Pigmented linear discoid lupus erythematosus following the lines of Blaschko: A retrospective study of a Chinese series.

BACKGROUND: Linear cutaneous lupus erythematosus is a rare subtype of lupus erythematosus (LE) that develops linear lesions following the lines of Blaschko. Linear cutaneous lupus erythematosus may present as various subtypes of LE, including linear discoid lupus erythematosus. There are few reports about pigmentedlinear discoid lupus erythematosus in the literature. AIMS: We aimed to summarize the clinical and pathological features of patients with pigmented linear discoid lupus erythematosus following the lines of Blaschko. METHODS: Eighteen patients with pigmented linear discoid lupus erythematosus attending the outpatient department of the Dermatology, Peking Union Medical College Hospital, China, were enrolled in the study. We recorded clinical data including sex, age at onset, disease duration, location and distribution of the lesions, symptoms, trigger factors, antinuclear antibody (ANA) testing, therapy, and therapeutic responses. Histopathological features were also summarized. RESULTS: All 18 patients presented with well-defined brownish pigmented linear or segmental macules or plaques, following the lines of Blaschko. All the lesions were located on the head or neck. Unilaterally distributed lesions were found in 94.4% of patients. Two patients showed low titers of ANA in a speckled pattern. No systemic involvement or progression to systemic LE was noted. The patients were clinically diagnosed as pigmented lichen planus (55.6%), pigmented linear discoid lupus erythematosus (33.3%), and linear morphea (11.1%) before histopathological examination. LIMITATIONS: The study was retrospective and direct immunofluorescence was not performed. Not all patients' information was available and 4 patients were lost to follow-up because their contact information was changed. CONCLUSION: Pigmented linear discoid lupus erythematosus mostly occurs on the head and neck. It manifests as brownish macules along the lines of Blaschko. Differentiation between pigmented linear discoid lupus erythematosus and other dermatoses that have a linear distribution can be difficult both clinically and pathologically, but histological details can help distinguish them.

Hutchinson-Gilford progeria syndrome.

Progeria is a rare genetic disorder characterized by premature aging, involving the skin, bones, heart, and blood vessels. We report a 4-year-old boy who presented with clinical manifestations of progeria. He had characteristic facies, prominent eyes, scalp and leg veins, senile look, loss of scalp hair, eyebrows and eyelashes, stunted growth, and sclerodermatous changes. The present case is reported due to its rarity.

Pigmentary demarcation lines on the face in Saudi women.

BACKGROUND: Pigmentary demarcation lines (PDL) are physiological abrupt transition lines between hyperpigmented skin and lighter areas. Recent evidence suggests that they involve the face. AIMS: To survey facial PDL in Saudi females referred to general dermatology clinics for various complaints and determine any associated risks. METHODS: Screening for facial lines was done in general dermatology clinics over a year. Whenever a patient was found to have facial PDL, a detailed questionnaire and examination were undertaken. RESULTS: Out of 1033 patients screened, 144 patients (14%) were found to have at least one of the facial PDLs. The median age of onset was 16 years. The most common line was F with 76 patients (53%). Family history was positive in 51 patients (35%). CONCLUSION: Facial PDL is a common and chronic pigmentary problem in Saudi women. It should be recognized and differentiated from other similar diseases like melasma. A significant proportion of patients have a milder presentation.

Prurigo pigmentosa: an underdiagnosed disease?

Prurigo pigmentosa is a distinctive inflammatory disease first described by the Japanese dermatologist Masaji Nagashima in 1971. It is typified by recurrent, pruritic erythematous macules, papules and papulovesicles that resolve leaving behind netlike pigmentation. The disease is rarely diagnosed outside Japan, because clinicians outside Japan are not well conversant with the criteria for its diagnosis. Only one patient from India has been published previously under the diagnosis of prurigo pigmentosa, a hint that the disease may be under-recognized in India. We present an account of our observations in patients diagnosed with prurigo pigmentosa who were of five different nationalities, namely, Japanese, German, Indonesian, Turkish and Iranian. With this article we seek to increase awareness for the condition among dermatologists in India and we provide criteria for its diagnosis, both clinically and histopathologically.

