Answer

Diagnosis: Childhood granulomatous periorifical dermatitis (CGPD).

Discussion

Childhood granulomatous periorificial dermatitis (CGPD) was first described in 1970 by Gianotti et al. ^[1] as an asymptomatic perioral eruption in children having a waxing and waning course. Later, it was reported by Marten et al., ^[2] and Frieden et al. ^[3] who named it as "granulomatous perioral dermatitis in children". The term "facial Afro-Carribean childhood eruption (FACE)" was coined by Williams et al. ^[4] in 1990, to reflect its occurrence commonly in Afro-Carribean children. Finally, in 1996 Knautz et al. ^[5] used the term CGPD to highlight the frequent perinasal or periocular involvement and to avoid use of the term FACE which limits the diagnosis to Afro-Carribean subjects.

The etiology of periorificial granulomatosis is unknown, but long-term use of topical steroids is known to be related to the disease. ^[6] Majority of the cases of this disease have been reported to occur in dark-skinned Afro-Carribean children. Only one Asian case has been reported so far. ^[7] The lesions begin as an asymptomatic, discrete 1- to 3-mm dome-shaped red or yellow-brown papules around the mouth, nose and eyes of an otherwise healthy child. Childhood granulomatous perioral dermatitis with involvement of the neck and upper trunk has also been reported. ^[8] Previous case reports relating CGPD to cutaneous sarcoidosis and granulomatous rosacea have been described; however, its etiology still remains controversial. ^[6]

Histopathologically, perifollicular or interfollicular dermal granulomatous infiltrates are observed consisting of epithelioid cells, multinucleated giant cells and surrounding lymphocytes in absence of caseation necrosis. The disease is benign and self-limited. However, resolution seems to be hastened with the use of rosacea medications, such as oral macrolides or tetracyclines, topical erythromycin or metronidazole ^[8] and avoidance of fluorinated steroids. A few case reports mention resolution of lesions with use of topical pimecrolimus.

Differential diagnosis includes perioral dermatitis (PD), rosacea, sarcoidosis, and lupus miliaris disseminatus faciei. PD is different from CGPD by its occurrence in young women in the form papules and pustules associated with scaling of the surrounding skin and sparing of the vermilion border. Rosacea usually affects the central third of the face and is characterized by flushing, telangiectasia and erythematous papulopustules and does not occur in children.

Sarcoidosis on the other hand is rare in children and when it occurs in children, systemic symptoms, such as fatigue, fever, cough and dyspnea, usually accompany. Multi-organ involvement which involves the lungs, lymph nodes and eyes has been seen to occur.

LMDF affects the face with a predeliction for peri-occular region and heals leaving behind a scar. Histopathologically, epithelioid cell granuloma with caseation necrosis is classically described; however, absence of caseating granuloma, granulomas with central neutrophillic abscess formation and perifollicular infiltrates of lymphocytes and scant neutrophils have also been described. ^[9]

However, CGPD is an asymptomatic monomorphic papular eruption around the mouth and eyes in children which involves the vermilion border. Therefore, once we have ruled out other conditions while making its diagnosis, we can be assured of its spontaneous healing without scar formation. ^[10]



The names of the winners(first three correct entries) are:

1. Bhushan S Madke, Mumbai
2. Humanshu Gupta, New Delhi
3. Geeti Khullar, Delhi

"Others who have sent correct answers are mentioned below:"

Anupam Das, Vinay K, Naveen Kumar Kansal, Shraddha Uprety, Dhiraj Kumar, Suresh Kumar K, Shouvik Ghosh, Mrinal Gupta, Vishal Chugh, Rashmi Sriram, Tirthankar Gayen, Akhilesh Shukla, Shahid Hassan, Vanya Narayan, Soumyajit Roychoudhury, Naveen Keswani, Bharati Sahu, Laxmisha Chandrashekar, S. Murugan, Pooja Aggarwal, Rishu Sarangal, Sarita Sanke, Akhilesh.A, Rameshwar Gutte, Dipti Das, Deblina Bhunia, Gayatri M.Karad, Hari Kishan Kumar.Y, Rinu Ruth George, Shvetha Jain, Mugdha Jhamwar, Shilpa Y K, Brahmita Monga, Aditi Jha, Anisha Sethi, Saurabh Singh, Balaji Govindan, Meghana Phiske, Anjali Pal, Vinitha Varghese