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CORRESPONDENCE
Year : 2023 | Volume
: 41
| Issue : 1 | Page : 50-51
Multiple hyperkeratotic white papules in a 6-year-old girl: A rare presentation of granuloma annulare
Yavuz Semiz1, Ezgi Aktas1, Sila Ozlem Aktas2, Ozben Yalcin2
1 Department of Dermatology, Okmeydanı Training and Research Hospital (Prof. Dr. Cemil Taşçıoğlu City Hospital), Istanbul, Turkey
2 Department of Pathology, Okmeydani Training and Research Hospital (Prof. Dr. Cemil Tasçioglu City Hospital), Istanbul, Turkey
Correspondence Address:
Dr. Yavuz Semiz
Department of Dermatology, Okmeydani Training and Research Hospital (Prof. Dr. Cemil Tasçioglu City Hospital), Kaptan Pasa Mah, Darülaceze Cad, SSK Okmeydani Hst, No: 25, 34384 Sisli, Istanbul
Turkey
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/ds.DS-D-22-00159
Dear Editor,
Granuloma annulare (GA) is an inflammatory skin disorder; its etiology and pathogenesis still remain unclear. It is more common during the fifth decade of life and among Caucasians (with a female predilection).[1],[2] GA in the pediatric population is also reported, mostly presenting as a deep subcutaneous variant of the disease.[2],[3] Here, we report a 6-year-old girl who presented with multiple hyperkeratotic white papules on the extremities, which were histopathologically consistent with GA.
A 6-year-old girl presented with multiple papules on the extremities that had been present for the previous 6 months. The family reported an increase in the number of lesions, the patient did not have any associated symptoms, and she had not received any treatment for the lesions during this period. In the dermatologic examination, multiple whitish keratotic papules on the upper and lower extremities of the patient were noted [Figure 1]a. Dermoscopic examination showed scales and white follicular plugs on a white basement [MoleMax HD digital dermoscopy; Derma Medical Systems, Wien, Austria; [Figure 1]b]. A punch biopsy was performed, and the histopathological examination with hematoxylin and eosin revealed orthokeratosis, acanthosis in the epidermis, keratotic plugs in follicular ostia, interstitial lymphocytic infiltration, and degenerated collagen. Alcian blue staining confirmed focal areas of increased mucin in the dermis [Figure 2].
Figure 1: (a) Multiple whitish keratotic papules on the arm lateral aspect of the right thigh. (b) Dermoscopic examination of the lesions: White follicular plugs on a white basement (MoleMax HD digital dermoscopy; Derma Medical Systems, Wien, Austria)
Figure 2: (a) Hyperkeratosis and orthokeratosis in the epidermis, keratotic plugs in follicular ostia, interstitial lymphocytic infiltration in the dermis (b) Collagenolytic areas and poor granuloma formation in the histiocytes (c) Focal areas of increased mucin in the dermis (H and E, a: ×40, b: ×100, c: Alcian blue staining ×100)Laboratory examinations, including serum fasting glucose and autoantibody tests, revealed no abnormalities.
With these clinical and histopathological findings, a diagnosis of GA was made. After the diagnosis, the patient's lesions spontaneously resolved within a few months. Follow-up schedule has been planned for the patient in the department of pediatric endocrinology in case of any possibility of the development of diabetes mellitus (DM) in the coming years.
GA was first reported in 1895 by Colcott-Fox[4] while the term “granuloma annulare” was introduced later in 1902 by Radcliffe Crocker.[5] GA is a noninfectious granulomatous skin reaction with an unknown etiopathogenesis; thus, diagnosis and treatment are still challenging.
