Cribriform ulcerations of the back

A 26-year-old woman presented with a 6-week history of cribriform back ulcers, some of which had central hyperkeratotic crusts (Fig 1), and atrophic hyperpigmented papules on the arms (Fig 2). Additionally, she had scarring alopecia (Fig 3) and livedo reticularis of both legs. Review of systems was positive for Raynaud phenomenon, photosensitivity, oral ulcers, and joint pain for many years. Skin biopsies from the back and the arm both found interface dermatitis, thickened collagen bundles, periadnexal inflammation, focal fat necrosis, and increased dermal mucin (Figs 4 and 5). Serologic testing found antinuclear antibody titer of 1:2560 and positive anti-RNP, anti-Ro, and anticardiolipin antibodies. Anti-Smith, anti–double-stranded DNA, complete blood count, urinalysis, and C3/C4 levels were normal.Fig 2View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig 3View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig 4View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig 5View Large Image Figure ViewerDownload Hi-res image Download (PPT) Question 1: What is the most likely diagnosis for the lesion on the back?A.SarcoidosisB.Lupus panniculitisC.BlastomycosisD.PsoriasisE.Pityriasis lichenoides et varioliformis acuta Answers:A.Sarcoidosis – Incorrect. Up to a third of patients with sarcoidosis can present with cutaneous findings, the most common being red-brown papules and plaques without secondary changes. Classically, these lesions would show noncaseating granuloma formation on biopsy, which is not present in this case.1Katta R. Cutaneous sarcoidosis: a dermatologic masquerader.Am Fam Physician. 2002; 65: 1581-1584PubMed Google ScholarB.Lupus panniculitis – Correct. In the setting of a positive antinuclear antibody titer, positive autoimmune review of systems, and presence of specific antibodies, systemic lupus erythematosus (SLE) should be strongly suspected. This patient qualifies for lupus diagnosis from both the 1997 American College of Radiology criteria and the updated 2019 European League Against Rheumatism/American College of Radiology classification criteria for SLE. Given the coexisting clinical manifestations of ulceration and hyaline fat necrosis on biopsy, a lupus panniculitis variant of SLE is the correct diagnosis. The cribriform scarring seen on the back is not a typical presentation of lupus panniculitis. More commonly, lupus panniculitis is characterized by painful subcutaneous nodules that often appear bound-down and atrophic, and may ulcerate. Discoid lupus lesions coexist in up to a third of cases.C.Blastomycosis – Incorrect. This fungal infection is endemic to the Ohio and Mississippi river valleys. Skin lesions usually occur secondary to a primary pulmonary blastomycosis caused by inhalation of spores from the soil. Blastomycosis presents with scaly, verrucuous papules and plaques, which may ulcerate. Often these plaques heal with cribriform scarring. Classic findings on pathology are marked psuedoepitheliomatous hyperplasia of the epidermis and yeast forms with broad-based budding and double-contoured walls.D.Psoriasis – Incorrect. The pathognomonic findings of psoriasis are salmon-colored plaques with overlying silvery-white scale. Skin findings may be associated with inflammatory arthritis and nail changes. Scarring alopecia and ulcerations are not characteristic.E.Pityriasis lichenoides et varioliformis acuta – Incorrect. This condition is characterized by recurrent crops of diffuse, asymptomatic vesicles and scaly papules, which spontaneously resolve within weeks. Although histopathology does show interface dermatitis, wedge-shaped lymphocytic infiltrates in the dermis are also characteristic. These patients typically do not have systemic symptoms or abnormalities in laboratory findings. Question 2: What is the most likely mechanism of this disease?A.Drug reactionB.Environmental triggers in the context of genetic susceptibilityC.Mast cell degranulationD.Bacterial invasion of sebaceous glandE.Ischemia Answers:A.Drug reaction – Incorrect. In a young patient with no ongoing medical issues that would necessitate use of drugs such as procainamide, hydralazine, or hydrochlorothiazide, drug-induced lupus erythematosus is an unlikely diagnosis. Clinical manifestations of drug-induced lupus include