Macular pigmentation of uncertain aetiology revisited: two case reports and a proposed algorithm for clinical classification

Ashy dermatosis, erythema dyschromicum perstans, lichen planus pigmentosus and idiopathic eruptive macular pigmentation are various types of acquired macular hyperpigmentation disorders of the skin described in literature. However, a global consensus on the definitions of these entities is lacking. We report two cases of acquired macular (hyper)pigmentation of uncertain aetiology diagnosed as ashy dermatosis and attempt to clarify the various confusing nosologies based on existing literature. We infer that acquired small and large macular pigmentation of uncertain aetiology should be considered separate from that associated with lichen planus. We also propose a diagnostic algorithm for patients with acquired macular hyperpigmentation.     

Erythema dyschromicum perstans in children: a report of 14 cases

BACKGROUND: Erythema dyschromicum perstans (EDP) or ashy dermatosis is a peculiar, slowly progressive, macular hyperpigmentation, which leaves a permanent discoloration. It is an acquired dermatosis that occurs most frequently in Central and South America. EDP usually appears in adults, but some isolated cases and small series have been reported in prepubertal children. METHODS: A retrospective review of cases of EDP in 10-year-old children or younger, attended in a pediatric hospital between 1990 and 2003. RESULTS: We present 14 cases of EDC in children 10 years and younger. With an additional 25 cases reported so far in the English language literature, a total of 39 children have been described. Unlike adult patients, who are most commonly of Hispanic origin, children with EDP are usually Caucasian. Other important facts in children with EDP are the absence of consistent trigger factors and an eventual improvement or resolution of the lesions in 50% of prepubertal patients. CONCLUSION: We suggest that EDP is a distinctive clinical entity, different from lichen planus and lichen planus pigmentosus, which may be identical to the so-called idiopathic eruptive macular pigmentation.     

Erythema dyschromicum perstans in early childhood

Erythema dyschromicum perstans (EDP) is a rare disorder characterized by asymptomatic, slowly progressive, ash-gray macular pigmentation of the skin which usually occurs from age 5 through adult life. We have experienced two cases of EDP in children aged 2 and 3, both exceptionally younger than the previously reported cases. We therefore suggest that EDP should be included in the differential diagnosis of pigmentary disorders occurring at an early age.     

Erythema dyschromicum perstans in a Japanese child

Erythema dyschromicum perstans (EDP) is asymptomatic slate-gray to blue-brown macules. Idiopathic eruptive macular pigmentation is asymptomatic brown nonconfluent macules. We describe electron microscopic studies of a 9-year-old Japanese girl with EDP. The ultrastructural figures indicated that the production of immature, small, irregular-shaped melanosomes in melanocytes and peripheral localization of melanosomes in keratinocytes caused the clinical appearance of EDP. The ultrastructural evidence distinguishes EDP from idiopathic eruptive macular pigmentation and suggests a distinct pathogenesis of the disease.     

Erythema dyschromicum perstans: a case report and review

Erythema dyschromicum perstans (EDP) is an acquired ashy dermatosis characterized by patches of blue-gray pigmentation over the trunk, extremities, and neck. These pigmentary changes may become chronic and disfiguring. At present, the etiology of EDP remains unclear, and there is no single established therapy. We describe a 21-year-old Hispanic man with extensive patches of EDP who improved with oral corticosteroid therapy.     

Lichen planus pigmentosus and its variants: review and update

Lichen planus pigmentosus (LPP) is considered a rare variant of lichen planus (LP). It is characterized by acquired dark brown to gray macular pigmentation located on sun‐exposed areas of the face, neck, and flexures, commonly found in dark‐skinned patients. In patients with LPP, an inflammatory lichenoid response results in marked pigmentary incontinence. It has been associated with hepatitis C virus, sun exposure, and contactants such as mustard oil and nickel. LPP‐inversus affects fair and dark skin, predominantly involving flexural and intertriginous areas, while sun‐exposed areas are spared; friction is an associated trigger. LPP along Blaschko's lines has been associated with susceptibility to genetic mosaicisms. LPP can present concomitantly with other variants of LP such as frontal fibrosing alopecia, as well as endocrinopathies, and autoimmune diseases. Treatment is difficult and consists of avoidance of triggers and topical and systemic medications in order to stop the inflammatory reaction and reduce pigmentation, improving aesthetic appearance and quality of life.     

