Rediscovering Perifollicular Elastolysis: A Hitherto Undocumented Entity in India.

Perifollicular elastolysis, also known as papular acne scars is a common but taken for granted entity. It appears as asymptomatic whitish yellow papules on the trunk and proximal arms. Histologically there is loss of elastin around the pilosebaceous follicles. The lesions are the result of a scarring process that appear as papules and are often mistaken for papules of acne and are treated. A knowledge of this entity which has never been described before in India is being reiterated.

What was known?

Perifollicular elastolysis is also called papular acne scars

They appear as multiple asymptomatic skin-colored or yellowish white papules most commonly on proximal arms and trunk

Histologically there is varying degree of loss of elastin around pilosebaceous follicles

Introduction

We describe four representative cases of perifollicular elastolysis (PE), also known as papular acne scars which are a hitherto unreported condition in Indian literature despite its not so uncommon occurrence. These lesions are present as multiple asymptomatic skin-colored to yellowish white papules usually on the trunk and proximal arms. Histologically they are characterized by varying degrees of loss of elastin around pilosebaceous follicles. These lesions should be explained to the patient as scars of acne that do not require active treatment.

Case Report

Four cases with similar clinical features are being reported. They all had lesions suggestive of inflammatory acne lesions on the face which had been treated by topical and systemic antibiotics for varying periods. On examination, they all had mild to moderate acne on the trunk and upper arms, multiple hyperpigmented macular scars, some of which had some crusting, which developed after the lesions resolved. They all pointed out what they described as “white heads” on the trunk and upper arms and said they had developed from inflamed lesions that resolved over a period of months. Upon close examination, the lesions were skin-colored or whitish, 1–6 mm in size, papular, soft to firm and nontender [Figure 1]. Except for one patient who had papules with an atrophic looking skin, the overlying skin in others was not wrinkled, and there was no compressibility. One patient had elongated papules and many lesions were located around follicular openings [Figure 2]. All patients reported no change in size or shape and did not respond to the topical therapy. They did not reveal any history of contact with any chemicals and did not indulge in any activity, which would produce acne or acneiform lesions. A histopathological examination revealed stretched out atrophic epidermis. The dermis showed dense connective tissue with few entrapped sebaceous glands. There was thinning of connective tissue around the sebaceous gland and fragmentation of elastic fibers on H and E staining of skin biopsy [Figure 3]. Elastic Van Gieson stain confirmed thinned out and fragmented elastic fibers around the sebaceous gland [Figure 4]. A clinical diagnosis of PE/"papular acne scars" was made.

Figure 1

Skin-colored papules on the back

Figure 2

Elongated skin-colored papules on the back

Figure 3

Wispy thin elastic fibers in close proximity to sebaceous gland (H and E, ×20)

Figure 4

Fragmentation of elastic fibers in close vicinity of sebaceous gland with thick collagen bundles (EVG, ×20)

Discussion

Perifollicular elastolysis has been variably described as “papular acne scars” or “postacne anetoderma-like scars.”[1234] Though these entities are documented, literature is sparse. Despite acne being one of the most common disorders in India, there is no Indian report on PE. These lesions often tend to go unnoticed by the dermatologist who treats active acne, associated pigmentation, and the easily recognized scars. PE, often get treated with topical antibiotics and benzoyl peroxide. These lesions can be few to numerous, occur on the upper back, upper chest or upper arms. They are asymptomatic, 1–4 mm in size, round to oval, and whitish, yellowish or skin-colored. Smaller ones mimic closed comedones. Many are perifollicular and some show a central dell.[23] Other follicular disorders may have a potential for similar scarring and there is also a report of atopic dermatitis presenting with PE.[5] However, the likelihood of acne as the antecedent pathology is very high because most of the patients give a positive history of truncal acne in the past or at present. In the study by Wilson et al. who coined the term “papular acne scars” the lesions were present in 57% of patients with acne whereas just 9% with a negative history for acne demonstrated the papules. Moreover, 81% of patients with these lesions gave a history of truncal acne.[3] The other point favoring the association of acne with these scars is that no prepubertal patient with these lesions has been documented. They further argued that a negative history of acne in patients presenting with these lesions is not reliable as a significant number of people develop acne in their adolescence and few may actually recall details or severity. Some such lesions resemble anetoderma with wrinkling of the overlying skin. Some lesions, especially larger ones are softer than smaller ones also appear herniated but they are palpable and not compressible unlike anetoderma. Papular elastorrhexis, an inadequately understood rare condition presents with whitish scattered firm papules on the trunk and upper extremities. However, it usually occurs in children and prepubertal age group is not folliculocentric and there is no history of truncal acne. It has been variably interpreted as a variant of nevus anelasticus, a connective tissue nevus or an incomplete form of Buschke-Ollendorff syndrome.[6]

The histology of these lesions, irrespective of their nomenclature, uniformly shows a decrease fragmentation or even near total loss of elastin tissue.[23467] Varadi and Saqueton who suggested the term. PE documented an elastase producing strain of Staphylococcal epidermidis from an affected hair follicle incriminating the elastase for degrading the elastin tissue.[2] However, in a later study Dick et al. did not note any elastolytic activity in the Propionibacterium acnes and S. epidermidis. They suggested that the tissue necrosis produced by leukocytes during the inflammatory phase may have subsequently regenerated with collagenous scar formation and absence of elastin fibers.[4] Collagen in all the three has been shown to have a different characteristic though. Though Wilson et al. found collagen fibers greatly reduced, Varadi and Saqueton found them unaltered. A report on papular elastorrhexis reported homogenized collagen in all of their cases.[235]

Conclusion

There is a distinct entity of scars in acne patients that present as 1–4 mm asymptomatic skin-colored, yellowish or whitish round to oval papules. The lesions do not respond to topical anti-acne treatment. Often these patients present with such scars with ongoing or burnt out inflammatory truncal acne. They are known by various names such as papular acne scars, PE, and papular elastorrhexis. Though they have subtle differences they all have underlying elastolysis, which is perifollicular in nature. Since these lesions are often interspersed with active acne they often tend to get treated topically. Not only should these lesions be recognized by the treating dermatologist but they should also be explained to the patient or else the patient may interpret them as inadequate/unsatisfactory treatment outcome and continue to treat themselves or seek treatment for these untreatable lesions.

What is new?

In spite of their not very uncommon occurrence they have never been reported in Indian literature

Most patients are not aware of this entity and continue to apply topical acne medications on these lesions leading to frustration and waste of money for the patient

Dermatologists in India with its large population of acne patients should be reminded of this condition to arrive at a correct diagnosis and also counsel the patient accordingly.