

The differential diagnosis for chronic paronychia includes several cancers that should be considered when the symptoms affect a single digit, the condition does not respond to treatment, or patients have a history of cancer.1,3,5 The following cancers may cause nail damage: malignant melanoma, squamous cell carcinoma, Kaposi sarcoma, bronchogenic carcinoma, leukemia cutis, subungual keratoacanthoma, myeloma-associated systemic amyloidosis, and digital papillary adenocarcinoma.5

Other conditions that may mimic chronic paronychia include herpetic whitlow, psoriasis, eczema, granuloma annulare, Reiter syndrome, dermatomyositis, pyogenic granuloma, hematoma from pulse oximetry, and, rarely, pemphigus vulgaris and food hypersensitivity.1

Treatment of Chronic Paronychia

Chronic paronychia is diagnosed based on clinical findings and patient history, which generally reveal exposure to irritants or other risk factors.1,5 Clinical history also may reveal episodic worsening of symptoms of paronychia after exposure to moisture.5 If the practitioner suspects malignancy, a nail biopsy may be needed.5 When infection is suspected, a culture for bacteria, fungi, and atypical mycobacteria may be indicated.5 Acute paronychia differs from chronic paronychia because it persists for less than 6 weeks, typically affects a single digit, and results in greater swelling and erythema.1,5

Chronic paronychia can be managed surgically or nonsurgically.1,3,5 It is essential to counsel the patient about avoidance of environmental triggers, including irritants, allergens, chemicals, nail manipulation, and moisture.3,5 Patients also may be advised, when appropriate, to use rubber gloves, keep nails short, and wear comfortable footwear to avoid toenail damage.3 Patients with diabetes should maintain proper glycemic control.3

Topical corticosteroids are used as first-line treatment and topical antifungals may be added; oral antifungals are not indicated.3,5 Tacrolimus ointment (1%) also can be used as an alternative to topical steroid therapy.3,9 Antibiotics (topical or systemic) may be necessary in the presence of secondary infections.3 Overall, response to therapy is slow, and chronic paronychia can take months to resolve, but patients should be advised to continue to avoid triggers and persist with treatment.3

In patients whose condition is unresponsive to nonsurgical measures, surgery is indicated to remove the inflamed tissue or a portion of the eponychium.3,5

The patient in this case was counseled to avoid acrylic nails and was prescribed clobetasol ointment twice daily for 2 weeks and ketoconazole cream twice daily for 4 weeks. This treatment helped improve the inflammation around her nails, although the nail dystrophy still was present at her 3-month follow-up visit.

References

1. Leggit JC. Acute and chronic paronychia. Am Fam Physician. 2017;96(1):44-51. 2. Diseases of the fingers : B-paronychia. Hospital (Lond 1886). 1891;10(254):226. 3. Relhan V, Goel K, Bansal S, Garg VK. Management of chronic paronychia. Indian J Dermatol. 2014;59(1):15-20. doi:10.4103/0019-5154.123482 4. Atıs¸ G, Göktay F, Altan Ferhatog˘lu Z, et al. A proposal for a new severity index for the evaluation of chronic paronychia. Skin Appendage Disord. 2018;5(1):32-37. doi:10.1159/000489024 5. Shafritz AB, Coppage JM. Acute and chronic paronychia of the hand. J Am Acad Orthop Surg. 2014;22(3):165-174. doi:10.5435/JAAOS-22-03-165 6. Bahunuthula RK, Thappa DM, Kumari R, Singh R, Munisamy M, Parija SC. Evaluation of role of Candida in patients with chronic paronychia. Indian J Dermatol Venereol Leprol. 2015;81(5):485-490. doi:10.4103/0378-6323.158635 7. Chow E, Goh CL. Epidemiology of chronic paronychia in a skin hospital in Singapore. Int J Dermatol. 1991;30(11):795-798. doi:10.1111/j.1365-4362.1991.tb04789.x 8. Tosti A, Piraccini BM, Ghetti E, Colombo MD. Topical steroids versus systemic antifungals in the treatment of chronic paronychia: an open, randomized double-blind and double dummy study. J Am Acad Dermatol. 2002;47(1):73-76. doi:10.1067/mjd.2002.122191 9. Rigopoulos D, Gregoriou S, Belyayeva E, Larios G, Kontochristopoulos G, Katsambas A. Efficacy and safety of tacrolimus ointment 0.1% vs. betamethasone 17-valerate 0.1% in the treatment of chronic paronychia: an unblinded randomized study. Br J Dermatol. 2009;160(4):858-860. doi:10.1111/j.1365-2133.2008.08988.x.

