Parapsoriasis is a term encompassing several disorders of the skin that possess a similar clinical presentation to psoriasis. They are considered to be lymphoproliferative in origin, as a number of patients progress to cutaneous T-cell lymphoma or mycosis fungoides. Pityriasis lichenoides, large plaque parapsoriasis (> than 5 cm in diameter) and small plaque parapsoriasis (< 5 cm) are the three main subtypes. The diagnosis is made on clinical grounds and a biopsy with a subsequent histopathological examination.
Presentation
The term parapsoriasis was initially coined more than 100 years ago and included pityriasis lichenoides, large plaque parapsoriasis, and small plaque parapsoriasis, which exhibit a similar clinical appearance but different etiology from psoriasis [1] [2]. The conditions that fit the clinical and histological description of parapsoriasis seem to represent a spectrum of a lymphoproliferative disorder because many studies have established that patients with either small plaque or large plaque psoriasis eventually progress to a T-cell cutaneous lymphoma (known as mycosis fungoides) [2] [3] [4]. Furthermore, infectious pathogens, such as human herpesvirus type 8 (HHV-8), were identified in a substantial number of lesions [5], implying that microorganisms might play an important role in the development of this clinical entity. As mentioned previously, three main subtypes of parapsoriasis are currently identified [1] [4] [6] [7]:
- Pityriasis lichenoides - It is described as a rare disorder of the skin with several clinical forms - pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC) and febrile ulceronecrotic Mucha-Habermann disease (FUMHD) [7]. Main findings include an acute eruption of small red papules on multiple sites that may leave residual hypo/hyperpigmentation and scarring after resolution (PLEVA), a slower onset of flat macules and papules that are reddish-to-brown in color (PLC), or severe generalized eruption of necrotic plaques, ulcers, and purpuric lesions that has a 25% mortality rate (FUMHD) [7].
- Small plaque parapsoriasis - The appearance of small (< 5 cm in diameter), pruritic macules, plaques, and patches on the extremities and the trunk in a relapsing and remitting fashion is the hallmark of small plaque parapsoriasis [1] [6]. They are more frequently encountered in middle-aged adults and a predominance toward male gender is observed [1] [6]. Digitate dermatosis, a separate type of small plaque parapsoriasis, is described when symmetrically distributed finger-like patches develop on the flanks [6].
- Large plaque parapsoriasis - In addition to a larger diameter (> 5 cm, but many patients present with lesions of > 10 cm), atrophy of the plaques is a typical sign of large-plaque parapsoriasis. The lower extremities (thighs and buttocks), as well as the flexures of all extremities, are the main location where skin lesions appear [6]. Retiform parapsoriasis is a term used to describe a web-like disseminated pattern of lesions [1].
Skin
- Xanthoma
[…] basal-cell carcinoma Merkel-cell carcinoma nevus sebaceous trichoepithelioma Pigmented Freckles lentigo melasma nevus melanoma Dermal and subcutaneous epidermal inclusion cyst hemangioma dermatofibroma (benign fibrous histiocytoma) keloid lipoma neurofibroma xanthoma [en.wikipedia.org]
Workup
A properly conducted physical examination and a meticulously obtained patient history are essential steps in making an initial diagnosis of parapsoriasis [6]. For this reason, physicians must obtain data regarding the progression of symptoms, their pattern of appearance (relapses, recurrences, etc.), and the location where they appear. A full inspection of the skin reveals crucial information about the lesions (their size, type, and stage), which is why the physical examination is perhaps the most important component of the workup. But because the diagnosis cannot be made solely on clinical grounds, and because progression to mycosis fungoides may occur in unrecognized patients (carrying a significantly poorer prognosis), a prompt sampling of the lesion and a histopathological evaluation is necessary. Small plaque parapsoriasis is distinguished by nonspecific epithelial hyperplasia, acanthosis, spongiosis, focal hyperkeratosis, and exocytosis [1] [6], whereas infiltration of lymphocytes, lichenoid findings, variable epithelial appearance (hyperplastic, atrophic, or even normal) and necrosis of keratinocytes are known hallmarks of large plaque parapsoriasis [1] [6]. However, lymphocytic infiltrates (although much milder) in the dermis have been identified in small plaque parapsoriasis as well [1], illustrating the frequent histological overlapping of the parapsoriasis subtypes.
