Atypical presentation of lichen sclerosus et atrophicus: depigmented confetti-like papules in the supraclavicular region

INTRODUCTION

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis with a predilection for the anogenital region, where it can lead to significant morbidity and impact quality of life. However, the presentation can be diverse and extends beyond the classic atrophic, ivory-white plaques, encompassing a variety of extragenital manifestations that pose diagnostic challenges. This case report details a presentation of LSA in a 30-year-old male, characterized by multiple depigmented papules in a confetti-like pattern over the supraclavicular areas.

CASE REPORT

A 30-year-old male presented to the dermatology clinic with a 2-year history of multiple depigmented papules over the supraclavicular region. He denied any history of trauma, burns, or other skin conditions in the affected area. The patient reported using indigenous medications prescribed by a traditional healer for the past year without any noticeable improvement. He reported no systemic symptoms such as fever, weight loss, or joint pain. His past medical history was unremarkable; he had no similar family history and had no known allergies. On physical examination, multiple small, well-defined, depigmented papules were observed over the supraclavicular areas, arranged in a distinctive confetti-like, bilaterally symmetrical pattern [Figures 1 and 2]. The surrounding skin appeared normal, without any signs of atrophy, sclerosis, or ulceration. There were no palpable lymph nodes in the cervical or supraclavicular regions. A dermoscopic examination of the lesions revealed follicular plugging and telangiectasia, observed as dilated blood vessels [Figure 3]. A 4-mm punch biopsy was performed from one of the depigmented papules. Histopathological examination of the biopsy specimen showed homogenization of collagen in the upper dermis, a characteristic feature of LSA, and there was lymphocytic infiltrate below the homogenized collagen and in the perifollicular area, further supporting the diagnosis [Figure 4]. The patient was started on topical psoralen with ultraviolet A therapy and ultrapotent topical corticosteroids. The lesions showed partial flattening, although the overall appearance remained largely unchanged.

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Figure 1:

Bilaterally symmetrical depigmented confetti-like papules.

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Figure 2:

Small closely grouped papules in the supraclavicular area.

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Figure 3:

Dermoscopy showing white structureless areas (blue star), telangiectasia (green star), and peppering (pink star) (DermLite DL4, polarized, 10x).

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Figure 4:

Hematoxylin and eosin stained section showing homogenized collagen in the upper dermis (green star), band like (orange star), and perifollicular lymphocytic (red star) infiltrates (4x).

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DISCUSSION

LSA is typically associated with atrophic, ivory-white plaques, primarily in the anogenital region. Extragenital lichen sclerosus contributes to approximately 15–20% of patients with LSA, commonly presenting in adult females as asymptomatic white opalescent papules and plaques on the neck, shoulders, and upper trunk.^[1] Atypical presentations include linear lesions, such as those on the forehead mimicking “en coup de sabre,”^[2] extensive keratotic plaques, as reported on the lower limbs,^[3] acrosyringeal variants presenting as non-coalescing punctate atrophic macules,^[4] bullous lesions,^[5] and involvement of less common sites such as the face, palms, soles, scalp, and lower limbs.^[1]

However, the presentation in this patient, with numerous small, discrete papules, posed a diagnostic challenge. A similar case with lesions over the supraclavicular area in a guttate pattern was described,^[6] but it was unilateral as opposed to bilaterally symmetrical lesions in this case. The differential diagnosis for depigmented papules in this distribution includes vitiligo, idiopathic guttate hypomelanosis (IGH), salt and pepper pigmentation in scleroderma, and post-inflammatory hypopigmentation (PIH). Vitiligo typically presents with sharply demarcated, depigmented macules or patches, often with a symmetrical distribution. IGH is characterized by small, round, or oval hypopigmented macules, primarily on the extremities. Salt and pepper pigmentation is characterized by perifollicular sparing. PIH usually follows an inflammatory insult and resolves over time. However, the dermoscopic findings of follicular plugging and telangiectasia, combined with the histopathological evidence of collagen homogenization and a lymphocytic infiltrate, confirmed the diagnosis of LSA in this patient. Dermoscopy played a crucial role in this case, revealing follicular plugging and telangiectasia. Follicular plugging, a hallmark of LSA, reflects altered keratinization and dermal fibrosis. Telangiectasia, the presence of dilated blood vessels, indicates underlying vascular changes associated with the disease process. Other dermoscopic features include chrysalis-like structures, rosettes, peppering, and white structureless areas.^[7] These findings, although not specific to LSA, provided valuable clues and guided the decision to perform a biopsy. All the abovementioned differential diagnoses show pigmentary changes in histopathology without specific changes in dermal collagen. Histopathology of typical LSA lesions shows epidermal atrophy, follicular plugging, basal cell vacoular change homogenization of upper dermal collagen, and band-like lymphocytic infiltrate beneath it.^[8,9] The pathogenesis of LSA remains poorly understood, but it is thought to involve autoimmune mechanisms, genetic predisposition, and environmental factors. Autoimmune factors, such as the presence of autoantibodies against extracellular matrix proteins, may contribute to the dermal fibrosis and inflammation observed in LSA. Genetic factors may also play a role, as familial clustering of LSA has been reported. Environmental triggers, such as trauma or infection, may initiate or exacerbate the disease process.^[1]

The treatment of extragenital LSA is challenging, and there is no consensus on the optimal management strategy. Topical corticosteroids, calcineurin inhibitors, and phototherapy are commonly used, but their efficacy in atypical presentations is not well established.^[10] This case serves as a valuable reminder of the diverse clinical spectrum of LSA, especially in terms of morphology and occurring in a male patient, and the importance of integrating clinical, dermoscopic, and histopathological findings for accurate diagnosis. It also highlights the need for further research to better understand the pathogenesis of extragenital LSA and to develop effective treatment strategies for these challenging cases. Future studies should focus on identifying the specific factors contributing to atypical presentations and evaluating the efficacy of different treatment modalities.

CONCLUSION

This atypical presentation of LSA in the supraclavicular region emphasizes the importance of considering LSA in the differential diagnosis of depigmented lesions in extragenital locations. The integration of clinical, dermoscopic, and histopathological findings is crucial for accurate diagnosis.