Nevoid telangiectasia patches in a 17-year-old boy

A 17-year-old-boy presented to the Dermatology service with a 5-month history of mildly pruritic rash over his trunk and limbs. On examination, several erythematous telangiectatic patches were noted over his chest, bilateral flanks, hips, and lower limbs (Fig 1, A and B). These were accompanied by tenosynovitis over bilateral hands. 5 months after the initial review, the telangiectatic patches regressed with development of hypopigmented sclerotic plaques over these sites (Fig 1, C-E). A skin biopsy was arranged, and histology revealed thick dermal collagen fibers, with a perivascular and peri-adnexal lymphohistiocytic infiltrate within the superficial to deep dermis (Fig 2, A and B). Question 1: What is the most likely diagnosis for these telangiectatic patches ?A.Port wine stainB.Unilateral nevoid telangiectasiaC.Telangiectasia macularis eruptiva perstansD.MorpheaE.Benign hereditary telangiectasia Answer:A.Port wine stain – Incorrect. These present from birth as erythematous or violaceous patches.B.Unilateral nevoid telangiectasia – Incorrect. These may be congenital or acquired, and are characterized by multiple, unilateral and linearly arranged, blanchable telangiectases in a dermatomal (most frequently involving the third and fourth cervical dermatomes) or Blaschkoid pattern.1Kreft B. Marsch W. Wohlrab J. Unilateral nevoid telangiectasia Syndrome.Dermatology. 2004; 209: 215-217https://doi.org/10.1159/000079892Crossref PubMed Scopus (27) Google ScholarC.Telangiectasia macularis eruptiva perstans – Incorrect. This is characterized by brownish erythematous macules and telangiectasia usually located at the chest and limbs.2Costa D.L. Moura H.H. Rodrigues R. Pineiro-Maceira J. Ramos-E-Silva M. Telangiectasia macularis eruptiva perstans: a rare form of adult mastocytosis.J Clin Aesthet Dermatol. 2011; 4: 52-54PubMed Google ScholarD.Morphea – Correct. This is consistent with the skin biopsy results. This case also highlights telangiectatic patches as a unique early presentation of morphea, before its evolution to well-recognized hypopigmented sclerotic plaques.E.Benign hereditary telangiectasia – Incorrect. Distribution of telangiectasia is generalized or in photo-exposed areas. Patients do not have any systemic involvement. Morphea, also known as localized scleroderma, is an autoimmune condition characterized by inflammation and sclerosis of the skin and underlying soft tissues.3Abbas L. Joseph A. Kunzler E. Jacobe H.T. Morphea: progress to date and the road ahead.Ann Transl Med. 2021; 9: 437https://doi.org/10.21037/atm-20-6222Crossref PubMed Google Scholar It is characterized by distinctive cutaneous inflammation and fibrosis, and damage which produce atrophy. Unlike systemic scleroderma, morphea does not often involve the extra-cutaneous systems and also lacks the specific antibodies as found in systemic scleroderma.3Abbas L. Joseph A. Kunzler E. Jacobe H.T. Morphea: progress to date and the road ahead.Ann Transl Med. 2021; 9: 437https://doi.org/10.21037/atm-20-6222Crossref PubMed Google Scholar While early presentations are often of an erythematous patch or edematous plaque, morphea can also appear as telangiectatic patches. Question 2: What subtype of morphea does this patient have?A.CircumscribedB.GeneralizedC.LinearD.PanscleroticE.Mixed Answer:A.Circumscribed – Incorrect. Otherwise also known as plaque morphea, it begins as an erythematous-to-violaceous, edematous plaque of several centimeters and is most often located on the trunk with 3 or less lesions.B.Generalized – Correct. Defined as 4 or more morpheaform plaques involving at least 2 different anatomic sites.C.Linear – Incorrect. One or more linear streaks of cutaneous induration that may involve the dermis, subcutaneous tissue, muscle, and underlying bone.D.Pansclerotic – Incorrect. A very rare variant presenting with extensive full-thickness skin involvement sparing acral skin.E.Mixed – Incorrect. A combination of 1 or more morphea subtypes. The more modern and clinically appropriate classification, the ‘Pauda criteria’, divides morphea into circumscribed, generalized, linear, pansclerotic, and mixed subtypes. Each subtype has different clinical manifestations and degree of involvement of the subcutaneous soft tissues.4Florez-Pollack S. Kunzler E. Jacobe H.T. Morphea: current concepts.Clin Dermatol. 2018; 36: 475-486https://doi.org/10.1016/j.clindermatol.2018.04.005Abstract Full Text Full Text PDF PubMed Scopus (49) Google Scholar The patient’s findings were consistent with generalized morphea. Question 3: Which is the most common extracutaneous manifestation associated with morphea?A.GastrointestinalB.NeurologicalC.Musculoskeletal/articularD.VascularE.Respiratory Answer:A.Gastrointestinal – Incorrect. This includes gastrointestinal reflux.B.Neurological – Incorrect. Such as in the en coup de sabre type, neurological abnormalities can include migraines, trigeminal neuralgia, seizures, behavioral changes, and learning disability.5Zulian F. Vallongo C. Woo P. et al.Localized scleroderma in childhood is not just a skin disease.Arthritis Rheum. 2005; 52: 2873-2881https://doi.org/10.1002/art.21264Crossref PubMed Scopus (260) Google ScholarC.Musculoskeletal/articular – Correct. This is the most common extracutaneous manifestation of morphea and includes sacroiliitis, generalized synovitis, and inflammatory arthritis.D.Vascular – Incorrect. This can include Raynaud’s phenomenon, deep vein thrombosis, and vasculitis.E.Respiratory – Incorrect. This includes restrictive pulmonary disease, bronchopneumonia. In addition to involvement of joints and deeper subcutaneous areas underlying the lesions of morphea, patients may also experience other non-localized musculoskeletal manifestations. In a study of 750 patients with morphea, 22.4% had extracutaneous manifestations which included articular (47.2%), neurologic (17.1%), vascular (9.3%), ocular (8.3%), gastrointestinal (6.2%), respiratory (2.6%), cardiac, and renal.5Zulian F. Vallongo C. Woo P. et al.Localized scleroderma in childhood is not just a skin disease.Arthritis Rheum. 2005; 52: 2873-2881https://doi.org/10.1002/art.21264Crossref PubMed Scopus (260) Google Scholar Our patient’s rheumatological manifestation, with his initial presentation of tenosynovitis, likely represented early extra-cutaneous manifestations of morphea. None disclosed.