J Rheum Dis.  2020 Apr;27(2):120-122. 10.4078/jrd.2020.27.2.120.

Rapidly Progressive Pansclerotic Morphea Associated With Raynaud Phenomenon

• Adil R
• El Kassimi I
• Nawal S
• Meryem Z
• Tbouda M
• Youssef S

• KMID: 2471954
• DOI: http://doi.org/10.4078/jrd.2020.27.2.120

Abstract

No abstract available.

MeSH Terms

Raynaud Disease*
Scleroderma, Localized*

Figure

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  Figure 1. (A) Extended plates at all trunk with sclerotic skin. (B) Atrophy and contractures in the arm and the forearm with a sclerotic hypopigmented. (C, D) Morpheus plaque with an ivo-ry-colored center of the but-tocks and thighs (after receiving the patient's consent form about publishing all photographic materials).

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  Figure 2. Raynaud phenomenon: episodic, symmetric, acral vasospasm, Note the discolor-ation of the fingers, Pallor: due to vasoconstriction of precapil-lary muscular arterioles (A), cyanosis: due to venous pooling and deoxygenation of venous blood (B), erythema: reactive hyperemia (C).

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  Figure 3. Skin biopsy showing. (A) Marked thickening of the dermis with sclerotic bands of collagen extending into the subcutaneous fat, atrophic epidermis topped by parakeratotic hyperkeratosis (H&E, ×100). (B) Atrophy of epidermis with fibrosis extending from the dermis to the hypodermis (H&E, ×200). (C) Fibrous dermis with a few gaping vessels surrounded of a mononuclear inflammatory infiltration (H&E, ×200).

Reference

1. Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol. 2006; 18:606–13.

2. Kim A, Marinkovich N, Vasquez R, Jacobe HT. Clinical features of patients with morphea and the pansclerotic subtype: a cross-sectional study from the morphea in adults and children cohort. J Rheumatol. 2014; 41:106–12.

3. Wollina U. Increased risk of cancer in patients with scleroderma: no risk in patients with morphoea? Ann Rheum Dis. 2004; 63:611–2.

4. Hardy J, Audouin-Pajot C, Abid A, Chiotasso D, Coustets B, Suc A, et al. [Pansclerotic morphea with a rapidly fatal outcome in an 11-year-old girl]. Ann Dermatol Venereol. 2016; 143:836–40. French.

5. Zhang A, Nocton J, Chiu Y. A case of pansclerotic morphea treated with tocilizumab. JAMA Dermatol. 2019; 155:388–9.