Treatment
METHODS: A retrospective study of treatment response in 116 patients diagnosed with MF and 71 patients with parapsoriasis and treated with topical nitrogen mustard from 1991 to 2009. [ncbi.nlm.nih.gov]
Furthermore, the treatment indications were similar for all patients, and the same doctors performed the treatment supervision and follow-ups. [scielo.br]
Prognosis
Although these various diseases have some similar clinical and histopathological features, they are obviously quite different from the standpoint of malignant potential and prognosis. [ncbi.nlm.nih.gov]
The prognosis of LPP is related to the progression and treatment of underlying mycoses fungoides Nevertheless, individuals with milder signs and symptoms have a better prognosis than those with severe signs and symptoms Regular follow up visits with the [dovemed.com]
The prognosis is excellent. [dermpedia.org]
Etiology
[…] characterized by papules and plaques or scaly patches resembling psoriasis The term applied to a group of relatively uncommon inflammatory, maculopapular, scaly eruptions of unknown etiology and resistant to conventional treatment. [icd9data.com]
The term applied to a group of relatively uncommon inflammatory, maculopapular, scaly eruptions of unknown etiology and resistant to conventional treatment. [icd10data.com]
Abstract The term parapsoriasis refers to a group of chronic asymptomatic scaly dermatoses of unknown etiology about which there is still controversy over the nosology and nomenclature of the different conditions that comprise the group, particularly [ncbi.nlm.nih.gov]
Parapsoriasis Summary Epidemiology Etiology Small plaque parapsoriasis Large plaque parapsoriasis Differential diagnoses [amboss.com]
Etiology and Pathophysiology LPP is a chronic inflammatory disorder likely caused by long-term stimulation of a specific subset of T cells by a corresponding antigen. [unboundmedicine.com]
Epidemiology
Epidemiology References:[1][2][3] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]
AIM: The aim of the study was to investigate the epidemiological and morphological data of parapsoriasis cases diagnosed at Emergency City Hospital, Timisoara, Romania for a period of 12 years. [ncbi.nlm.nih.gov]
Epidemiology Incidence There are no good data reflecting the true incidence of LPP. [unboundmedicine.com]
Again, because this disorder is difficult to diagnose, there are no concrete epidemiological statistics. But, of those with LPP, about 10% will progress to a malignant form. The cause of parapsoriasis is unknown. [aocd.org]
Pathophysiology
Etiology and Pathophysiology LPP is a chronic inflammatory disorder likely caused by long-term stimulation of a specific subset of T cells by a corresponding antigen. [unboundmedicine.com]
Pathophysiology of Parapsoriasis The initiating cause of parapsoriasis is unknown, but the diseases likely represent different stages in a continuum of lymphoproliferative disorders from chronic dermatitis to frank malignancy of cutaneous T-cell lymphoma [emedicine.com]
Prevention
General Prevention There are no known preventative measures. [unboundmedicine.com]
Prevention There is no known way to prevent psoriasis. Keeping the skin clean and moist and avoiding your specific psoriasis triggers may help reduce the number of flare-ups. Doctors recommend daily baths or showers for persons with psoriasis. [checkorphan.org]
Prevention This skin disease can unfortunately not be prevented. [health.ccm.net]
References
- Baderca F, Chiticariu E, Baudis M, Solovan C. Biopsying parapsoriasis: quo vadis? Are morphological stains enough or are ancillary tests needed? Rom J Morphol Embryol. 2014;55(3 Suppl):1085-1092.
- Sarveswari KN, Yesudian P. The conundrum of parapsoriasis versus patch stage of mycosis fungoides. Indian J Dermatol Venereol Leprol. 2009;75(3):229-235.
- Väkevä L, Sarna S, Vaalasti A, Pukkala E, Kariniemi AL, Ranki A. A retrospective study of the probability of the evolution of parapsoriasis en plaques into mycosis fungoides. Acta Derm Venereol. 2005;85(4):318-323.
- Salava A, Pereira P, Aho V, et al. Skin Microbiome in Small- and Large-plaque Parapsoriasis. Acta Derm Venereol. 2017 Feb 8.
- Kreuter A, Bischoff S, Skrygan M, et al. High association of human herpesvirus 8 in large-plaque parapsoriasis and mycosis fungoides. Arch Dermatol. 2008;144(8):1011–1016.
- Lewin J, Latkowski JA. Digitate dermatosis (small-plaque parapsoriasis). Dermatol Online J. 2012;18(12):3.
- Khachemoune A, Blyumin ML. Pityriasis lichenoides: pathophysiology, classification, and treatment. Am J Clin Dermatol. 2007;8(1):29-36.