Childhood granulomatous periorificial dermatitis (CGPD), is a rare benign granulomatous skin disease of unknown cause. The disorder was first described in 1970 by Gianotti in a

case series A case series (also known as a clinical series) is a type of medical research study that tracks subjects with a known exposure, such as patients who have received a similar treatment, or examines their medical records for exposure and outcome. Cas ...

of five children. CGPD is more common in boys than girls.

Signs and symptoms

CGPD is characterized by the presence of small, raised, dome-shaped, flesh-colored or yellow-brown

papule A papule is a small, well-defined bump in the skin. It may have a rounded, pointed or flat top, and may have a dip. It can appear with a stalk, be thread-like or look warty. It can be soft or firm and its surface may be rough or smooth. Some h ...

s primarily distributed around the mouth, eyes, and nose. Affected children may also have papules on the ears, eyelids, cheeks, forehead, and nose. CGPD skin lesions rarely affect areas of the skin other than the face.

Cause

The cause of CGPD is unknown.

Diagnosis

The approach to diagnosing CGPD is controversial. Certain

dermatologist Dermatology is the branch of medicine dealing with the skin.''Random House Webster's Unabridged Dictionary.'' Random House, Inc. 2001. Page 537. . It is a speciality with both medical and surgical aspects. A dermatologist is a specialist medica ...

s suggest that ruling out infectious and allergic causes of similar skin eruptions and a

skin biopsy Skin biopsy is a biopsy technique in which a skin lesion is removed to be sent to a pathologist to render a microscopic diagnosis. It is usually done under local anesthetic in a physician's office, and results are often available in 4 to 10 days. ...

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 Sarcoidosis (also known as ''Besnier-Boeck-Schaumann disease'') is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph nodes. Less commonly a ...

. On microscopy, non- tuberculous granulomas with surrounding

lymphocyte A lymphocyte is a type of white blood cell (leukocyte) in the immune system of most vertebrates. Lymphocytes include natural killer cells (which function in cell-mediated, cytotoxic innate immunity), T cells (for cell-mediated, cytotoxic ad ...

s clustered around hair follicles may be seen; additionally, infiltrates of epithelioid macrophages, lymphocytes, and

giant cell A giant cell (also known as multinucleated giant cell, or multinucleate giant cell) is a mass formed by the union of several distinct cells (usually histiocytes), often forming a granuloma. Although there is typically a focus on the pathologica ...

s may also be seen. Several conditions exhibit skin findings similar to those of CGPD. These conditions include

perioral dermatitis Perioral dermatitis, also known as periorificial dermatitis, is a common type of skin rash. Symptoms include multiple small (1–2 mm) bumps and blisters sometimes with background redness and scale, localized to the skin around the mouth and ...

, acne vulgaris, granulomatous rosacea,

contact dermatitis Contact dermatitis is a type of acute or chronic inflammation of the skin caused by exposure to chemical or physical agents. Symptoms of contact dermatitis can include itchy or dry skin, a red rash, bumps, blisters, or swelling. These rashes are ...

folliculitis Folliculitis is the infection and inflammation of one or more hair follicles. The condition may occur anywhere on hair-covered skin. The rash may appear as pimples that come to white tips on the face, chest, back, arms, legs, buttocks, or head. ...

, atopic dermatitis, cheilitis, medication-induced acneiform eruptions,

lupus miliaris disseminatus faciei Lupus miliaris disseminatus faciei, also known as acne agminata, is a disease with a similar appearance to acne vulgaris.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). Page 676. McGraw-Hill. . The cause of ...

, benign cephalic histiocytosis, granulosis rubra nasi,

xanthoma A xanthoma (pl. xanthomas or xanthomata) (condition: xanthomatosis) is a deposition of yellowish cholesterol-rich material that can appear anywhere in the body in various disease states. They are cutaneous manifestations of lipidosis in which lip ...

zinc deficiency Zinc deficiency is defined either as insufficient zinc to meet the needs of the body, or as a serum zinc level below the normal range. However, since a decrease in the serum concentration is only detectable after long-term or severe depletion, s ...

glucagonoma Glucagonoma is a very rare tumor of the pancreatic alpha cells that results in the overproduction of the hormone, glucagon. Typically associated with a rash called necrolytic migratory erythema, weight loss, and mild diabetes mellitus, most people ...

, cutaneous

, and scabies.

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 Isotretinoin, also known as 13-''cis''-retinoic acid and sold under the brand name Accutane among others, is a medication primarily used to treat severe acne. It is also used to prevent certain skin cancers ( squamous-cell carcinoma), and in t ...