Erythema dyschromicum perstans in prepubertal children

Erythema dyschromicum perstans (EDP) is a rare disorder of pigmentation that is most common in Hispanic patients. In adults, EDP has a slow onset and is unlikely to resolve spontaneously. The etiology and clinical course in children is poorly defined. Physical examinations, chart reviews, and telephone interviews were performed for eight pediatric patients with EDP who were followed at Children's Memorial Hospital in Chicago between 1990 and 1998. All the patients available for long-term follow-up (five of the eight) experienced complete clearance without recurrence in an average of 2.5 years. In all of our patients, the onset was noted from July to December. The administration of aminopenicillins was coincident with the development of EDP in two of the patients. Review of the English-language literature reveals that 25 prepubertal children have previously been reported. Including our patients, 69% of prepubertal children with EDP experienced resolution. We concluded that the clinical course of childhood (prepubertal) EDP differs from that of adult EDP, and it is more likely to resolve within 2-3 years.     

The skin manifestations of congenital and acquired hemostatic disorders

The authors review the literature data on skin symptoms of the most prevalent disorders induced by congenital or acquired defects of blood coagulation. The range of skin changes in hemostasis disorders includes hemorrhages of 5 various types, thromboses, pigmentation disorders, and pustular involvement. Early detection of skin symptoms of hemostasis disorders will improve the diagnosis and timely correction of such conditions.     

Erythema dyschromicum perstans: response to dapsone therapy

Erythema dyschromicum perstans (EDP) is a rare disorder characterized by asymptomatic, slowly progressive, ash-gray macular pigmentation of the skin, which usually occurs from age 5 years through adult life. Most cases reported to date are of Latin American and Indian patients. Rare cases have been reported from Turkey. No treatment of choice is presently available. Various therapies have been tried, including sun protection, chemical peels, antibiotics, corticosteroids, vitamins, isoniazid, griseofulvin, and chloroquine, without any benefit. Some authors have suggested the therapeutic efficacy of clofazimine and dapsone on EDP. We report a case of EDP that responded remarkably well to treatment with dapsone.     

Erythema Dyschromicum Perstans: Report of a New Case and Critical Review of the Literature

Erythema dyschromicum perstans (EDP), described by Convit et al. in 1961, is a rare dermatosis. Its relationship with ashy dermatosis (AD), described by Ramirez in 1957, is still a matter of debate. We report a typical case of EDP. The patient, of North African origin, had a dyschromic (hypo- and hyperpigmented) eruption on the chest and limbs for 2 years. The lesions were occasionally surrounded by a papular border which spread slowly and centrifugally. Histological examination showed a lichenoid infiltrate. A carcinoma of the lung was simultaneously discovered. No treatment was given. EDP is infrequent and often considered identical to ashy dermatosis in the literarure. However, the clinical aspects of the two diseases differ. The main features of these two diseases are reviewed and compared on the basis of a literature review. We conclude that EDP and AD are distinct clinical entities.     

Clinicopathological characteristics of lichen planus pigmentosus and its response to tacrolimus ointment: an open label,non‐randomized,prospective study

