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. 2016;7(4):1000356.
doi: 10.4172/2155-9554.1000356. Epub 2016 Jun 5.

Somatic Mosaicism in Blaschkolinear Inflammatory Disorders

Nicholas Theodosakis  1 , Lauren Levy  1 , Shawn Cowper  1   2 , Rossitza Lazova  1   2 , Lisa Kugelman  1 , Amanda Zubek  1 , Keith Choate  1   2   3
Affiliations

Somatic Mosaicism in Blaschkolinear Inflammatory Disorders

Nicholas Theodosakis et al. J Clin Exp Dermatol Res. 2016.

Abstract

Linear lichen planus (LP) is a rare dermatologic disease in which lichenoid lesions conform to a blaschkolinear distribution, most commonly on the extremities. Linear discoid lupus erythematosus (DLE) is a cutaneous manifestation of lupus that also conforms to Blaschko's lines. Blaschkolinear disorders have been shown to result from somatic mosaicism, most recently in nevus sebaceus, epidermal nevi, and syringocystadenoma papilliferum. In linear LP and DLE, presentation of papules along Blaschko's lines suggests that these disorders can result from keratinocytic genetic mosaicism. Their onset later in life suggests that a secondary trigger is necessary to drive inflammatory reactions to these linear lesions. To date causative mutations have not be identified. We report 2 cases of linear lichen planus and 1 case of linear discoid lupus erythematosus, all histologically confirmed. Both LP patients have experienced episodic regression and recurrence of their lesions in precisely the same distribution, with moderate symptomatic benefit from topical steroids and non-steroidal anti-inflammatories. The DLE patient showed gradual response to hydroxychloroquine over the course of 14 months. These cases highlight linear inflammatory diseases which represent localized variants of disorders for which there are few efficacious therapies. Their linear presentations suggest that they result from somatic mosaicism and genetic investigation of such disorders may reveal relevant therapeutic targets.

Keywords: Blaschkolinear; Genodermatoses; Linear.

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Figures

Figure 1:
Figure 1:
Right Medial Leg Linear Lichen Planus, Case 1: (A) Right medial thigh with approximately one dozen 1–2mm light pink planar papules surrounded by reticulated hyperpigmentation along lines of Blaschko, (B) A biopsy from the right calf showed a patchy lichenoid lymphocytic infiltrate focally obscuring the dermal epidermal junction in association with vacuolar alteration of the basal layer keratinocytes with occasional necrotic keratinocytes and focal overlying parakeratosis. Mild acanthosis, hypergranulosis, and hyperkeratosis noted. Numerous melanophages were seen in the papillary dermis. These findings were consistent with the diagnosis of lichen planus.
Figure 2:
Figure 2:
Nasal Linear Lichen Planus, Case 2: (A) Left nasal dorsum with a linear 6 cm × 1 cm linear, thin brown plaque with follicular sparing and perifollicular hyperpigmentation, (B) Skin revealed a patchy interface dermatitis involving the basal aspect of the epidermis and follicular infundibular epithelium. Individual necrotic keratinocytes, some with surrounding lymphocyte “satellitosis,” were noted. Some were superficial to the basal layer, extending occasionally into the stratum granulosum. The infiltrate in the dermis consisted of small lymphocytes with rare plasma cells. Melanophages were conspicuous in the papillary dermis. Hair follicular ostia were patulous and plugged by basket woven orthokeratin.
Figure 3:
Figure 3:
Post-treatment Linear Discoid Lupus, Right Cheek, Case 3 Right cheek with an approximately 5 cm long hyperpigmented patch resulting from resolution of DLE lesion following Blaschko’s lines treated with hydroxychloroquine.
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