Lymphomatoid Papulosis Type C of the Eyelid in a Young Girl: A Case Report and Review of Literature

Abstract

We report an unusual presentation of lymphomatoid papulosis(LyP) type C in a young girl. Complete systemic work up and histopathological evaluation is mandatory in cases of suspicious lesions, not responding to conservative treatment.

Self-healing Eyelid Nodule: A Quiz

“…Along with cutaneous anaplastic large cell lymphoma (cALCL), LyP belongs to the continuous spectrum of cutaneous CD30 + lymphoproliferative disorders, the second most common group of primary cutaneous T-cell lymphomas (CTCL). Originally described by Macaulay (1) as a "self-healing, rhythmic and paradoxical eruption, histologically malignant but clinically benign", it is currently well known that LyP is a relapsing but indolent skin disease that may be histologically indistinguishable from aggressive cutaneous lymphocytic proliferations (2)(3)(4)(5)(6)(7)(8)(9)(10). Achieving an accurate clinicopathological correlation is therefore absolutely mandatory for a conclusive diagnosis.…”

“…To date, 5 histopathological subtypes of LyP, respectively named from A to E, have been defined (2)(3)(4)(5)(6)(7)(8)(9)(10). Overlapping patterns in the same specimen or in the same patient are observed in up to 10% of skin biopsies generally between the types A and C, the most common variants of LyP.…”

“…Since Lemagne et al (5) described the first patient with a rapidly growing ulcerated tumour of the outer canthus as the presenting feature of this condition in 1987, a total of 6 cases of eyelid LyP have been reported worldwide (5)(6)(7)(8)(9)(10). Three of them showed simultaneous lesions over their head, neck, trunk, and limbs (6,9,10) while the rest consisted of solitary eyelid LyP lesions characteristically accompanied by a previous or subsequent history of several waxing and waning necrotic papulonodules (5,7,8), as we noticed in our patient. This important finding is probably the most helpful clinical trait to consider LyP when atypical single presentations appear, differentiating it from cALCL and other much more common and aggressive papulonecrotic eyelid proliferations, such as keratoacanthoma and squamous cell carcinoma (2).…”

“…Clinically, LyP is characterised by outbreaks of papular or nodular lesions that usually become necrotic and typically heal spontaneously within a few weeks leaving an atrophic or hypopigmented scar (1)(2)(3)(4)(5)(6)(7)(8)(9)(10). Although this entity may be easily confused with other skin diseases such as arthropod-bites, prurigo, pyodermas, pityriasis lichenoides, or even cutaneous malignancies, its chronic recurrent course and especially its self-healing nature are both the differential clinical clues.…”

Eyelid involvement of primary cutaneous lymphomas: A clinical perspective

“…Lymphomatoid papulosis (LyP) is a low‐grade CTCL, classified in the group of primary cutaneous CD30+ lymphoproliferative disorders 8 . An asymmetrically distributed or agminated polymorphic eruption of lesions of variable size and number may occur anywhere on the body including the face and eyelids 31–38 . Rapidly growing reddish‐brown or purplish papules (Figure 6b) with haemorrhage, necrosis, erosion or crusting are the typical presentation, but subcutaneous nodules and large tumours showing ulceration (Figure 6c) may also occur 35,38 .…”