IntroductionNevus lipomatosus cutaneous superficialis (NLCS) is a rare benign hamartomatous skin tumor characterized by dermal deposition of mature adipose tissue. It’s classified in two types: the classical form with multiple soft, pedunculated, cerebriform papules and nodules that coalesce into plaques, and the solitary form that consists of a solitary papule or nodule. In this study, eight cases of NLCS are reported.MethodsThe study was a retrospective case series including all patients with histopathologically documented NLCS who attended the Dermatology Department of Charles Nicolle hospital between January 1997 and December 2010. The objective of our study was to determine the epidemioclinical characteristics, the histopathologic features, and the treatment of this hamartoma. Patients included three males and five females aged between 7 and 41 years.ResultsIn four cases hamartoma was present since childhood, and in the other four cases it appeared in the third and fourth decades. Classical form was noted in seven cases and the solitary form in one case. Lesions involved limbs in four patients and trunk in four patients. Seven patients underwent surgical excision, and for one case no treatment was proposed.ConclusionThe multiple or classical form is largely predominant in our study. Habitually, NLCS has an asymptomatic course. Treatment is usually not necessary unless for cosmetic reasons; surgical excision is curative and recurrence after is rare.

Our study illustrates the severity of erythroderma. It alters heavily the quality of life of patients which is initially altered by the pre-existent dermatosis. It may be life threatening as mortality rate is high.

Cicatricial pemphigoid is an extremely rare condition in children, since only 17 cases have been reported in the English literature, so far. We describe a new case in a 20-month-old boy, who is to our knowledge the youngest patient reported yet. The disorder had begun 10 months before he was referred to our department by mucosal crusted erosions of the oral and nasal cavities and conjunctivae. Cutaneous examination showed buccal erosions with limited mouth opening, entropion of the lower eyelids, trichiasis, cicatricial cornea, synechia of the nasal cavities and hypopigmented lesions of the abdomen. There were no anal or genital lesions. Cicatricial pemphigoid was confirmed by positive direct and indirect immunofluorescence on mucous biopsy. Systemic corticosteroids (2 mg/kg/day), maintained for 12 months, had led to complete healing of lesions. But due to cicatrization, synechia of the nasal cavities and corneal opacities, leading to a dramatic visual loss, have occurred. Dapsone 25 mg/day and topical ocular cyclosporine are now maintained to avoid relapse. Our review of the literature of all cases of CP showed that ocular and to a less degree, vulvar lesions are the most severe ones, due to the serious complications with scar formation.

Our study illustrates the poor prognosis of pemphigus by a long duration to disease control, a high initial dose of oral steroid, a high rate of relapse and a short remission period. Only mucosal involvement at presentation was identified as a poor prognostic factor.

Cutaneous manifestations are the hallmarks of OE. As vital organ involvement has been reported, close monitoring and continuous surveillance is warranted.