Linear Forehead Dermatitis

 The patient is an otherwise healthy 30 yo man in good general health.  For two months he has had a slightly pruritic dermatitis on his forehead.

Clinically, we considered En Coup de Sabre (Linear scleroderma)

Dermatoscopic Image: (Courtesy of Makayla Powers PA-C)

Punch biopsy showed:
Lichenoid interface and perifollicular dermatitis with postinflammatory pigment alteration.  No features of localized scleroderma.  These findings are suggestive of discoid L.E. or linear L.P.
Photomics courtesy of David Jones, MD, Berkshire Medical Center, Pittsfield, Massachusetts.

Treatment was initiated with clobetasol ointment.  Will switch to tacrolimus ointment if response is good.

 References
1. Rodriguez E et. Al. Acute Onset Linear Lichen Planus Pigmentosus of the Forehead: A Case Series  J Drugs Ddermatol 2023 Jan 1;22(1):94-97.  Free Full Text

2. Khelifa E.  Linear sclerodermic lupus erythematosus, a distinct variant of linear morphea and chronic cutaneous lupus erythematous. Int J Dermatol 2011 Dec;50(12):1491-5. PMID 22097995

3. Reference suggested by Dr. Sontheimer.  Dao DP, Sahni DR, Sontheimer DR. Linear discoid lupus erythematous simulating en coup de sabre morphea in a female chronic granulomatous disease carrier. Dermal Online J. 2023 Dec 15;29(6). Free Full Text. 

4. Das A et al. Linear lesions in dermatology: a clinicoaetiopathological study. Clin Exp Dermatol 2021 Dec;46(8):1452-1461. PMID: 34022084 

5. Urur YG. Et. Al. Dermoscopic Characteristics of Cutaneous Lupus Erythematosus According to Subtype, Lesion Location, Lesion Duration, and CLASI Score. Dermatol Pract Concep. 2024 Jan 1;14(1):e2024040. Free Full Text.

Linear Scleroderma in a 40 year-old Woman

Presented by Hamish McDougall
Cape Breton, Nova Scotia

The patient is a 37 year old woman with a four year history of a slowly progressively asymptomatic area of induration on the posterior aspect of the left thigh (photo).  A biopsy showed thickened collagen bundles in the reticular dermis and a sparse superficial and deep lymphoplasmacytic infiltrate consistent with morphea.

Strangely, her father-in-law is seriously ill with systemic sclerosis.  He lives far from the patient and her husband.  At this time, we have discovered no common exposures the patient and her father-in-law have.  The patient lives in an endemic area for Lyme Disease and serological testing will be offered.

Questions:
Is there any value in obtaining serologies, other than Lyme studies, on this woman?
What treatment might be of value? 
Do you have alternate diagnoses?

References:
1. Localized Scleroderma Review Article (Like a chapter in a text book)

2. Morphea Sculpted in Silica: A Case Report of Limited Cutaneous Systemic Sclerosis in a Woman with Long-Time Exposure to Silica Dust.

Pedro Gomes J, Shoenfeld Y.  Free Full Text.

3.  "Borrelia-associated early-onset morphea": a particular type of scleroderma in childhood and adolescence with high titer antinuclear antibodies? Results of a cohort analysis and presentation of three cases.

Prinz JC, et. al. J Am Acad Dermatol. 2009 Feb;60(2):248-55. CONCLUSION:B burgdorferi infection may be relevant for the induction of a distinct autoimmune type of scleroderma; it may be called "Borrelia-associated early onset morphea" and is characterized by the combination of disease onset at younger age, infection with B burgdorferi, and evident autoimmune phenomena as reflected by high-titer antinuclear antibodies. As exemplified by the case reports, it may take a particularly severe course and require treatment of both infection and skin inflammation.