The most common, classical, and localized form of the disease is characterized by isolated, skin-colored-to-erythematous circinate papules and plaques.[2] Fifty percent of the patients show spontaneous regression within 2 years.[2],[6] Four main clinicopathological variants of GA have been described: localized GA, disseminated GA, perforating GA, and subcutaneous (deep) GA.[2] Papular GA is another variant that manifests as asymptomatic, skin-colored, or hypopigmented firm papules (1–3 mm). Although GA is a disorder of adulthood, papular GA most commonly affects children, usually on the dorsal aspect of the hands.[6]
Histopathological findings of GA include a focus of necrobiosis surrounded by palisading histiocytes, mucin deposition, multinucleated giant cells, and a dermal infiltrate of eosinophils, lymphocytes, and neutrophils. Among the histopathological findings of GA, degraded collagen, histiocytic infiltrate, and the presence of mucin seems to be common among all subtypes.[2]
The diagnosis of GA can be easily made in most cases along with the differential diagnoses of other granulomatous disorders. Still, some rare presentations of GA have been reported in the literature, including cases mimicking dermatofibroma, dermatomyositis, papulonecrotic tuberculid, psoriasis, tinea cruris, and sarcoidosis on scar tissue.[2] Although variable appearances of vessels on a pinkish-reddish background were reported to be a characteristic dermoscopic feature of GA, it has no pathognomonic dermoscopic features.[7],[8] Based on reported data from confusing cases, clinicopathological correlation is crucial before making a diagnosis of GA. Our patient presented with multiple whitish keratotic papules on the upper and lower extremities. On dermoscopic examination of the papules, scales and white follicular plugs on a white basement were noted. Keratotic plugs in follicular ostia were noted in histopathological examination and clinically presented as follicular plugs. Interstitial lymphocytic infiltration degenerated collagen and focal areas of increased mucin in the dermis were also observed consistent with GA.
In a recent case–control study conducted among 177 adult GA patients, a significant link was noted between GA and autoimmune disease, DM, hyperlipidemia, hypothyroidism, and ischemic heart disease.[1] Similarly, in the pediatric population, DM, and autoimmune diseases were suggested to be associated with GA.[8]
In a study, investigating this association in the pediatric population 2 of 34 pediatric GA patients were shown to develop type 1 DM after 60 months of follow-up.[9] Similarly, several cases presenting as GA preceding the onset of autoimmune disease have been reported.[8] At the time of diagnosis, our patient did not have DM or autoimmune diseases confirmed by physical and/or laboratory examination. She is under follow-up in the department of pediatric endocrinology in case of the development of mentioned diseases.
The use of various local and systemic agents (e.g., topical and intralesional corticosteroids, topical calcineurin inhibitors, apremilast, methotrexate, biologic therapies, pentoxifylline, antimicrobials, phototherapy, and tofacitinib) was reported as GA treatments; however, none of them have been proven to be reliable or efficient, and in several cases, the patients did not respond to any of these treatments.[2] Fortunately, a spontaneous resolution of GA is expected over months to years.[2],[3]
Our patient is a 6-year-old girl presenting with multiple white hyperkeratotic papules on the upper and lower extremities and with atypical dermoscopical findings. The clinical features of our patient were not characteristic of GA, whereas the histopathologic examination showed typical GA features. Thus, a diagnosis of GA was made, and a spontaneous resolution of the lesions was observed, which is also an expected finding in GA. The case presented could have been classified as papular GA; however, the lesions were not similar to those reported as papular GA in the literature. As a result, we report this case as a rare presentation of GA, as hyperkeratotic white papules and white follicular plugs, reflection of keratotic plugs in follicular ostia in the histopathologic examination, were shown in the dermoscopic examination of a girl with no associated comorbidities. The patient is still under follow-up in the departments of dermatology and pediatric endocrinology, and to date, no new lesions or associated DM or other diseases have developed. We wanted to report this case to emphasize the importance of establishing a clinicopathologic correlation before establishing a diagnosis.
Declaration of patient consent
The authors certify that they have obtained appropriate patient's guardian consent form. In the form, the guardian has given the consent for the child's images and other clinical information to be reported in the journal. The guardian understands that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
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