Atypical presentations of pityriasis rosea: case presentations

JEADV (2005) 19, 120– 126 DOI: 10.1111/j.1468-3083.2004.01105.x CASE REPOR T Atypical presentations of pityriasis rosea: case presentations Blackwell Publishing, Ltd. A Chuh,†* V Zawar,‡ A Lee† †Department of Community and Family Medicine, The Chinese University of Hong Kong and Prince of Wales Hospital, Shatin, Hong Kong SAR, China, ‡Department of Dermatology, NDMVPS Medical College and Research Centre, Nashik, Maharashtra State, India. *Corresponding author, The Bonham Surgery, Shop B5, Ning Yeung Terrace, 78 Bonham Road, Ground Floor, Hong Kong, tel. +852 25590420, 852 25174081; fax +852 23703330; E-mail: [email protected] ABSTRAC T Atypical cases of pityriasis rosea (PR) are fairly common and less readily recognized than typical eruptions. We present four patients for whom we believe atypical PR is the most likely diagnosis. A 33-year-old man had purpuric lesions bilaterally on the legs with classical rash on the trunk. A 28-year-old woman had intensely pruritic and urticarial lesions. A 10-year-old girl had hundreds of small papular lesions 1–3 mm in size. A pregnant woman aged 26 had oral haemorrhagic ulcers with classical PR eruption on her trunk. The oral ulcers erupted and remitted at the same time as the generalized eruption. We reviewed the literature and proposed a classification based on rash morphology, rash size, rash distribution, number of lesions, site of lesions, severity of symptoms and course of the eruption. We believe that it is difficult to make a clear division to define typical and atypical PR, and that it is important not to ascribe any unusual or atypical skin eruption with PR unless other dermatoses have been excluded. Key words: Camille Melchoir Gibert, exanthem, herald patch, Langer’s skin cleavage lines, peripheral collarette scaling, purpura Received: 31 March 2004, accepted 9 April 2004 Introduction Pityriasis rosea (PR), first named as such by Gibert in 1860,1,2 is a fairly common dermatosis. Typical clinical features are depicted in figs 1–3. The overall incidence of is 6.8 per 1000 dermatological patients.3–11 A study in Minnesota12 revealed that 43% of all cases were diagnosed by internal physicians or general practitioners. A crosssectional study in students aged 10–29 in Burkina Faso13 reported the prevalence to be 0.6%. An epidemiological study in Hong Kong14 retrieved records of 41 patients with PR in six primary care practices over 3 years. However, primary care physicians have been reported to significantly underdiagnose PR.15 PR has significant impacts on the quality of life in adults 16 and children. 17 Atypical cases of PR are fairly common.18,19 In this article, we present several patients with atypical PR, and review the literature describing atypical PR cases. 120 Case reports Patient 1 A 33-year-old man had a generalized pruritic eruption for 3 weeks, with no preceding coryzal symptoms and no herald patch. He had schizophrenia, but his psychiatric medications had not been changed recently. Examination revealed discrete scaly patches on his trunk and four extremities, extending to the forearms and legs. The rash of the trunk showed peripheral collarette scaling and orientation along lines of skin cleavage (fig. 4a). Purpuric lesions were seen bilaterally on the legs (fig. 4b). No evidence of trauma was noted. The face, scalp, palms, soles and mucous membranes were uninvolved. His complete blood picture, liver function tests and clotting profile were normal. VDRL, HIV antibodies and antinuclear antibodies were negative. We prescribed topical calamine lotions and a sedating antihistamine at night. Complete remission of his lesions was seen after 4 weeks. © 2004 European Academy of Dermatology and Venereology Atypical presentations of pityriasis rosea 121 fig. 1 Herald patch and secondary eruption in typical pityriasis rosea. The herald patch is seen lateral and superior to the left nipple. fig. 2 Peripheral collarette scaling in typical pityriasis rosea. We believe that the purpuric lesions on the legs were most likely PR lesions. However, vasculitis due to other causes or a pigmented purpuric dermatosis is also possible. A limitation in our diagnosis is that we did not perform lesional biopsy to exclude other dermatoses. Patient 2 A 28-year-old female hospital nurse had a generalized pruritic eruption for 1 week. She had a sore throat, headache and malaise before rash onset. There was no herald patch. She was single and claimed to be sexually inactive. Examination