An unusual variant of perniosis: A case series of “cold-associated perniosis of the thighs” without equestrian activity

Pinar Incel Uysal; Neslihan Akdogan; Servet Guresci; Sezer Kulacoglu; Başak Yalçin

doi:10.4103/ds.ds_8_19

CASE REPORT

Year : 2019 | Volume : 37 | Issue : 4 | Page : 217-221

An unusual variant of perniosis: A case series of “cold-associated perniosis of the thighs” without equestrian activity

Pinar Incel Uysal¹, Neslihan Akdogan¹, Servet Guresci², Sezer Kulacoglu², Başak Yalçin¹
¹ Department of Dermatology, Ankara Numune Training and Research Hospital, Ankara, Turkey
² Department of Pathology, Ankara Numune Training and Research Hospital, Ankara, Turkey

Date of Submission	29-Apr-2018
Date of Decision	29-Jan-2019
Date of Acceptance	09-Mar-2019
Date of Web Publication	16-Dec-2019

Correspondence Address:
Dr. Pinar Incel Uysal
Department of Dermatology, Ankara Numune Training and Research Hospital, Talatpasa Bulvari, 06100 Altindag, Ankara
Turkey

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ds.ds_8_19

Abstract

The role of cold exposure in the etiology of perniosis was clearly identified among susceptible individuals. However, few authors have newly reported purple-red “equestrian-type lesions” localized on thighs in patients without equestrian activity and suggested to use the reputation; “cold-induced perniosis of thighs.” We conducted a retrospective chart review of patients who have presented to our department with cold perniosis-like lesions exclusively localized on the thighs. Clinical and histopathological features of five female patients aged 16–31 years without equestrian activity indicated the diagnosis of cold-induced perniosis of the thighs. Cold-associated perniosis may be appropriate diagnosis for the patients presenting with symmetrically distributed painful, itching, or burning violaceous plaques and patches on the thighs for several weeks. The condition primarily affects overweight women and does not require outdoor activity. Microscopic presentation of the disorder is variable and very similar to acral idiopathic perniosis and chilblain lupus erythematosus.

Keywords: Chilblain, cold panniculitis, cold-associated dermatoses, perniosis of thighs

How to cite this article:
Uysal PI, Akdogan N, Guresci S, Kulacoglu S, Yalçin B. An unusual variant of perniosis: A case series of “cold-associated perniosis of the thighs” without equestrian activity. Dermatol Sin 2019;37:217-21

How to cite this URL:
Uysal PI, Akdogan N, Guresci S, Kulacoglu S, Yalçin B. An unusual variant of perniosis: A case series of “cold-associated perniosis of the thighs” without equestrian activity. Dermatol Sin [serial online] 2019 [cited 2023 Mar 21];37:217-21. Available from: https://www.dermsinica.org/text.asp?2019/37/4/217/273103

Introduction

Cold-induced dermatoses refer to a spectrum of diseases including perniosis/chilblain, cryoglobulinemia, frostbite, cold-induced urticaria, and cold panniculitis. Cold-induced dermatoses, including perniosis, chilblain lupus erythematosus, and well-developed panniculitis, may demonstrate a wide range of histopathological changes.

Equestrian cold panniculitis was first described in 1980, typically affecting horse riders due to prolonged skin exposure to the cold.^[1] Subsequently, this condition has been referred to in the literature as equestrian perniosis (EP).^[2],[3],[4] However, the clinicopathological characteristics of the disease are not specific to horse riding alone; they have also been observed and reported in association with other outdoor leisure activities.^[5] Moreover, a similar condition has been reported which possesses overlapping histopathological features with cutaneous lupus, induced following ice-pack application.^[6],[7] Thus, the term “cold-associated perniosis of the thighs” has recently been proposed to cover this range of related conditions.^[8],[9]

Herein, we describe the cases of five female patients aged <35 years presenting with similar erythematous or violaceous urticarial plaques and nodules on the upper lateral thigh during winter months (December–February) between 2015 and 2017. None of the patients reported participating in any equestrian activity. All patients were overweight, with a history of wearing tight-fitting clothes and prolonged exposure to cold climates. Therefore, we would like to emphasize this condition, which has a benign course and may show clinical and histopathological characteristics which overlap with acral perniosis and chilblain lupus erythematosus.

