Lower Lip Squamous Cell Carcinoma in Disabling Pansclerotic Morphea of Childhood

Abstract

Disabling pansclerotic morphea of childhood (DPMC) is an aggressive form of cutaneous scleroderma that involves all layers of the skin, extending through the dermis and subcutaneous tissues to involve muscle, tendon, and even bone. As DPMC is extremely rare, its association with skin squamous cell carcinoma (SCC) is rarer still. To our knowledge there are only two cases of SCC in patients with DPMC that developed within areas of chronic skin ulceration. We report the first case of lower lip squamous cell carcinoma arising in a young woman with DPMC and discuss the carcinogenic pathway that may have led to its occurrence.

Update on morphea

“…SCC of the skin has also been reported to involve sites not affected by chronic wounds. 15,16 Mixed variant morphea occurs in up to 15% of patients with morphea and is simply a combination of two or more of the subtypes described above. 4 When examining a patient with ''scleroderma,'' differentiating morphea from systemic sclerosis is the physician's paramount task.…”

“…Both adults (10.6%) and children (12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23).8%) with morphea have an increased prevalence of autoimmune disorders in their families when compared to the general public. 4,5 One percent of patients with morphea have a first or second degree relative with morphea.…”

Metastatic Squamous Cell Carcinoma in a Patient with Disabling Pansclerotic Morphea of Childhood

“…French physician Jean‐Nicolas Marjolin described the appearance of skin cancer in chronic ulcers for the first time in 1828. SCC can also appear in localized morphea, with or without a chronic ulcer . Ultraviolet radiation does not appear to be essential in the pathogenesis since SCC develops in nonphotoexposed sites .…”

“…SCC can also appear in localized morphea, with or without a chronic ulcer . Ultraviolet radiation does not appear to be essential in the pathogenesis since SCC develops in nonphotoexposed sites . In one experiment it was observed that transplanted wounds in pigs grew tumors in animals who were receptors of wounds with longer evolution time.…”

“…In one experiment it was observed that transplanted wounds in pigs grew tumors in animals who were receptors of wounds with longer evolution time. It is thought that chronic inflammatory processes occurring in ulcers give rise to SCC and that these repair processes can influence its development , although the exact etiology of SCC in morphea is unknown and probably multifactorial.…”