revealed palpable weal-like lesions on her trunk and proximal aspects of the four extremities (fig. 5a,b). Close examination revealed peripheral collarette scaling. Most lesions follow the lines of skin cleavage. She was diagnosed to have urticarial PR, and was recruited into a study investigating the association of human herpesvirus (HHV) 6 and 7 infections and PR.20 Her clinical features have been reported previously.21 fig. 3 (a,b) Trunk and proximal limb distribution, symmetry and rash orientation along lines of skin cleavage in typical pityriasis rosea. Her complete blood picture was normal. VDRL was negative. The polymerase chain reaction for HHV-6 and -7 DNA was negative in peripheral blood mononuclear cells and in plasma, in both acute and convalescent blood specimens. She was prescribed oral fexofenadine 180 mg as a single morning dose, and oral chlorpheniramine 4 mg at night. Complete symptomatic remission was seen after 4 weeks. We believe that the most likely diagnosis is urticarial PR. However, we have not performed a lesional biopsy to exclude urticarial vasculitis, annular erythema and subacute cutaneous lupus erythematosus. Moreover, fig. 5(a,b) do not illustrate urticarial PR adequately as the patient had already been on treatment for 1 week when such photographs were taken. Patient 3 A 10-year-old girl had a sudden eruption of pruritic lesions, preceded 5 days previously by malaise and coryzal symptoms. One papule on her back had erupted 7 days previously. © 2004 European Academy of Dermatology and Venereology JEADV (2005) 19, 120–126 122 Chuh et al. fig. 5 (a,b) Patient 2 with urticarial pityriasis rosea. Spontaneous remission of all papules was noted after 8 weeks. We believe that the most likely diagnosis is papular PR. Patient 4 fig. 4 (a,b) Patient 1 with typical eruption of pityriasis rosea on trunk and atypical purpuric lesion on legs. Examination revealed hundreds of small papular lesions 1–3 mm in diameter over her trunk and proximal aspects of the four extremities (fig. 6a). Peripheral collarette scaling and orientation along lines of skin cleavage were evident for the larger lesions (fig. 6b). The complete blood picture was normal. VDRL, antibodies against Epstein–Barr virus, early, capsid, and nuclear antigens, and HIV antibodies were all negative. A 26-year-old woman during the first trimester of her pregnancy had a generalized pruritic eruption for 2 weeks. She noticed one lesion on her back 7 days before the generalized eruption. Her past health was good. Drug history was unremarkable. Examination revealed discrete patches on her trunk and proximal aspects of the four extremities. Peripheral collarette scaling was noted, and some lesions were orientated along lines of skin cleavage. The mucosal surface of her lower lips was erythematous, with two small non-tender haemorrhagic ulcers present (fig. 7). Such lesions appeared at the same time as the generalized eruption. Other mucosal surfaces were normal. Her VDRL and HIV antibodies were negative. She was prescribed topical betamethasone ointment for the lesions on the trunk. After 3 weeks the mucosal lesions on her lower lip resolved spontaneously while at the same time the lesions on the trunk cleared. © 2004 European Academy of Dermatology and Venereology JEADV (2005) 19, 120–126 Atypical presentations of pityriasis rosea 123 fig. 7 (a) Patient 4 with discrete patches of pityriasis rosea eruption on her trunk. (b) Mucosal lesions on the lower lip of patient 4. fig. 6 (a,b) Patient 3 with small papular pityriasis rosea. Peripheral collarette scaling and orientation along lines of skin cleavage were seen in the herald patch (black arrow) and some of the other lesions (white arrows). We believe that the oral lesions were most likely part of the clinical picture of PR as they erupted and remitted at the same time as the generalized PR eruption. However, it is also entirely possible that these were incidental aphthous ulcers unrelated to PR. Moreover, immunomodulation in PR could have secondarily caused the aphthous ulcers. bullae.27 It is commoner in children and young adults, and may be severely pruritic and extensive.23 Purpuric PR usually presents with macular purpuric lesions. Palatal petechiae may be present.29 Histopathological evidence of accompanying allergic vasculitic is usually absent.29 Haemorrhagic and purpuric PR are different terminologies describing the same condition.30 Urticarial PR, also known as PR urticata, presents with raised lesions resembling urticarial weals. It is often accompanied