Journal of Dermatology and Dermatologic Diseases

Systemic sclerosis, also known as Systemic Scleroderma is a multisystem disorder characterized by fibrosis of the dermis and vascular abnormalities. It is rare disorder, with an incidence of about 2-8 per 1,000,000 populations, and is even more unusual during childhood. It belongs under the spectrum of Scleroderma, a group of disorders that present with indurated patches or plaques on the skin. We are presenting a case of a 6-year-old male with diffuse indurated hidebound skin, Raynaud’s phenomenon, flexion deformity of the digits, microstomia and a beak like nose with onset at 3 years of age. Biopsy revealed prominent sclerosis of the papillary and deep reticular dermis and few to absent adnexal structures in the dermis. Clinical and histological findings of the case were compatible with a diagnosis of Scleroderma. However, testing of autoantibodies such as anti-nuclear antibodies (ANA) and anti-Scl-70 yielded negative results. Additionally, systemic manifestations like pulmonary or renal involvement, which are often encountered in patients with Systemic Sclerosis, were notably absent in our case. Despite heterogeneity of its presentation, careful medical history, thorough dermatological evaluation and histopathological correlation still remains the cornerstone of diagnosis of Systemic Sclerosis. Finally, a multidisciplinary approach to management cannot be overemphasized in these patients.

Subcutaneous leiomyosarcomas are rare tumors representing 1% to 2% of all superficial soft tissue malignancies. Although they can appear anywhere in the body, they most often occur in the lower limbs. The incidence of subcutaneous LMS located at the upper back is very rare.

We report an historic case of a 47-year-old man admitted to the dermatology department for the management of a large subcutaneous upper back leiomyosarcoma, measuring 08 cm of size, confirmed by histopathology.

Background: Pachyonychia Congenita (PC) is a chronically debilitating and lifelong genetic disease that typically causes constant, disabling pain. PC appears to be rare, but its prevalence is unsubstantiated by large-scale epidemiologic studies. We conducted the first national prevalence study of a cohort of PC patients, those managed annually by US dermatologists.

Methods: Potential study participants were randomly selected from a national panel of patient-care dermatologists and invited to participate in a brief study of a patient condition that would be disclosed at the study website.

Results: Of the 423 dermatologists contacted, 400 participated, of whom 53% reported managing at least one PC patient during the past 12 months, an annual prevalence of 6.4/10,000 patients (extrapolated to 8,900 to 9,800 nationally), according to the study model.

Conclusions: Study findings indicate PC is likely to be far more prevalent than previous estimates in the literature and that the frequency and level of disability caused by pain-related symptoms may be under-recognized by the treating dermatologist. Additional research is needed to determine the extent to which PC diagnosis has been or could be genetically confirmed.

Skin lightening refers to the use of depigmenting agents to transform the skin complexion to a lighter tone. Therapeutic skin lightening may be undertaken in controlled medical situations to manage certain hyper-pigmentation disorders such as melasma, solar lentigo and vitiligo. On the other hand, cosmetic skin lightening is the use of skin lightening agents solely for aesthetic purpose to obtain a reduction in the skin’s physiologic pigmentation. Motivations for engaging in cosmetic skin lightening practice in sub-Saharan Africa has been alluded to a number of political, social and aesthetic purposes. The practice of cosmetic skin lightening is not limited by gender, age, socio-economic status or educational qualifications however; it is most prevalent among women in sub-Saharan region Africa. Common depigmenting agents identified in most topical skin lighteners in the region have been reported to contain hydroquinone and corticosteroids although they are not often stated on the ingredients lists. This review will explore the practice of cosmetic skin lightening in sub-Saharan Africa, motivations for engaging in the practice and examine mechanisms of actions of hydroquinone and corticosteroids.