joint pain and serositis. Skin eruptions, photosensitivity, and Raynaud phenomenon are not typically seen with drug-induced lupus.2Solhjoo M. Ho C.H. Chauhan K. Drug-Induced Lupus Erythematosus.in: StatPearls [Internet]. StatPearls Publishing, Treasure Island (FL)2019https://www.ncbi.nlm.nih.gov/books/NBK441889/Google ScholarB.Environmental triggers in the context of genetic susceptibility – Correct. The exact pathogenesis of cutaneous lupus is not known, but most agree that genetic predisposition and environmental influences, such as ultraviolet light, are involved. For example, ultraviolet radiation can activate proinflammatory cytokines, which induce cell apoptosis. Leukocytes are then recruited to the skin, leading to the characteristic lesions and infiltrate on biopsy.3Maidhof W. Hilas O. Lupus: an overview of the disease and management options.P T. 2012; 37: 240-249PubMed Google Scholar Additionally, ethnic background and gene mutations influence the clinical heterogeneity seen in lupus patients.C.Mast cell degranulation – Incorrect. Mast cell degranulation releases histamine and other preformed mediators of inflammation, which lead to the development of urticaria. This does not account for the presentation seen above.D.Bacterial invasion of sebaceous glands – Incorrect. Bacteria in sebaceous glands are one of the causes of acne vulgaris and do not account for the presentation seen above.E.Ischemia – Incorrect. Although SLE may cause ischemic changes in the cardiovascular system, which can lead to acute coronary events, it is not an etiology for the origin of the disease.3Maidhof W. Hilas O. Lupus: an overview of the disease and management options.P T. 2012; 37: 240-249PubMed Google Scholar Question 3: Which of the following treatment regimens could be used to treat this condition?A.Narrowband ultraviolet B phototherapyB.IsoniazidC.HydroxychloroquineD.Tranexamic acidE.Sulfamethoxazole-trimethoprim Answers:A.Narrowband ultraviolet B phototherapy – Incorrect. Studies have highlighted the importance of photoprotection in the prevention of lupus flares. Narrowband ultraviolet B, thus, may trigger or exacerbate a flare of lupus. Lupus patients require frequent application of broad-spectrum, high sun protection factor sunscreens, including physical blockers containing titanium dioxide or zinc oxide. Photoprotective clothing and sun avoidance are also critical.B.Isoniazid – Incorrect. Isoniazid is an antibiotic used in the treatment and prevention of tuberculosis and plays no direct role in the treatment of lupus. Additionally, isoniazid can cause drug-induced systemic lupus, which is characterized by fevers, serositis, arthritis, and a positive antihistone antibody. Cutaneous symptoms are often absent.2Solhjoo M. Ho C.H. Chauhan K. Drug-Induced Lupus Erythematosus.in: StatPearls [Internet]. StatPearls Publishing, Treasure Island (FL)2019https://www.ncbi.nlm.nih.gov/books/NBK441889/Google ScholarC.Hydroxychloroquine – Correct. This antimalarial medication is the gold standard in treating cutaneous forms of lupus, although the mechanism of therapeutic action is not well understood.3Maidhof W. Hilas O. Lupus: an overview of the disease and management options.P T. 2012; 37: 240-249PubMed Google Scholar,4Okon L.G. Werth V.P. Cutaneous lupus erythematosus: diagnosis and treatment.Best Pract Res Clin Rheumatol. 2013; 27: 391-404Crossref PubMed Scopus (144) Google ScholarD.Tranexamic acid – Incorrect. Tranexamic acid is indicated for use as an antifibrinolytic agent in a variety of conditions such as trauma or childbirth but has not been approved for use in SLE. Additionally, given the increased risk of thromboembolic phenomena in lupus patients due to presence of antiphospholipid antibody and other procoagulants in the serum, use of this medication would be ill advised.5Myers S.P. Kutcher M.E. Rosengart M.R. et al.Tranexamic acid administration is associated with an increased risk of posttraumatic venous thromboembolism.J Trauma Acute Care Surg. 2019; 86: 20-27Crossref PubMed Scopus (88) Google ScholarE.Sulfamethoxazole-trimethoprim – Incorrect. Sulfamethoxazole-trimethoprim is a sulfonamide antibiotic and plays no direct role in the treatment of lupus. Additionally, this antibiotic may exacerbate cytopenias. It is also photosensitizing and may trigger a lupus flare.