CASE #2 Lichen Planus

Lichen planus (LP) is a relatively common inflammatory dermatosis first discovered in 1869 by Erasmus Wilson,1 although the condition previously was reported as “lichen ruber” by Hebra.1-3 The Greek word leichen refers to tree moss and the Latin word planus means planar, referring to the condition’s characteristic flat-topped appearance.1,3 LP was described further clinically in 1892 and 1895, and its histopathology was first described by Darier in 1909.1

Although LP occurs in people of any ethnic background and both sexes, it is slightly more prevalent in women and is much more common in adults than children.2-5 The incidence of LP is estimated to be less than 1%, and it accounts for up to 1.2% of dermatology referrals.3 However, a form of cutaneous LP, actinic LP, is highly prevalent in tropical regions, such as the Middle East, East Africa, and India.3 Mucosal involvement is present in up to 65% of cases of cutaneous LP and can be the only clinical manifestation in 15% to 35% of patients.2

The etiology of LP is thought to be primarily immunemediated.2-4 Cytotoxic CD8+ T cells attack basal keratinocytes and CD4+ helper T cells recruit cytokines,2,3 leading to increased levels of interleukin (IL)-1α, IL-6, and IL-8.6 Other inflammatory cytokines involved include tumor necrosis factor-α and interferon-γ. 6 In the dermis and epidermis, there are increased numbers of dendritic cells expressing FXIIIa and S-100-positive Langerhans cells.2,7 The skin’s basement membrane is damaged by the CD8+ cell-mediated destruction of basal keratinocytes, which then allows more CD8+ cells to infiltrate and continuously attack basal keratinocytes. This cycle is hypothesized to explain the chronic nature of LP.3

LP is associated with a number of viruses such as hepatitis B virus, human herpesviruses 6 and 7, and varicella zoster.3 LP is linked most widely to hepatitis C virus (HCV) infection.3-5 A meta-analysis has shown that patients with LP are 5 times more likely to test positive for HCV than controls.5 In addition, interferon therapy for HCV is linked to LP; some patients experience improvement of LP lesions while on this treatment and others have disease flares.3,4,8 Several familial cases of LP have been reported, suggesting a genetic predisposition.3,5

Other risk factors include certain medications, such as antimalarial drugs, β-blockers, thiazides, angiotensin-converting enzyme inhibitors, and nonsteroidal anti-inflammatory drugs, all of which can trigger lichenoid eruptions.3,4 Anxiety, depression, and stress also are associated with LP.3 Additional conditions linked to LP include thymomas, primary biliary cirrhosis, multiple sclerosis, primary sclerosing cholangitis, diabetes mellitus, ulcerative colitis, and Laugier–Hunziker syndrome.3,6

Classically, LP lesions are characterized by violaceous, flattopped papules. The surfaces of these lesions contain fine, white, reticulated lines referred to as Wickham striae. LP commonly appears on the dorsal hands, flexural aspect of wrists and forearms, shins, and presacral area. LP also may be generalized, although this occurs rarely.

A tool used to identify clinical characteristics of cutaneous LP is the 6 Ps: purple, polygonal, planar, pruritic, papules, and

Dermatology Clinic

plaques.3,4,8 Lesions are often itchy and commonly lead to postinflammatory hyperpigmentation.5 Lesions may display Koebner phenomenon, in which lesions arise on the skin at sites of prior traumatic injury.5 Other clinical variants of LP include actinic, annular, atrophic, bullous, hypertrophic, inverse, linear, and ulcerative LP.2-4,8

Histology of LP characteristically shows compact hyperkeratosis without parakeratosis, wedge-shaped hypergranulosis, basal cell liquefaction, colloid bodies, dense “band-like” inflammatory infiltrate (lymphocytes and histiocytes) in the papillary dermis at the dermoepidermal junction, irregular acanthosis with saw-tooth rete ridges, and pigment incontinence.2,5,6

Lichenoid drug eruptions potentially can be differentiated from idiopathic LP by the presence of eosinophils and/or plasma cells in the infiltrate, focal parakeratosis, focal interruption of the granular layer, and a deep perivascular infiltrate.2

