Pityriasis lichenoides represents a unique group of inflammatory skin disorders that include pityriasis lichenoides et varioliformis acuta (PLEVA), febrile ulceronecrotic Mucha-Habermann disease (a subtype of PLEVA), and pityriasis lichenoides chronica. The history, epidemiology, clinical features, pathophysiology, and treatment of this group of conditions are reviewed in this manuscript.

Pityriasis lichenoides and its subtypes.

https://www.cidjournal.com/article/S0738-081X(04)00147-6/pdf

Treatment of nonhealing ulcers with allografts

Pityriasis lichenoides (PL) is an uncommon, acquired spectrum of skin conditions that poses various challenges to patients as well as clinicians. It is a difficult and debatable disorder to diagnose, categorize, and treat. Besides these inherent obstacles, PL merits awareness because of its potential to progress to cutaneous lymphoma or an ulceronecrotic presentation, both of which carry a significant risk of mortality. The scope of PL presentations is delineated along a continuum of multiple variants including pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC), and febrile ulceronecrotic Mucha-Habermann disease (FUMHD). Classification of these presentations as separate subsets is debatable in view of their overlapping clinical, histopathologic, and etiologic features. PLEVA generally presents as an acute-to-subacute skin eruption of multiple, small, red papules that develops into polymorphic lesions and vacillates with periods of varying remissions as well as possible sequelae of hyper/hypopigmentation and varicella-like scars. PLC has a more gradual manifestation of very small red-to-brown flat maculopapules with mica-like scale; it also follows a relapsing course but with long periods of remission. FUMHD is an acute and severe generalized eruption of purpuric and ulceronecrotic plaques with associated systemic involvement and a mortality rate of up to 25%; hence, it should be approached as a dermatologic emergency.Histopathological evaluation of PL usually reveals dermal, wedge-shaped, lymphocytic infiltrate, epidermal spongiosis, parakeratosis, and variable necrosis of keratinocytes. PLC demonstrates more subtle histology whereas, at the other end of the spectrum, febrile ulceronecrotic FUMHD exhibits the most exaggerated histological features. The pathogenic mechanism behind PL is unclear although infectious or drug-related hypersensitivity reactions versus premycotic lymphoproliferative disorder are the mainstay theories. The foremost therapies for PLEVA and PLC are phototherapy, systemic antibacterials, and topical corticosteroids. Aggressive treatment with immunosuppressant and/or immunomodulating agents as well as intensive supportive care are recommended for FUMHD. We first describe a representative case of a 14-year-old boy with PLC who was successfully treated with narrow-band UVB. We then review the pathophysiology, classification, and treatment of PL.

Pityriasis lichenoides: pathophysiology, classification, and treatment.

BACKGROUND Various biologic dressings have been developed in an effort to find the ideal skin substitute for use in acute and chronic wounds. There are many potential uses for such dressings, but no panaceas exist. Because millions of health care dollars are spent each year on wound care, and a great deal of patient morbidity occurs from these wounds, the search for new and better dressings is likely to continue. OBJECTIVE To review the current evidence regarding the utility, outcomes, and adverse effects of the available biologic dressings, with a particular focus on use in acute surgical wounds and applicability to dermatologic surgery. MATERIALS AND METHODS PubMed literature search and review of data on biologic dressings with particular attention to the past 2 decades. Emphasis was placed on peer-reviewed manuscripts and larger series. CONCLUSIONS There is extensive literature regarding the use of biologic dressings in chronic wounds, such as venous leg ulcers and burns, but studies evaluating these dressings in acute surgical wounds and dermatologic surgery have been limited. There appear to be specific surgical settings in which such dressings may be of particular use, in addition to limitations of their use. Additional studies, particularly randomized and comparative trials, would be highly desirable.

