Nodular Lesions of Self-healing Juvenile Cutaneous Mucinosis

Abstract

Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder of unknown origin, which occurs in children in good health. It is characterized by the multiplication of transient cutaneous papules and nodules, mainly located on the head and periarticular areas that spontaneously resolve. Histological features of SHJCM have been well described; therefore, the diagnosis is usually made easily when papules are biopsied. We report a series of 3 new cases of SHJCM presenting mainly with nodular lesions. Histological examination of these nodules showed either lesions consistent with nodular or proliferative fasciitis or nonspecific panniculitis. Mucinous deposits were present but often inconspicuous, so could be disregarded. We wanted to emphasize this misleading presentation because a biopsy for histological examination is always mandatory in cases of proliferating nodules to rule out malignant tumors. Therefore, the diagnosis always requires discussion between pathologists and clinicians to rapidly reassure the parents and avoid inappropriate therapy.

Proliferating subcutaneous nodules in a 12-year-old girl

“…Our case highlights an important histological variant of SHJCM, likely to represent a late stage of evolution, in which mucin is inconspicuous and thus diagnosis can be difficult. This finding is similar to that in three recently reported cases of SHJCM . The proliferation of plump, round, histiocyte‐like cells and spindle‐shaped myofibroblast‐like cells seen in our patient's second biopsy could have been misinterpreted as proliferative fasciitis.…”

“…Since the first description of SHJCM in 1973, 1 only 18 cases have been reported. 2 Periorbital oedema and systemic symptoms such as fever and arthralgia have been associated with SHJCM, although they were not present in our case. Compared with previously reported cases, our patient was older and her skin lesions were atypically located nodules with no papules.…”

“…SHJCM is a rare, benign disorder of unknown origin that typically presents in children with the development of multiple asymptomatic papules and nodules affecting the head, limbs or periarticular areas. Since the first description of SHJCM in 1973, only 18 cases have been reported . Periorbital oedema and systemic symptoms such as fever and arthralgia have been associated with SHJCM, although they were not present in our case.…”

New Developmental Pattern of Lesions in Alcohol - Related Adult - Type Self - Healing Cutaneous Mucinosis: A Short Report and Review of the Literatures

“…Cutaneous mucinosis has various clinical types, such as SHJCM and congenital forms (13). In previous studies, different types of SHJCM, such as the nodular type (14)(15)(16), focal type (6,17), familial type (10), recurrent type (18) with different lesion site presentation (1,15), have been report. Proper approach to this disorder and its mimickers have been presented (3,19,20).…”

“…The association between self -healing cutaneous mucinosis and underlying disorders, like nephroblastoma and epilepsy, has been proposed (5,21). Approximately 20 case studies have been written about SHJCM (14,23). The current patient had 3 major clinical presentations of cutaneous mucinosis, including nodular and papular lesions, and periorbital edema.…”