Introduction: Morpheq, also known as localized scleroderma, describes a distinctive inflammatory skin disorder that ultimately leads to sclerosis. It is differentiated from systemic scleroderma by the absence of vasculopathy and organ involvement. Initial erythema may precede the sclerotic stage by a few months causing initial diagnostic confusion. High index of suspicion and knowledge of disease evolution are essential. We report a case of morphea and its progression, the diagnostic approach and the importance of early treatment and long-term monitoring. Case Summary: A 3l-year-old Filipino female who presented with multiple erythematous plagues on the trunk and extremities and arthralgia was initially diagnosed with cutaneous drug reaction. Prompt treatment led to partial relief of symptoms. However, two months later, eruption of multiple ivory-white small patches and plaques were noted on the same affected areas prompting an impression of morphea. Serum markers revealed elevated antinuclear antibody levels and negative anti-Scl70/anti-centromere serum autoantibodies. Skin biopsy showed homogenized thick dermal collagen bundles confirming the diagnosis of morphea. Topical therapy with calcipotriol + betamethasone dipropionate ointment showed remarkable improvement with decrease in erythema and softening of the lesions while adjunct narrowband-UVB phototherapy also provided relief due to its ability to reduce collagen synthesis and cytokine production. Conclusion Morphea may be easily misdiagnosed during the early stages especially if sclerosis ensues late in the disease. Characteristic clinical appearance of erythematous plaques with violaceous borders may not always be present. Histologic examination and serum autoantibodies help exclude other disorders with the same clinical and histopathological spectrum. Treatment is individualized depending on the severity and depth of skin involvement, early treatment and monitoring should be initiated before complications arise.
Scleroderma, Localized ; Fibrosis

Morphea is a rare, chronic inflammatory condition that affects the skin and subcutaneous tissues with an unclear etiology. Genetic predisposition, autoimmune dysregulation, and environmental factors play a role in its pathogenesis. It affects both adults and children and presents as erythematous patches or plaques that develop sclerotic centers with a violaceous border. Early diagnosis and treatment are crucial to minimize damage and physical sequelae.

We present here a 30-year old female who presented with a solitary, violet-hue in color, indurated plaque on her left forearm after wearing a metal smartwatch for 4 months. She experienced on and off episodes of overheating from the watch but continued wearing it. There was no associated pruritus, tenderness, or loss of sensation. Anti-dsDNA showed a borderline positive result. Vitamin D levels were below the lower limit revealing a severe Vitamin D deficiency. Dermoscopy revealed fibrotic beams, branching vessels and an erythematous to pink background. Histopathologic analysis showed superficial and deep perivascular and periadnexal infiltrates of lymphocytes and plasma cells with compact collagen bundles and notable loss of periadnexal fat. The patient was started on topical halobetasol then shifted to tacrolimus 0.01% and started on targeted NB-UVB. Excellent response was seen after 9 sessions of phototherapy. There was a decrease in induration, size and no further progression.

Morphea is a rare inflammatory condition without a clear etiology and early diagnosis and treatment are important. This case highlights the relationship between gadget trauma and the development of Morphea.

