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Solitary Morphea Profunda: A Case Report
S F Sobhan1, M Shafiquzzaman, M R Islam
1Dr Sobnam Faria Sobhan, Registrar, Department of Dermatology and Venereology, Mymensingh Medical College Hospital (MMCH), Mymensingh, Bangladesh;
Abstract:
Morphea is an autoimmune connective tissue disease that leads to sclerosis of skin and underlying subcutaneous tissue due to excessive collagen deposition. It is also called localized scleroderma. Morphea profunda is one of the morphological variants of morphea. It involves the deep dermis, subcutaneous tissue, fascia and muscle. Morphea profunda is characterized by one or more deep sclerotic plaque, thickened skin with hypo or hyper-pigmentation occurring mostly in abdomen and extremities. Lesions usually progress slowly and remain stable for years. Diagnosis of morphea is made by characteristic clinical appearance but for determination of depth and degree of involvement, histopathology is required in most cases. There is a morphea specific skin scoring system LoSCAT by which grading of the disease is done clinically. Treatment of morphea includes topical and systemic medication like Tacrolimus (0.1%), Imiquimod, Calcipotriol, methotrexate and PUVA. Our patient a 12 year old girl presented with a depressed hypopigmented lesion on left arm which she noticed incidentally. The lesion appeared on the lateral aspect of left upper arm and gradually extending deep and also increasing in size for last few months with sparse hair and loss of sensation over the lesion. Disease activity and severity was assessed by LoSCAT, a morphea scoring system. By LoSCAT it was found that the disease was moderately active but there is greater damage caused by the disease. On routine investigation all parameters are normal and was ANA was (-ve). The diagnosis was confirmed by skin biopsy for histopathology. The patient was treated with methotrexate.
Insights
Morphea profunda, a deep form of localized scleroderma, presents as sclerotic plaques affecting skin and deeper tissues. Methotrexate effectively treated a 12-year-old girl with this rare autoimmune condition.
Area of Science:
- Dermatology
- Autoimmune Diseases
- Connective Tissue Disorders
Background:
- Morphea is an autoimmune disease causing skin and subcutaneous tissue sclerosis due to excess collagen.
- Morphea profunda affects deep dermis, subcutaneous tissue, fascia, and muscle, presenting as deep sclerotic plaques.
- Diagnosis relies on clinical appearance, with histopathology crucial for assessing depth and severity.
Purpose of the Study:
- To present a case of morphea profunda in a pediatric patient.
- To highlight the diagnostic and management approach for morphea profunda.
Main Methods:
- Clinical assessment including the LoSCAT (Localized Scleroderma Cutaneous Assessment Tool) scoring system.
- Confirmation of diagnosis via skin biopsy for histopathology.
- Treatment with systemic methotrexate.
Main Results:
- A 12-year-old girl presented with a depressed, hypopigmented lesion on her left arm, showing signs of deep tissue involvement.
- The LoSCAT score indicated moderate disease activity with significant tissue damage.
- Histopathology confirmed morphea profunda, and the patient responded to methotrexate treatment.
Conclusions:
- Morphea profunda requires thorough evaluation, including histopathology, for accurate diagnosis and staging.
- Systemic methotrexate is a viable treatment option for morphea profunda in pediatric cases.
- Early diagnosis and appropriate management are crucial for minimizing long-term damage in morphea profunda.
