Childhood granulomatous periorificial dermatitis (CGPD) is a rare benign granulomatous skin disease of unknown cause.[1] The disorder was first described in 1970 by Gianotti in a case series of five children.[2] CGPD is more common in boys than girls.[3]
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.[1] Affected children may also have papules on the ears, eyelids, cheeks, forehead, and nose.[1] CGPD skin lesions rarely affect areas of the skin other than the face.[1]
The approach to diagnosing CGPD is controversial.[2] 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.[2] 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.[2][3]
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.[2] Topical antibiotics such as metronidazole and erythromycin have been used for CGPD.[2] Oral antibiotics of the tetracycline class such as minocycline, doxycycline, and tetracycline have been recommended for CGPD.[1]Trimethoprim/sulfamethoxazole has also been used.[2] The use of oral systemic antibiotics is limited by side effects such as nausea, vomiting, and sensitivity of the skin to sunlight.[1] 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.[1]
The use of calcineurin inhibitor creams such as tacrolimus or pimecrolimus on the skin is controversial and results have been mixed.[1] Certain studies found the use of topical calcineurin inhibitors led to resolution of CGPD whereas others saw incomplete resolution or prolonged symptoms.[1] Topical azelaic acid has been used successfully to treat CGPD.[1] Immediate discontinuation of topical corticosteroids is recommended since corticosteroids are thought to worsen CGPD.[2]
Prognosis
CGPD is known to be a temporary skin disease with a benign course.[1] 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.[1]
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.[1][3] 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.[2] CGPD is more common in boys than girls.[3]
History
Gianotti et al. first described CGPD in five Italian children in 1970.[3] 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)".[3] 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.[3]
^ abcdefghijZalaudek, I; Di Stefani, A; Ferrara, G; Argenziano, G (April 2005). "Childhood granulomatous periorificial dermatitis: a controversial disease". Journal of the German Society of Dermatology (Review). 3 (4): 252–5. doi:10.1111/j.1610-0387.2005.05009.x. PMID16370472. S2CID9802651.