There are no serum or laboratory tests that can be used to diagnose LP. The condition is diagnosed clinically, and, if needed, diagnosis can be confirmed with a punch biopsy.4-6 When LP is suspected, dermoscopy may be used to look for Wickham striae on the surface of the lesions.2 Clinicians also should examine areas commonly affected by LP, including the nails, scalp, genitalia, and oral mucosa.5 Given its strong association with HCV, serum testing for HCV should be considered in patients diagnosed with LP.5

Several conditions must be considered in the differential diagnosis of LP, such as discoid lupus, erythema dyschromicum perstans, pityriasis rosea, psoriasis, secondary syphilis, and other lichenoid dermatoses.4,8 Unlike LP, lichen nitidus tends to appear in younger patients, rarely involves the oral mucosa, and lacks Wickham striae.1,4,5,8 Lichen striatus primarily occurs in younger patients, and the lesions are generally lighter than those of LP, appearing pink, skin colored, or tan, and often occur as a single linear streak of lesions on an extremity.8

Lichenoid drug eruptions are most difficult to differentiate from cutaneous LP because the conditions may appear identical clinically and histologically. Lichenoid drug eruptions potentially may be distinguished from idiopathic LP by the following: generalized and symmetric distribution, photo distribution, lack of Wickham striae, and greater psoriasiform or eczematous morphology.3 Chronic graft-vs-host disease (GVHD) after stem cell transplantation also can result in lesions identical to idiopathic LP.5,8 In cases of suspected drug eruption or GVHD, medication history and medical history, respectively, could help distinguish these conditions from LP.3

Cutaneous LP can resolve on its own after 1 year, but treatment may help lesions resolve more quickly.4,5 Drug-induced LP must be considered in all cases and suspected offending medications should be withdrawn. The first-line therapy for cutaneous LP is potent topical glucocorticoids such as clobetasol. Additional well-described therapies include intralesional and systemic corticosteroids, oral retinoids such as acitretin, phototherapy, topical calcineurin inhibitors, antimalarial agents, and in treatment-resistant cases, oral immunosuppressive agents (eg, methotrexate, cyclosporine, mycophenolate mofetil, azathioprine).2,4,5,8

The condition of the patient in this case was confirmed via punch biopsy, and she was prescribed topical clobetasol ointment. At her 3-month follow-up visit, the active lesions were resolved for the most part, with some residual postinflammatory hyperpigmentation. ■

Cutaneous lichen planus is characterized by the 6 Ps: purple, polygonal, planar, pruritic, papules, and plaques.

Dina Zamil, BS, is a medical student at Baylor College of Medicine, Tara L. Braun, MD, is a resident in the Department of Dermatology at Baylor College of Medicine, and Christopher Rizk, MD, is a dermatologist affiliated with Baylor College of Medicine in Houston, Texas.

References

1. Gupta S, Jawanda MK. Oral lichen planus: an update on etiology, pathogenesis, clinical presentation, diagnosis and management. Indian J Dermatol. 2015;60(3):222-229. doi:10.4103/0019-5154.156315 2. Marshman G. Lichen planus. Australas J Dermatol. 1998;39(1):1-11; quiz 12-13. doi:10.1111/j.1440-0960.1998.tb01233.x 3. Tziotzios C, Lee JYW, Brier T, et al. Lichen planus and lichenoid dermatoses: clinical overview and molecular basis. J Am Acad Dermatol. 2018;79(5):789804. doi:10.1016/j.jaad.2018.02.010 4. Katta R. Lichen planus. Am Fam Physician. 2000;61(11):3319-3324, 3327-3328. 5. Le Cleach L, Chosidow O. Clinical practice. Lichen planus. N Engl J Med. 2012;366(8):723-732. doi:10.1056/NEJMcp1103641 6. Lehman JS, Tollefson MM, Gibson LE. Lichen planus. Int J Dermatol. 2009;48(7):682-694. doi:10.1111/j.1365-4632.2009.04062.x 7. Akasu R, From L, Kahn HJ. Lymphocyte and macrophage subsets in active and inactive lesions of lichen planus. Am J Dermatopathol. 1993;15(3):217-223. doi:10.1097/00000372-199306000-00004 8. Shiohara T, Mizukawa Y. Lichen planus and lichenoid dermatoses. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology, 4th ed. Elsevier; 2018;11:188-207.