Background Lichen planus pigmentosus (LPP) is an uncommon variant of lichen planus, for which no effective treatment is available. Objectives The aim of this study was to determine the clinical, epidemiological and histopathological characteristics of LPP patients in Kuwait. Methods Thirty‐three LPP patients who attended the Dermatology outpatient clinics at Farwaniya Hospital, Kuwait from the year 2002 to 2008 were studied for clinical, epidemiological and histopathological findings. Thirteen of these patients were treated with topical tacrolimus 0.03%. Ointment applied topically twice daily for the duration varying from 6 to 12 weeks. Results Of the 33 patients, 21 were men and 12 were women. The duration of eruption ranged from 6 weeks to 3 years. The face and neck were the commonest sites, affecting 18 (54.5%) patients. The pattern of pigmentation was diffuse in 18 (54.54%) patients, reticular in seven (21.2%), blotchy in five (15.2%), linear in two (6.1%) and perifollicular in one (3%). Twenty patients had positive serology for hepatitis C virus (HCV), with significantly higher serum liver enzymes (ALT and AST). Of the 13 patients, who were treated with tacrolimus Ointment, seven (53.8%) showed appreciable lightening of the pigmentation after an average of 12 weeks. Conclusions We conclude that HCV may be one of the factors associated with LPP, in those who have a tendency to develop LPP. However, this possible association should be interpreted carefully. In addition, tacrolimus ointment could have a beneficial role in the treatment of LPP.     

Eyelash trichomegaly: review of congenital, acquired, and drug-associated etiologies for elongation of the eyelashes

Eyelash trichomegaly is defined as eyelashes which are found to be of increased length, thickness, and pigmentation. This unique finding can be present at birth as part of a variety of congenital syndromes or as a benign familial trait. There are also acquired conditions and drugs that are known to cause these changes. Case reports and clinical studies in the medical literature concerning eyelash trichomegaly were investigated and summarized to compile a comprehensive review of the etiology of eyelash trichomegaly. Previously published reviews and studies that report on the finding of increased generalized hair growth and which do not specifically mention eyelashes were not included. Trichomegaly of the eyelashes may occur as a key feature among rare congenital syndromes, develop in association with certain acquired diseases, or present as an intended or treatment-related adverse drug effect. Eyelash trichomegaly may be present from birth or manifest later in life in association with acquired diseases or drug therapy. The relevance of this finding may be benign, however eyelash trichomegaly in some individuals can be a symptom for immune dysfunction or a clinical measure of response to drug therapy.     

Management of congenital ichthyoses: European guidelines of care,part one

These guidelines for the management of congenital ichthyoses have been developed by a multidisciplinary group of European experts following a systematic review of the current literature, an expert conference held in Toulouse in 2016 and a consensus on the discussions. They summarize evidence and expert-based recommendations and are intended to help clinicians with the management of these rare and often complex diseases. These guidelines comprise two sections. This is part one, covering topical therapies, systemic therapies, psychosocial management, communicating the diagnosis and genetic counselling.     

Management of congenital ichthyoses: European guidelines of care,part two

These guidelines for the management of congenital ichthyoses have been developed by a multidisciplinary group of European experts following a systematic review of the current literature, an expert conference held in Toulouse in 2016, and a consensus on the discussions. These guidelines summarize evidence and expert-based recommendations and intend to help clinicians with the management of these rare and often complex diseases. These guidelines comprise two sections. This is part two, covering the management of complications and the particularities of some forms of congenital ichthyosis.     

Primär kutane CD4+ klein‐ bis mittelgroßzellige pleomorphe T‐Zell‐Lymphoproliferation: Wo stehen wir? Eine systematische Übersicht

Primary cutaneous CD4+ small/medium pleomorphic T‐cell lymphoproliferative disorder (PCSMP‐TLPD) is a provisional entity with uncertain malignant potential according to the latest revision of the WHO classification for lymphoid neoplasms. We conducted a systematic literature review of all previously reported cases of PCSMP‐TLPD to highlight their typical and atypical features. The main features of PCSMP‐TLPD and its possible clinicopathologic overlap with similar disorders are also discussed. It is hoped that this review will provide a useful outline of this condition and the most important differential diagnoses. Finally, we recommend a rigorous consensus among cutaneous lymphoma experts in drafting diagnostic criteria and the best case definition.     

Do you know this syndrome?