Condom leukoderma.

Contact dermatitis from natural latex of condom has been reported and is attributed to latex sensitivity. Chemical leukoderma from rubber condom is probably not reported. Here we present a case of chemical leukoderma in a 32-year-old male who developed depigmentation around the shaft of the penis in a circumferential pattern. Since the lesion was solitary and the site corresponded to the point of maximum contact of the condom, a diagnosis of contact leukoderma due to latex condom was thought of. Patch testing was done with mercaptobenzothiazole (MBT), dusting powder present in the condom and condom latex as such. The patient tested positive (3+) with mercaptobenzothiazole and the condom latex. On discontinuation of condom use and with UVB phototherapy, lesions repigmented in eight weeks.

Tuberous sclerosis complex associated with dyschromatosis universalis hereditaria.

Tuberous sclerosis is an autosomal dominant disease due to mutations in two genetic loci, characterized by hamartoma formation in the skin, nervous system, heart, kidney and other organs. Dyschromatosis universalis hereditaria is an autosomal dominant genodermatosis, characterized by small hyperpigmented and hypopigmented macules, uniformly distributed over the entire body. The face is rarely involved and the palms, soles and mucous membranes are usually spared. We report a case of tuberous sclerosis with dyschromatosis universalis hereditaria, with hyperpigmented and hypopigmented macules affecting the palms, soles and oral mucosa. To our knowledge, this is the first reported case of such an association.

Hyperpigmented dermal macules in children following the administration of Maloprim for malaria chemoprophylaxis.

The occurrence of an unexpected side effect following the use of Maloprim (pyrimethamine/dapsone) for malaria chemosuppression in 3-59 months old children in Sierra Leone is reported. As part of a trial of chemoprophylaxis and insecticide-impregnated bed nets, 2000 children received either Maloprim or placebo; 4% of children who received Maloprim fortnightly for more than 3 months developed hyperpigmented macules, whereas none of the children who received placebo did so. Histopathological examination of full thickness skin biopsies showed macrophages containing melanin in the dermal layer. Clustering of cases was noted among siblings, suggesting the possible involvement of genetic factors in the pathogenesis of these skin reactions. One child was accidentally re-exposed to Maloprim after the drug had been withdrawn and he developed a severe reaction. No other serious side effect was noted. Hyperpigmented lesions similar to those reported in this study have been described previously in patients with leprosy treated with dapsone, and the dapsone component of Maloprim is the likely cause of the skin reactions seen in children given this drug for malaria chemoprophylaxis.

Hypopigmented lesions in early leprosy--a clinical and histological study.

26 Patients of leprosy presenting with hypopigmented lesions were divided on morphological grounds into 3 Sub groups, Group I (9 patients) with well-defined single patch with moderate to complete sensory loss; Group II (8 patients) with single ill-defined lesion having partial sensory loss; and Group III (9 patients) having multiple hypo-pigmented patches with mild to moderate sensory loss. Epidermal atrophy was a conspicuous histological finding in all groups. Only patients in Group I showed epitheloid cells in dermal infiltrate with erosion of epidermis in one case. This group may be labelled as maculoanesthetic leprosy. Patients in Group II and III showed mononuclear cell infiltrate in dermis, around neurovascular bundles and appendages. They were histologically consistent with indeterminate leprosy. Follow-up biopsy after six to eight months of treatment showed healing of the lesion of reduction in the infiltrate in most cases.

Primary hyperpigmented cutaneous lesions in tuberculoid leprosy.

Two cases of tuberculoid leprosy with primary hyperpigmented anaesthetic lesions are reported and subject is reviewed.

Leprous lesion with hyperpigmented border.

Two cases of leprosy--one Tuberculoid and the other Borderline are reported. The Tuberculoid patch shows sharply defined hyperpigmented border whereas in the Borderline patient, diffuse hyperpigmentation is present around the lesion. The cases are reported for documentation due to rare clinical pressentation.

Hypopigmentation in leprosy.

Hypopigmentation of macular leprosy lesions was not found to correlate with cellular infiltrate and AFB load in tissues. It is felt that hypopigmentation is in some way related to neural involvement.