Diagnosis
A diagnosis of perioral dermatitis is typically made based on the characteristics of the rash. A skin biopsy is usually not required to make the diagnosis but can be helpful to rule out other skin diseases which may resemble perioral dermatitis. Extended patch testing may be useful to also rule out allergic contact causes.Other skin diseases that may resemble perioral dermatitis include:

Rosacea
Acne vulgaris
Seborrheic dermatitis
Allergic contact dermatitis
Irritant contact dermatitis
Angular cheilitis

Treatment
Multiple treatment regimes are available and treatment algorithms have been proposed.Perioral dermatitis will usually resolve within a few months without medication, by limiting the use of irritants, including products with fragrance, cosmetics, benzoyl peroxide, occlusive sunscreens, and various acne products. This is called zero treatment. Topical corticosteroids should be stopped entirely if possible. If the flare proves intolerable, temporary use of a less potent topical corticosteroid can often be helpful. Medication
A number of medications, either applied directly to the skin or taken by mouth, may hasten recovery. These include tetracycline, doxycycline, and erythromycin. Erythromycin may be used as a cream. Doxycycline is most often the first antibiotic drug choice, given at a daily dosage of 100 mg for up to a month before considering tapering off or stopping. Sometimes, longer duration of low doses of doxycycline are required.Metronidazole is less effective, but is available in a gel and can be applied twice daily. If the perioral dermatitis was triggered by a topical steroid then pimecrolimus cream has been suggested as effective in improving symptoms. However, this has also been documented to cause the condition.
The two, however, are clinically, radiographically, and genetically distinct from one another. Treatment
People with Pyle disease are often asymptomatic. Dental anomalies may require orthodontic interventions. Skeletal anomalies may require orthopedic surgery. Epidemiology
Pyle disease is thought to be a rare disorder, although its prevalence is unknown. More than 25 cases have been described in the medical literature.It has been described in four German families originating from the same town in Bohemia and in a 7-year-old Japanese girl. Research
Cortical-bone fragility is a common feature in osteoporosis that is linked to nonvertebral fractures. Regulation of cortical-bone homeostasis has proved elusive. The study of genetic disorders of the skeleton can yield insights that fuel experimental therapeutic approaches to the treatment of rare disorders and common skeletal ailments. The study showed that Pyle’s disease was caused by a deficiency of sFRP4, that cortical-bone and trabecular-bone homeostasis were governed by different mechanisms, and that sFRP4-mediated cross-regulation between Wnt and BMP signaling was critical for achieving proper cortical-bone thickness and stability.Recently, homozygous mutations in secreted frizzled-related protein 4 gene (SFRP4) gene were found to underlie this condition. Sequencing of coding regions of SFRP4 gene from an 11-year-old female with PYL was performed. A novel homozygous nonsense variant, c.183C>G (p.Y61*) was observed. Segregation analysis in the patient revealed a germline mutation, resulting in reduced protein formation. References


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