by intense pruritus.22 Atypical size of lesions Types of atypical pityriasis rosea The early classification of atypical PR was complicated.22 We propose a simplified classification as follows. Atypical morphology of lesions Atypical rash morphology includes rashes in vesicular,23–27 purpuric27–30 or haemorrhagic,31 and urticarial22 forms. Vesicular PR usually presents as a generalized eruption of 2–6 mm vesicles, each having a central bulla, or a rosette of vesicles and PR gigantea of Darier that has enormous plaques is rare. In a patient described in 1915,32 the herald patch was the same size and shape of a pear. The clinical course is similar to typical PR.22 Papular PR is more often seen in children.33 Numerous small papules 1–2 mm in diameter may be seen together with classical PR patches.34 Atypical distribution of lesions In PR inversus, the face, axillae and groin are predominantly affected.35 In the limb-girdle type, the eruption is restricted to © 2004 European Academy of Dermatology and Venereology JEADV (2005) 19, 120–126 124 Chuh et al. the shoulders or hips.34 Cases with relatively asymmetrical rash distribution, or strictly right-36 or left-sided22 involvements have been reported. Atypical number of lesions The trunk is usually the predilected site in localized PR. The rash morphology and disease course are the same as for typical PR.37 Pityriasis circinata et marginata is sometimes considered a special form of PR.4,22,38 This is mainly seen in adults, with fewer and larger lesions often localized to the axillae or inguinal region. Atypical site of lesions Involvements of the face, scalp, hands and feet are not rare in PR.22 In a series of 202 patients with PR,39 involvements of face and neck were seen in eight (4%) patients. Involvements of finger and toe tips,40 scalp, eyelids and penis22 have been reported. The oral cavity is another atypical site for PR lesions.41–46 Oral lesions may be white, haemorrhagic, erosive or bullous.41 Intraoral lesions are usually asymptomatic. They either follow a similar course as cutaneous PR lesions,47 or subside several days before the cutaneous eruption.4 Atypical severity of symptoms PR may cause no pruritus at all.48 On the other hand, the term PR irritata is sometimes coined to describe patients complaining of severe itch, pain and a burning sensation.49 Atypical course of the eruption In a series of 826 patients with PR,48 the rate of relapse was 2.8%. In the population study with 939 patients,12 17 (1.8%) patients had recurrent PR after an average of 4.5 years followup. A single case of annual relapse for five consecutive years has been reported.50 Drug-induced pityriasis rosea-like rashes Many drugs, including captopril,51–54 gold,54–57 isotretinoin,58 non-steroidal anti-inflammatory agents,59,60 omeprazole,61 terbinafine62 and tyrosine kinase inhibitor63 have been implicated in causing PR-like rashes. However, in many such cases, the rashes described only resemble PR remotely, and are best considered as separate clinical entities. Discussion It is important that physicians are aware of the wide spectrum of PR variants so that appropriate management and reassurance can be offered. In a previous report to validate diagnostic criteria for PR,64 we listed three mandatory clinical features (discrete circular or oval lesions, scaling on most lesions, peripheral collarette scaling) and three optional clinical features (trunk and proximal limb distribution, orientation along skin cleavage lines, herald patch). In our series,64 only four of 18 patients with PR exhibited all six features unequivocally. We believe that it is unnecessary for an eruption to exhibit all six features to be termed classical PR, and that it is difficult to make a clear division between typical and atypical PR. PR can occur concomitantly with other cutaneous and mucous membrane diseases. It is important not to ascribe or associate any unusual or atypical skin eruption with PR unless other dermatoses have been excluded. A limitation in the presentation of our four cases is that lesional histopathology was not investigated for any of the patients. It is fortunate that spontaneous rash resolution was seen in all four patients, without need for active intervention. Retrospectively, we believe that for atypical eruptions without a definite diagnosis, it is still safer to consider lesional biopsy and other investigations so that important differential diagnoses will not be missed. References 1 Percival GH. 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