Morphea as an isotopic response to pigmented purpuric dermatosis and lichen striatus.

Morphea is an uncommon inflammatory and fibrosing disorder that has a polymorphous clinical presentation. We report two cases of morphea developing as an isotopic response after a preceding benign skin disease, accompanied by a review of the literature. This case series highlights the importance of return to care recommendations for benign skin conditions such lichen striatus and pigmented purpuric dermatoses due to the rare possibility of subsequent morphea development.

Clinical and epidemiological profile of lichen striatus in children-Experience from a tertiary care hospital.

We analyzed records of 30 patients with lichen striatus (age < 18 years) in this retrospective study. Seventy percent were females and 30% were males with a mean age of diagnosis of 5.38 ± 4.22 years. The most common age group affected was 0-4 years. The mean duration of lichen striatus was 6.66 ± 4.22 months. Atopy was present in 9 (30%) patients. Although LS is a benign self-limited dermatosis, long-term prospective studies with a greater number of patients will help in better understanding of the disease including its etiopathogenesis and association with atopy.

Rapid and successful effects of combining dupilumab with ultraviolet B radiation therapy in the treatment of Papuloerythroderma of Ofuji.

Papuloerythroderma of Ofuji (PEO) is an uncommon disease characterised by widespread erythroderma composed of intensely pruritic solid papules coalescing into plaques sparing the skin folds (deck-chair sign). The pathogenesis of PEO remains unclear, although T helper (Th) 2 and Th22 cells may play an important role. Dupilumab is an interleukin (IL)-4 receptor α-antagonist that effectively reduces Th2 responses, which has drawn increasing attention in the treatment of PEO patients. Here, we reported a successful case of dupilumab treatment in combination with ultraviolet B (UVB) radiation therapy, which is well known and effective for chronic itch. The patient had a significant decrease in visual analogue scale (VAS) score and eosinophil after only 1 week of treatment, which may be due to the combination effect.

Pityriasis rubra pilaris with erythema gyratum repens-like eruption and resolution with ustekinumab.

Erythema gyratum repens (EGR) is a rare paraneoplastic disorder often preceding the diagnosis of underlying malignancy by 9 months on average, while pityriasis rubra pilaris (PRP) is an uncommon papulosquamous inflammatory disease. We present the case of a 58-year-old woman with an EGR-like eruption transforming from resolving PRP, without associated malignancy. Her rash dramatically resolved within a month of ustekinumab initiation, which supports this presentation as a unique entity.

Familial Dyskeratosis Comedones without Dyskeratosis-A Rare Entity.

A 42-year-old woman presented with multiple, discrete, symmetric hyperkeratotic papules and comedo-like lesions over the cheek and upper part of the back. These lesions first began to appear at the age of 20 years. Since then, new lesions have periodically appeared with spontaneous regression. Keratinous material could be extruded, leaving behind pock-like scars. The scalp, palms, soles, and mucous membranes were spared. Because the lesions were asymptomatic, the patient did not seek any treatment (Figure 1 and 2). (SKINmed. 2022;20:152-153).

Dowling-Degos Disease Presenting With Associated Epidermal Inclusion Cysts: A Case Report and Review of the Literature.

ABSTRACT: Dowling-Degos Disease (DDD) is a rare and disfiguring autosomal dominant genodermatosis characterized by reticulate hyperpigmented macules or follicular comedone-like papules in the intertriginous areas that typically presents in the third or fourth decade of life. It is a progressive disease that is often treatment-resistant. Although its association with hidradenitis suppurativa has been well described, DDD has also been less commonly reported in conjunction with other dermatologic diseases with unknown etiologic associations. Herein, we present a case of DDD with associated epidermal inclusion cysts and conduct a literature review of dermatologic conditions reported in association with DDD.

Lichen striatus colocalized with a Spitz nevus.

A 7-year-old healthy girl presented with an 11-month history of an asymptomatic red, dome-shaped papule on her right medial elbow and 6-month history of linearly distributed, few millimeter, flesh-colored papules extending from and including the red papule. Histopathology demonstrated features of both a Spitz nevus and lichen striatus. The Spitz nevus was removed with a punch biopsy and the lichen striatus subsequently resolved. To our knowledge, co-localization of a Spitz nevus with lichen striatus has not been previously reported and highlights the potential association between the immunogenicity of Spitz nevi and the development of lichen striatus.

Papuloerythroderma of Ofuji.

Papuloerythroderma of Ofuji (PEO) is a rare skin condition first described in 1984 and characterized by diffuse erythroderma composed of papules coalescing into plaques with sparing of skin folds, known as the deck-chair sign. The disease is almost exclusively seen in the elderly and affects men more frequently than women. Common laboratory findings include peripheral and tissue eosinophilia, elevated levels of immunoglobulin E, and lymphopenia. The diagnosis entails exclusion of potentially causative pathologies, including drug intake, atopy, malignancy, and infection. These factors have frequently been found in association with PEO, but their role in the etiopathogenesis of the disease is poorly understood. A dysregulated immune system, with particular involvement of T-helper (Th)2 and Th22 cells, seems to be important in the development of PEO. Controversy exists as to whether PEO exists as an independent entity or as a clinical pattern of a variety of distinct conditions. Treatment necessitates first addressing any coexisting circumstances that may have a causal relationship with PEO. In idiopathic cases, topical and oral corticosteroids, ultraviolet light therapies, and immunosuppressive/immunomodulating therapies have been used with variable results. Future studies are needed to further understand the disease process and to establish guidelines for diagnostic workup and treatment.

