Case Report: Pansclerotic Morphea-Clinical Features, Differential Diagnoses and Modern Treatment Concepts

Abstract

Pansclerotic morphea (PSM) is a rare skin disease characterized by progressive stiffening of the skin with or without the typical superficial skin changes usually seen in morphea (localized scleroderma). Standard therapy, consisting of a combination of systemic glucocorticoids and methotrexate or mycophenolate mofetil, does rarely stop disease progression, which may lead to severe cutaneous sclerosis and secondary contractures. Little is known about the efficacy of newer biologicals such as abatacept, a fusion protein antibody against CTLA-4, or tocilizumab, a fully humanized IL-6R antibody, in the treatment of this pathology. We present the case of an 8 years old girl with an unusual, progressive stiffening of the skin, which was eventually diagnosed as pansclerotic morphea. A treatment with systemic glucocorticoids and methotrexate combined with tocilizumab led to a good clinical response within 2 months after initiation. In this paper, we discuss differential diagnoses to be considered and this new promising treatment option based on a case review of the literature.

Sun Sign in the Ultrasound Diagnosis and Activity Assessment of Morphea

“…At the mid (sclerotic‐predominant) stage with deep involvement, differential diagnoses include the chronic phase of lipodermatosclerosis; chronic graft‐versus‐host disease; late eosinophilic fasciitis; porphyria cutanea tarda, and dermatofibrosarcoma protuberans, among others. Differential diagnoses of the late stage of morphea with dermal involvement include late mycosis fungoides; late lichen sclerosus; acrodermatitis chronica atrophicans, vitiligo, late lichen plano pilaris; late lupus erythematosus profundus; lipodystrophy: intramuscular or intralesional corticosteroid injection and fat atrophy secondary to trauma 8–11 …”

“…Differential diagnoses of the late stage of morphea with dermal involvement include late mycosis fungoides; late lichen sclerosus; acrodermatitis chronica atrophicans, vitiligo, late lichen plano pilaris; late lupus erythematosus profundus; lipodystrophy: intramuscular or intralesional corticosteroid injection and fat atrophy secondary to trauma. [8][9][10][11] Nevertheless, to date, there are reports on the ultrasonographic appearance of some of these conditions such as vascular malformations, lipodermatosclerosis, eosinophilic fasciitis, keloids, chronic graft versus host disease, lipodystrophy, mycosis fungoides, and dermatofibrosarcoma protuberans. Eosinophilic fasciitis and chronic graft versus host disease present some similar ultrasonographic findings to morphea, which support the hypothesis that these entities may be variants of the presentation of morphea.…”

A rare and challenging presentation of adult‐onset pansclerotic morphea

“…Most evidence points towards the sparing of face, hands and feet as a hallmark of PSM. [2][3][4][5][6] In our case, this involvement was so important to the point of resembling epidermolysis bullosa.…”

Boosting Awareness of Sclerotic Skin Diseases: Exploring the Inflammatory Overlap of Sclerotic Skin Disease and COVID‐19 Vaccination

“…Morphea, also known as localized scleroderma, is a rare inflammatory disorder causing sclerosis of the skin and underlying subcutaneous tissues. [8][9][10][11] Excessive collagen deposition is triggered by abnormal innate and adaptive immune system activation. 8,9 Morphea is commonly categorized into linear, circumscribed, generalized, guttate, and mixed type based on its anatomic distribution.…”