Case Reports

The patients presenting with erythematous or violaceous urticarial plaques and nodules on the upper lateral thigh during winter months (between December 2015 and February 2017) were retrieved from files at the Department of Dermatology, Ankara Numune Training and Research Hospital, Turkey. All patients had undergone biopsy. Five patients with distinct clinicopathological characteristics were studied. Patient history, demographic data, clinical characteristics, laboratory analyses, and histochemical and immunohistochemical parameters were all reviewed.

Clinicopathological characteristics of the five patients are summarized in [Table 1]. Five females, aged between 16 and 31 (mean age: 20.6) years, presented at our department with similar violaceous and erythematous eruptions localized on the upper lateral thigh [Figure 1]a-e]. One of the patient had lesions on the posterior aspects of the thighs [Figure 1]f. All lesions were bilateral and symmetrical. One patient also had a symmetrical lesion on the anteromedial thigh [Figure 1]e. Focal erosion was observed in four of the five cases. Four of the five patients were overweight but otherwise healthy (median body mass index [BMI]: 28.7 kg/m²). Patient 1 had a history of hypothyroidism. One patient was a smoker. None of the patients had practiced any outdoor activities such as horse riding, cycling, or river walking. All five patients reported prolonged or intermittent exposure to cold climates, either at home or at work, because of poor heating. The mean duration of lesions being present before consultation with medical staff was 6 (3–12) weeks. Two patients (Patient 3 and 4) had experienced similar eruptions in previous years (once each). A burning sensation was the most common symptom reported by patients. Equestrian cold panniculitis was suspected for each patient; urticarial vasculitis, lupus panniculitis, and erythema annulare centrifugum were the other presumptive diagnoses.

Table 1: Characteristics of the participants

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Figure 1: Multiple violaceous plaques some with central ulceration on the upper posterolateral thighs of the Patient 1 (a) and Patient 2 (b and c). Erythematous patches and plaques involving upper lateral thighs of the Patient 3 (d). Symmetrically localized erythematous nodules and plaques on both anteroinferior and posterolateral thighs of Patient 4 (e and f). Histopathological images of the Patient 4 (g-i). Dermal perivascular lymphoid infiltration (g, H and E stain, ×40). Perieccrine mononuclear infiltration (h, H and E stain, ×200). Interstitial mucin deposition between dermal collagen bundles (i, Alcian Blue stain, ×100)

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Histopathological characteristics are also shown in [Table 1]. No end-stage panniculitis was observed in any of the patient biopsy samples during histopathological examination. The main histopathological findings were of superficial and deep perivascular mononuclear infiltration and periadnexal lymphoid infiltration [Figure 1]g, [Figure 1]h, [Figure 1]i. Perifollicular infiltration was observed in two patients, and vacuolar changes were observed in one patient. Mucin accumulation was detected in two patients [Table 1] and [Figure 1]h. Direct immunofluorescence examination was unremarkable in one of the five patients.

Routine laboratory analyses (total blood count, routine biochemistry, and urinalysis) were unremarkable for all patients. Additional laboratory investigations showed elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels in three patients. One patient (Patient 4) had cryoglobulinemia, and two patients had slightly elevated complement 4 levels. Antinuclear antibodies and extractable nuclear antigen antibodies (anti-SSA, anti-SSB, anti-Sm, anti-Jo1, anti-Scl70, and anti-Sm/RNP) were all negative for all patients.

All five patients were treated with a topical corticosteroid (betamethasone valerate or mometasone furoate). Three of the patients (Patients 1, 2, and 4) presenting with severe symptoms were prescribed oral pentoxifylline (PTX) (3 × 400 mg/day, orally). Patients were advised on preventive measures, including wearing loose clothing and reducing cold exposure. All lesions resolved without any residual pigmentation or scarring after 2–4 weeks. Recurrence was noted in Patient 4 after 1 year.

Discussion

Perniosis is characterized by acral violaceous lesions, presenting with dermal edema and lymphocytic, perivascular, and perieccrine infiltration on microscopic examination.^[10] Primary and secondary forms both exist. EP is a rare condition characterized by localized disease presenting with painful, itchy, or burning erythematous or purplish papules, plaques, or nodules of the upper thigh. EP is commonly associated with horse riding and/or other outdoor activities in cold or humid environments.^[5] However, few reports have demonstrated cases that are not associated with equestrian activities.^{[8],[11],[12]} Accordingly, there was no association with any of these activities in the patients we studied.

