Dermatology: Practical and Conceptual Image Letter | Dermatol Pract Concept. 2023;13(4):e2023206 1 Sudden-Onset Pain in Fibrous Hamartoma of Infancy in a Child: An Atypical Presentation Prabin P. Dev 1, Prashant Verma1, Shruti Sharma2, Niti Khunger1 1 Department of Dermatology & STD, Vardhman Mahavir Medical College & Safdarjung Hospital, New Delhi, India 2 National Institute of Pathology, Indian Council of Medical Research, Department of Health Research, Ministry of Health and Family Welfare, Govt. of India, New Delhi, India Citation: Dev PP, Verma P, Sharma S, Khunger N. Sudden-Onset Pain In Fibrous Hamartoma Of Infancy In A Child: An Atypical Presentation. Dermatol Pract Concept. 2023;13(4):e2023206. DOI: https://doi.org/10.5826/dpc.1304a206 Accepted: May 5, 2023; Published: October 2023 Copyright: ©2023 Dev et al. This is an open-access article distributed under the terms of the Creative Commons Attribution- NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original authors and source are credited. Funding: None. Competing Interests: None. Authorship: All authors have contributed significantly to this publication. Corresponding Author: Prabin P. Dev; Department of Dermatology & STD, Vardhman Mahavir Medical College & Safdarjung Hospital, K-16, Green Park extension, South Delhi, India; e-mail- drprabindev@gmail.com; Telephone: 9496669799 Case Presentation A 10-year-old male child presented with a slowly progressive plaque on his right posterior thigh that had been increasing in size over the last 9 years, and which turned painful in the last 2 weeks. On examination, an ill-defined plaque mea- suring 4 cm x3 cm with a hyper-pigmented, nodular surface was observed (Figure 1A). The lesion was firm, adherent to underlying structures, associated with tenderness and a local rise in temperature. Biopsy revealed follicular plugging, low papillomatosis, mild perivascular chronic infiltrate, and thick benign interlacing fibrous bands with muscle bundles extend- ing into the adipose tissue in the lower dermis and subcutis (Figure 1, B and C). MRI revealed diffuse soft tissue swelling in the thigh. A diagnosis of fibrous hamartoma of infancy (FHI) was made. The child underwent complete excision of the mass followed by split skin graft closure and is now under regular follow-up with no recurrence since last 6 months. Teaching Point FHI is a rare fibroproliferative lesion usually occurring within first two years of life with higher predilection in males [1]. FHI presents as a rapidly growing, firm mass of the subcutis in the axilla, shoulder, upper arm, inguinal region, or chest wall [1]. Our patient had the lesion on the posterior thigh. There are only three reported cases of FHI in the thigh [1]. The presentation of our patient was unique, as sudden onset of excruciating pain had not been previously reported. Although histopathology did not reveal the underlying cause of the pain, we hypothesize that nerve entrapment or unnoticed trauma leading to infection could be possible causes. Histopathology 2 Image Letter | Dermatol Pract Concept. 2023;13(4):e2023206 shows characteristic triphasic pattern of fibro-collagenous tis- sue, primitive mesenchymal cells, and mature fat [2]. Also, the surface changes in the plaque seen in our patient correlated with the papillomatosis on histopathology. Although FHI can be affixed to underlying tissue, it is usually benign. Very rarely, FHI can present with sarcomatous morphology [2]. Untreated cases, like in our patient, have the potential to continue grow- ing without regression. Local excision is the successful treat- ment for FHI, with a low incidence of local recurrence. References 1. Vinayak RS, Kumar S, Chandana S, Trivedi P. Fibrous hamar- toma of infancy. Indian Dermatol Online J. 2011;2(1):25-27. DOI: 10.4103/2229-5178.79867. PMID: 23130211. PMCID: PMC3481792. 2. Al-Ibraheemi A, Martinez A, Weiss SW, et al. Fibrous hamartoma of infancy: a clinicopathologic study of 145 cases, including 2 with sarcomatous features. Mod Pathol. 2017;30(4):474-485. DOI: 10.1038/modpathol.2016.215. PMID: 28059097. Figure 1. (A) Ill-defined plaque measuring 4x3 cm with a hyper-pigmented, nodular surface on right posterior thigh. (B) Photomicrograph shows epidermis with low papillomatosis. Dermis appears sclerosed with mild chronic inflammation (H&E; 40X). (C) Photomicrograph shows deeper part of dermis and subcutis. Thick benign interlacing fibrous bands are seen extending into the subcutis (H&E; 40X).