Biologic Dressings: Current Applications and Limitations in Dermatologic Surgery

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA). PLEVA patients only very rarely have systemic signs; the cutaneous lesions are usually asymptomatic, but may be pruritic and may heal with scarring. FUMHD often starts out as classic PLEVA, but goes on to develop widespread ulceronecrotic lesions and is associated with a high mortality rate. Whether Pityriasis lichenoides chronica (PLC) and PLEVA form a spectrum rather than single entities of clonal lymphoproliferative diseases has been discussed. Recently, it has been proposed that FUMHD, too, is a clonal lymphoproliferative disorder. Here, we report two cases of FUMHD with monoclonal T-cell population, as detected by Southern blot analysis. We propose that clonal FUMHD represents a cutaneous T-cell lymphoma entity.

https://www.jaad.org/article/S0190-9622(04)01561-0/pdf

Febrile ulceronecrotic Mucha-Habermann disease with clonality: a cutaneous T-cell lymphoma entity?

Background: Chronic insomnia disorder is highly prevalent, disabling and costly. Cognitive-behavioural therapy for insomnia (CBT-I) is recommended as the first-line treatment. CBT-I may contain various educational, cognitive and behavioural strategies delivered in a range of formats, but the effect of each component remains unclear. Methods: We performed a systematic review and component network meta-analysis (cNMA) of CBT-I trials for chronic insomnia. We searched PubMed, CENTRAL, PsycINFO and ICTRP for randomised controlled trials published from database inception to 14th May 2022, comparing any form of CBT-I against each other or a control condition for chronic insomnia disorder in adults (aged [≥]18 years). We included insomnia both with and without comorbidities. Concomitant treatments were allowed if they were equally distributed among the arms. Two independent reviewers identified components, extracted data, and assessed trial quality. Primary outcome of interest in this study was treatment efficacy (remission defined as reaching a satisfactory state at endpoint measured by any validated self-reported scale) at post-treatment. (PROSPERO; CRD42022324233) Findings: We identified 226 trials, including 29,982 participants. Mean age was 45.7 years and 71% were women. The results suggests that critical components of CBT-I are cognitive restructuring (incremental odds ratio[iOR] 1.63 [95% confidence interval 1.25 to 2.14]), sleep restriction (iOR 1.44 [1.00 to 2.06]) and stimulus control (iOR 1.44 [1.00 to 2.07]) Sleep hygiene education was not essential (iOR 1.05 [0.79 to 1.38]) and relaxation procedures may be counterproductive (iOR 0.81 [0.64 to 1.03]). Face-to-face, therapist-led program was found to be most beneficial (iOR 1.86 [1.21to 2.85]). The overall risk of bias was low in 8% of the trials, some concerns in 56%, and high in 36%. Interpretation: This cNMA suggests that effective and efficient CBT-I packages can include cognitive restructuring, sleep restriction and stimulus control, but not relaxation.

/pdf/dismantling-cognitive-behavioural-therapy-for-chronic-1hxxm4kl.pdf

Dismantling cognitive-behavioural therapy for chronic insomnia in adults with or without comorbidities: a systematic review and component network meta-analysis

We report a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) in a 21-year-old man. This disease is a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) and is characterized by the sudden onset of diffuse ulcerations associated with high fever and systemic symptoms. It is sometimes lethal especially in elderly patients. In the present case, intense generalized maculopapular erythematous plaques with central necrosis developed progressively in association with a high fever. Initial treatment with systemic betamethasone had been unsuccessful and the skin lesions, which covered about 50% of the body surface, became severely ulcerated. Although the development of new lesions had ceased spontaneously, widespread ulceration of the skin remained. Debridement of the necrotic skin and skin grafting using cultured epidermal autografts and meshed allografts of cadaver skin led to prompt reepithelization.

A case of febrile ulceronecrotic Mucha-Habermann disease requiring debridement of necrotic skin and epidermal autograft

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Dismantling cognitive-behavioural therapy for chronic insomnia in adults with or without comorbidities: a systematic review and component network meta-analysis

A case of febrile ulceronecrotic Mucha-Habermann disease requiring debridement of necrotic skin and epidermal autograft