Objective: To summarize the clinical and imaging features of linear scleroderma en coup de saber (LSCS) with central nervous system involvement in children. Methods: The clinical data(clinical manifestations and imaging features) of 6 children diagnosed with LSCS with central nervous system involvement who were admitted to Beijing Children's Hospital Affiliated to Capital Medical University from May 2019 to November 2021 were retrospectively analyzed. Results: The 6 patients were all female, aged 6.8 (3.3, 11.0) years at the time of diagnosis, and aged 3.0 (1.7, 4.1) years at the time of discovery of facial skin lesions. Facial skin lesions appeared before neurological symptoms in 5 cases, and neurological symptoms appeared 2 months before skin lesions in 1 case. All the patients had "sword wound" skin lesions on the forehead with alopecia. Neurological manifestations included epileptic seizures in 6 cases, focal neurological defects in 5 cases, and headaches in 2 cases. The intracranial lesions were all ipsilateral to the skin lesions. The magnetic resonance imaging (MRI) of 6 cases showed abnormal signals mainly involving white matter in 1 hemisphere, and 3 cases showed local encephalomalacia. The scattered low signal was observed in 5 cases on susceptibility weighted imaging. Localized brain parenchyma or leptomeninges enhancement was seen on Gadolinium-enhanced sequences in 5 cases. Scattered foci of calcification on the affected side were seen on cranial CT in 4 cases. Skin biopsy was performed in 2 cases. Part of the lesion of the brain was removed in 1 case, and the pathological findings suggested small vasculitis, which was consistent with skin pathological changes. All patients received symptomatic treatment with antiepileptic drugs. Oral prednisone combined with methotrexate was given in 4 cases, and 1 case was given oral prednisone only. One case was presumed to be in the resting stage of the disease due to significant cerebral atrophy in half of the brain, and only antiepileptic drugs were added. The patients were followed up for 6-36 months. The skin lesions of scleroderma and alopecia did not progress in 5 cases, and hemifacial atrophy was developed in 1 case, which was considered to be combined with Parry-Romberg syndrome. The seizures were controlled in 4 cases. One case had reduced seizure frequency but left hemiplegia. One patient still had intractable epilepsy and paroxysmal headache. Conclusions: LSCS with central nervous system involvement is more common in girls, with seizures and neurological defects as the main manifestations. Intracranial lesions are mostly ipsilateral to the skin lesions. Cerebral microbleeds, calcification, and encephalomalacia foci are common, and the pathological changes in skin and intracranial lesions are consistent with small-vessel vasculitis. Prednisone combined with methotrexate treatment has shown some efficacy, but some children remain with refractory epilepsy and neurological deficit symptoms.
Child ; Humans ; Female ; Scleroderma, Localized ; Anticonvulsants ; Methotrexate ; Prednisone ; Retrospective Studies ; Seizures ; Drug Resistant Epilepsy ; Calcinosis ; Alopecia ; Brain ; Encephalomalacia ; Headache

Introduction: Morphea, is a rare autoimmune disease presenting with fibrotic changes in the dermis and subcutis. It is a benign condition associated with significant atrophy and sclerosis leading to disfigurement, flexure contractures, and impaired function. Ultraviolet A1 and photochemotherapy are highly effective treatment options but are not readily available in the country. Narrowband ultraviolet B (NBUVB), on the other hand, is readily available, affordable, and safe to use. Case summary: Three patients diagnosed with different variants of morphea (bilateral generalize morphea, unilateral generalized morphea, and circumscribed morphea). underwent 30 sessions of NBUVB. Treatment response was assessed using tightness and itch Visual Analogue Scale (VAS), Modified Skin Score (MSS), photographic comparison, ultrasonographic measurement, and histopathologic analysis. NBUVB treatment resulted to 14-60% decrease in the tightness and itch VAS. MSS was also reduced by 35-50%. The size, pigmentation, and erythema of the lesions also decreased. Ultrasonography showed an improvement in the thickness of lesions after treatment. Histopathologic study showed less packed collagen with increase in inter-bundle spaces. Conclusion Response to treatment was influenced by the age of the lesion and anatomical location. More chronic lesions tend to have less response. Lesions on the face exhibited the greatest improvement while lesions on the lower extremities had the least improvement. This is the first case series study in the country that uses NBUVB as treatment for morphea. The improvement of the sclerotic and atrophic lesions treated with narrowband UVB treatment may be an acceptable substitute for UVA1 and PUVA.
Scleroderma, Localized ; Phototherapy

