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Childhood granulomatous periorificial dermatitis
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Synonyms Gianotti-type perioral dermatitis or Facial Afro-Caribbean childhood eruption
Pronounce
Field Dermatology
Symptoms
Complications
Onset
Duration
Types
Causes
Risks
Diagnosis
Differential diagnosis
Prevention
Treatment
Medication
Prognosis
Frequency
Deaths


Childhood granulomatous periorificial dermatitis (CGPD), is a rare benign granulomatous skin disease of unknown cause.<ref name="Dessinioti2014">,

 Acneiform eruptions, 
 Clinics in Dermatology, 
 
 Vol. 32(Issue: 1),
 pp. 24–34,
 DOI: 10.1016/j.clindermatol.2013.05.023,
 PMID: 24314375,</ref> The disorder was first described in 1970 by Gianotti in a case series of five children.<ref name="Zalaudek2005">, 
 Childhood granulomatous periorificial dermatitis: a controversial disease, 
 Journal of the German Society of Dermatology, 
 
 Vol. 3(Issue: 4),
 pp. 252–5,
 
 PMID: 16370472,</ref> CGPD is more common in boys than girls.<ref name="Kim2011"/> 

Signs and symptoms

CGPD is characterized by the presence of small, raised, dome-shaped, flesh-colored or yellow-brown papules primarily distributed around the mouth, eyes, and nose.<ref name="Dessinioti2014"/> Affected children may also have papules on the ears, eyelids, cheeks, forehead, and nose.<ref name="Dessinioti2014"/> CGPD skin lesions rarely affect areas of the skin other than the face.<ref name="Dessinioti2014"/>

Cause

The cause of CGPD is unknown.<ref name="Kim2011">,

 Childhood granulomatous periorificial dermatitis, 
 Annals of Dermatology, 
 
 Vol. 23(Issue: 3),
 pp. 386–8,
 DOI: 10.5021/ad.2011.23.3.386,
 PMID: 21909215,
 PMC: 3162274,</ref>

Diagnosis

The approach to diagnosing CGPD is controversial.<ref name="Zalaudek2005"/> Certain dermatologists suggest that ruling out infectious and allergic causes of similar skin eruptions and a skin biopsy demonstrating features consistent with CGPD is adequate for diagnosis. In contrast, other dermatologists advocate for performing a complete history and physical examination and obtaining laboratories and appropriate imaging to rule out cutaneous sarcoidosis.<ref name="Zalaudek2005"/> On microscopy, non-tuberculous granulomas with surrounding lymphocytes clustered around hair follicles may be seen; additionally, infiltrates of epithelioid macrophages, lymphocytes, and giant cells may also be seen.<ref name="Zalaudek2005"/><ref name="Kim2011"/>

Several conditions exhibit skin findings similar to those of CGPD. These conditions include perioral dermatitis, acne vulgaris, granulomatous rosacea, contact dermatitis, folliculitis, atopic dermatitis, cheilitis, medication-induced acneiform eruptions, lupus miliaris disseminatus faciei, benign cephalic histiocytosis, granulosis rubra nasi, xanthomas, zinc deficiency, glucagonoma, cutaneous sarcoidosis, and scabies.<ref name="Zalaudek2005"/><ref name="Kim2011"/>

Treatment

Treatment recommendations for CGPD vary and may include observation without treatment, stopping the use of topical corticosteroids, and the use of topical or oral antibiotics as well as isotretinoin.<ref name="Zalaudek2005"/> Topical antibiotics such as metronidazole and erythromycin have been used for CGPD.<ref name="Zalaudek2005"/> Oral antibiotics of the tetracycline class such as minocycline, doxycycline, and tetracycline have been recommended for CGPD.<ref name="Dessinioti2014"/> Trimethoprim/sulfamethoxazole has also been used.<ref name="Zalaudek2005"/> The use of oral systemic antibiotics is limited by side effects such as nausea, vomiting, and sensitivity of the skin to sunlight.<ref name="Dessinioti2014"/> Tetracycline antibiotics are not recommended for children under the age of 8 since tetracyclines are known to deposit in teeth (thereby staining them) and impair bone growth in children.<ref name="Dessinioti2014"/>

The use of calcineurin inhibitor creams such as tacrolimus or pimecrolimus on the skin is controversial and results have been mixed.<ref name="Dessinioti2014"/> Certain studies found the use of topical calcineurin inhibitors led to resolution of CGPD whereas others saw incomplete resolution or prolonged symptoms.<ref name="Dessinioti2014"/> Topical azelaic acid has been used successfully to treat CGPD.<ref name="Dessinioti2014"/> Immediate discontinuation of topical corticosteroids is recommended since corticosteroids are thought to worsen CGPD.<ref name="Zalaudek2005"/>

Prognosis

CGPD is known to be a temporary skin disease with a benign course.<ref name="Dessinioti2014"/> The skin papules typically resolve after a few months to a few years. After CGPD resolves, the skin may return to normal without scarring or may have small atrophic depressions with collagen loss, milia, or small pit-like scars.<ref name="Dessinioti2014"/>

Epidemiology

CGPD occurs most often in children of Afro-Caribbean descent before puberty though reports of this disease occurring in Asian and Caucasian children have also been described.<ref name="Dessinioti2014"/><ref name="Kim2011"/> Due to the limited number of reported cases, it remains controversial whether CGPD occurs more often in African children than in children of other races.<ref name="Zalaudek2005"/> CGPD is more common in boys than girls.<ref name="Kim2011"/>

History

Gianotti et. al first described CGPD in five Italian children in 1970.<ref name="Kim2011"/> In 1990, Williams et. al described a similar skin eruption in five children of Afro-Caribbean descent and coined the proposed term "facial Afro-Caribbean childhood eruption (FACE)".<ref name="Kim2011"/> Subsequently, another article by Katz and Lesher first introduced the term CGPD since some reported cases were not found in children of Afro-Caribbean descent and to avoid confusion with perioral dermatitis.<ref name="Kim2011"/>

See also

Perioral dermatitis

References