Dermatitis periorale granulomatous of childhood L71.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Childhood granulomatous periorificial dermatitis; Dermatitis; FACE; facial Afro-Caribbean childhood eruption (FACE); Gianotti-type perioral dermatitis; granulomatous; granulomatous perioral dermatitis; Perioral granulomatous dermatitis of childhood; periorifizielle; sarcoid-like granulomatous dermatitis

History
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Gianotti, 1970

Definition
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Rare, self-limiting, granulomatous disease of the perioral, perinasal and ocular facial skin with occasional extrafacial involvement, occurring mainly in African-American and Caribbean children. S.a.o. rosacea, lupoid.

Etiopathogenesis
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Unknown. Hypothesis: Non-specific granulomatous response to various topical and systemic substances such as topical fluorinated corticosteroids, antiseptic solutions, formaldehyde, vaccines. Variant of perioral dermatitis?

Manifestation
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No sex preference.

Clinical features
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Grouped, monomorphic yellow-brown, asymptomatic, 0.1-0.3 cm large papules on partly reddened skin, rarely scaling, possibly diffuse effluvium. No general symptoms. Extracutaneous manifestations with blepharitis and conjunctivitis are possible.

Histology
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Perifollicular non-cheesy granulomas or granulomatous infiltrates with epithelioid histiocytes in the dermis; partly focal erpidermal spongiosis.

Diagnosis
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Clinic, Histology

Differential diagnosis
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  • Rosacea granulomatosa (inappropriate age)
  • Sarcoidosis (no lung involvement)
  • Mycoses (fungus detection)
  • Mycobacterioses (pathogen detection)
  • Blue syndrome (= familial juvenile systemic granulomatosis).

External therapy
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Metronidazole, erythromycin, ichthyol ointment (1-2%), therapy duration several weeks to months.

Cave! Contraindicated: Local and systemic corticosteroids.

Internal therapy
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Macrolides, e.g. erythromycin.

Progression/forecast
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Self-limiting; complete healing after a few months up to 3 years with the possibility of leaving flat atrophic scars. Accelerated healing under systemic and/or local antibiotic therapy.

Literature
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  1. Antony FC et al (2002) Childhood granulomatous periorificial dermatitis in an Asian girl--a variant of sarcoid? Clin Exp Dermatol 27: 275-276
  2. Frieden IJ et al (1989) Granulomatous perioral dermatitis in children. Arch Dermatol 125: 369-373
  3. Gianotti F et al (1970) Particuliere dermatite peri-orale infantile: oberservations sur cinq cas. Bull Soc Fr Dermatol Syph 77: 341
  4. Hafeez ZH (2003) Perioral dermatitis: an update. Int J Dermatol 42: 514-517
  5. Smitt JH et al (1991) Granulomatous perioral dermatitis (facial Afro-Caribbean childhood eruption [FACE]). Br J Dermatol 125: 399
  6. Urbatsch AJ et al (2002) Extrafacial and generalized granulomatous periorificial dermatitis. Arch Dermatol 138: 1354-1358
  7. Zalaudek I et al (2005) Childhood granulomatous periorificial dermatitis: a controversial disease. JDDG 3: 252-255

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020