Atypical Case of Combined Types I and II Scleredema Mimicking Morphea on Histopathology

Abstract

Scleredema is a rare sclerotic skin disorder that typically develops in conjunction with diabetes, monoclonal gammopathy, or infection and commonly involves the neck, back, trunk, and arms. Scleredema can be categorized into three types according to its cause. The local examination of the lesion is characterized by non-pitting induration that follows a symmetrical spread with sparing of the hands and feet. We present a case of a 19-year-old female patient that presented to the outpatient clinic complaining of facial skin lesions that have been progressive for four years. The skin lesions were found to involve the neck, back, trunk and both arms sparing the hands and there was no systemic involvement of the disease. The patient is non-diabetic but reported frequent attacks of tonsillitis over the past months. Initially, punch biopsy showed no signs of scleredema; however, repeated biopsy at follow-up confirmed the presence of mucin deposits which are suggestive features of scleredema. Due to the similarities with various other diseases, the diagnosis requires clinical and histopathological exclusion which makes the diagnosis quite challenging. It almost always starts in the neck but can present initially in rare cases from the face spreading symmetrically. Close follow-up and continuous monitoring are necessary for systemic organ involvement.

Keywords: atypical presentation; scleredema; scleredema of buschke; type 1 scleredema; type 2 scleredema.

Figure 1. (A) Diffuse sclerosis (dark pigmentation) on the neck. (B) Diffuse sclerosis on the back (tiny spots of dark pigmentation).

Figure 2. Hands of the patient are spared from the lesions.

Figure 3. Histopathology slide of a 4-mm punch biopsy from the right forearm showing mildly spaced collagen bundles (magnification x40).

Figure 4. Second biopsy shows mild mucin deposition between the collagen (magnification x40).