Congenital hypotrichosis and Stargardt macular dystrophy are rare autosomal recessive disorder of unknown etiology respectively characterized by hair loss, macular degeneration and severe progressive vision reduction. There are few reports in the literature with this association. Studies show that the defective gene is on the chromosome I6q22.1 and involve cadherin molecule in the pathogenesis. Early recognition of these disorders often starts with hair changes and should alert the dermatologist for an eye examination thereby avoiding more severe ocular defect.     

Confusion in determination of two types of cutaneous adverse reactions to drugs,maculopapular eruption and erythema multiforme,among the experts: A proposal of standardized terminology

The clinical classification of cutaneous adverse reactions by drugs should be clearly distinguished to avoid conceptual confusion and inconsistency. Although dermatologists appear to have established a roughly common consensus for cutaneous adverse reactions, some types are more rigorously defined than other, possibly misleading classifications. To assess the consensus on the clinical classifications, we investigated the concordance rate of diagnosis by Japanese experts through a snap visual inspection of various clinical pictures exhibiting erythema multiforme and maculopapular eruption types of cutaneous adverse reactions. The experts were shown images on a screen and were then asked to decide whether to classify cases as maculopapular eruption or erythema multiforme type, and the concordance rates were calculated. Overall, the mean concordance rate was 71.6% (standard deviation, 17.3%), and only 33.8% of cases had a 90% or more concordance rate. Our study shows that the determinations of erythema multiforme and maculopapular eruption types by the existing classification criteria were confusing even among experts, which prompted us to standardize the terminology. We propose clinically defining erythema multiforme type as generalized macules mainly of 1 cm or more with a tendency of elevation and coalescence, and maculopapular eruption type as generalized erythema other than erythema multiforme type. Currently, the clinical definitions of cutaneous adverse reactions are poorly described, which may be problematic upon analyzing large volumes of data. Our proposal for a new terminology will enhance the accuracy and consistency of information for the correct analysis of cutaneous adverse reactions.     

Vitiligo: an evidence‐based update. Report of the 13th Evidence Based Update Meeting, 23 May 2013, Loughborough,U.K.

The Evidence Based Update (EBU) meetings are annual one‐day meetings held by the Centre of Evidence Based Dermatology at the University of Nottingham. The aim of the meeting is to discuss high‐quality evidence, mainly in the form of systematic reviews and randomized controlled trials, on a different topic each year. The meetings are designed to be interactive with a panel discussion between international experts and delegates forming a key part of the meeting. The 13th EBU meeting was on vitiligo and took place on 23 May 2013 in Loughborough, U.K. The most recent research including new and unpublished studies was presented on the classification of vitiligo, the evidence behind different treatment options and current guidelines for vitiligo.     

The color of skin: brown diseases of the skin,nails, and mucosa

Brown diseases comprise disorders leading to hyperpigmentation in skin and nails. Melasma is an acquired skin disorder that is characterized by brownish macules that typically occur on the face. Schamberg disease, also known as progressive pigmented purpura, is characterized by brown pigmentation with pepper spots on their edges. We summarize the epidemiology, pathogenesis, histologic features, and treatment choices for additional brown diseases, including melasma, pigmented purpuric dermatoses, postinflammatory hyperpigmentation, drug-induced hyperpigmentation, and pigmentations due to systemic or physiologic conditions.     

Erythema dyschromicum perstans

A 39-year-old Hispanic man presented with widespread, symmetric, hyperpigmented, brown-gray-blue, oval-to-circular, discrete and coalescing patches on the upper extremities, trunk, neck and face, many with an associated annular erythematous border. Histopathologic features showed an interface dermatitis, thin lichenoid and superficial perivascular infiltrates, and dermal melanophages. These changes were consistent with a diagnosis of erythema dyschromicum perstans (EDP). The etiology of EDP is unknown, but many associations have been described. The rim of erythema that appears during the inflammatory phase of the disorder distinguishes it from other disorders of hyperpigmentation. There is no consistently effective treatment for EDP. We highlight the use of narrow-band UV B phototherapy in the treatment of this disorder, which has shown good success in our patient thus far.