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Case Note

Case Note: Papular Eruption on the Nose

History

A 38-year-old male presents for management of numerous papules predominantly on the nose.

Initial evaluation

  • Healthy appearing African American male
  • No significant past medical history
  • Social history is unremarkable
  • Review of systems reveals a chronic persistent cough over the past 4-5 months and mild fatigue, with no ocular complaints, no palpitations, and no other systemic complaints
    • Cough is dry and nonproductive (occasionally evident during exam)
  • Presents with numerous erythematous-to-slightly violaceous papules concentrated on the nasal tip and around the alar rim, with papules also scattered on the cheeks and around the eyes and mouth
    • Lesions first noted by the patient a bout 4 months ago
    • Lesions are asymptomatic
    • Over-the-counter topical steroids have provided no relief
    • Detailed examination reveals a few scaly papules within a tattoo on the arm
  • There is no lymphadenopathy or hepatosplenomegaly on exam
  • The patient's eyes appear red with conjunctival injection
  • Image 1
    Image 1

Enter your diagnosis

Diagnosis

  • A diagnosis of sarcoidosis is rendered

Initial Treatment

  • It is recommend that the patient undergoes chest imaging and pulmonary function testing and sees a pulmonologist for suspected pulmonary sarcoidosis
  • Patient is referred to an ophthalmologist for clinical evidence of active ocular inflammation and the risk of asymptomatic uveitis progressing to visual impairment
  • A biopsy is requested of a representative skin lesion (arm tattoo lesion)
  • Routine blood work to be undertaken including complete blood count and comprehensive metabolic profile

Follow-up evaluation strategy

Follow-up evaluation:

  • The patient has hilar adenopathy on chest X-ray but normal pulmonary function test
    • He is started on a course of oral corticosteroids by his new pulmonologist with significant improvement
  • On ocular exam the patient has uveitis and is treated with topical corticosteroids
  • The patient is sent for additional testing for extracutaneous disease, including cardiac evaluation, vitamin D testing, thyroid testing, hepatic and renal monitoring
    • All results come back normal or negative
  • The skin lesions improve somewhat with systemic corticosteroids, but recur as the dose is tapered
  • Topical and intralesional corticosteroid injections are tried with good response
  • The patient is monitored closely by multiple doctors, as African American patients with facial cutaneous lesions tend to have persistent disease which fails to self-resolve


Further recommendations:

  • As well as referral to a pulmonologist and a ophthalmologist, referral to an otolaryngologist should also be considered in patients with nasal involvement
  • Patients who have tattoo-associated lesions should be counseled to avoid more tattoos
  • As was prescribed in this present case, topical corticosteroids may be administered (hydrocortisone 2.5% to the face, triamcinolone 0.1% to the body, clobetasol 0.05% to thick lesions on areas of thick skin such as the elbows or back)
  • As was further indicated in this present case, intralesional corticosteroid injections may be administered (triamcinolone; strength varying by anatomic site, size, and depth of lesion)
     

Subsequent treatment steps:

  • Treatment is a stepwise progression and multiple treatments may be used together
    • Patients can continue to receive topical and/or intralesional corticosteroids along with antimalarials and/or tetracycline class antibiotics while proceeding to more aggressive/suppressive therapy
  • Methotrexate may be beneficial to patients with widespread skin disease or skin disease that fails to respond to first-line treatment
  • Thalidomide is an alternate option for patients with widespread skin disease, particularly with recalcitrant facial lesions
    • Given the teratogenicity of the drug, patients and providers must be registered in the Risk Evaluation and Mitigation Strategy (REMS) program to use thalidomide
      • REMS, formerly the System for Thalidomide Education and Prescribing Safety (STEPS) program, is a US Food and Drug Administration program for pharmaceutical risk management
  • Patients with the most severe disease, or those who fail to respond to methotrexate, may be candidates for treatment with tumor necrosis factor alpha (TNF-α) inhibitors, particularly infliximab or adalimumab

General discussion

Sarcoidosis is a complex multisystem inflammatory disease characterized by non-caseating granulomatous inflammation. The vast majority of patients have lung involvement (90-95%), the skin and eye being the second and third most commonly affected organ systems. Skin involvement occurs in approximately 30% of patients with sarcoidosis, although the incidence and severity can vary by ethnic group. While sarcoidosis occurs in all ethnic groups and age ranges, with an overall prevalence of 10-40 cases per 100,000 persons, the annual incidence in African Americans is significantly higher, while that in Japanese is markedly lower. Scandinavia has the world's highest prevalence, but patients of African American ancestry are more likely to have chronic cutaneous disease and present to a dermatologist for evaluation and management. The incidence peaks in patients in their 30s-40s, and some populations display a bimodal distribution. The disease is slightly more common in women than in men. The disease phenotype may vary by ethnic group, with African American patients having more chronic cutaneous disease, Northern Europeans/Scandinavians having higher rates of erythema nodosum and acute but self-limited presentations of disease, and Japanese patients having higher rates of ocular and cardiac involvement.
 

