What's the Diagnosis?: Systemic Lupus Erythematosus

Column Author: Sean Reynolds, MBBCH | Associate Program Director, Pediatric Dermatology Fellowship

Column Editor: Angela L. Myers, MD, MPH | Division Director, Infectious Diseases; Medical Director, Center for Wellbeing

A 5-year-old boy with a history of systemic lupus erythematosus (SLE) presented to the dermatology clinic for evaluation of a new rash. Mother noted that the rash developed one month ago, initially appearing as “bumps” around his nose, which gradually spread around his mouth and eyes. She observed that some lesions became larger and pink, resembling “pimples.” The rash does not seem to bother the patient, and it has not bled, drained or itched. Initially, the mother used oatmeal soap to wash the area but has been using warm water for the past few days without improvement.

She denies any recent illness, travel history, exposure to sick contacts, family members with similar rashes, new soaps or detergents, or any other known rash triggers.

The patient was diagnosed with SLE two years ago after presenting with fevers and a rash. His condition has since been well controlled with an oral prednisolone taper, hydroxychloroquine, mycophenolate mofetil and rituximab infusions.

1. Which of the following is the most likely diagnosis?
   A) Cutaneous lupus
   B) Lip licker’s dermatitis
   C) Tinea faciei (ringworm)
   D) Periorificial dermatitis

1. D) Periorificial dermatitis
   This patient presents with a one-month history of a facial papular eruption. On examination, he has skin-colored to pink 2-3 mm papules, with occasional larger pink to red inflammatory papules. These are distributed around the mouth and nose, with a few small skin-colored papules on the lower eyelids. The morphology and distribution of the lesions are suggestive of periorificial dermatitis.

Periorificial dermatitis is an often-missed and frequently misdiagnosed facial eruption. It consists of erythematous papules and papulopustules typically distributed around the perioral, nasolabial and periocular areas. As the papules resolve, they may leave residual erythema or scale. Large inflammatory papules or nodules may also occur. This patients likely had “granulomatous periorificial dermatitis,” a variant which presents with skin colored to pink papules without pustules or scale and is more common in patients with skin of color.

While the cause is unknown, periorificial dermatitis is thought to represent a childhood form of rosacea, particularly ocular rosacea, as both conditions share similar histological features on biopsy. This similarity may also explain the increased incidence of blepharitis, conjunctivitis, chalazion and hordeolum (stye) in children with periorificial dermatitis and adults with ocular rosacea.

The course of periorificial dermatitis is usually self-limited, though resolution may take months to years without treatment.

Differential Diagnosis:

• Cutaneous lupus: Although cutaneous lupus should be considered given the patient’s history, it is unlikely in this case because the patient’s nasolabial and perioral papules would be an unusual presentation for cutaneous lupus. The more common variants of cutaneous lupus include acute cutaneous lupus (e.g., “butterfly” or malar rash), subacute cutaneous lupus (photodistributed annular or round, scaly papules and plaques), and discoid lupus (erythematous or violaceous papules or plaques with hyperpigmentation, hypopigmentation and atrophy).
• Lip-licker’s dermatitis: This condition is a common perioral eruption caused by irritant contact dermatitis from saliva due to repeated licking of the lips. The constant wet-dry cycle of saliva disrupts the normal skin barrier, leading to inflammation. The eruption typically involves erythema, dryness, and scaling of the lips and surrounding skin, corresponding to the area reachable by the patient’s tongue. Unlike periorificial dermatitis, where the lesions extend beyond the reach of the tongue and involve the nasolabial and periocular areas, lip-licker’s dermatitis is generally confined to the perioral region. Additionally, periorificial dermatitis does not typically involve the vermilion border of the lips or the skin immediately adjacent to the vermilion border and generally presents as clusters of small papules or pustules, which are not usually seen in lip-licker’s dermatitis.
• Tinea faciei (ringworm): Fungal infections of the face typically do not restrict themselves to the perioral, nasolabial or periocular areas. Instead, they usually present as round or annular scaly plaques, not as small papules as seen in this patient.

2. Which of the following can trigger or worsen the eruption?
   A) Inhaled budesonide inhaler
   B) Intranasal fluticasone spray
   C) Oral prednisolone
   D) Topical triamcinolone ointment
   E) All of the above

1. E) All of the above

Corticosteroid exposure is an important consideration in patients with suspected periorificial dermatitis. In a study of pediatric patients, over 70% had a history of exposure to topical, inhaled or oral steroids. When periorificial dermatitis is misdiagnosed as another dermatosis, a trial of topical steroids can significantly worsen the eruption. Discontinuation of topical steroids is recommended, although some patients may experience a transient “rebound flare” before improvement is seen. Strategies to mitigate exposure to inhaled corticosteroids include switching from a mask spacer to a mouth spacer (when age-appropriate) or using a gentle cleanser to wash the face after inhaled or intranasal steroid use. However, in some cases, such as this patient’s, discontinuing steroid exposure may not be feasible or appropriate, and “treating through” the exposure may be necessary.

3. Which of the following treatments is most appropriate?A) Topical steroids
   B) Topical ketoconazole cream
   C) Topical calcineurin inhibitor
   D) Topical zinc oxide cream

1. C) Topical calcineurin inhibitor

Several oral and topical treatment options can be effective in treating periorificial dermatitis. A recent study showed that approximately two-thirds of patients achieved clearance with twice-daily use of the topical calcineurin inhibitor pimecrolimus. Topical tacrolimus is another effective alternative. Topical antibiotics, such as metronidazole and erythromycin, are also effective and can be used alone or in combination with other treatments. For more severe or recalcitrant cases, oral erythromycin or tetracyclines (in patients older than 8 years) may be used. Topical ketoconazole and zinc oxide cream are not usually effective in treating the condition.

Treatment should be continued for a minimum of six to eight weeks, with gradual tapering, to avoid the rapid rebound often seen with shorter courses of therapy.

References:

1. Ollech A, Yousif R, Kruse L, et al. Topical calcineurin inhibitors for pediatric periorificial dermatitis. J Am Acad Dermatol. 2020;82(6):1409-1414. PMID: 32032693. doi:10.1016/j.jaad.2020.01.064
2. Goel NS, Burkhart CN, Morrell DS. Pediatric periorificial dermatitis: clinical course and treatment outcomes in 222 patients. Pediatr Dermatol. 2015;32(3):333-336. PMID: 25847356. doi:10.1111/pde.12534
3. Nguyen V, Eichenfield LF. Periorificial dermatitis in children and adolescents. J Am Acad Dermatol. 2006;55(5):781-785. PMID: 17052482. doi:10.1016/j.jaad.2006.05.031
4. Tempark T, Shwayder TA. Perioral dermatitis: a review of the condition with special attention to treatment options. Am J Clin Dermatol. 2014;15(2):101-113. PMID: 24623018. doi:10.1007/s40257-014-0067-7