Scleredema Diabeticorum is a rare fibromucinous connective tissue disease, with only 27 cases reported in the Philippines. This condition is linked to diabetes mellitus, characterized by skin thickening and induration, particularly on the neck and upper back. This condition results from excessive collagen deposition and is often seen in patients with poorly controlled blood glucose levels. Timely identification and intervention are crucial for enhancing patient outcomes.

This is a case of a 46-year-old Filipino woman, initially treated as a case of psoriasis vulgaris, with a 3-year history of skin thickening and induration on the upper back and nape with progressive limited range of motion. These findings were not elicited upon initial consultation, only to be unveiled later, adding complexity to her condition. Further investigation revealed an elevated HbA1C of 10.7%. Histopathology showed thickened collagen bundles, leading to a diagnosis of scleredema diabeticorum. She underwent UVA1 phototherapy, with a total of 40 sessions, twice a week with the maximum dose of 90 J/cm2 and oral Methotrexate with a dose of 10mg/week. The patient showed significant improvement in induration and range of motion. Patient was referred to Internal Medicine for co-management.

In conclusion, this case highlights the complexity of diagnosing scleredema diabeticorum in a patient initially treated for psoriasis vulgaris. The discovery of skin induration and elevated HbA1C emphasizes the need for thorough re-evaluation in chronic conditions. The patient’s positive response to UVA1 phototherapy and methotrexate demonstrates its effectiveness, reinforcing the importance of continuous evaluation in complex dermatological cases.

No abstract available.
Cyclosporine* ; Scleredema Adultorum*

No abstract available.
Scleredema Adultorum* ; Streptococcal Infections*

Foot Diseases ; etiology ; Humans ; Scleredema Adultorum ; Scleroderma, Systemic ; complications ; Skin

Scleredema is a rare skin disease of unknown cause involving the face, neck, upper portion of the trunk, and proximal upper extremities. The clinical features of it are nonpitting, solid emema or stiffness of the neck, which may be sudden or insidious onset. Preceeding upper respiratory infection or diabetes mellitus have been frequently discribed to be associated with the disease. A monoclonal gammopathy has been frequently observed in patients with scleredema. We present a case of scleredema developed the face, upper portion of the back and shoulder in 16-year-old girl who had a preceeding upper respiratory infection.
Adolescent ; Diabetes Mellitus ; Female ; Humans ; Neck ; Paraproteinemias ; Scleredema Adultorum* ; Shoulder ; Skin Diseases ; Upper Extremity

BACKGROUND: Scleredema is a rare connective tissue disorder, characterized by hard and thick skin. It is well known that collagen synthesis is increased in scleredema skin, but there has been no study on the change of elastic fibers. OBJECTIVE: The purpose of this study was to compare the changes of collagen and elastic fibers in scleredema and normal skin. METHODS: We retrospectively evaluated the biopsy specimens of 10 patients diagnosed with scleredema. The control group was selected by matching each scleredema case with same site and similar ages from the biopsy specimens which showed normal dermis. A computerized image analysis system was used to measure the fraction of collagen and elastic fibers and calculate the ratio of elastic fibers to collagen fibers in each group. RESULTS: The skin of scleredema was found to have a significant increase in the percentage of collagen fibers in the dermis compared to the control group. On the other hand, the percentage of elastic fibers was significantly decreased in the scleredema group (p<0.05). CONCLUSION: In contrast to scleroderma which shows an increase in dermal elastic tissue and collagen fibers, we concluded that there is a decrease of elastic fibers in scleredema and that the pathogenesis and molecular involvement are different in the two diseases. we also found that the computerized image analysis system was a useful tool in the measurement of connective tissue components.
Biopsy ; Collagen* ; Connective Tissue ; Dermis ; Elastic Tissue* ; Hand ; Humans ; Retrospective Studies ; Scleredema Adultorum* ; Skin

The diseases which present with cutaneous sclerodermatous changes are scleroderma, eosinophilic fasciitis, mixed connective tissue disease, sclerederma adultorum, scleromyxedema and cutaneous midline mucinosis. This paper reviews the characteristics and differential diagnosis among of the above mentioned diseases.
Diagnosis, Differential ; Fasciitis/diagnosis ; Human ; Myxedema/diagnosis ; Scleredema Adultorum/diagnosis ; Scleroderma, Circumscribed/diagnosis* ; Scleroderma, Systemic/diagnosis

Scleredema and acanthosis nigricans are two distinct dermatological disorders that have been reported in association with insulin resistance. Few reported cases have shown an association between scleredema and acanthosis nigricans. A 53-year-old obese woman with diabetes mellitus presented with a 4-year history of an indurated plaque on the nape and upper mid-back. She also presented with a 20-year history of hyperpigmented, velvety plaques on the neck and both axillae. We report a diabetic patient who developed scleredema and acanthosis nigricans.
Acanthosis Nigricans ; Axilla ; Diabetes Mellitus ; Female ; Humans ; Insulin Resistance ; Middle Aged ; Neck ; Scleredema Adultorum

Scleredema is a rare scleradematosis of unknown cause involving the face, neck, upper portion of the trunk, and proximal upper extremities. The clinical features of the disease are nonpitting indurated edema or stiffness of the neck, which may be sudden or insidious in onset. Diabetes mellitus and its complications have been frequently described to be associated with this disease. We present a case of scleredema developed on the nape, upper portion of the back and shoulder in 60-year-old man who had been suffered from diabetes mellitus for the past ten years.
Diabetes Mellitus ; Edema ; Humans ; Middle Aged ; Neck ; Scleredema Adultorum* ; Shoulder ; Upper Extremity

BACKGROUND: Scleredema is a rare connective disorder with unknown etiology. There were no comprehensive studies about the clinical and histopathological features of scleredema in Korea. OBJECTIVE: Our purpose was to find the clinical and pathological features of scleredema. METHODS: The 31 patients with scleredema at our institution over ten years were retrieved. We reviewed medical records, clinical photographs, and histopathology slides of these patients, retrospectively. RESULTS: 1. The male to female ratio was 23:8 (2.9:1). 2. Most of the patients had the insidious onset and chronic localized lesion on the nape and upper back. 3. In sixteen patients (16/31, 51.6%), the diabetes mellitus was accompanied. In these patients, the male predominance (14/16, 87.5%) was significant. 4. The positive staining was in 75%(15/20) of the tissues with alcian blue or mucicarmine staining. 5. In four patients, localized electron beam therapy markedly improved the skin lesion without serious adverse effects. CONCLUSION: A preceding respiratory infection was uncommon. Almost all patients had insidious onset and chronic course. Diabetes mellitus was commonly accompanied, especially in male patients. Electron beam therapy appears to be effective in the treatment of scleredema.
Alcian Blue ; Diabetes Mellitus ; Female ; Humans ; Korea ; Male ; Medical Records ; Retrospective Studies ; Scleredema Adultorum* ; Skin