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CASE REPORT |
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| Year : 2022 | Volume
: 67
| Issue : 6 | Page : 747-751 |
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| Clinical characteristics of 9 adult patients with granulomatous periorificial dermatitis and comparison with childhood granulomatous periorificial dermatitis |
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Soyun Cho1, Bo Ri Kim2, Ji Su Lee1, Jung Im Na2, Sang Woong Youn2
1 From the Department of Dermatology, Seoul Metropolitan Government National University (SMG-SNU) Boramae Medical Center; Department of Dermatology, SNU College of Medicine, Seoul, Korea
2 Department of Dermatology, SNU College of Medicine, Seoul; Department of Dermatology, SNU Bundang Hospital, Gyeonggi, Korea
| Date of Web Publication | 23-Feb-2023 |
Correspondence Address:
Sang Woong Youn
Department of Dermatology, Seoul National University Bundang Hospital, 82 Gumi-Ro 173 Beon-Gil, Seongnam, Gyeonggi 13620
Korea
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijd.ijd_14_21
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 Abstract | | |
To our knowledge, there have been no previous reports of granulomatous periorificial dermatitis (GPD) in adult patients in contrast to childhood GPD (CGPD). We report cases of 9 adult patients with GPD with regards to the clinical and histopathological characteristics and their management. GPD in adults may be an entity that is actually underdiagnosed, especially in middle-aged females. It is a benign disorder, albeit requiring a relatively long-term treatment. Unlike CGPD, GPD in adults is frequently accompanied by pruritus with predilection for the eyelid and should be treated initially with oral medication.
Keywords: Adults, childhood granulomatous periorificial dermatitis, granulomatous periorificial dermatitis, papule, perioral dermatitis, periorificial dermatitis
How to cite this article:
Cho S, Kim BR, Lee JS, Na JI, Youn SW. Clinical characteristics of 9 adult patients with granulomatous periorificial dermatitis and comparison with childhood granulomatous periorificial dermatitis. Indian J Dermatol 2022;67:747-51 |
How to cite this URL:
Cho S, Kim BR, Lee JS, Na JI, Youn SW. Clinical characteristics of 9 adult patients with granulomatous periorificial dermatitis and comparison with childhood granulomatous periorificial dermatitis. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 23];67:747-51. Available from: https://www.e-ijd.org/text.asp?2022/67/6/747/370278 |
*Soyun Cho & Bo Ri Kim contributed equally to this article.
| Introduction | |  |
Granulomatous periorificial dermatitis (GPD), an uncommon variant of periorificial dermatitis or perioral dermatitis, is characterized by monomorphous, small, papular eruptions around the eyes, nose and mouth that histopathologically show a granulomatous pattern. It occurs most commonly in prepubertal children, garnering the term childhood GPD (CGPD), but a few cases involving adults have been reported.[1],[2],[3],[4] In fact, adult GPD may be underdiagnosed due to limited data and uncertain terminology, as it is more commonly seen than estimated in the literature. We experienced nine cases of adult GPD and report them here in an attempt to elucidate the clinical characteristics of adult GPD and determine whether or not differences exist between adults and children.
| Case Summary | | |
Nine adults and 19 children who were diagnosed as having GPD with compatible clinical and histopathologic findings between 2009 and 2018 were identified and retrospectively reviewed. Clinical features and images of GPD in adults are summarized in [Table 1] and [Figure 1]. All nine patients were female (median age 49 years; range 27– 73). The median duration of the skin condition was 4.5 months (range, 1 month to 1 year). All had relatively monomorphous flesh-colored to erythematous papules on normal skin that were concentrated around the eyes, nose and mouth, with frequent involvement of the nasolabial fold. Two had only periorbital lesions and one had only perinasal lesions. Histology revealed non-caseating perifollicular granulomas in the dermis with lymphohistiocytic infiltration within and around the granulomas in all patients. Microscopic polarized light exam for foreign body material was negative in all cases. Special stains for fungi and acid-fast bacilli revealed no microorganisms. All patients were otherwise healthy, and laboratory work-up including blood angiotensin-converting enzyme and chest X-ray were normal. Lesions resolved without scarring by at least 2 months of oral doxycycline treatment in four patients, or by oral steroid or hydroxychloroquine in three patients. Topical therapy was not effective in most patients. | Table 1: Demographic and clinical data of adult patients with granulomatous periorificial dermatitis
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 | Figure 1: Clinical and histopathological images of adult GPD patients. (a) Monomorphous, pinhead-sized erythematous papules around the eyes, nose and mouth, also along the nasolabial fold. (b) Skin specimens reveal non-caseating perifollicular granulomas in the upper dermis with extensive lymphohistiocytic infiltration within and around the granulomas (hematoxylin and eosin, ×40 and ×100). Langhans type giant cells and many lymphocytes are seen within the granulomas (hematoxylin and eosin, ×400)
