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morphea; localized cutaneous scleroderma; linear scleroderma

Cutaneous sclerosis, localized, linear or generalized, limited to the skin. Classification: 1) localized (1-3 lesions) 2) linear 3) generalized: 4 lesions affecting two separate anatomic sites 4) pansclerotic Etiology: 1) unknown 2) may be associated with infection due to Borrelia burgdorferi 3) rare complication of immune checkpoint inhibitors [8] Epidemiology: 1) onset between 20-50 years in 75% 2) linear morphea has earlier onset 3) pansclerotic morphea begins before age 14 4) female:male ratio 3:1 Pathology: 1) generally limited to the skin 2) pansclerotic form involving 1) dermis 2) fat 3) fascia 4) muscle 5) bone 3) destruction of underlying structures may result in growth disturbance in children 4) dermatopathology: a) epidermis: normal to atrophic with loss of rete ridges b) dermis 1] initially, edematous with swelling & degeneration of collagen fibers 2] later, lesions become homogeneous & eosinophilic 3] slight perivascular or diffuse infiltrate with: a] lymphocytes b] plasma cells c] macrophages 4] later, dermis thickened with few fibroblasts & dense collagen 5] inflammatory infiltrate at dermal-subcutis junction 6] dermal appendages progressively disappear 7] pansclerotic form a] fibrosis with disappearance of subcutaneous tissue b] sclerosis of fascia * histopathology image [4] Clinical manifestations: 1) morphea plaques [3] - occur on the face, head, neck, trunk (especially around the belt line), & lower extremities - usually oval & initially are indurated, with a yellow to brown color - when lesions are active, they are surrounded by a violaceous halo - over time, plaques may appear more atrophic & less indurated [3] - lesions may be mildly painful or painless 2) discrete sclerotic plaques a) early: violaceous plaques b) later: white, shiny center with erythematous or violaceous periphery c) may be solitary, linear or generalized 1] circumscribed a] generally involves trunk [3] b] limbs c] face d] genitalia e] axillae (less common) f] perineum (less common) g] areola (less common) 2] linear: a] extremities b] head with or without hemiatrophy of face 3] generalized (pansclerotic) a] trunk b] extremities c] face d] scalp e] sparing of fingertips & toes (no Raynaud's phenomenon) [3] d) size 2-16 cm in diameter e) hair follicles & sweat duct orifices often disappear f) plaques are hard in texture g) plaques are often hyperesthetic f) rarely accompanied by atrophy of underlying structures g) hemiatrophy of the tongue with linear morphea 3) scarring alopecia with scalp involvement of plaque 4) may begin as pruritic pink rash [3] 3) lichen sclerosus et atrophicus 5) nail dystrophy with linear lesions of extremities or pansclerotic form 6) purpura, telangiectasia & rarely bullae may be seen later in the course of the disorder 7) systemic manifestations (internal organ involvement) extremely rare [3] 8) Raynaud's phenomenon extremely rare [3] * images [4,5] Laboratory: 1) serology for Lyme disease 2) biopsy with silver stain to r/o Borrelia burgdorferi Special laboratory: - pulmonary function testing - doppler echocardiogram if evidence of pulmonary hypertension - right heart catheterization if further evaluation of pulmonary hypertension is needed [3] Radiology: - morphea affecting the neck, face, or scalp should undergo MRI of the head to evaluate for possible involvement of adjacent brain tissue [3] Complications: 1) generalized (pansclerotic) a) flexion contractures of the hands & feet b) atrophy & fibrosis of muscle c) restricted respiration - pulmonary hypertension 2) linear a) atrophy ocular tissues b) atrophy of bone Differential diagnosis: 1) progressive systemic sclerosis (scleroderma) 2) limited cutaneous systemic sclerosis - associated with scleroderma autoantibodies, specifically anticentromere antibody, thus anti-nuclear antibody positive - mnifestations may include calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, & telangiectasis - localized plaques are not a feature [3] 3) scleredema: - characterized by fibro-mucinous induration of the upper back, neck, upper arms, & occasionally the face - most commonly seen in association with diabetes (scleredema diabeticorum) or paraproteinemia. 4) eosinophilic fasciitis - eosinophilic fasciitis involves the fascia, not specifically the skin - affects the extremities with painful diffuse induration - spares the hands & feet - skin may also have an orange peel appearance 5) eosinophilic myalgia syndrome 1) acrodermatitis chronica atrophicans Management: 1) no effective treatment 2) symptomatic management as indicated 3) systemic glucocorticoids 4) treatment of Lyme disease a) high dose parenteral penicillin or ceftriaxone b) concurrent systemic glucocorticoids to reduce sclerosis 5) toclizumab for glucocorticoid-resistant morphea due to immune checkpoint inhibitor [8]

General

systemic sclerosis

References

  1. Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 274-77
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 177
  3. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018. - Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025
  4. Nguyen JV, Elston DM (images) Medscape: Morphea http://emedicine.medscape.com/article/1065782-overview
  5. DermNet NZ. Morphoea (images) http://www.dermnetnz.org/immune/morphoea.html
  6. Tratenberg M, Gutwein F, Rao V, Sperber K, Wasserrman A, Ash J. Localized Scleroderma: A Clinical Review. Curr Rheumatol Rev. 2017;13(2):86-92. Review. PMID: 27604889
  7. Gu A, Li J, Zhang LT Corrugated Indurated Short Cords on the Neck of a Teenage Girl. JAMA Dermatol. Published online April 14, 2021 PMID: 33851971 https://jamanetwork.com/journals/jamadermatology/fullarticle/2778394
  8. Blaise M, Cardot-Leccia N, Seitz-Polski B et al Tocilizumab for Corticosteroid-Refractory Immune Checkpoint Inhibitor- Induced Generalized Morphea. JAMA Dermatol. Published online November 30, 2022. PMID: 36449277 https://jamanetwork.com/journals/jamadermatology/fullarticle/2798842
  9. Papara C, De Luca DA, Bieber K, et al. Morphea: the 2023 update. Front Med (Lausanne). 2023;10:1108623. PMID: 36860340