|Year : 2018 | Volume
| Issue : 2 | Page : 110-113
Erythema dyschromicum perstans in pregnancy
Shagufta Rather, Atiya Yaseen, Sameena Batool, Iffat Hassan
Department of Dermatology, Venereology and Leprology Government Medical College and Hospital, Srinagar, Jammu and Kashmir, India
|Date of Web Publication||14-Dec-2018|
Dr. Shagufta Rather
Department of Dermatology, Venereology and Leprology, Government Medical College and Hospital, Srinagar 190001, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
Erythema dyschromicum perstans is a slowly progressive acquired dermatoses characterized by macular hyperpigmentation. There is no racial, genetic, or sex predilection. It occurs in adults, with some isolated cases and small series occurring in prepubertal children. The pigmentary disorder has never been reported in patients during pregnancy. We report a singular case of the disorder in a pregnant woman.
Keywords: Ashy dermatoses, erythema dyschromicum perstans, macular hyperpigmentation
|How to cite this article:|
Rather S, Yaseen A, Batool S, Hassan I. Erythema dyschromicum perstans in pregnancy. Pigment Int 2018;5:110-3
| Introduction|| |
Erythema dyschromicum perstans (EDP) is an uncommon, somewhat dubious cutaneous eruption of unknown etiology first described by Ramirez in 1957. Clinically, the condition is characterized by slowly progressive, symmetrical gray brown to blue gray macules, sometimes with an erythematous or elevated border, which is seen most commonly over the face, neck, trunk, and proximal arms. Although the condition has been widely described, especially in Latin Americans and Asians affecting all age groups and both sexes, but to the best of our knowledge, not a lone case has been reported during pregnancy so far.
| Case report|| |
A 28-year-old primigravida presented with asymptomatic, slowly progressive diffuse darkening of the trunk, arms, dorsal hands, proximal thighs, and upper neck unfurling onto the jawline, appearing at 10–12 weeks of gestation. The lesions slowly extended peripherally becoming confluent and affected almost the whole body. There was no history of preceding dermatitis, itching or photosensitivity, and the use of any over-the-counter drugs, Oral Contraceptive Pills (OCPs), or herbal supplements. She denied contact with any new substance and starting any new activity prior to the onset of lesions. A full review of all systems revealed no abnormality.
Systemic examination was normal. Cutaneous examination revealed confluent, symmetric ashy gray macules with polycyclic margins over the trunk, neck, face, upper limb, and proximal thighs [Figure 1] sparing the palms and soles. Most lesions displayed an elevated, erythematous, nonscaly border. Darier’s sign was negative. Hair, nails, and mucosae were spared.
Complete blood count, liver and renal chemistry, thyroid profile, stool analysis for parasites, serology for human immunodeficiency virus (HIV) 1 and 2, and hepatitis B, C, antinuclear antibody assessments were unremarkable. A skin biopsy specimen from the pigmented macules on the right thigh divulged focal changes such as moderate compact hyperkeratosis, epidermal thinning, a vacuolar alteration of the basal layer with mild-to-moderate lymphohistiocytic infiltrate intermixed with melanophages in the upper dermis and the absence of colloid bodies [Figure 2]. The clinical and histopathological attributes were corroborative with the diagnosis of EDP.
|Figure 2: H&E stained sections of EDP (40×) showing compact hyperkeratosis, epidermal thinning, vacuolar alteration of the basal layer with lymphohistiocytic infiltrate intermixed with melanophages in the upper dermis|
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| Discussion|| |
Since first described by Ramirez in El Salvador in 1957, EDP, also known as ashy dermatosis, has been a controversial entity. The terms EDP, ashy dermatosis, idiopathic eruptive macular pigmentation, and lichen planus pigmentosus (LPP) are often used interchangeably for various look-alike dermatoses. Sulzberger proposed the term “erythema dyschromicum perstans” while examining Convit’s patients with lesions similar to those described by Ramirez, sans the raised erythematous margins in the latter. The entity LPP introduced by Bhutani is characterized by hyperpigmented dark brown or slate gray macules distributed mostly over the exposed areas and flexures. There are exacerbations and remissions occasionally accompanied by pruritus. The lesions lack the erythematous border seen in EDP. The clinical association of this disorder with lesions of classical lichen planus and demonstration of colloid bodies on histopathology prompted the authors to coin a separate term LPP. They suggested that ashy dermatosis is similar to LPP, the former being a macular variant of the latter.
Though several of the clinical and histologic features described in our case fit into LPP, a remarkable exception is that our patient had no preceding or concomitant lesions of lichen planus, a prominent raised erythematous border was seen, and colloid bodies could not be demonstrated on histopathology.
Zaynoun et al. reviewed the literature and proposed clinical diagnostic criteria to delineate EDP from ashy dermatosis and other simulating disorders:
- Ashy dermatosis: Patients with idiopathic eruptive hyperpigmented macules, irrespective of the presence or absence of interfacial dermatitis histologically.
- EDP: Patients with lesions similar to those of ashy dermatosis, but who have or have had lesions with erythematous borders.
- Simulators: Known dermatological diseases such as lichen planus, LPP, postinflammatory hyperpigmentation, generalized melasma, mastocytosis, and others [Table 1].
|Table 1: Differences between erythema dyschromicum perstans and its close simulators|
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The etiology of EDP is largely unspecified. Various infections, for example, whipworm infection, HIV, and internal disorders have been implicated. Cell Mediated Immunity (CMI) may have a role to play. Cases have been reported after the ingestion of ammonium nitrate, radiographic contrast media, pesticides, and some medications such as ethambutol, benzodiazepines, and penicillin. An immunological response to any of these factors might define the extent of lesional inflammation of the skin based on the genetic profile. Mexican patients with the allele HLA-DR4 have been found to have a higher genetic susceptibility to acquiring EDP. Albeit the condition has not been reported in pregnancy so far, a reduction in cell-mediated immunity, increased production of estrogens, progesterone, or melanocyte-stimulating hormone during gestation may be determinant.,
Clinically, this rare inflammatory skin disorder is characterized by asymptomatic, slowly progressing, ashy-gray, macular hyperpigmentation, symmetrically distributed on the trunk, the proximal aspects of the extremities, the neck, and face. The expanding macules have a slightly raised, erythematous border in the initial inflammatory stage, which is considered to be the hallmark of the disease. However, Chang et al. observed the peripheral erythematous borders only in 12 out of 68 (17.6%) patients with EDP. In most cases, the disease starts in adult life, but isolated cases and small series have been reported in children. The histopathology of EDP is not pathognomonic. The most relevant histopathological findings are a dermal perivascular lymphocytic infiltrate with numerous melanophages. Direct immunofluorescence microscopy studies have demonstrated colloid staining of IgM and C4, as well as the presence of fibrinogen at the dermoepidermal junction. Immunohistologic findings in EDP include keratinocytes having Ia antigen expression, strong OKT4 and OKT6 staining of Langerhans’ cells, and the dermal infiltrate of the helper/inducer and suppressor/cytotoxic phenotypes of T-cells.
The treatment of EDP is largely unsatisfactory and remains a challenge. Overall, response to several therapeutic modalities including clofazimine and dapsone therapy is said to be variable or inconsistent. Lately, topical tarolimus has been found to be an effective and a safe therapeutic option. In our patient, other than sun avoidance, no other treatment was offered. The patient was later lost to follow-up.
The case is highlighted because it is the first reported case of EDP occurring during pregnancy, which might further help us to elucidate the pathomechanism of this enigmatic 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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]