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In addition, a comparison of clinical characteristics of GPD between adults and children is shown in [Table 2]. All adult cases were female, whereas childhood cases were almost equal in sex ratio, with slightly more girls (52.6%) than boys (47.4%). In both groups, the most commonly affected locations were periorbital, perinasal, and perioral altogether, followed by periorbital alone in adults (33.3%) and perinasal and perioral in children (15.8%). The majority of adults (77.8%) complained of symptoms such as pruritus or burning sensation, but most children (73.7%) had no symptoms. Topical therapy was not effective in most adults but was efficacious in children. Oral antibiotics were effective in both groups. Scarring was observed in none of the patients who were followed up until clearance of lesions. | Table 2: Comparison of clinical characteristics of adult and pediatric patients with granulomatous periorificial dermatitis
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| Discussion | | |
To the best of our knowledge, adult GPD cases have not been reported as a separate entity in the literature. A few reported cases demonstrate clinical and histological features identical to our adult cases and thus may be in fact GPD.[1],[2],[3],[4] Because GPD can only be confirmed by comprehensive evaluation including histological examination, clinical features, and etiopathogenesis to differentiate from granulomatous rosacea, sarcoidosis, lupus miliaris disseminatus faciei (LMDF), and perioral dermatitis, it is difficult to reach a definite diagnosis.[5]
GPD differs from classical perioral dermatitis in that there is a lack of pustules and the presence of discrete yellow–brown papules, less prominent erythema and scaling, and histologically perifollicular granulomas.[5] However, GPD and perioral dermatitis can be considered as different stages of the same entity in predisposed patients, or GPD as a variant of perioral dermatitis because clinical pictures with characteristic periorificial lesion distribution are very similar. Although there is overlapping in the histology and treatment choice of GPD and granulomatous rosacea, granulomatous rosacea is usually accompanied by vascular symptoms of rosacea without the periorificial distribution. Sarcoidosis is almost always associated with systemic involvement and symptoms such as fever, cough, and fatigue, and histologically sarcoidosis is characterized by “naked” granulomas without the inflammatory cells within and around the granulomas. LMDF is a chronic papular eruption in the central face with a predilection for the eyelids, appearing similar to GPD; however, histology of LMDF is characterized by well-formed granuloma with central caseation necrosis.[6]
The etiology of GPD is unknown; however, based on the peculiar distribution and histopathology, unrecognized, prolonged exposure to allergens or irritants may lead to chronic inflammation of vellus follicles around the eyes, nose, and mouth and develop into GPD.[7] Unlike perioral dermatitis, our patients did not have a history of antecedent topical or inhalant steroid use.
CGPD that affects prepubescent child has been reported in darker populations, including Italian, black, Japanese and Korean children, and cases that occurred in Afro-Caribbean children were termed facial Afro-Caribbean childhood eruption (FACE).[8] However, recent reports demonstrate that CGPD occurs in Caucasians as well.[9] CGPD affects both sexes almost equally, with slightly more girls (58%) than boys (42%), as shown in our study.[10] In contrast, adult GPD patients in the present series were all women, possibly reflecting the role of sex hormones in the adult form of GPD. The limited number of patients in this case series is not sufficient to reach such a conclusion, though.
GPD is a distinct facial papular disorder with periorificial distribution in middle-aged adults, mostly in females, as well as in children. It is the benignity of the condition with a good clinical outcome for anti-acne-rosacea medication, albeit requiring a relatively long-term treatment. Unlike CGPD, GPD in adults is frequently accompanied by pruritus with predilection for the eyelids and should be treated initially with oral doxycycline rather than topical metronidazole. More reports need to be gathered to gain more insight into this rare, peculiar condition.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Moncourier M, Pralong P, Pinel N, Templier I, Leccia MT. Granulomatous periocular eruption. Ann Dermatol Venereol 2017;144:430-3.  |
| 2. | El-Saad El-Rifaie M. Perioral dermatitis with epithelioid cell granulomas in a woman: A possible new etiology. Acta Derm Venereol 1980;60:359-60. |
| 3. | Husz S, Korom I. Periocular dermatitis: A micropapular sarcoid-like granulomatous dermatitis in a woman. Dermatologica 1981;162:424-8. |
| 4. | Falk ES. Sarcoid-like granulomatous periocular dermatitis treated with tetracycline. Acta Derm Venereol 1985;65:270-2. |
| 5. | Kim YJ, Shin JW, Lee JS, Park YL, Whang KU, Lee SY. Childhood granulomatous periorificial dermatitis. Ann Dermatol 2011;23:386-8. |
| 6. | Rocas D, Kanitakis J. Lupus miliaris disseminatus faciei: Report of a new case and brief literature review. Dermatol Online J 2013;19:4. |
| 7. | Plewig G, Kligman AM. Acne and Rosacea. Springer Berlin, Heidelberg; 2000. p. 30-1. |
| 8. | Williams HC, Ashworth J, Pembroke AC, Breathnach SM. FACE--facial Afro-Caribbean childhood eruption. Clin Exp Dermatol 1990;15:163-6. |
| 9. | Zalaudek I, Di Stefani A, Ferrara G, Argenziano G. Childhood granulomatous periorificial dermatitis: A controversial disease. J Dtsch Dermatol Ges 2005;3:252-5. |
| 10. | Nguyen V, Eichenfield LF. Periorificial dermatitis in children and adolescents. J Am Acad Dermatol 2006;55:781-5. |
[Figure 1]
[Table 1], [Table 2] |
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