1/13. Treatment of cutaneous leishmaniasis with 20% paromomycin ointment.Cutaneous leishmaniasis is an infectious disease caused by flagellate protozoa of the genus Leishmania. In Mediterranean countries, the most common causative agents are Leishmania (L.) major, L. infantum and L. tropica. In croatia, cutaneous leishmaniasis is a rare disease, the last case being reported in 1988. Our patient was a 5-year-old boy with a left cheek skin lesion in the form of papule with central exulceration, hyperkeratotic crust and erythema of a 6-month duration. The diagnosis of cutaneous leishmaniasis was based on history data (stay in the southernmost region of croatia and multiple mosquito bites), light microscopic histology (dense infiltrates of large histiocytes with extracellular bodies), and positive montenegro (leishmanin) test. A new therapy with aminosidine (paromomycin), an aminoglycoside antibiotic, in the form of ointment at a concentration of 20%, was for the first time used in croatia. Four-week therapy resulted in complete regression of the skin lesions with residual hyperpigmentation. During therapy, no local or systemic side effects were observed. Thus, topical therapy with paromomycin could be considered an efficient therapeutic alternative in the management of cutaneous leishmaniasis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/13. Grzybowski's generalized eruptive keratocanthomas: a case report.A 47-year old woman with Grzybowski's generalised eruptive keratoacanthomas is described. There was no history of skin disease in her family, except for an uncle's basal cell carcinoma. From 1995 she developed multiple lesions of various size, ranging from hundreds of small follicular lesions to large typical keratoacanthomas up to 5 cm in diameter, scleroderma-like facial skin and marked ectropion. Histological examination of small and large skin lesions was typical of keratoacanthoma, and no human papillomavirus was detected by polymerase chain reaction. Oral treatment with acitretin had no effect. Both cyclophosphamide and methotrexate therapy were refused by the patient despite the progressive course of the disease. Blepharoplastic surgery had some effect on eye symptoms. The etiology of this rare disease is unknown, but is probably related to some genetic defect.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/13. Neonatal lupus erythematosus.Neonatal lupus erythematosus (NLE) is a rare disease characterized by the transplacental passage from the mother to the fetus of autoantibodies, in particular anti-Ro(SS-A), anti-La(SS-B), or both. The majority of infants with NLE exhibit isolated congenital heart block, cutaneous lesions analogous to those of adult subacute cutaneous lupus erythematosus, or both. We report a case of NLE in a 3-month-old male infant, born to a clinically asymptomatic mother, presenting small, annular, erythematous plaques with sharp, hyperkeratotic borders and central clearing localized at the eyebrow region. Both the infant and the mother were positive for anti-Ro(SS-A).- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/13. Is demodex really non-pathogenic?Although usually considered a non-pathogenic parasite in parasitological textbooks, Demodex folliculorum has been implicated as a causative agent for some dermatological conditions, such as rosacea-like eruptions and some types of blepharitis. Several anecdotal reports have demonstrated unequivocal tissue damage directly related to the presence of the parasite. However, this seems to be exceedingly rare, in contrast with the marked prevalence of this infestation. We have had the opportunity to observe one of such cases. A 38-year-old woman presented with rosacea-like papular lesions in her right cheek. Histopathological examination revealed granulomatous dermal inflammation with a well-preserved mite phagocytized by a multinucleated giant cell. This finding may be taken as an evidence for the pathogenicity of the parasite, inasmuch as it does not explain how such a common parasite is able to produce such a rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/13. Idiopathic eruptive macular pigmentation: report of 10 cases.Idiopathic eruptive macular pigmentation (IEMP) is a rare disease. IEMP is characterized by asymptomatic, pigmented macules involving the neck, trunk, and proximal extremities. This study describes 10 cases of idiopathic eruptive macular pigmentation seen during a 9-year period at the Asan Medical Center, Seoul, korea. We present these characteristic consecutive cases to provide more insight into the clinical picture and course of IEMP. skin lesions of 8 patients were multiple brown macules involving the trunk, face, neck, and extremities. In 2 patients, multiple dark brown macules and patches were noted. The age of onset varied from 1 to 20 years. Tentative diagnoses were usually ashy dermatosis (erythema dyschromicum perstans), fixed drug eruption, or mastocytosis. The history of any erythema and drug medication was absent. Darier's sign was absent. skin biopsy specimens showed increased pigmentation of the basal layer in an otherwise normal epidermis. Pigmentary incontinence, melanophages, and mild perivascular lymphohistiocytic infiltrate in the papillary dermis were also revealed. mast cells could not be found. The lesions gradually disappeared during a period of several months to years. The alleged rarity of IEMP may be partially caused by medical unfamiliarity with this entity, despite its clinical and histopathologic characteristic picture. Treatment of IEMP is unnecessary because spontaneous resolution of the lesions can be expected within several months to a few years.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/13. Nodular amyloidosis: case report and literature review.BACKGROUND: amyloidosis refers to a group of depositional diseases that are classified into two main types: systemic and localized. Large nodules of localized cutaneous amyloidosis of the nasal ala and surrounding skin are rare and the treatment is often unsatisfactory. OBJECTIVE: We report a case of rapidly enlarging, localized, nodular cutaneous amyloidosis of the nose and the surrounding skin with a brief review of the current literature regarding treatment of this rare disease. CONCLUSION: Nodular amyloidosis can be treated successfully with cold steel excision in combination with carbon dioxide laser. Close followup of these patients is warranted, as nodular amyloidosis may be the precursor to systemic amyloidosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/13. dapsone in rosacea fulminans.rosacea fulminans is a rare disease with female predominance characterized by abrupt onset of pustules, papules, and confluent nodules on the face. The conventional treatment consists of systemic glucocorticoids and isotretinoin. We present the case of a 56-year-old woman with a marked facial papulopustular eruption that had followed an initial period of severe seborrhoea. Conventional treatment produced no clear improvement. dapsone treatment achieved complete healing in 5 weeks.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/13. Necrotising fasciitis in the head and neck region.Necrotising fasciitis is an uncommon entity in present day medicine. Our review of the literature did not reveal any case involving the head and neck region. A case of this rare disease involving the head and neck region is presented.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/13. Atrophia maculosa varioliformis cutis.Atrophia maculosa varioliformis cutis is a rare disease characterized by spontaneously formed facial scars in young adults. Its etiology is unknown; there may be an underlying defect of dermal elastin. We discuss a patient with this unusual disorder and review its literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/13. granuloma faciale: Is it a new indication for pimecrolimus? A case report.granuloma faciale is a benign, chronic disease which is characterized by red-brown facial nodules and plaques. This quite rare disease is mostly seen in middle-aged males. A 41-year-old female patient consulted our department with a facial lesion of 4 years' duration. A dermatological examination revealed a pink-brown plaque of 1x5 cm over the nasal dorsum extending to the left malar region. Her personal and family histories were unremarkable. The routine hemogram and biochemical tests were normal, antinuclear antibody was negative. In the histopathological examination of the biopsy material which was taken from the lesion, the epidermis was intact; grenz zone was observed in the papillary dermis and with diffuse infiltrate with leukocytoclastic vasculitis and eosinophils, polymorphonuclear leukocytes and lymphocytes in the dermis were observed. With these findings, the patient was diagnosed with granuloma faciale and treatment with topical pimecrolimus cream 1% was started. The patient applied this treatment twice a day for 2 months and a dramatic recovery was observed after this period. The case is discussed in comparison with the literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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