The etiopathogenesis of sarcoidosis remains unknown. The current leading theory speculates that patients inherit a genetic predisposition for an exuberant inflammatory response to an unknown or group of unknown antigens; when exposed, patients mount an inflammatory response, representing the onset of the disease. Putative antigens include an unknown autoantigen, misfolded serum amyloid A proteins, infectious agents (including mycobacteria or Propionibacterium acnes), particulate matter, or heavy metals/dust/debris. Whatever the inciting exposure, these genetically predisposed patients mount an abnormal immune response.
 

Sarcoidosis is characterized by granulomatous inflammation - affected organs develop infiltrates of macrophages and lymphocytes that aggregate into non-caseating granulomas composed of groups of histiocytes and giant cells, with a sparse rim of lymphocytes (so-called 'naked' granulomas). These foci of granulomatous infiltrates may occur throughout the body; the distribution of inflammation determines the disease phenotype. In some patients, the inflammatory response is acute and self-limited; other patients develop persistent inflammation; and some patients develop inflammation that leads to scarring, fibrosis, and permanent organ dysfunction.
 

Approximately 60% of patients with sarcoidosis will spontaneously remit within the first 2-3 years of disease presentation. Of the remaining patients, some will develop scarring and fibrosis with permanent organ damage, while some patients will have persistent ongoing areas of active inflammation. It is these sites of active disease that are amenable to therapeutic intervention.
 

Cutaneous sarcoidosis generally affects 25-30% of patients with the disease; this number varies depending on the ethnic group. Cutaneous sarcoidosis is generally divided into 'specific lesions', those that display characteristic granulomas on biopsy, and 'sarcoid non-specific lesions', which generally refers to erythema nodosum. Erythema nodosum (EN) is most often seen in patients of Northern European ancestry, presenting with tender red-brown subcutaneous nodules symmetrically distributed on the anterior shins. EN is most often seen in the setting of a specific subtype of sarcoidosis, Löfgren's syndrome, wherein patients present with fevers, arthritis/arthralgias, hilar adenopathy, and EN. This is almost invariably seen in Caucasian patients and portends a good prognosis; most patients with Löfgren's syndrome will spontaneously resolve their disease, although they may require symptomatic treatment during the acute inflammatory phase.
 

Cutaneous sarcoidosis-specific lesions are protean and can present with almost any type of morphology, including papules, plaques, annular lesions, lichenoid papules, verrucous/hyperkeratotic papules, psoriasiform/papulosquamous plaques, ichthyosiform patches, subcutaneous nodules, scar- and tattoo-associated papules, ulcers, hypopigmented patches, erythroderma, and scarring or non-scaring alopecia. In short, patients with virtually any skin lesion and findings or symptoms concerning for systemic sarcoidosis may have cutaneous involvement, and physicians should maintain a high index of suspicion and low threshold to perform skin biopsy for confirmation. The most common cutaneous findings are erythematous-to-violaceous papules, generally concentrated around the nose, ala, and periorbital, or perioral areas. Plaques are also quite common. Lupus pernio is a specific morphologic pattern of sarcoidosis worthy of special mention. Common in African Americans, patients with lupus pernio have violaceous scaly plaques and nodules of the nose or central cheeks. Patients with lupus pernio are more likely to have chronic, refractory disease that often persists for many years, with some patients having lifelong skin involvement. These patients are often refractory to most standard treatments. Beyond papules, plaques, and lupus pernio, scar- and tattoo-associated lesions are also common. The other morphologic types are less common, but given the widely varied nature of cutaneous sarcoidal lesions and the easy accessibility of skin lesions and low morbidity associated with skin biopsy, dermatologists are often involved in helping make the diagnosis of sarcoidosis by skin biopsy to demonstrate the characteristic granulomas.
 

Ninety percent or more of patients with sarcoidosis will have lung involvement; this can range from asymptomatic hilar adenopathy picked up incidentally on routine chest imaging, to mild chronic cough or dyspnea, to fulminant pulmonary involvement with massive infiltrates requiring aggressive therapy, oxygen supplementation, and eventual organ transplantation. Ocular involvement can range from asymptomatic disease to significant inflammation, putting vision at risk; uveitis is the most common manifestation. Hepatic involvement is common but rarely clinically relevant. 

Further reading

Chen ES, Moller DR. Sarcoidosis-scientific progress and clinical challenges. Nat Rev Rheumatol 2011;7:457-67.

Haimovic A, Sanchez M, Judson MA, et al. Sarcoidosis: a comprehensive review and update for the dermatologist. Part I: Cutaneous Disease. J Am Acad Dermatol 2012;66:699.e1-18.

Haimovic A, Sanchez M, Judson MA, et al. Sarcoidosis: a comprehensive review and update for the dermatologist. Part I: Extracutaneous Disease. J Am Acad Dermatol 2012;66:719.e1-10.

Judson MA. The clinical features of sarcoidosis: a comprehensive review. Clinic Rev Allerg Immunol 2015;49:63-78.

Wanat KA, Rosenbach M. A practical approach to cutaneous sarcoidosis. Am J Clin Dermatol 2014;15:283-97.

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