. Topical antibiotics such as

metronidazole Metronidazole, sold under the brand name Flagyl among others, is an antibiotic and antiprotozoal medication. It is used either alone or with other antibiotics to treat pelvic inflammatory disease, endocarditis, and bacterial vaginosis. It i ...

and erythromycin have been used for CGPD. Oral antibiotics of the

tetracycline Tetracycline, sold under various brand names, is an oral antibiotic in the tetracyclines family of medications, used to treat a number of infections, including acne, cholera, brucellosis, plague, malaria, and syphilis. Common side effects in ...

class such as

minocycline Minocycline, sold under the brand name Minocin among others, is a tetracycline antibiotic medication used to treat a number of bacterial infections such as pneumonia. It is generally less preferred than the tetracycline doxycycline. It is also ...

, doxycycline, and tetracycline have been recommended for CGPD.

Trimethoprim/sulfamethoxazole Trimethoprim/sulfamethoxazole, sold under the brand name Bactrim among others, is a fixed-dose combination antibiotic medication used to treat a variety of bacterial infections. It consists of one part trimethoprim to five parts sulfamethoxa ...

has also been used. The use of oral systemic antibiotics is limited by side effects such as

nausea Nausea is a diffuse sensation of unease and discomfort, sometimes perceived as an urge to vomit. While not painful, it can be a debilitating symptom if prolonged and has been described as placing discomfort on the chest, abdomen, or back of the ...

vomiting Vomiting (also known as emesis and throwing up) is the involuntary, forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose. Vomiting can be the result of ailments like food poisoning, gastroenteri ...

, and sensitivity of the skin to sunlight. 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. The use of

calcineurin inhibitor Immunosuppressive drugs, also known as immunosuppressive agents, immunosuppressants and antirejection medications, are drugs that inhibit or prevent activity of the immune system. Classification Immunosuppressive drugs can be classified into ...

creams such as

tacrolimus Tacrolimus, sold under the brand name Prograf among others, is an immunosuppressive drug. After allogeneic organ transplant, the risk of organ rejection is moderate. To lower the risk of organ rejection, tacrolimus is given. The drug can also ...

pimecrolimus Pimecrolimus is an immunomodulating agent of the calcineurin inhibitor class used in the treatment of atopic dermatitis (eczema). It is available as a topical cream, once marketed by Novartis (however, Galderma has been promoting the compound i ...

on the skin is controversial and results have been mixed. Certain studies found the use of topical calcineurin inhibitors led to resolution of CGPD whereas others saw incomplete resolution or prolonged symptoms. Topical

azelaic acid Azelaic acid (AzA) is an organic compound with the formula HOOC(CH2)7 COOH. This saturated dicarboxylic acid exists as a white powder. It is found in wheat, rye, and barley. It is a precursor to diverse industrial products including polymers an ...

has been used successfully to treat CGPD. Immediate discontinuation of topical corticosteroids is recommended since corticosteroids are thought to worsen CGPD.

Prognosis

CGPD is known to be a temporary skin disease with a benign course. 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 Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations (which can destroy the gene to build up the organ), poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply t ...

depressions with

collagen loss Collagen is a protein that is an important part of connective tissues in the body. It is a rigid, non-soluble and fibrous protein that adds up to one third of the proteins found in the human body. Collagen is mostly made up of molecules that are pa ...

milia A milium (''plural'' milia), also called a milk spot or an oil seed, is a clog of the eccrine sweat gland. It is a keratin-filled cyst that can appear just under the epidermis or on the roof of the mouth.Freedberg, et al. (2003). ''Fitzpatrick' ...

, or small pit-like scars.

Epidemiology

CGPD occurs most often in children of Afro-Caribbean descent before

puberty Puberty is the process of physical changes through which a child's body matures into an adult body capable of sexual reproduction. It is initiated by hormonal signals from the brain to the gonads: the ovaries in a girl, the testes in a bo ...

though reports of this disease occurring in

Asia Asia (, ) is one of the world's most notable geographical regions, which is either considered a continent in its own right or a subcontinent of Eurasia, which shares the continental landmass of Afro-Eurasia with Africa. Asia covers an are ...

n and

Caucasian Caucasian may refer to: Anthropology *Anything from the Caucasus region ** ** ** ''Caucasian Exarchate'' (1917–1920), an ecclesiastical exarchate of the Russian Orthodox Church in the Caucasus region * * * Languages * Northwest Caucasian l ...

children have also been described. 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. CGPD is more common in boys than girls.

History

Gianotti et al. first described CGPD in five Italian children in 1970. 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)". 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.

References

{{reflist Cutaneous conditions Dermatitis Idiopathic diseases Pediatrics Rare diseases