The microscopic picture of cold-associated perniosis of the thigh may represent several overlapping features. Lesions usually show superficial and deep perivascular, periadnexal, and perineural dermal mononuclear infiltration.^[2],[3] Subtle epidermal changes, vacuolar degeneration of the basal membrane, and dermal mucin deposition can sometimes also be observed in biopsy specimens.^[8],[9],[13] Cases with histopathological features mimicking lupus have also been reported.^[9] Distinction between conditions may be challenging; however, the presence of spongiosis, papillary edema, and perieccrine infiltrate favors the diagnosis of perniosis.^[14] One of our cases (Patient 3) presented a histopathological picture resembling lupus. However, this patient did not meet any clinical or laboratory lupus criterion, and clinicopathological correlation led instead to a diagnosis of isolated pernio. Superficial lymphocytic lobular panniculitis is occasionally present in perniosis of the thigh, idiopathic perniosis, and chilblain lupus. Similar to previous reports, fully formed lobular panniculitis was not evident in our patients. Immunohistochemical analyses revealed a predominance of CD3+ lymphocytic cells, with sparse CD20+ cells in two patients (Patients 2 and 3) [Table 1].

Our cases had very similar clinical manifestations and histopathological characteristics to those described previously by Ferrara and Cerroni.^[8] In accordance with their report, exclusive upper posterolateral thigh involvement was common among our patients. In addition, the most common lesions were violaceous to red plaques with burning sensation, pain, and itchiness. However, in our report, these lesions were not associated with the equestrian activity. Furthermore, the patients were all overweight, female, and wore tight-fitting clothes. All lesions occurred in the winter months, and all patients had a history of exposure to cold conditions. Elevated ESR and CRP levels were observed in three of the five patients. Notably, cryoglobulinemia was detected in one patient; in the literature, there is some debate regarding the association between perniosis and cryoglobulinemia.^[10],[15] Due to the lack of any other supportive microscopic or clinical findings of cryoglobulinemia in our patient (Patient 4), this was not considered the primary cause of the lesions. Chronic perniosis (lasting >4 weeks) has previously been associated with connective tissue disease (CTD).^[10] However, despite the mean duration of the lesions in our patients being 6 weeks, no CTD was established in association with the condition in any patients. All patients studied were overweight. On contrary to a previous suggestion that low BMI is a risk factor for chilblains/acral perniosis, high BMI seems more likely to be correlated with perniosis localized to the thigh or flank.^[16] This correlation may be explained by impaired skin microvasculature or by a cold-induced vasodilation (CIVD) response due to thicker subcutaneous fat tissue. CIVD can be defined as acute increase in peripheral blood flow during cold exposures. It is suggested that CIVD acts as a protective thermoregulatory mechanism against cold injuries.^[17] In addition to the fingers and toes, this paradoxical and cyclic response have been shown in the face and forearm.^[18],[19] CIVD seems to be affected by individual risk factors including age, gender, diet, and alcohol consumption. In addition, recent study has shown that individuals with low body fat have higher local cold tolerance than the others^[20] In this context, we assume that impaired CIVD may be responsible for individual tendency in patients with recurrent attacks, which we observed in two of our patients. PTX has potential immunologic and anti-inflammatory effects in the treatment of perniosis, and its safety and efficacy are both supported by the literature.^[21] Even when lesions tended to resolve with proper clothing and avoiding exposure to cold conditions, faster improvements were observed in patients who received oral PTX therapy [Table 1].

Conclusion

Based on the available data and the data presented here, we consider that the proposed term of “cold-associated perniosis of the thighs” is more accurate and descriptive for this particular disorder. There is still a paucity of data regarding this rare condition. The condition tends to have a mild course, and preventive measures are usually effective in controlling it. We believe that this entity deserves particular attention and should be taken into account in the differential diagnosis of cold-induced dermatoses. Therefore, we believe that this report will increase awareness and lead future reports to provide a better understanding of the characteristics of the disorder.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest

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