Dermatosis específicas de la embarazada (DEE). Propuesta de nueva clasificación / Pregnant specific dermatoses (DEE). Proposal for a new classification

En el presente trabajo se realiza una breve descripción de las dermatosis específicas de la embarazada (DEE). Se considera que la afección específica más frecuente son las pápulas y placas urticarianas pruriginosas del embarazo (PPUPE), y en segundo lugar los prurigos vinculados del embarazo (PVE). Se aporta una propuesta de nueva clasificación de las dermatosis específicas de la embarazada (DEE). En la clasificación se consideran dos grupos de afecciones. Grupo A: dermatosis específicas de la embarazada (DEE), y dentro de él: a) como enfermedad más frecuente las pápulas y placas urticarianas pruriginosas del embarazo (PPUPE); b) seguidas de los prurigos, tanto sea el precoz como el tardío, a los cuales se los une bajo el término de prurigos vinculados del embarazo (PVE) como entidad también frecuente c) la foliculitis pruriginosa del embarazo (FPE). Como afecciones menos frecuentes dentro de este grupo: a) dermatitis papulosa de Spangler (DPS); b) erupción toxémica del embarazo (ETE); c) herpes gestationis (HG); d) impétigo herpetiforme (IH). Grupo B: afección específica de la embarazada sin lesiones en piel: colestasis intrahepática del embarazo (CIE). (AU)

In the present work, a brief description of the specific dermatoses of the pregnant woman (DEE) is made. The most common specific condition is considered to be pruritic urticarial papules and plaques of pregnancy (PPUPE), and secondly, prurigos associated with pregnancy (PVE). A proposal for a new classification of pregnant-specific dermatoses (DEE) is provided. Two groups of conditions are considered in the classification. Group A: pregnant-specific dermatosis (EED), and within it: a) as the most frequent disease, pruritic urticarial papules and plaques of pregnancy (PPUPE); b) followed by prurigos, both early and late, to which they are associated under the term of linked prurigos of pregnancy (PVE); as also frequent entity; c) the pruritic folliculitis of pregnancy (FPE). As less frequent conditions within this group: a) Spangler's papular dermatitis (DPS); b) toxic eruption of pregnancy (TEE); c) herpes gestationis (HG); d) impetigo herpetiformis (IH). Group B: specific condition of the pregnant woman without skin lesions: intrahepatic cholestasis of pregnancy (ICD). (AU)

A Systematic Review on the Treatment of Dermatosis Papulosa Nigra.

Dermatosis papulosa nigra (DPN) is a benign skin condition that is primarily reported in skin of color patients. While prevalent, treatment options are limited and the benign course of DPNs may cause them to be overlooked by clinicians. However, large and multiple lesions in cosmetically sensitive areas may be emotionally and socially distressful to patients. There are few literature reviews examining treatment options for this condition. A literature search was performed using PubMed, Medline, Embase, and Web of Science databases. 67 articles were identified and 15 studies met the inclusion criteria. Our findings demonstrate that laser therapy is becoming increasingly utilized as a safe and efficacious treatment for DPNs in skin of color patients. J Drugs Dermatol. 20(4):467-472. doi:10.36849/JDD.2021.5555.

Dowling-Degos disease: a review.

Dowling-Degos disease is a rare autosomal dominant genodermatosis. It is characterized by acquired reticulate hyperpigmentation over the flexures, comedone-like follicular papules, and pitted perioral scars that usually develop during adulthood. Mutations in genes affecting melanosome transfer, and melanocyte and keratinocyte differentiation have been implicated in the pathogenesis of this disease. These genes include KRT5, POFUT1, POGLUT1 and, most recently, PSENEN. Dowling-Degos disease can be found in isolation or with other associated findings, most notably hidradenitis suppurativa. This condition belongs to a spectrum of conditions that all result in reticulate hyperpigmentation that at times are hard to distinguish from each other. The most closely linked entity is Galli-Galli, which is clinically indistinguishable from Dowling-Degos disease and can only be distinguished by the presence of acantholysis on microscopy. Unfortunately, Dowling-Degos disease is generally progressive and recalcitrant to treatment.

Differentiation of inflammatory papulosquamous skin diseases based on skin biophysical and ultrasonographic properties: A decision tree model.

BACKGROUND: The biophysical and ultrasonographic properties of the skin change in papulosquamous diseases. AIMS: : To identify biophysical and ultrasonographic properties for the differentiation of five main groups of papulosquamous skin diseases. METHODS: Fifteen biophysical and ultrasonographic parameters were measured by multiprobe adapter system and high-frequency ultrasonography in active lesions and normal control skin in patients with chronic eczema, psoriasis, lichen planus, pityriasis rosea and parapsoriasis/mycosis fungoides. Using histological diagnosis as a gold standard, a decision tree analysis was performed based on the mean percentage changes of these parameters [(lesion-control/control) ×100] for differentiation of the diseases. RESULTS: The accuracy of the decision tree model for differentiation of five diseases was 67% which developed based on changes in stratum corneum hydration, epidermal thickness, skin pH, melanin index, R0 (reciprocal of firmness) and erythema. Among the flowcharts for pairs of diseases, three models for differentiation had high accuracy (> 95%): those of psoriasis from lichen planus, pityriasis rosea, and parapsoriasis/mycosis fungoides. LIMITATIONS: Validation studies on a larger sample size in situations where the diagnosis is unclear are needed to confirm the accuracy and applicability of decision trees. CONCLUSION: Skin biophysical and ultrasonographic properties may help in the differentiation of papulosquamous diseases as simple and non-invasive tools.