Linear scleroderma en coup de sabre (LScs) is a variant of localized scleroderma. This disease typically occurs in patients in their 20s or younger individuals and predominantly occurs in the forehead area. A 26-year-old man with linear scleroderma was surgically treated at our center with Medpor (porous polyethylene) and dermal fat graft for the forehead lesion. After 26 months of postoperative follow-up, the depressed lesion that appeared scarred as well as the margins improved significantly. The surgical treatment of LScs using Medpor and dermal fat graft is an effective treatment modality that can increase patient satisfaction.
Adult ; Cicatrix ; Follow-Up Studies ; Forehead ; Humans ; Patient Satisfaction ; Scleroderma, Localized ; Transplants

No abstract available.
Scleroderma, Localized

Although a few reports have noted the concurrent presentation of morphea and vitiligo at distinctly separate sites in the same patient, it is extremely rare that these two conditions occur at the same sites in a patient. We report the case of a 10-year-old Korean girl with morphea and vitiligo and those lesions occurred at the same sites and progressed simultaneously. An autoimmunity and a cutaneous mosaicism was considered to be involved in such an unique presentation as the pathogenesis is concerned.
Autoimmunity ; Child ; Female ; Humans ; Mosaicism ; Scleroderma, Localized* ; Vitiligo*

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis presenting as white papules and atrophic patches in the anogenital region. Extragenital LSA is rare and commonly affects the neck, thighs, and the trunk. A 68-year-old woman presented with a well-demarcated, whitish linear plaque on the forehead that was observed several months prior to presentation. A biopsy specimen showed hyperkeratosis and thinning of the epidermis and homogenization of collagen in the papillary dermis with a subepidermal cleft. Mild periappendageal lymphocytic infiltration and eccrine gland atrophy secondary to thickening of collagen bundles were observed in the deep dermis. Based on the clinical and histopathological findings, this patient was diagnosed with extragenital LSA and concomitant morphea along the Blaschko's lines.
Aged ; Atrophy ; Biopsy ; Collagen ; Dermis ; Eccrine Glands ; Epidermis ; Female ; Forehead* ; Humans ; Lichen Sclerosus et Atrophicus* ; Lichens* ; Neck ; Scleroderma, Localized ; Skin Diseases ; Thigh

BACKGROUND: Periostin is a novel matricellular protein expressed in many tissues, including bone, periodontal ligament, and skin. Although its expression is prominent in various fibrotic conditions, studies of periostin in localized scleroderma are rare. OBJECTIVE: To investigate the expression of periostin and other molecules in localized scleroderma. METHODS: A retrospective study of 14 patients with confirmed mature stage localized scleroderma was undertaken. Fourteen age-matched and biopsy site-matched subjects with normal skin were included as controls. Collagen fiber deposition, periostin, procollagen, transforming growth factor-β, and matrix metalloproteinase (MMP)-1 expression were assessed and compared between the two groups. Co-localization of α-smooth muscle actin and periostin was evaluated using confocal microscopy. RESULTS: Periostin was predominantly expressed along the dermo-epidermal junction in the controls. Conversely, patients with localized scleroderma demonstrated increased collagen fiber deposition and periostin expression that was more widely distributed along the entire dermis. MMP-1 staining showed increased expression in the epidermis and dermis of patients compared to scanty expression in the controls. A semi-quantitative evaluation showed a higher proportion of excessive collagen bundle deposition (57.1% vs. 7.1%, p=0.013), diffuse periostin positivity (42.9% vs. 0%, p=0.016), and moderate MMP-1 positivity (71.4% vs. 7.1%, p=0.001) in patients than in the controls. CONCLUSION: Compared to the controls, patients with localized scleroderma had enhanced periostin expression corresponding to increased collagen fiber deposition and unexpected overexpression of MMP-1. The results of this human in vivo study may implicate the pathogenesis of localized scleroderma.
Actins ; Biopsy ; Collagen ; Dermis ; Epidermis ; Humans ; Microscopy, Confocal ; Periodontal Ligament ; Procollagen ; Retrospective Studies ; Scleroderma, Localized* ; Sclerosis ; Skin

No abstract available.
Diagnostic Errors* ; Scleroderma, Localized* ; Vitiligo*