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.

Human ; Female ; Middle Aged: 45-64 Yrs Old ; Scleredema ; Scleredema Adultorum ; Scleredema Diabeticorum ; Diabetes Mellitus

Mycosis fungoides (MF) is a primary cutaneous T-cell lymphoma that may, although rarely, present as asymptomatic hypopigmented lesions. On the other hand, vitiligo is an autoimmune disorder characterized by asymptomatic depigmented patches. Few reports have documented both conditions occurring in a single patient, making this an intriguing diagnostic and management dilemma.

This is a case of a 26-year old female with a 10-year history of persistent hypopigmented, depigmented and erythematous patches and thin plaques [Figures 1a and 2a]. Histopathology showed epidermotropism of mononuclear cells on H&E stain [Figure 3] and a positive CD-3 stain for T cells, confirming mycosis fungoides [Figure 4b]. No evidence of lymphadenopathy and visceral involvement was found on both the chest and abdominal CT scans. The depigmented patches were likewise biopsied showing lack of Melan-A staining and absence of epidermal melanocytes, consistent with vitiligo [Figure 4a]. Final diagnoses were both mycosis fungoides, stage IB and vitiligo. The patient underwent narrowband UVB phototherapy and was started on topical corticosteroids [Figures 1b and 2b]. Improvement was evident over a three-month period, with body surface area decreasing from 77% to 46%. As the patient is young, has normal LDH levels, and shows no signs of extracutaneous disease, prognosis is promising.

This rare case of mycosis fungoides and vitiligo occurring together presents notable diagnostic and management challenges. Some studies hypothesize that the aberrant T cells involved in MF might contribute to the T cell-mediated destruction of melanocytes, similar to the mechanisms seen in vitiligo.

Human ; Female ; Adult: 25-44 Yrs Old ; Mycosis Fungoides ; Vitiligo ; Phototherapy

INTRODUCTION

Lamellar ichthyosis (LI) is an inherited rare disorder characterized by generalized scaling presenting at birth and persisting throughout life. It presents at birth with a collodion membrane, later developing into large, brown scales across the skin. LI requires ongoing treatment and monitoring due to physical and psychosocial impacts. This study highlights siblings who showed substantial quality-of-life improvements with oral retinoid therapy.

OBJECTIVE

To present and discuss a detailed case summary, explore management options, and evaluate the treatment outcomes.

CASE SUMMARY

We report on a pair of siblings, a 24-year-old male and a 19-year-old female, who were born encased in a collodion membrane, and later presented with large, brown, plate-like scales all over the body. Skin changes were accompanied by intermittent heat intolerance and mild ectropion, which subsequently caused impaired quality of life while growing up. Histopathology results were consistent with lamellar ichthyosis. Both siblings responded well to oral Acitretin at 0.5 mglkglday, showing significant shedding of thick scales and a reduction of ectropion within the first two weeks of therapy.

CONCLUSION

Lamellar ichthyosis, a severe, lifelong disorder with psychosocial repercussions, requires long-standing, continual therapy. Maximizing treatment options with oral acitretin, addressing the psychosocial implications of the disease and getting patients actively involved in its management results in better treatment outcomes.

Human ; Male ; Female ; Young Adult: 19-24 Yrs Old ; Ichthyosis, Lamellar ; Acitretin